The Toronto Notes 2011 Comprehensive medical reference and review for the Medical Council of Canada Qualifying Exam Part 1 and the United States Medical Licensing Exam Step 2
27th Edition Editors-in-Chief: Yingming Amy Chen and Christopher Tran
Wherever the art of medicine is loved, there is also a love of humanity. – Hippocrates
Toronto Notes for Medical Students, Inc. Toronto, Ontario, Canada
Twenty-seventh Edition Copyright © 2011 – Toronto Notes for Medical Students, Inc. Toronto, Ontario, Canada. Typeset and production by Type & Graphics Inc. ISBN 978-0-9809397-4-3 (27th ed.) All rights reserved. Printed in Toronto, Ontario, Canada. Toronto Notes 2011 is provided for the sole use of the purchaser. It is made available on the condition that the information contained herein will not be sold or photocopied. No part of this publication may be used or reproduced in any form or by any means without prior written permission from the publisher. Every effort has been made to obtain permission for all copyrighted material contained herein. Previous editions copyright © 1985 to 2010. Cover design: John Sauvé and Stephanie VandenBerg Illustrations: Biomedical Communications, University of Toronto Clipart images: Copyright TASK FORCE ImageGALLERY, © NVTech Inc., Type & Graphics Inc. Notice: THIS PUBLICATION HAS NOT BEEN AUTHORED, REVIEWED OR OTHERWISE SUPPORTED BY THE MEDICAL COUNCIL OF CANADA NOR DOES IT RECEIVE ENDORSEMENT BY THE MEDICAL COUNCIL AS REVIEW MATERIAL FOR THE MCCQE. THIS PUBLICATION HAS NOT BEEN AUTHORED, REVIEWED OR OTHERWISE SUPPORTED BY THE NATIONAL BOARD OF MEDICAL EXAMINERS U.S.A. NOR DOES IT RECEIVE ENDORSEMENT BY THE NATIONAL BOARD AS REVIEW MATERIAL FOR THE USMLE. The editors of this edition have taken every effort to ensure that the information contained herein is accurate and conforms to the standards accepted at the time of publication. However, due to the constantly changing nature of the medical sciences and the possibility of human error, the reader is encouraged to exercise individual clinical judgement and consult with other sources of information that may become available with continuing research. The authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this textbook, atlas, or software and make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the publication. In particular, the reader is advised to check the manufacturer’s insert of all pharmacologic products before administration.
Library of Congress Cataloging-in-Publication Data is available upon request
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Toronto Notes 2011
To the contributors of past, present and future editions, and To the families, friends, and loved ones of those who participated in the Toronto Notes 2011 production – your support and understanding were invaluable in the success of this project! Thank You!
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Note: Many of you have wondered about the Toronto Notes logo, which is based on the rod of Asclepius, the Greek god of medicine. The rod of Asclepius consists of a single serpent entwined around a staff. This icon symbolizes both rebirth, by way of a snake shedding its skin, and also authority, by way of the staff. In ancient Greek mythology, Asclepius was the son of Apollo and a skilled practitioner of medicine who learned the medical arts from the centaur Chiron. Asclepius’ healing abilities were so great that he was said to be able to bring back people from the dead. These powers displeased the gods, who punished Asclepius by placing him in the sky as the constellation Orphiuchus. The rod of Asclepius is at times confused with the caduceus, or wand, of Hermes, a staff entwined with two serpents and often depicted with wings. The caduceus is often used as a symbol of medicine or medical professionals, but there is little historical basis for this symbolism. As you may have guessed, our logo uses the rod of Asclepius that is modified to also resemble the CN Tower – our way of recognizing the university and community in which we have been privileged to learn the art and science of medicine. Thomas O’Brien, HBSc, MD Class of 2009 M.D. Program, University of Toronto
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Preface – From the Editors Dear Readers, Toronto Notes is a concise and comprehensive medical review revised annually by the University of Toronto graduating medical class under the guidance of leading experts within the Faculty of Medicine. This reference text started as a compilation of notes written by and shared among University of Toronto students to study for their medical licensing exams. After 27 years, it has become a reputable study guide used by medical students across Canada and around the world. This transformation could not have happened without the dedication of students who ensure that each edition contains the latest evidence-based information on 29 subspecialties of Medicine. This year has been no different: over 100 staff worked tirelessly to complete this edition. Since its inception, Toronto Notes has been a nonprofit organization where revenue is used to fund University of Toronto medical student initiatives including community outreach, international health projects, charitable performances, and graduating class scholarships and bursaries. On behalf of the Toronto Notes production team, we would like to thank the support from thousands of medical students around the world by purchasing our book.
As readers, we appreciate your feedback. This year, we have spent considerable time improving the scientific and writing accuracy of our publications. Many of those changes originated from your comments submitted online on our website. Having travelled across the country for electives, we appreciate how so many medical students value Toronto Notes for studying during clinical rotations and exam preparation. We therefore understand the trust placed in our publication and realize our crucial task in upholding the accuracy of our content. Thank you very much for purchasing the 2011 edition of Toronto Notes. We hope it will serve you well in all of your future medical endeavours. Sincerely,
Yingming Amy Chen
Christopher Tran
Editors-in-Chief Toronto Notes 2011
The 27th edition of Toronto Notes offers exciting new changes to further enhance your exam preparation. We have included over 20 new original medical illustrations, updated our online colour atlas with new images and annotations, and revised our evidence-based medicine reviews. We also have a new learning module: the Approach to Ultrasound in the Emergency Department, created to help medical students understand the basics of ultrasound imaging in trauma and other emergencies. In addition, we have included over 50 Objective Structured Clinical Examination (OSCE) scenarios with history and physical exam checklists for practice purposes that can be found online. We have also separated our popular Clinical Handbook into four pocket reference guides for easy portability.
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Toronto Notes 2011
Student Contributors Editors-in-Chief Yingming Amy Chen Christopher Tran
Production Managers Alicia Mattia Kathryn Isaac
Handbook Editors Reema Shah Alon Vaisman
Atlas Editors Jeanne Huo Cheryl Lee
Website and Mobile Resources Payal Agarwal Greg Rampersad
Associate Editors: Medicine Doreen Ezeife Nigel Tan
Associate Editors: Surgery Alaina Garbens Dupe Oyewumi
Associate Editors: Primary Medicine and Other Specialties Christopher Kitamura Michelle Lam
EBM Editor: Medicine Steven Wong
EBM Editor: Surgery Adam Gladwish
EBM Editor: Primary Medicine and Other Specialties Janine Hutson
Chapter Editors: Medicine Mina Atia Helen Cheung Derek Chew Katie Connolly Mark Davis Yehoshua Gleicher Denise Jaworsky Lorraine Jensen Christopher Kandel Shelley Kraus Kayi Li Shiying Liu Anya McLaren Ines Menjak Melanie Ostreicher Tara Rastin Elissa Rennert-May Courtney Scott Rupal Shah Adil Shamji Emily Siu Vithika Sivabalasundaram Roseanne St. Bernard Dave Sussman Julie Thorne Pamela Tsao Daniel Vilensky Michael Ward Bertha Wong Lianne Wong
Chapter Editors: Surgery Ryan Austin Brian Ballios Samir Bidnur Frederick Cheng Annie Doan Chris Farlinger Alexander Huang Imran Jivraj Faazil Kassam Yonah Krakowsky Kay Lam Pamela Lau Hubert Lee Lindsay MacKenzie Alireza Mansouri Jayant Ramakrishna Deborah Sasges Caroline Scott Anthony So Peter Szasz Jenna Tessolini Nathalie Wong-Chong Rebecca Zener
Chapter Editors: Primary Medicine and Other Specialties Trevor Arnason Heidi de Boer Shuo Chen Hannah Chiu Nicole Coles Roopan Gill Jacqueline Holiff Gillian Lindzon Venetia Lo Melissa Loh Donald Ly Rachel Markin Rebecca Menzies Farheen Mussani Babak Rashidi Michael Romano Daniel Rosenfield Tamar Rubin Sharon Sadry John Sauve Julia Sharp Vincent Spano Mitch Vainberg Christian van der Pol Melinda White Karen-Rose Wilson Jacqueline Wong Nancy Xi Elizabeth Yeboah Susanna Zachara Ryan Zufelt
Copy Editors Tara Adirim Sara Ahmed Sarah Erdman Nisha Andany Michal Bodhanowicz Romy Cho Andrew Chou Heathcliff D’Sa Kelly Emms Sarah Erdman David Evans Zac Feilchenfeld Tym Frank Jessica Green Lara Hart Aasim Hasany Lowell Henriques Alicia Mattia Kelly McGowan Ariel Mendlowitz Maike Milkereit Abhi Rewari Dan Segal Stephanie Vandenburg Diana Wu
Biomedical Communications Production Editors Walid Aziz Carly Vanderlee
Illustrators Diego Accorsi Leanne Chan Shelley Chen Enid Hajderi Janice Joo Paul Kelly
Beatrice Lau Tabby Lulham Julie Man Carly Vanderlee Lucy Zhang
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Faculty Contributors, University of Toronto All contributing professors have been appointed at the University of Toronto. Anne M.R. Agur, BSc (OT), MSc, PhD Professor, Division of Anatomy, Department of Surgery Ruby Alvi, MD, CCFP, MHSc Assistant Professor, Department of Family and Community Medicine Stacey Bernstein, MD, FRCPC Associate Professor, Department of Pediatrics The Hospital for Sick Children
Jason Dodge, MD, MEd, FRCSC Assistant Professor, Division of Gynecologic Oncology, Department of Obstetrics and Gynecology Princess Margaret Hospital Andrew Dueck, MD, MSc, FRCSC, FACS, RPVI Assistant Professor, Division of Vascular Surgery Sunnybrook Health Sciences Centre
Matthew Binnie, MD, FRCP Assistant Professor, Division of Respirology, Department of Medicine St. Michael’s Hospital
Marc A. Freeman, MD, FRCPC Program Director, Nuclear Medicine Department of Medical Imaging Mount Sinai Hospital, University Health Network, Women’s College Hospital
Andrea Boggild, MSc, MD, DTMH, FRCPC Tropical Disease Unit Toronto General Hospital
Wayne Gold, MD, FRCPC Division of Infectious Diseases, Department of Medicine Toronto General Hospital
David K. Chan, MD, MEd, FRCPC Assistant Professor, Division of Neurology, Department of Medicine St. Michael’s Hospital
Barry J. Goldlist, MD, FRCPC, FACP, AGSF Professor and Director, Division of Geriatric Medicine, Department of Medicine University Health Network
Alice Y.Y. Cheng, MD, FRCPC Assistant Professor, Endocrinology and Metabolism, Department of Medicine St. Michael’s Hospital and Credit Valley Hospital Chi-Ming Chow, MDCM, MSc, FRCPC, FACC, FASE Associate Professor, Division of Cardiology, Department of Medicine St. Michael’s Hospital Tae-Bong Chung, MD, FRCPC Assistant Professor, Department of Medical Imaging Mount Sinai Hospital Tulin Cil, MD, MEd, FRCSC Assistant Professor, Division of General Surgery, Department of Surgery Princess Margaret Hospital Isabella Devito, MD, FRCPC Assistant Professor, Department of Anesthesia and Pain Management University Health Network and Mount Sinai Hospital
Jeremy A. Hall, MD, MEd, FRCSC Orthopaedic Trauma and Upper Extremity Surgeon St. Michael’s Hospital Philip C. Hébert, MA, PhD, MD, FCFPC Associate Professor, Department of Family and Community Medicine Ethics Consultant, Clinical Ethics Centre Sunnybrook Health Sciences Centre Sender Herschorn, MD, FRCSC Professor and Chair, University Division of Urology Sunnybrook Health Sciences Centre and Women’s College Hospital Janey Hsiao, MD Division of Hematology, Department of Medicine Sunnybrook Health Sciences Centre and Women’s College Hospital
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Jonathan C. Irish, MD, MSc, FRCSC, FACS Professor, Department of Otolaryngology – Head and Neck Surgery Chief, Department of Surgical Oncology University Health Network Nasir Jaffer, MD, FRCPC Associate Professor, Faculty of Medicine Department of Medical Imaging Mount Sinai Hospital, University Health Network, Women’s College Hospital Keith A. Jarvi, MD, FRCSC Professor, Division of Urology, Department of Surgery Director, Murray Koffler Urologic Wellness Centre Mount Sinai Hospital Dana Jerome, MD, MEd, FRCPC Assistant Professor, Division of Rheumatology, Department of Medicine Women’s College Hospital Ian Johnson, MD, MSc, FRCPC Associate Professor, Dalla Lana School of Public Health Scientific Advisor, Ontario Agency for Health Protection and Promotion David Juurlink, BPhm, MD, PhD, FRCPC Assistant Professor of Medicine, Pediatrics, and Health Policy, Management, and Evaluation Head, Division of Clinical Pharmacology and Toxicology Sunnybrook Health Sciences Centre Gabor Kandel, MD, FRCPC Associate Professor, Division of Gastroenterology, Department of Medicine St. Michael’s Hospital Simon J. Kingsley, MD, CCFP, EM Lecturer, University of Toronto Emergency Physician, St. Michael’s Hospital Young M. Kim, MD, DPhil Clinical Research Fellow, Division of Cardiovascular Surgery St. Michael’s Hospital
Toronto Notes 2011
Sari L. Kives, MD, FRCSC Assistant Professor, Department of Obstetrics and Gynecology St. Michael’s Hospital, Hospital for Sick Children
Ali Naraghi, MD, FRCR Associate Professor, Department of Medical Imaging University Health Network, Women’s College Hospital
Wai-Ching Lam, MD, FRCSC Associate Professor, Department of Ophthalmology and Vision Science Toronto Western Hospital
Markku T. Nousiainen, BA, MS, Med, MD, FRCSC Assistant Professor, Division of Orthopedic Surgery, Department of Surgery Sunnybrook Health Sciences Centre, Holland Orthopedic & Arthritic Centre
Perla Lansang, MD, FRCPC Assistant Professor, Division of Dermatology Sunnybrook Health Sciences Centre Jodi Lofchy, MD, FRCPC Associate Professor, Department of Psychiatry Director, Psychiatry Emergency Services University Health Network Armando Lorenzo, MD, FRCSC Assistant Professor. Division of Urology, Department of Surgery Hospital for Sick Children Ryan Mai, MD, FRCPC Department of Anesthesia St. Michael’s Hospital Todd Mainprize, MD, FRCSC Neurosurgeon, Department of Neurosurgery Sunnybrook Health Science Centre Heather McDonald-Blumer, MD, MSc, FRCPC Program Director, Division of Rheumatology, Department of Medicine Program Director, Core Internal Medicine University of Toronto Filomena Meffe, MD, FRCSC, MSc Associate Professor, Department of Obstetrics and Gynecology Director, Undergraduate Medical Education St. Michael’s Hospital Azadeh Moaveni, MD, CCFP Lecturer, Department of Family and Community Medicine Undergraduate Hospital Program Director Toronto Western Hospital
Andrea V. Page, MD, FRCPC Division of Infectious Diseases, Department of Medicine University Health Network Blake C. Papsin, MD, MSc, FRCSC, FACS, FAAP Cochlear Americas Chair in Auditory Development Professor, Department of Otolaryngology – Head and Neck Surgery The Hospital for Sick Children Division of Plastic and Reconstructive Surgery University of Toronto Vikramaditya Prabhudesai, MBBS, MSc, FRCR Division of Medical Imaging St. Michael’s Hospital Ramesh Prasad, MBBS, MSc, FRCPC, FACP Associate Professor, Division of Nephrology, Department of Medicine St. Michael’s Hospital
Gemini Tanna, MD, FRCPC Division of Nephrology, Department of Medicine Sunnybrook Health Sciences Centre John Teshima, MD, FRCPC, MEd Assistant Professor, Department of Psychiatry Sunnybrook Health Sciences Centre David R. Urbach, MD, MSc, FRCSC, FACS Covidien Chair, Minimally Invasive Surgery Associate Professor of Surgery and Health Policy, Management and Evaluation University Health Network Taufik A. Valiante, MD PhD FRCSC Assistant Professor, Division of Neurosurgery, Department of Surgery Co-Director, Epilepsy Program University Health Network Herbert P. von Schroeder, MD, MSc, FRCSC Associate Professor, Divisions of Orthopedic and Plastic Surgery, Department of Surgery University Health Network Michael Weinstein, MD, FRCPC, FAAP Assistant Professor and Director, Department of Pediatrics The Hospital for Sick Children Michael Wiley, BSc, MSc, PhD Professor and Division Chair, Division of Anatomy, Department of Surgery
Martin Schreiber, MD, FRCPC, MEd Associate Professor, Division of Nephrology, Department of Medicine St. Michael’s Hospital
David Wong, MD, FRCPC Assistant Professor, Division of Gastroenterology, Department of Medicine University Health Network
Fran E. Scott, MD, CCFP, FRCPC, MSc Associate Professor, Division of Epidemiology Dalla Lana School of Public Health
Anna Woo, MD SM, FRCPC, FACC Associate Professor, Division of Cardiology, Department of Medicine Toronto General Hospital
Darrell H.S. Tan, MD, FRCPC Division of Infectious Diseases, Department of Medicine St. Michael’s Hospital
Eugene Yu, MD Assistant Professor, Medical Imaging and Otolaryngology-Head and Neck Surgery Division of Neuroradiology, Department of Medical Imaging University Health Network
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Toronto Notes 2011
Table of Contents Index Abbreviations 1.
Ethical, Legal and Organizational Aspects of Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ELOAM
2.
Anesthesia and Perioperative Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A
3.
Cardiology and Cardiovascular Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . C
4.
Clinical Pharmacology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . CP
5.
Dermatology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . D
6.
Diagnostic Medical Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . DM
7.
Emergency Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ER
8.
Endocrinology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . E
9.
Family Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . FM
10. Gastroenterology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . G 11. General Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . GS 12. Geriatric Medicine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . GM 13. Gynecology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . GY 14. Hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . H 15. Infectious Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ID 16. Nephrology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . NP 17. Neurology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . N 18. Neurosurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . NS 19. Obstetrics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . OB 20. Ophthalmology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . OP 21. Orthopaedics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . OR 22. Otolaryngology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . OT 23. Pediatrics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . P 24. Plastic Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . PL 25. Population and Community Health . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . PH 26. Psychiatry . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . PS 27. Respirology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . R 28. Rheumatology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . RH 29. Urology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . U 30. Common Unit Conversions 31. Commonly Measured Laboratory Values 32. List of Abbreviations 33. Index Toronto Notes 2011
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How to Use This Book This book has been designed to remain as one book or to be taken apart into smaller booklets. Identify the beginning and end of a particular section, then carefully bend the pages along the perforated line next to the spine of the book. Then tear the pages out along the perforation. The layout of Toronto Notes 2011 allows easy identification of important information. These items are indicated by icons interspersed throughout the text:
Icon
Significance The ‘key’ icon, found next to headings in the text, identifies key objectives and causal conditions as defined by the Medical Council of Canada or the National Board of Medical Examiners USA. If it appears beside a ‘black-bar’ title, all subsequent subheadings should be considered key topics. The ‘pearl’ icon, found in the sidebar, identifies concise, important information which will aid in the diagnosis and/ or management of conditions discussed in the accompanying text. The ‘light bulb’ icon indicates helpful mnemonic devices and other memory aids. The ‘flag’ icon indicates information or findings that require urgent management or specialist referral. The ‘camera’ icon indicates topics that correspond with images found in the Colour Photo Atlas available online. The ‘X-ray’ icon indicates topics that correspond to information or images contained within the Radiology Atlas located online. The ‘computer’ icon indicates topics that correspond with electronic resources such as Functional Neuroanatomy™ or ECG Made Simple™ located online.
Chapter Divisions To aid in studying and finding relevant material quickly, each chapter is organized in the following general framework: Basic Anatomy/Physiology Review • features the high-yield, salient background information students are often assumed to have remembered from their early medical school education Common Differential Diagnoses • aims to outline a clinically useful framework to tackle the common presentations and problems faced in the area of expertise Diagnoses • the bulk of the book • etiology, epidemiology, pathophysiology, clinical features, investigations, management, complications, and prognosis Common Medications • a quick reference section for review of medications commonly prescribed
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Ethical, Legal and Organizational Aspects of Medicine Trevor Arnason and Shuo Chen, chapter editors Christoper Kitamura and Michelle Lam, associate editors Janine Hutson, EBM editor Dr. Philip Hébert, staff editor
Legal Matters. . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 The Doctor-Patient Relationship under the Law Consent under the Law Power of Attorney (POA) Confidentiality and Reporting Requirements Privacy of Medical Records Negligence and Liability Physician Competence and Conduct Ethics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 Principles of Ethics Code of Ethics Confidentiality Consent Assessing Capacity Truth Telling Resource Allocation Research Ethics Physician-Industry Relations Doctor-Patient Relationship Personal and Professional Conduct Areas of Controversy Organization of Health Care in Canada. . . . . . 16 History Key Principles of the Canada Health Act Health Care Expenditure and Delivery in Canada Role of the Provincial Licensing Authorities Distinction Between Licensure and Certification Role of Professional Associations The US Health Care System. . . . . . . . . . . . . . . 18 Health Care Expenditure and Delivery in the US Access to Health Services Health Care Reform Physician Responsibilities Regarding Death . . . . . . . . . . . . . . . . . . . . . . . . 20 Role of the Coroner Palliative and End-of-Life Care References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
Further information on these topics can be found in the Objectives of the Considerations of the Legal, Ethical and Organizational Aspects of the Practice of Medicine (CLEO) – which can be downloaded free of charge from the Medical Council of Canada website at www.mcc.ca/pdf/cleo.pdf Toronto Notes 2011
ELOAM1
ELOAM2
Legal Matters
Toronto Notes 2011
Legal Matters The Doctor-Patient Relationship under the Law N.B.: Canadian law applicable to medical practice varies between jurisdictions and changes over time. • Criminal law is nationwide, but noncriminal (civil) law varies between provinces. • This section is meant to serve only as a guide; students and physicians should ensure that their practices conform to local and current laws.
Fiduciary duty: obligation to act in the patient’s best interests
• laws which regulate doctor-patient relationship function to protect patients • derived from three sources: 1) common law (in Quebec, the Civil Code of Quebec), 2) statutes and 3) the Constitution • common law: body of legal rules and principles derived from judges’ decisions that forms the basis of the Anglo-Canadian legal system, includes: tort law that allows patients to recover damages for wrongful acts committed against them. The most important are 1) negligence (see Negligence and Liability, ELOAM6) and 2) battery (the application of force to a person’s body without their consent) the doctor-patient relationship is a contract of contractual rights and obligations that if breached may result in the award of damages a doctor also has a fiduciary duty to their patient • statutes are laws passed by provincial legislatures and the federal parliament, for example: in Ontario, the Health Care Consent Act regulates consent to treatment (see Consent under the Law, ELOAM2) the Personal Health Information Protection Act regulates the collection, use and disclosure of health records (see Privacy of Medical Records, ELOAM5) • the Constitution is the supreme law of Canada; all other laws must be consistent with it the Canadian Charter of Rights and Freedoms guarantees the rights of life, liberty, security of the person, and equality under the law (among others) these rights are subject only to such reasonable limits as can be demonstrably justified in a free and democratic society
Consent under the Law • consent of the patient must be obtained before any medical intervention is provided. Consent can be: oral or written, although written is usually preferred implied (e.g. a patient holding out their arm for an immunization) or expressed • consent is an ongoing process and can be withdrawn or changed after it is given • Health Care Consent Act covers consent to treatment, admission to a facility, and personal assistance services (e.g. home care)
Major Exceptions to Consent • Emergencies • Communicable diseases • Mental Health legislation
Exceptions to Consent 1. Emergencies treatment can be provided without consent where a patient is experiencing severe suffering, OR where a delay in treatment would lead to serious harm or death AND consent cannot be obtained from the patient or their substitute decision maker (SDM) emergency treatment should not violate a prior expressed wish of the patient (e.g. a signed Jehovah’s Witness card) if patient incapable, MD must document reasons for incapacity and why situation is emergent if a SDM is not available, MD can treat without consent until the SDM is available or the situation is no longer emergent 2. Legislation Mental Health legislation allows for: the detention of patients without their consent (see Consent, ELOAM8) psychiatric outpatients to be compelled to adhere to a care plan in accordance with Community Treatment Orders (see Psychiatry, PS51) Public Health legislation allows medical officers of health to detain, examine, and treat patients without their consent (e.g. a patient with TB refusing to take medication) to prevent transmission of communicable diseases (see Population and Community Health, PH4) 3. Special Situations public health emergencies (e.g. an epidemic or communicable disease treatment) warrant for information by police
Toronto Notes 2011
Legal Matters
Four Basic Requirements of Valid Consent 1. Voluntary consent must be given free of coercion or pressure the physician must not deliberately mislead the patient about the proposed treatment 2. Capable the patient must be able to understand the nature and effect of the proposed treatment 3. Specific the consent provided is specific to the procedure being proposed and to the provider who will carry out the procedure (i.e. the patient must be informed if students will be involved in providing the treatment) 4. Informed sufficient information must be provided to allow the patient to make choices in accordance with their wishes. This information should include: the nature of the treatment or investigation proposed and its expected effects all significant risks and special or unusual risks alternative treatments or investigations and their anticipated effects and significant risks the consequences of declining treatment risks that are common sense need not be disclosed (i.e. bruising after venipuncture) the reasonable person test – the physician must provide all information that would be needed “by a reasonable person in the patient’s position” to be able to make a decision disclose common adverse events (>1/200 chance of occurrence) and serious risks (e.g. death) even if remote it is the physician’s responsibility to make reasonable attempts to ensure that the patient understands the information physicians cannot withhold information about a therapeutic option based on personal conscience (e.g. not discussing the option of emergency contraception) Consequences of Failure to Obtain Valid Consent • treatment without consent is battery, even if the treatment is life-saving • treatment of a patient on the basis of poorly informed consent may constitute negligence • the onus of proof that valid consent was not obtained rests with the plaintiff (usually the patient) Capacity and Substitute Decision Makers (see Assessing Capacity, ELOAM9) • capable patients are entitled to make their own decisions • capacity assessments must be conducted by a physician and, if appropriate, in consultation with other health care professionals (e.g. another physician, a mental health nurse) • capable patients can refuse treatment even if it leads to serious harm or death; however, decisions that put patients at risk of serious harm or death require careful scrutiny Substitute Decision Makers (SDMs) • SDM must follow the following principles when giving informed consent: act in accordance with wishes previously expressed by the patient while capable if wishes unknown, act in the patient’s best interest, taking the following into account: 1. values and beliefs held by the patient while capable 2. whether well-being is likely to improve with vs. without treatment 3. whether the expected benefit outweighs the risk of harm 4. whether a less intrusive treatment would be as beneficial as the one proposed the final decision of the SDM may and should be challenged by the MD if the MD believes the SDM is not abiding by the above principles • most provinces have legislated hierarchies for SDMs; the hierarchy in Ontario is: 1. legally appointed guardian 2. appointed attorney for personal care, if a power of attorney confers authority for treatment consent (see Power of Attorney, ELOAM4) 3. representative appointed by the Consent and Capacity Board 4. spouse or partner 5. child (age 16 or older) or parent (unless the parent has only a right of access) 6. parent with only a right of access 7. sibling 8. other relative(s) 9. public guardian and trustee Treatment of the Incapable Patient • obtain informed consent from SDM • an incapable patient can only be detained against his/her will to receive treatment if he/she meets criteria for certification under the Mental Health Act (see Psychiatry, PS51). In such a situation: document assessment in chart notify patient of assessment using appropriate Mental Health Form(s) (Form 42) notify Rights Advisor
ELOAM3
The Supreme Court of Canada expects physicians to disclose the risks that a “reasonable” person would want to know. In practice, this means disclosing minor risks that are common as well as serious risks that happen infrequently.
Consent Treatment without consent = battery, including if NO consent or if WRONG procedure Treatment with poor or invalid consent = negligence
If the MD feels the SDM is not acting in the patient’s best interest, the MD can apply to Consent and Capacity Board for another SDM.
Administration of treatment for an incapable patient in an emergency situation is applicable if the patient is: 1. Experiencing extreme suffering 2. At risk of sustaining serious bodily harm if treatment is not administered promptly
ELOAM4
There is no age of consent.
Other Types of Capacity Not Covered by the HCCA • Testamentary (ability to make a will) • Fitness (ability to stand trial) • Financial (ability to manage property – Form 21 of the Mental Health Act) • Personal (ability to care for oneself on a daily basis)
Legal Matters
Toronto Notes 2011
Pediatric Aspects of Capacity Covered by the HCCA • no age of consent; consent depends on patient’s decision-making ability (capacity) N.B. PEI, NB, QC, SK, BC have specific age of consent, but common law and case law deem underage legal minors capable, allowing them to make their own choices • infants and children are assumed to lack mature decision-making capacity for consent but they should still be involved (i.e. be provided with information appropriate to their comprehension level) • adolescents are usually treated as adults • preferably, assent should still be obtained from patient, even if not capable of giving consent • in the event that the physician believes the SDM is not acting in the child’s best interest, an appeal must be made to the local child welfare authorities • under normal circumstances, parents have right of access to the child’s medical record Criteria for Financial Competence • covered by the Mental Health Act and Substitute Decision Act • patient must: appreciate importance of financial capability and reason for exam have realistic appreciation of own strengths/weaknesses in managing finances understand nature and extent of assets, liabilities, income, and expenses have recently demonstrated ability to make reasonable financial decisions and be expected to do so in future have appropriately used available resources and indicate willingness to do so in future • if MD determines the patient incapable of managing property, a Form 21 is completed and the Public Guardian and Trustee becomes the temporary guardian until a substitute can be found; eligible substitute guardians are patient’s spouse/partner, relative, or attorney • Form 21 can only be filled out if the patient is an inpatient of a psychiatric facility; to continue financial incapacity after discharge, fill out Form 24 Instructional Advance Directives • allow patients to exert control over their care once they are no longer capable • the patient sets out their decisions about future health care, including who they would allow to make treatment decisions on their behalf and what types of interventions they would want • takes effect once the patient is incapable with respect to treatment decisions • in Ontario, a person can appoint a power of attorney for personal care to carry out his/her advance directives • patients should be encouraged to review these documents with their family and physicians and to reevaluate them often to ensure they are current with their wishes
Power of Attorney (POA) LEGAL TERMS AND DEFINITIONS • all Guardians and Attorneys for Personal Care have fiduciary duties for the dependent person Power of Attorney for Personal Care • a legal document in which one person gives another the authority to make personal care decisions (health care, nutrition, shelter, clothing, hygiene, safety) on their behalf if they become mentally incapable Guardian of the Person • someone who is appointed by the Court to make decisions on behalf of an incapable person in some or all areas of personal care, in the absence of a POA for personal care Continuing Power of Attorney for Property • a legal document in which a person gives another the legal authority to make decisions about their finances if they become unable to make those decisions Guardian of Property • someone who is appointed by the Public Guardian and Trustee or the Courts to look after an incapable person’s property or finances Public Guardian and Trustee • acts as a SDM of last resort on behalf of mentally incapable people who do not have another individual to act on their behalf
Toronto Notes 2011
Legal Matters
ELOAM5
Confidentiality and Reporting Requirements • the legal duty to maintain patient confidentiality is imposed by provincial health information legislation and precedent-setting cases in the common law • the right to confidentiality is not absolute • disclosure of health information can take place with the patient’s consent or without the patient’s consent in certain circumstances defined by statutory and common law Statutory Reporting Obligations • legislation has defined specific instances where public interest overrides the patient right to confidentiality; varies by province, but may include: 1. suspected child abuse or neglect – report to local child welfare authorities (e.g. Children’s Aid Society) 2. fitness to drive a vehicle or fly an airplane – report to provincial Ministry of Transportation (see Geriatric Medicine, GM9) 3. communicable diseases – report to local public health authority (see Population and Community Health, PH27) 4. improper conduct of other physicians or health professionals – report to college or regulatory body of the health professional (sexual impropriety by physicians is required reporting in some provinces) 5. vital statistics must be reported; reporting varies by province (in Ontario, births are required to be reported within 30 days to Office of Registrar General or local municipality; death certificates must be completed by a MD then forwarded to municipal authorities) 6. reporting to coroners (see Physician Responsibilities Regarding Death, ELOAM20) • physicians who fail to report in these situations are subject to prosecution and penalty, and may be liable if a third party has been harmed Duty to Protect/Warn • the physician has a duty to protect the public from a known dangerous patient; this may involve taking appropriate clinical action (e.g. involuntary detainment of violent patients for clinical assessment), informing the police, or warning the potential victim(s) if a patient expresses an intent to harm • first established by a Supreme Court of California decision in 1976; supported by Canadian courts • obliged by the CMA Code of Ethics and recognized by some provincial/territorial regulatory authorities • concerns of breaching confidentiality should not prevent the MD from exercising the duty to protect; however, the disclosed information should not exceed that required to protect others • applies in a situation where: 1. there is a clear risk to identifiable person(s); 2. there is a risk of serious bodily harm or death; and 3. the danger is imminent (i.e. more likely to occur than not) Disclosure for Legal Proceedings • disclosure of health records can be compelled by a court order, warrant, or subpoena
Legal Aspects of Confidentiality Advice should always be sought from provincial licensing authorities and/or legal counsel when in doubt.
Ontario’s Medical Expert Panel on Duty to Warn Ferris et al., 1998 There should be a duty to inform when a patient reveals that he/she intends to do serious harm to another person(s) and it is more likely than not that the threat will be carried out. Where a threat is directed at a person or group and there is a specific plan that is concrete and capable of commission and the method for carrying it out is available to the threatener, the physician should immediately notify the police and, in appropriate circumstances, the potential victim. The report should include the threat, the situation, the physician’s opinion and the information upon which it is based.
Privacy of Medical Records • privacy of health information is protected by professional codes of ethics, provincial and federal legislation, the Canadian Charter of Rights and Freedoms, and the fiduciary duty • the federal government created the Personal Information Protection and Electronic Documents Act (PIPEDA), which established principles for the collection, use, and disclosure of information that is part of commercial activity (e.g. physician practices, pharmacies, private labs) • PIPEDA has been superseded by provincial legislation in many provinces, such as the Ontario Personal Health Information Protection Act, which applies more specifically to health information Duties of Physicians with Regards to the Privacy of Health Information • inform patients of information-handling practices through various means (e.g. posting notices, brochures and pamphlets, and/or through discussions with patients) • obtain the patient’s express consent to disclose information to third parties under Ontario privacy legislation, the patient’s express consent needs not be obtained to share information between health care team members involved in the “circle of care.” However, the patient may withdraw consent for this sharing of information. • provide the patient with access to their entire medical record; exceptions include instances where there is potential for harm to the patient or a third party • provide secure storage of information and implement measures to limit access to patient records • ensure proper destruction of information that is no longer necessary
CMA Code of Ethics • Protect the health information of your patients. • Provide information reasonable in the circumstances to patients about the reasons for the collection, use and disclosure of their health information. • Be aware of your patient’s rights with respect to the collection, use, disclosure and access to their health information; ensure that such information is recorded accurately.
ELOAM6
Legal Matters
Toronto Notes 2011
Negligence and Liability • negligence: breach of a legal duty of care which results in damage legal finding, not a medical one • physicians may be found negligent when the following four conditions are met: 1. the physician owed a duty of care to the patient (the existence of a doctor-patient relationship generally suffices) 2. the duty of care was breached (e.g. by failure to provide the standard of care) 3. the patient was injured or harmed 4. the harm or injury was caused by the breach of the duty of care • the standard of care is one that would reasonably be expected under similar circumstances of an ordinary, prudent physician of the same training, experience, and specialization • errors of judgement are not necessarily negligent making the wrong diagnosis is not negligent if a reasonable doctor might have made the same mistake in the same circumstances failure to reconsider the diagnosis if the patient does not respond to treatment may be negligent • physicians can be held liable for the negligent actions of their employees or other individuals they are supervising
Physician Competence and Conduct
CMA Code of Ethics Report any unprofessional conduct by colleagues to the appropriate authority.
• the competence and conduct of physicians is legally regulated in certain respects to protect patients and society • physicians are legally required to maintain a license with the appropriate authority • physicians must ensure that patients have access to continuous on-call coverage and are never abandoned • sexual conduct with patients, even when consented to by the patient, is a serious matter that can lead to criminal, civil, and disciplinary action sexual conduct includes intercourse, undue touching, inappropriate reference to sexual matters, sexual jokes, and physician presence when capable patients undress or dress physicians may have a personal relationship with a patient providing a year has passed since the last therapeutic contact physicians are prohibited from personal relationships with patients whom they saw for psychotherapy in Ontario, physicians must report any colleagues of whom they have information regarding sexual impropriety • physicians must maintain adequate records for each patient, including: showing that care has been continuous and comprehensive minimal standards for record-keeping include diagnosis, differential diagnosis, appropriate tests and referrals, coherent patient record (full standards available at www.cpso.on.ca) keeping records for 10 years in most jurisdictions although the medical record is the property of the physician or an institution, the patient or the patient’s delegate must be allowed full access to information in the medical record upon (usually written) request • in the hospital, physicians must ensure their own competence, respect hospital by-laws and regulations, practice only within the limits of granted privileges, cooperate with other hospital personnel, and maintain adequate hospital records
Toronto Notes 2011
Ethics
ELOAM7
Ethics Principles of Ethics • ethics deals with 1) the principles and values that help define what is morally right and wrong, and 2) the rights, duties and obligations of individuals and groups • there are two broad approaches to ethics: consequentialism and deontology consequentialism distinguishes right from wrong according to an action’s outcomes (i.e. the right thing to do is minimize suffering) while deontology is rule or duty-based (i.e. it is always wrong to punish the innocent) there is no one agreed upon ethical theory but most contemporary writers combine both approaches most widely used approach is ‘principlism’ The Four Principles Approach to Medical Ethics (‘principlism’) 1. Respect for Autonomy recognizes an individual’s right and ability to decide for themselves according to his/her beliefs and values respecting and promoting an individual patient’s values in decision making to empower him or her a patient’s decision may differ from the recommendation of the physician and the physician should understand, appreciate, and respect the patient’s perspective patients are not expected to act in ways considered reasonable by others as long as they do not harm others (this principle is not applicable to newborn children or situations where informed consent and choice are not possible or may not be appropriate) autonomy also requires showing fidelity to incapable patients’ prior capable views if known, and treating them with worth and dignity 2. Beneficence acting in the patient’s ‘best interests’, where these represent the patient’s values, beliefs, and preferences, so far as these are known the aim is to minimize harmful outcomes and maximize beneficial ones physicians recommend treatment based on evidence and professional experience to patients and help them weigh the risks and benefits of various options autonomy should be integrated with the physician’s conception of a competent patient’s best interests paramount in situations where consent/choice is not possible or may not be appropriate 3. Non-Maleficence obligation to avoid causing harm; primum non nocere (“First, do no harm”) efforts should be made to reduce error and adverse events and ensure patient safety a limit condition of the Beneficence principle 4. Justice fair distribution of benefits and harms within a community, regardless of geography or privilege scarce resources are distributed based on the needs of patients and the benefit they would receive from obtaining a specific resource (e.g. organs for transplantation are fairly distributed if they go to those who are the most unwell, who are the most likely to survive the longest with the transplant, and who have waited the longest to receive a transplant) concept of fairness: Is the patient receiving what he/she deserves? How do treatment decisions impact on others? respects rules of fair play and basic human rights, such as freedom from persecution and the right to have one’s interests considered and respected
Code of Ethics • the CMA has developed and approved a Code of Ethics that acts as a common ethical framework for Canadian physicians sources include the Hippocratic Oath, developments in human rights, recent bioethical discussion may set out different standards of behaviour than does the law prepared by physicians for physicians based on the fundamental ethical principles of medicine statements are general in nature applies to physicians, residents, and medical students
Four Ethical Principles 1. Autonomy 2. Beneficence 3. Non-maleficence 4. Justice
Autonomy vs. Competence Autonomy: the right that patients have to make decisions according to their beliefs and preferences Competence: the ability or capacity to make a specific decision for one’s self
Adverse Event (AE) An unintended injury or complication from health care management resulting in disability, death or prolonged hospital stay.
The Canadian Adverse Events Study: The Incidence of Adverse Events among Hospital Patients in Canada CMAJ 2004; 170(11):1678-86 Study: Review of random sample of charts in four randomly selected Canadian hospitals for the fiscal year 2000. Patients: 4174 patient charts sampled, 3745 eligible charts (>18 years of age; nonpsychiatric, nonobstetric, minimum 24 hour admission). Results: AE rate was 7.5% per 100 hospital admissions (95% CI 5.7-9.3). Highly preventable AEs occurred in 36.9% of patients with AEs (95% CI 32.0-41.8%) and death occurred in 20.8% (95% CI 7.8%-33.8%). An estimated 1521 additional hospital days were associated with AEs. Patients with AEs were significantly older than those without (mean age [and standard deviation] 64.9 [16.7] v. 62.0 [18.4] years; p=0.016). Men and women experienced equal rates of AEs. Conclusions: The overall incidence rate of AEs of 7.5% suggests that, of the almost 2.5 million annual hospital admissions in Canada similar to the type studied, about 185 000 are associated with an AE and close to 70 000 of these are potentially preventable.
ELOAM8
The CMA Code of Ethics is a quasi-legal standard for physicians. If the law sets a minimal moral standard for doctors, the Code ratchets up these standards.
Ethics
Toronto Notes 2011
• CMA policy statements address specific ethical issues not mentioned by the code (e.g. abortion, transplantation, and euthanasia) • the American Medical Association (AMA) has a Code of Medical Ethics articulates the values of medicine as a profession defines medicine’s integrity and is the source of the profession’s authority to self-regulate considered an evolving document that changes as new questions arise concerning medicine’s core values and its application to daily practice • the AMA develops policy positions (“AMA Policy”) regarding health care issues, the health care system, internal organizational structure, decision-making processes, and medical science and technology
Confidentiality Reasons to Breach Confidentiality • Child abuse • Fitness to drive • Communicable disease • Coroner report • Duty to inform/warn
CMA Code of Ethics “Disclose your patients’ personal health information to third parties only with their consent, or as provided for by law, such as when the maintenance of confidentiality would result in a significant risk of substantial harm to others or, in the case of incompetent patients, to the patients themselves. In such cases take all reasonable steps to inform the patients that the usual requirements for confidentiality will be breached.”
• a full and open exchange of information between patient and physician is central to a therapeutic relationship • privacy is a right of patients (which they may forego), while confidentiality is a duty of doctors (which they must respect barring patient consent or the requirements of the law) • if inappropriately breached by a doctor, he/she can be sanctioned by the hospital, by the court or by his or her regulatory authority (see Confidentiality and Reporting Requirements, ELOAM5) • based on the ethical principal of patient autonomy, patients have the right to: control their own information expect information concerning them will receive proper protection from unauthorized access by others (see Privacy of Medical Records, ELOAM5) • confidentiality may be ethically and legally breached in certain circumstances, e.g. the threat of harm to others (see Confidentiality and Reporting Requirements, ELOAM5) • unlike the solicitor-client privilege, there is no ‘physician-patient privilege’ by which a physician, even a psychiatrist, can promise the patient absolute confidentiality • physicians should seek advice from their local health authority or the Canadian Medical Protective Association (CMPA) before disclosing HIV status of a patient to someone else many jurisdictions make mandatory not only the reporting of serious communicable diseases (e.g. AIDS), but also the reporting of those who harbour the agent of the communicable disease physicians failing to abide by such regulations could be subject to professional or civil actions
Consent CPSO Policy Consent Obtaining valid consent before carrying out medical, therapeutic and diagnostic procedures has long been recognized as an elementary step in fulfilling the doctor’s obligations to the patient.
• the autonomous authorization of a medical intervention by a patient • applies to acceptance and refusal of treatment • also see Consent under the Law, ELOAM2 Ethical Principles Underlying Consent • usually the principle of respect for patient autonomy overrides the principle of beneficence • where a patient cannot make an autonomous decision, it is the duty of the SDM (or the physician in an emergency) to act on the patient’s known prior wishes or, failing that, to act in the patient’s best interests • there is a duty to discover, if possible, what the patient would have wanted when capable • central to determining best interests is understanding the patient’s values, beliefs, and cultural or religious background since these may affect the patient’s perception of treatments as beneficial or harmful • more recently expressed wishes should take priority over remote ones • patient preferences may be given orally (i.e. do not have to be in written form) Obtaining Consent • a signed consent form only documents the consent – it does not replace the process for obtaining valid consent (see Figure 1) • what matters is what the patient understands and appreciates, not what the signed consent form states • consent can be withdrawn at any point, unless stopping a procedure would put the patient at risk of serious harm • consent is not required in certain situations (see Consent under the Law, ELOAM2)
Toronto Notes 2011
Ethics
No
Is this a “treatment”?
ELOAM9
Use common law
Yes Is the patient capable to make this decision?
No
Is this an emergency?
Discuss involvement of SDM
No
Does patient dispute the finding of incapacity
Yes
Review Board
No
Do not treat
No
Yes Is there an SDM available?
Yes
Is the SDM capable to make this decision?
No Treat as emergency
Does the patient consent?
Yes
No
No Treat as emergency
Yes
Does the SDM consent? Yes Treat
Do not treat
Yes Treat
Figure 1. Consent Flowchart
SDM = substitute decision maker Adapted by P. Hébert from Sunnybrook Health Sciences Centre Consent Guidelines
Assessing Capacity • a person is presumed capable unless there is good evidence to the contrary • capacity is the ability to: understand information relevant to a treatment decision appreciate the reasonably foreseeable consequences of a decision or lack of a decision • capacity is specific for each decision (e.g. a person may be capable to consent to having a chest x-ray, but not for a bronchoscopy) • most Canadian jurisdictions distinguish capacity to make health care decisions from capacity to make financial decisions. A patient may be deemed capable of one, but not the other. • capacity can change over time (e.g. temporary incapacity secondary to delirium) • clinical capacity assessment may include: specific capacity assessment (i.e. capacity specific to the decision at hand) 1. effective disclosure of information and evaluation of patient’s reason for decision 2. understanding of: – his/her condition – the nature of the proposed treatment – alternatives to the treatment – the consequences of accepting and rejecting the treatment – the risks and benefits of the various options (test: can the patient recite back what you have disclosed?) 3. for the appreciation needed for decision making capacity, a person must: – acknowledge the condition that affects him/herself – be able to assess how the various options would affect him or her – be able to reach a decision and adhere to it, and make a choice, not based primarily upon delusional belief (test: are their beliefs responsive to evidence?) general impressions input from psychiatrists, neurologists, etc. • employ “Aid to Capacity Evaluation” (see Table 1)
CPSO Policy Capacity Capacity is an essential component of valid consent, and obtaining valid consent is a policy of the CMA and other professional bodies.
ELOAM10
Ethics
Toronto Notes 2011
Table 1. Aid to Capacity Evaluation Ability to understand the medical problem Ability to understand the proposed treatment Ability to understand the alternatives (if any) to the proposed treatment Ability to understand the option of refusing treatment or of it being withheld or withdrawn Ability to appreciate the reasonably foreseeable consequences of accepting the proposed treatment Ability to appreciate the reasonably foreseeable consequences of refusing the proposed treatment Ability to make a decision that is not substantially based on delusions or depression Adapted from Etchells et al. (1996).
• a decision of incapacity may warrant further assessment by psychiatrist(s), legal review boards (e.g. in Ontario, the Consent and Capacity Review Board), or the courts • judicial review is open to patients if found incapable • see Consent under the Law, ELOAM2 • ethical principles underlying capacity patient autonomy and respect for persons physician beneficence requires that incapable persons be protected from making harmful decisions even patients found incapable to make a specific decision should still be involved in that decision as much as possible (seek assent and cooperation and explore reasons for dissent) people should be allowed to make their own informed decisions, or to appoint their own SDM agreement or disagreement does not equal capacity
Truth Telling Ethical Basis • helps to promote and maintain a trusting physician-patient relationship • patients have a right to be told important information that physicians have regarding their care • enables patients to make informed decisions about health care and their lives CPSO Policy Truth Telling Physicians should provide patients with whatever information that will, from the patient’s perspective, have a bearing on medical decision-making and communicate that information in a way that is comprehensible to the patient.
Protocol to Break Bad News: SPIKES S Setting the scene and listening skills P patient’s perception of condition and seriousness I Invitation from patient to give information K Knowledge – giving medical facts E Explore emotions and empathize S Strategy and summary WF Baile and R Buckman, 2000.
Legal Basis • required for valid patient consent (see Consent under the Law, ELOAM2) goal is to disclose information that a reasonable person in the patient’s position would need in order to make an informed decision (“standard of disclosure”) • withholding information can be a breach of fiduciary duty and duty of care • obtaining consent on the basis of misleading information can be seen as negligent Evidence about Truth Telling • most patients want to know what is wrong with them • although many patients want to protect family members from bad news, they themselves would want to be informed in the same situation • truth telling improves compliance and health outcomes • informed patients are more satisfied with their care • negative consequences of truth telling can include decreased emotional well-being, anxiety, worry, social stigmatization, and loss of insurability Challenges in Truth Telling • medical error many jurisdictions and professional associations expect and require physicians to disclose medical error; any event that harms or threatens to harm patients must be disclosed to the patient or the patient’s family and reported to the appropriate health authorities physicians should disclose to patients the occurrence of adverse events or errors caused by medical management, but should not suggest that they resulted from negligence because: a) negligence is a legal determination and b) error is not equal to negligence (see Negligence and Liability, ELOAM6) disclosure allows the injured patient to seek appropriate corrective treatment promptly physicians should avoid simple attributions as to cause and sole responsibility of others or oneself physicians should offer apologies or empathic expressions of regret (“I wish things had turned out differently”) as these can increase trust and are not admissions of guilt or liability uniform Apology Acts across Canada protect apologies, both as expressions of regret and admissions of responsibility, from being used as evidence of liability and negligence • breaking bad news adequate support should be provided along with the disclosure of difficult news SPIKES protocol was developed to facilitate “breaking bad news”
Toronto Notes 2011
Ethics
ELOAM11
Arguments Against Truth Telling • may go against certain cultural norms and expectations • may lead to patient harm and increased anxiety • 10-20% of patients prefer not to be informed • medical uncertainty may result in the disclosure of uncertain or inaccurate information Exceptions to Truth Telling • waiving the right to know: patient declines information that would normally be disclosed • physicians should explore such decisions and determine if patient is capable • strong cultural or ethnic preferences for withholding information should be explored with the patient and it should be ascertained if the patient subscribes to these preferences • if patients continue to decline important information, the patient’s consent to inform someone else on their behalf should be sought • a patient may waive their right to know the truth about their situation when disclosure would in itself cause physical or mental harm to the patient a strong cultural component exists that must be respected and acknowledged the patient is incapacitated he/she is in a medical emergency the more weighty the consequences for the patient from non-disclosure, the more carefully one must consider the right to ignorance; arguably, such a patient could be considered as incapacitated and a SDM may need to be found to whom disclosure can be made • the doctrine of therapeutic privilege is rarely acceptable in Canadian courts refers to the withholding of information by the clinician in the belief that disclosure of the information would lead to anxiety, psychological distress or even self-harm. Such concerns have been found to be exaggerated and should not be grounds to deceive patients. clinicians should avoid invoking therapeutic privilege and allow patients to make decisions based on an accurate description of their medical situation
Resource Allocation • the distribution of goods and services to programs and people • physicians have the duty to inform patients about therapeutic options even if they are not available • ethics relate to justice: physicians must make health care resources available to patients in a manner which is fair and equitable, without bias or discrimination need and benefit are morally relevant criteria for resource allocation gender, sexual orientation, religion, level of education or age alone are morally irrelevant criteria • ethical dilemmas that arise when deciding how best to allocate resources fair chances versus best outcome – favouring best outcome vs. giving all patients fair access to limited resources (e.g. transplant list prioritization) priorities problem – how much priority should treating the sickest patients receive? aggregation problem – modest benefits to many vs. significant benefits to few democracy problem – when to rely on a fair democratic process as the only way to arrive at a decision • guidelines for appropriately allocating resources the physician’s primary obligation is to protect and promote the welfare and best interests of his or her patients choose interventions known to be beneficial on the basis of evidence of effectiveness seek the tests or treatments that will accomplish the diagnostic or therapeutic goal for the least cost advocate for one’s patients but avoid manipulating the system to gain unfair advantage for them resolve conflicting claims for scarce resources justly, on the basis of morally relevant criteria such as need and benefit, using fair and publicly defensible procedures inform patients of the impact of cost constraints on care, but in a sensitive way seek resolution of unacceptable shortages at the level of hospital management or government
CPSO Policy Resource Allocation Physicians should “recognize [their] responsibility to promote fair access to health care resources” and should “use health care resources prudently.”
ELOAM12
Ethics
Toronto Notes 2011
Research Ethics Guiding Principles for Research Ethics 1. Respect for persons (i.e. informed consent) 2. Beneficence (i.e. balancing benefits and harms) 3. Justice (i.e. avoiding exploitation or unjustified exclusion)
• involves the systematic analysis of ethical dilemmas arising during research involving human subjects to ensure that: study participants are protected clinical research is conducted to serve the interests of the participants and/or society as a whole • major ethical dilemmas arise when a physician’s obligation to the patient comes into conflict with other obligations and incentives • any exceptions to disclosure for therapeutic consent do not apply in an experimental situation Table 2. Ethical Principles for Research Involving Human Subjects Patient’s participation in research should not put him/her at a known or probable disadvantage with respect to medical care
Informed Consent for Research • The nature of informed consent differs in the contexts of research and clinical practice in that the potential research subject must be informed about: � Purpose of the study � Source of funding � Nature and relative probability of harms and benefits � Nature of the physician’s participation including any compensation • Proposals for research must be submitted to a research ethics board to be scientifically and ethically evaluated and approved.
Participant’s voluntary and informed choice is usually required Consent may not be required in special circumstances: chart reviews without patient contact; emergency situations for which there is no accepted or helpful standard of care and the proposed intervention is not likely to cause more harm than such patients already face Access to the treatment that is considered standard Placebo-controlled trials are generally acceptable where patients still receive the standard of care and are informed about the placebo arm and what that entails Must employ a scientifically valid design to answer the research question Scientific rigour ensured via peer review, expert opinion Must demonstrate sufficient value to justify the risk posed to participants Must be conducted honestly (i.e. carried out as stated in the approved protocol) Findings must be reported promptly and accurately without exaggeration, to allow practicing clinicians to draw reasonable conclusions Patients must not be enticed into risky research by the lure of money and investigators must not trade the interests of patients for disproportionate recompense by a sponsor; both participants and investigators are due fair recompense for their time and efforts Any significant interventional trial ought to have a data safety monitoring board that is independent of the sponsor and can ensure safety of the ongoing trial Laid out in the Declaration of Helsinki, the Belmont Report, etc.
Physician-Industry Relations • health care delivery in Canada involves collaboration between physicians and the pharmaceutical and health supply industries in the areas of research, education, and clinical evaluation packages (e.g. product samples) • physicians have a responsibility to ensure that their participation in such collaborative efforts is in keeping with their duties to their patients and society • gifts or free products from the pharmaceutical industry are inappropriate sponsorship for travel and fees for conference attendance may be accepted only where the physician is a conference presenter and not just in attendance physicians receiving such sponsorship must disclose this at presentations or in written articles • CMA and CPSO guidelines for ethically appropriate physician-industry relations the primary goal should be the advancement of the health of Canadians relationships should be guided by the CMA Code of Ethics the physician’s primary obligation is to the patient physicians should avoid any self-interest in their prescribing and referral practices physicians should always maintain professional autonomy, independence, and commitment to the scientific method • the AMA Code of Medical Ethics has a number of opinions on “Practice Matters” including “Industry representatives in clinical settings,” “Financial incentives and the practice of medicine,” and “Gifts to physicians from industry,” (see www.ama-assn.org/ama/pub/physicianresources/medical-ethics/code-medical-ethics.shtml)
Doctor-Patient Relationship • a partnership based on the physician providing expert opinion, information, options, and interventions that allows the patient to make informed choices about their health care • within this relationship, the doctor and patient share the goals of positive health outcomes, good communication, honesty, flexibility, sensitivity, informed consent, and respect • this relationship has the potential to be unequal due to a power difference patients are ill and lack medical knowledge physicians possess medical knowledge and skills and have their patients’ trust
Toronto Notes 2011
Ethics
• due to the nature of the doctor-patient relationship, the physician will: place the best interests of the patient first establish a relationship of trust with the patient follow through on undertakings made to the patient in good faith • the physician will accept or refuse patients requesting care: without consideration of race, gender, age, sexual orientation, financial means, religion or nationality without arbitrary exclusion of any particular group of patients, such as those known to be difficult or afflicted with serious disease except in emergency situations, in which case care must be rendered • once having accepted a patient into care, the physician may terminate the relationship providing: it is not an emergency care has been transferred adequate notice has been given to allow the patient to make alternative arrangements they have other options to find ‘medically necessary care’ • the physician will not exploit the doctor-patient relationship for personal advantage – financial, academic or otherwise • the physician will disclose limitations to the patient where personal beliefs or inclinations limit the treatment the physician is able to offer • the physician will maintain and respect professional boundaries at all times including physical, emotional, and sexual boundaries regarding treatment of themselves, their families, and friends
ELOAM13
CPSO Policy: Treating Self and Family Members Physicians will not diagnose or treat themselves or family members except for minor conditions or in emergencies and then only if no other physician is readily available.
CPSO Policy: Ending the PhysicianPatient Relationship Discontinuing services that are needed is an act of professional misconduct unless done by patient request, alternative services are arranged, or adequate notice has been given.
Personal and Professional Conduct CanMEDS Competencies • a framework of professional competencies established by the Medical Council of Canada (MCC) as objectives for the Medical Council of Canada Qualifying Exam (MCCQE) • further information on MCC objectives can be found at www.mcc.ca 1. Communicator; Culturally Aware • display sensitivity to people of all ages, races, cultures, religions, sexual orientations, and genders • accept or refuse patients without consideration of age, race, culture, religion, sexual orientation, and gender • understand the variation in values and morals and their impact on approaches to care and decision-making • elicit patients’ beliefs, concerns, and expectations about their illness • conduct patient-centered interviews, ensure patient comprehension 2. Collaborator • respect all members of the health care team • identify the roles and competencies of each member, and delegate tasks appropriately • consult other physicians and health care professionals effectively and appropriately • consult with patients and families regarding continuing care plans • be able to outline co-ordination of services (e.g. Public Health, Home Care, Social Services, Workers’ Compensation, Children’s Aid Society, etc.) 3. Health Advocate • identify determinants of health: biological (e.g. genes, impact of lifestyle) physical (e.g. food, shelter, working conditions) social (education, employment, culture, access to care) • influence public health and health policy to protect, maintain, and promote the health of individuals and the community 4. Manager • meet regulatory requirements in an office practice (e.g. medical record-keeping, narcotic control, infection control, etc.) • be prudent in utilization of health care resources, based on anticipated cost-benefit balance • regulate work schedule such that time is available for continuing education 5. Professional • maintain standards of excellence in clinical care and ethical conduct • exhibit appropriate personal and interpersonal behaviour • enhance clinical competence through lifelong learning • accept responsibility for personal actions • do not exploit the physician-patient relationship for personal advantage (e.g. financial, academic) 6. Scholar • commitment to critical appraisal, constructive skepticism • participate in the learning of peers and others (e.g. students, health care professionals, patients)
Professional Considerations Elderly Patient • Identify their resuscitation options (CPR vs. DNR), if applicable • Check for documentation of advance directives and POA where applicable • For further details, see Geriatric Medicine, GM11 Pediatric Patient • Identify the primary decision-maker (parents, guardian, wards-of-state, emancipated) • Regarding capacity assessment see Pediatric Aspects of Capacity Covered by the HCCA (ELOAM4) • Be wary of custody issues if applicable Terminally Ill or Palliative Patient • Consider the SPIKES approach to breaking bad news • What are their goals of care, i.e. disease vs. symptom management? • Identify advance directives, POA, or SDM, if applicable • Check for documentation of resuscitation options (CPR vs. DNR) and likelihood of success • For further details, see Geriatric Medicine, GM11 Incapable Patient • If not already present, perform a formal capacity assessment • Identify if the patient has a SDM or who has their POA • Check the patient’s chart for any Mental Health Forms (e.g. Form 1) or any forms they may have on their person (e.g. Form 42)
ELOAM14
Ethics
Toronto Notes 2011
Areas of Controversy Dealing with Controversial and Ethical Issues in Practice • Discuss in a non-judgmental manner • Ensure patients have full access to relevant and necessary information • Identify if certain options lie outside of your moral boundaries and refer to another physician if appropriate • Consult with appropriate ethics committees or boards • Protect freedom of moral choice for students or trainees Source: MCC-CLEO Objectives, 1998
Euthanasia: Ethically Appropriate Actions • Respect competent decisions to forgo treatment • Provide appropriate palliative measures • Decline requests for euthanasia and assisted suicide
Euthanasia and Physician-Assisted Suicide • euthanasia: a deliberate act undertaken by one person with the intention of ending the life of another person to relieve that person’s suffering where the act is the cause of death • physician-assisted suicide: the act of intentionally killing oneself with the assistance of a physician who deliberately provides the knowledge and/or the means • ethical issues and arguments: right to make autonomous choices about the time and manner of own death belief that there is no ethical difference between the acts of euthanasia/assisted suicide and foregoing life-sustaining treatments belief that these acts benefit terminally ill patients by relieving suffering patient autonomy has limits death should be the consequence of the morally justified withdrawal of life-sustaining treatments only in cases where there is a fatal underlying condition, and it is the condition (not the withdrawal of treatment) that causes death • law Canada: euthanasia and physician-assisted suicide are punishable offences under the Criminal Code of Canada US: euthanasia is punishable under general homicide laws; Oregon and Washington are the only states to have enacted legislation allowing physicians to actively assist patients who wish to end their lives • the use of palliative sedation with opioids in end-of-life care, knowing that death may occur as an unintended consequence (principle of double effect) is distinguished from euthanasia and assisted suicide where death is the primary intent • the appropriate withdrawal of life-support is distinguished from euthanasia and assisted suicide as it is seen as allowing the underlying disease to take its ‘natural course’ Maternal-Fetal Conflict of Rights • conflict between maternal autonomy and the best interests of the fetus • ethical issues and arguments principle of reproductive freedom: women have the right to make their own reproductive choices coercion of a woman to accept efforts to promote fetal well-being is an unacceptable infringement of her personal autonomy • law: upholds a woman’s right to life, liberty, and security of person and does not recognize fetal rights if a woman is competent and refuses medical advice, her decision must be respected even if the fetus will suffer the fetus does not have legal rights until it is born alive and with complete delivery from the body of the woman • Royal Commission on New Reproductive Technologies recommendations: medical treatment must never be imposed upon a competent pregnant woman against her wishes no law should be used to confine a pregnant woman in the interest of her fetus the conduct of a pregnant woman in relation to her fetus should not be criminalized child welfare should never be used to control a woman’s behaviour during pregnancy civil liability should never be imposed upon a woman for harm done to her fetus during pregnancy • ethically appropriate actions a woman is permitted to refuse HIV testing during pregnancy, even if vertical transmission to fetus results a woman is permitted to refuse Caesarean section in labour that is not progressing, despite evidence of fetal distress
Advanced Reproductive Technologies: Ethically Appropriate Actions • Educate patients and address contributors to infertility (e.g. stress, alcohol, medications, etc.) • Investigate and treat underlying health problems causing infertility • Wait at least one year before initiating treatment with ART (exceptions – advanced age or specific indicators of infertility) • Educate and prepare patients for potential negative outcomes of ART
Advanced Reproductive Technologies (ART) • includes non-coital insemination, hormonal ovarian stimulation, and in vitro fertilization (IVF) • ethical issues and arguments donor anonymity vs. child-centred reproduction (i.e. knowledge about genetic medical history) preimplantation genetic testing for diagnosis before pregnancy lack of sufficient data regarding efficacy and complications to provide the full disclosure needed for truly informed consent use of new techniques without patients appreciating their experimental nature embryo status – the Supreme Court of Canada maintains that fetuses are “unique” but not persons under law; this view would likely apply to embryos as well access to ART private vs. public funding social factors limiting access to ART (e.g. same-sex couples) commercialization of reproduction; reimbursement of gamete donors is currently illegal in Canada
Toronto Notes 2011
Ethics
Fetal Tissue • pluripotent stem cells have been derived from human embryonic and fetal tissue • potential uses of stem cells in research: studying human development and factors that direct cell specialization evaluating drugs for efficacy and safety in human models cell therapy: using stem cells grown in vitro to repair or replace degenerated/destroyed/ malignant tissues (e.g. Parkinson’s disease) genetic treatment aimed at altering somatic cells (i.e. myocardial or immunological cells) is acceptable and ongoing genetic treatment aimed at altering germ cells is prohibited in Canada and elsewhere • Tri-Council Policy Statement: Ethical Conduct for Research Involving Humans (Government of Canada, 2003) embryo research is permitted up to 14 days post-fertilization embryos created for reproductive purposes that are no longer required may be used gamete providers must give free and informed consent for research use no commercial transactions in the creation and use of the embryos is permitted creation of embryos solely for research purposes is prohibited human cloning is strictly prohibited • risks of coercion must be minimized: may not pressure fertility treatment team to generate more embryos than necessary only discuss option of using fetal tissue for research after free and informed choice to have a therapeutic abortion has been made physicians responsible for fertility treatment may not be part of a stem cell research team
ELOAM15
The CMA remains neutral on the issue of embryonic stem cell research.
Abortion • abortion: the active termination of a pregnancy before fetal viability fetal viability: fetus >500 g, or >23-24 weeks gestational age in the case of multiple pregnancy, selective termination of the non-viable or less viable fetus is allowed • ethical and legal issues and arguments: according to common law, the rights of a fetus are not equal to those of a human being who should have input into the abortion decision (e.g. male partners, patient’s guardians) no law currently regulates abortion in Canada – it is a woman’s medical decision to be made in consultation with whom she wishes; no mandatory role for spouse/family • CMA policy on induced abortion: induced abortion should not be used as an alternative to contraception counselling on contraception must be readily available full and immediate counselling services must be provided in the event of unwanted pregnancy there should be no delay in the provision of abortion services no patient should be compelled to have a pregnancy terminated physicians should not be compelled to participate in abortion – if morally opposed, the physician should inform the patient so she may consult another physician no discrimination should be directed towards either physicians who do not perform or assist at induced abortions or physicians who do induced abortion should be uniformly available to all women in Canada and health care insurance should cover all the costs (N.B. the upper limit of gestational age for which coverage is provided varies between provinces) Genetic Testing • uses: confirm a clinical diagnosis detect genetic predisposition to a disease allows preventative steps to be taken and helps patient prepare for the future give parents the option to terminate a pregnancy or begin early treatment • ethical dilemmas arise because of the nature of genetic information: it has individual and familial implications it pertains to future disease it often identifies disorders for which there are no effective treatments or preventive steps • ethical issues and arguments: obtaining informed consent is difficult due to the complexity of genetic information doctor’s duty to maintain confidentiality vs. duty to warn family members risk of social discrimination (e.g. insurance) and psychological harm • law: no current specific legislation exists testing requires informed consent no standard of care exists for clinical genetics but physicians are legally obligated to inform patients that prenatal testing exists and is available breach of confidentiality – duty to warn family members only acceptable if can likely prevent serious harm, such as if treatment or prevention is available (e.g. familial adenomatous polyposis)
Genetic Testing: Ethically Appropriate Actions • Thorough discussion and realistic planning with patient before testing is done • Genetic counselling for delivery of complex information, supportive discussion
ELOAM16
Organization of Health Care in Canada
Toronto Notes 2011
Organization of Health Care in Canada • one federal, three territorial, and ten provincial systems • federal system provides care to Aboriginal groups, the RCMP, and the armed forces • financed by both the public (70%) and private (30%) sectors • each provincial plan must cover all medically necessary health services delivered in hospitals and by physicians; may choose to cover additional services such as home care and prescription drugs • non-insured health services and fees are either covered by private insurance or by the individual • workers’ compensation funds cover treatment for work-related injuries and diseases The current legal foundation of the Canadian health system is based on three statutes: 1. Constitution Act (1867) – deals primarily with the jurisdictional power between federal and provincial governments 2. Canada Health Act (1984) – outlines the national terms and conditions 3. Canada Health and Social Transfer Act (1996) – sets the conditions for fiscal transfers from the federal government to the provinces and territories
History 1867
British North America Act (now Constitution Act) establishes Canada as a confederacy • government has minimal role in health care at this time • “establishment, maintenance, and management of hospitals” under provincial jurisdiction
1947
Saskatchewan introduces universal hospital insurance • based on taxes and premiums • other provinces follow
1957
Federal government passes Hospital Insurance and Diagnostic Services Act • provinces with universal hospital insurance receives federal funds • federal government pays for approximately 50% of insured services
1962
Saskatchewan implements universal medical care insurance • physician services included
1965
Royal Commission on Health Services (Hall Commission) recommends federal leadership and financial support with provincial government operation
1966 Medical Care Act passed by federal government • federal government contribution maintained at 50% on average, with poorer provinces receiving more funds • medical insurance must be �Comprehensive �Portable �Universal�Publicly administered 1977 Established Programs Financing Act passed by federal government • federal government gives “tax points” to provinces by reducing federal taxes and allowing provinces to collect more • funding no longer tied to direct services g federal influence wanes • provinces bear greater costs and impose restrictions on physicians • physicians respond with “extra-billing:” patients pay a supplementary fee 1984
Canada Health Act passed by federal government • replaced Medical Care Act and Hospital Insurance and Diagnostic Services Act • extra-billing banned by new fifth criterion: Accessibility
1996 Canada Health and Social Transfer Act passed by federal government • federal government gives provinces a single grant for health care, social programs, and post-secondary education; division of resources at provinces’ discretion 1999 Social Union Framework Agreement signed by the Prime Minister and all Premiers and territorial leaders except Quebec • federal and provincial/territorial governments vow to concentrate their efforts to modernize Canadian social policy 2001 Kirby and Romanow Commissions appointed Kirby Commission (final report, October 2002) • one-member committee of the Senate: examined history of health care system in Canada, pressures and constraints of current health care system, role of federal government, and health care systems in foreign jurisdictions Romanow Commission (final report, November 2002) • one-member royal commission (former Saskatchewan Premier Roy Romanow) appointed by the Prime Minister to inquire into and undertake dialogue with Canadians on the future of Canada’s public health care system
Toronto Notes 2011
Organization of Health Care in Canada
ELOAM17
2003 First Ministers’ Accord on Health Care Renewal signed • First Ministers agreed on an action plan to improve access to quality care for all Canadians and to prepare an annual public report on primary and home care • 1st Health Council (composed of government and expert/public representatives) appointed to improve accountability in the health care system 2004 First Ministers’ Meeting on the Future of Health Care produces a 10-year plan • priorities include reductions in waiting times, development of a national pharmacare plan, and primary care reform 2005 Chaoulli v. Quebec, Supreme Court of Canada Decision • ruled that banning private insurance is unconstitutional under the Quebec Charter of Rights, given that patients do not have access to those services under the public system in a timely way
Key Principles of the Canada Health Act 1. Public Administration provincial health care programs must be administered by public authorities 2. Comprehensiveness provincial health care programs must cover all necessary diagnostic, physician, and hospital services 3. Universality all eligible residents must be entitled to health care services 4. Portability emergency health services must be available to Canadians who are outside their home province, paid for by the home province 5. Accessibility user fees, charges, or other obstructions to insured health care services are not permitted
The federal government can reduce its contributions to provinces that violate the key principles of the Canada Health Act.
Health Care Expenditure and Delivery in Canada • projected total health care expenditure in 2006 was $148 billion, 10.0% of the GDP, approx. $3678 USD per capita; this includes out-of-pocket, government-funded and third-party expenditures (Canadian Institute of Health Information) • the 2006 Canadian health care expenditure increased 5.8% over 2005 spending (Canadian Institute of Health Information) • the 2006 Canadian health care expenditure as a percentage of GDP ranked eighth out of 30 Organization for Economic Cooperation and Development (OECD) member nations • 70.4% of health care spending came from public sector sources in 2006, as compared to 45.8% in the US • in 2006 there were 2.1 physicians per 1000 population, ranking 26th out of OECD member countries Delivery of Health Care • hospital services in Canada are publicly funded but delivered through private, not-for-profit institutions owned and operated by communities, religious organizations, and regional health authorities • this differs from other countries, such as the US (a mix of public and private funding, as well as private-for-profit and private not-for-profit delivery) and the UK (primarily public funding and delivery)
Public Health $10.6, 6.6%
Capital $7.3, 4.5%
Administration $5.6, 3.5%
Other Health Spending $10.3, 6.3%
Hospitals $45.4, 28.2%
Other Institutions $16.4, 10.2%
Drugs $26.5, 15.5%
Other Professionals $17.3, 10.8%
Physicians $21.5, 13.4%
Figure 2. Health Expenditure in Canada by Use of Funds (Billions of Dollars), 2007
Source: Canadian Institute for Health Information, National Health Expenditure Trends, 1975 to 2009
Private Health Insurance 12% Household (Out of Pocket) 15% Municipal Governments 1% Social Security Funds 1% Federal Direct 4%
Non-Consumption 3%
Provincial Territorial Governments 65%
Figure 3. Canadian Health Care Dollars by Source of Funds, 2007
Source: Canadian Institute for Health Information, National Health Expenditure Trends, 1975 to 2009
Payments for Care at Private For-Profit and Private Not-for-Profit Hospitals: A Systematic Review and Meta-analysis CMAJ 2004; 170(12):1817-24 Meta-analysis of 8 US observational studies involving more than 350 000 patients. Concluded that care provided by private for-profit hospitals was more expensive (Relative payments for care=1.19; 95% CI=1.07-1.33; p=0.001). If half of Canadian hospitals were converted to private for-profit institutions, an extra $3.6 billion would be paid annually.
ELOAM18
Organization of Health Care in Canada/The US Health Care System
Toronto Notes 2011
Role of the Provincial Licensing Authorities • the medical profession in Canada self-regulates under the authority of provincial legislation; Canada is the only country in the world where the medical profession still regulates itself • physicians in each province are self-regulated by a licensing authority (see Role of Professional Associations); membership is mandatory to practice in that province • system of self-regulation is based on the premise that the licensing authority must act first and foremost in the interest of the public • Licensing Authority functions include issuing non-transferable licenses allow doctors to practice only in that province maintaining ethical, legal, and competency standards and developing policies to guide doctors investigating complaints against doctors disciplining doctors guilty of professional misconduct or incompetence (in most Canadian jurisdictions there is zero tolerance for sexual misconduct by physicians, resulting in harsh penalties including permanent suspension from the profession)
Distinction Between Licensure and Certification Certification by the LMCC plus either the RCPSC or CFPC is a minimum requirement for licensure by most provincial licensing authorities.
• provincial licensing authorities provide non-transferable licensure to physicians • the Medical Council of Canada (MCC) certifies physicians certification is known as the Licentiate of the MCC (LMCC) LMCC is acquired by passing the MCC Qualifying Examination Parts I and II • the Royal College of Physicians and Surgeons of Canada (RCPSC) certifies specialists who complete an accredited residency program and pass the appropriate exam voluntary membership of RCPSC is designated FRCPC or FRCSC (Fellow of the Royal College of Physicians/Surgeons of Canada) • the College of Family Physicians of Canada (CFPC) certifies family physicians who complete an accredited residency program and pass the Certification Examination in Family Medicine • the RCPSC and CFPC are responsible for monitoring ongoing continuing medical education (CME) and professional development
Role of Professional Associations • provincial medical associations represent the economic and professional interests of doctors; membership is voluntary, although fee payment is mandatory in some provinces • the Canadian Medical Association (CMA) is a national association that provides leadership to doctors and advocates for access to high quality health care in Canada; membership is voluntary and requires provincial medical association membership • the CMA represents physicians’ concerns at the national level, while the provincial medical associations negotiate fee and benefit schedules with provincial governments • medical residents are represented nationally by the Canadian Association of Interns and Residents, and provincially by Provincial Housestaff Organizations, which uphold the economic and professional interests of residents • medical students are represented at their universities by student societies; these bodies collectively form the Canadian Federation of Medical Students; francophone medical schools participate in the Federation of Quebec Medical Student Societies • the Canadian Medical Protective Association (CMPA), a physician-run organization, is a voluntary insurance association that protects the integrity of member physicians by providing legal defense against allegations of malpractice or negligence and by providing risk management and educational programs, and general advice
The US Health Care System • the United States health care system is market-based • it is funded and delivered by a mixture of the public, private, and voluntary sectors; private-forprofit is the prevailing method of delivery • public funding is derived from taxes raised at both the federal and state government levels
Toronto Notes 2011
The US Health Care System
ELOAM19
Health Care Expenditure and Delivery in the US • health care spending in the US represents a large economic sector health care comprises over 15% of the gross domestic product (GDP) (highest in the OECD), amounting to $6714 USD per capita in 2006 one advantage is the widespread availability of technology – the US has 4 times as many MRI machines per capita than Canada • the US scores poorly on some indicators of population health, with a life expectancy below the OECD average and infant mortality above the OECD average. Possible factors that account for this discrepancy are: poor health of large uninsured population high cost of health care administration the provision of inefficient high-cost, high-intensity care the higher-spending regions in the US do not provide any better quality of care, access to care, health outcomes or satisfaction with care when compared to the lower-spending regions • the US has the highest level of obesity of all OECD nations at 34.3%; this has major implications for future health care spending Other Federal – 4% Medicaid (State) – 7%
Other State/Local – 3%
Home Health – 3%
H
Private Health Insurance 36%
Medicaid (Federal) – 9% Medicare 22%
Nursing Home – 7%
Other Private – 4%
Physicians and Clinical Services – 26%
Public (45%)
Figure 4. Personal Health Care Expenditure in USA by Source of Funds, 2007
Source: National Center for Health Statistics. Health, United States, 2009: With Special Feature on Medical Technology. Hyattsville, MD. 2010.
Hospital – 37%
Prescription Drugs – 12%
Out-of-pocket 14% Private (55%)
Other – 10%
Dental – 5%
Figure 5. Personal Health Care Expenditure in USA by Type, 2007
Source: National Center for Health Statistics. Health, United States, 2009: With Special Feature on Medical Technology. Hyattsville, MD. 2010.
Access to Health Services • 70% of Americans under the age of 65 have private health insurance, either employer-sponsored or individually purchased; 12% receive health care through public health insurance; 18%, mainly the poor, have no health insurance • access to publicly funded health services occurs primarily through two programs, Medicare and Medicaid (see Table 3), which were created by the 1965 Social Security Act • other federal government-funded health programs include the Military Health Services System, the Veterans Affairs Health Services System, the Indian Health Service, and the Prison Health Service Table 3. Medicare and Medicaid Program Information Medicare
Medicaid
Eligibility
People over the age of 65 People with end stage renal disease People of any age meeting the Medicare definition of disability
People who receive funds through social assistance programs Pregnant women People with developmental disabilities Low-income children through the 1997 State Children’s Health Insurance Program
Coverage
Basic “Part A” providing inpatient hospital care, home care, limited skilled nursing facility care, and hospice care Supplemental “Part B” covers outpatient physician and clinic services, and requires payment of a further monthly fee
Basic coverage involves inpatient and outpatient hospital care, laboratory and x-ray services, skilled nursing care, home care, physician services, dental services, and family planning Financing for Medicaid is provided jointly by the federal and state governments, and program details vary greatly between states
Co-payment To help pay for out-of-pocket expenditures, and to cover many of the services not insured by Medicare, the majority of Medicare beneficiaries buy supplemental private health insurance Source: Centers for Medicare and Medicaid Services; www.cms.gov
States may impose deductibles, coinsurance, or co-payments on some Medicaid recipients for certain services Medicaid is not health insurance – coverage is unreliable as improvement in an individual’s financial status can lead to a loss of Medicaid eligibility
Cost of Health Care Administration in the United States and Canada NEJM 2003; 349:768-75 Administrative costs were estimated from data on insurance overhead, employers’ costs to manage benefits, and the administrative costs of hospitals, practitioners’ offices, nursing homes, and home care. In 1999, the cost of U.S. health administration was $1,059 per capita, more than three times greater than the cost in Canada ($307 per capita).
ELOAM20
The US Health Care System/Physician Responsibilities Regarding Death
Toronto Notes 2011
Health Care Reform • Patient Protection and Affordable Care Act and the Health Care and Education Reconciliation Act of 2010 are federal statutes signed into law in March 2010 that include a number of new healthcare provisions to be implemented over 8 years expand Medicaid eligibility, provide subsidies for insurance premiums and incentives for businesses to provide health care benefits, prohibit denial of coverage/claims for pre-existing conditions, and establish health-insurance exchanges costs are offset by a number of health care related taxes, including a tax penalty for citizens with no health insurance (low income persons are exempt)
Physician Responsibilities Regarding Death • physicians are required by law to complete a medical certificate of death unless the coroner needs notification (see Role of the Coroner); failure to report death is a criminal offence
Role of the Coroner Notify Coroner if Death Occurs due to: • Violence, negligence, misconduct • Pregnancy • Sudden or unexpected causes • Disease NOT treated • Cause other than disease • Suspicious circumstances
• Coroner’s Act (specific to Ontario, similar in other provinces) requires physicians to notify a coroner or police officer if death occurs: due to violence, negligence, misconduct, misadventure, or malpractice during pregnancy or is attributable to pregnancy suddenly and unexpectedly from disease which was not treated by a legally qualified medical practitioner from any cause other than disease under suspicious circumstances • coroner investigates these deaths, as well as deaths that occur in psychiatric institutions, jails, foster homes, nursing homes, hospitals to which a person was transferred from a facility, institution or home, etc. • in consultation with forensic pathologists and other specialists, the coroner establishes: the identity of the deceased where and when the death occurred the medical cause of death the means of death (i.e. natural, accidental, suicide, homicide or undetermined) • coroners do not make decisions regarding criminality or legal responsibility
Palliative and End-of-Life Care • focus of care is comfort and respect for person nearing death and maximizing quality of life for patient, family, loved ones • appropriate for any patient at any stage of a life-threatening illness • may occur in a hospital, hospice, in the community or at home • often an interdisciplinary team of caregivers • addresses the medical, psychosocial, and spiritual dimensions of care
Toronto Notes 2011
References
References Baile WF, Buckman R, Lenzi R, et al. (2000). SPIKES - A six-step protocol for delivering bad news: application to the patient with cancer. Oncologist. 5(4): 302-11. Baker GR, Norton PG, Flintoft V, Blais R, Brown A, Cox J, Etchells E, Ghali WA, Hebert P, Majumdar SR, O’Beirne M, Palacios-Derflingher L, Reid RJ, Sheps S, Tamblyn R. The Canadian Adverse Events Study: the incidence of adverse events among hospital patients in Canada. CMAJ 2004;170(11):1678-86. Canadian Institute for Health Information, National Health Expenditure Trends, 1975 to 2009 (Ottawa, Ont.: CIHI, 2009). Canadian Medical Association Journal “Bioethics for Clinicians” Series. Canadian Medical Association www.cma.ca. Canadian Public Health Association and WHO. Ottawa Charter for Health Promotion. Ottawa: Health and Welfare Canada, 1986. CMA Code of Ethics, Canadian Medical Association www.cma.ca. Centers for Medicare and Medicaid Services www.cms.hhs.gov. College of Physicians and Surgeons of Ontario www.cpso.on.ca. CPSO Policy Statements, College of Physicians and Surgeons of Ontario www.cpso.on.ca/policies/mandatory.htm. Democratic Policy Committee. Patient Protection and Affordable Care Act. http://dpc.senate.gov/dpcdoc-sen_health_care_bill.cfm Devereaux PJ, Choi PT, Lacchetti C, Weaver B, Schunemann HJ, Haines T, Lavis JN, Grant BJ, Haslam DR, Bhandari M, Sullivan T, Cook DJ, Walter SD, Meade M, Khan H, Bhatnagar N, Guyatt GH. A systematic review and meta-analysis of studies comparing mortality rates of private for-profit and private not-for-profit hospitals. CMAJ 2002;166(11):1399-406. Devereaux PJ, Heels-Ansdell D, Lacchetti C, Haines T, Burns KE, Cook DJ, Ravindran N, Walter SD, McDonald H, Stone SB, Patel R, Bhandari M, Schunemann HJ, Choi PT, Bayoumi AM, Lavis JN, Sullivan T, Stoddart G, Guyatt GH. Payments for care at private for-profit and private not-for-profit hospitals: a systematic review and metaanalysis. CMAJ 2004;170(12):1817-24. Etchells E, Sharpe G, Elliott C and Singer PA. Bioethics for clinicians: 3. Capacity. CMAJ 1996,155(6):657-661. Ferris LE, Barkun H, Carlisle J, Hoffman B, Katz C, and Silverman M. Defining the physician’s duty to warn: consensus statement of Ontario’s Medical Expert Panel on Duty to Inform. CMAJ 1998;158:1473-1479. Fisher ES, Wennberg DE, Stukel TA, Gottlieb DJ, Lucas FL, Pinder EL. The implications of regional variations in Medicare spending. Part 1: the content, quality, and accessibility of care. Ann Intern Med 2003;138:273-87. Fisher ES, Wennberg DE, Stukel TA, Gottlieb DJ, Lucas FL, Pinder EL. The implications of regional variations in Medicare spending. Part 2: health outcomes and satisfaction with care. Ann Intern Med 2003;138:288-98. Government of Canada: Interagency Advisory Panel on Research Ethics www.pre.ethics.gc.ca/english/index.cfm. Health Care Consent Act, 1996. S.O. 1996, c. 2, Sched. A. Health Protection and Promotion Act, R.S.O. 1990., c.H.7; O. Re.g. 559/91, amended to O. Re.g. 96/03. Hebert P. Doing Right: A Practical Guide to Ethics for Physicians and Medical Trainees, Toronto, Oxford University Press; 1996. Hebert PC, Hoffmaster B, Glass KC, Singer PA. Bioethics for clinicians: 7. Truth telling. CMAJ 1997;156:225-8. Institute of Medicine. Care Without Coverage: Too Little, Too Late. Washington D.C., The National Academies Press; 2002. Online (www.iom.edu) Kirby, M. The Kirby Commission: The Health of Canadians – The Federal Role. 2002. Online at the Senate Canada website: http://www.parl.gc.ca/37/2/parlbus/ commbus/senate/Com-e/soci-e/rep-e/repoct02vol6-e.htm. Medical Council of Canada. Objectives of the considerations of the legal, ethical and organizational aspects of the practice of medicine. 1999. Online at the Medical Council of Canada website (www.mcc.ca). National Center for Health Statistics, Center for Disease Control and Prevention website (www.cdc.gov/nchs). National Center for Health Statistics. Health, United States, 2009: With Special Feature on Medical Technology. Hyattsville, MD. 2010. Naylor CD. Health care in Canada: incrementalism under fiscal duress. Health Affairs 1999;18:9-26. OECD. Health Data, 2008. www.oecd.org. Ontario Medical Association www.oma.org. Ontario’s Office of the Chief Coroner www.mpss.jus.gov.on.ca. Physician Privacy Toolkit (2004), Ontario Medical Association www.oma.org/phealth/privacymain.htm. Romanow, R. The Romanow Report: Royal Commission on the Future of Health Care in Canada. 2002. Online at the Senate Canada website: http://www.hc-sc.gc.ca/ hcs-sss/alt_formats/hpb-dgps/pdf/hhr/romanow-eng.pdf. Shah CP. Public health and preventive medicine in Canada. 5th ed. Toronto: Elsevier Canada; 2003. p 357-360, 426. Reprinted by permission of Elsevier Canada, 2006. WHO. World Health Report 2005. http://www.who.int/whr/en. Woolhandler S, Campbell T, Himmelstein DU. Costs of Health Care Administration in the United States and Canada. NEJM 2003; 349:768-75.
ELOAM21
ELOAM22 Toronto Notes 2011
Notes
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A
Anesthesia and Peri-Operative Medicine Alexander Huang, Lindsay MacKenzie and Pamela Lau, chapter editors Alaina Garbens and Modupe Oyewumi, associate editors Adam Gladwish, EBM editor Dr. Isabella Devito and Dr. Ryan Mai, staff editors Anesthesia Basics . . . . . . . . . . . . . . . . . . . . . . . . 2 Pre-Operative Assessment. . . . . . . . . . . . . . . . . 2 History and Physical Pre-Operative Investigations Fasting Guidelines American Society of Anesthesiology (ASA) Classification Pre-Operative Optimization. . . . . . . . . . . . . . . . 4 Medications Hypertension Coronary Artery Disease (CAD) Endocrine Disorders Respiratory Diseases Aspiration Monitoring. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Induction Agents. . . . . . . . . . . . . . . . . . . . . . . . . 6 Intravenous Agents Volatile Inhalational Agents Muscle Relaxants and Reversing Agents
Local Anesthesia. . . . . . . . . . . . . . . . . . . . . . . . 21 Local Anesthetic Agents Local Infiltration, Hematoma Blocks Topical Anesthetics Obstetrical Anesthesia . . . . . . . . . . . . . . . . . . . 22 Pediatric Anesthesia . . . . . . . . . . . . . . . . . . . . . 23 Uncommon Complications. . . . . . . . . . . . . . . . 24 Malignant Hyperthermia (MH) Common Medications. . . . . . . . . . . . . . . . . . . . 25 Intravenous Induction Agents Opioids Volatile Inhalational Agents Depolarizing Muscle Relaxants Non-Depolarizing Muscle Relaxants Reversal Agents for Non-Depolarizing Relaxants Local Anesthetic Agents References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28
Airway Management. . . . . . . . . . . . . . . . . . . . . . 7 Airway Anatomy Review Tracheal Intubation Rapid Sequence Induction (RSI) Difficult Airway Intraoperative Management. . . . . . . . . . . . . . . 10 Oxygen Therapy Ventilation Temperature Heart Rate Blood Pressure Fluid Balance and Resuscitation IV Fluids Blood Products Extubation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 Post-Operative Care . . . . . . . . . . . . . . . . . . . . . 17 Pain Management . . . . . . . . . . . . . . . . . . . . . . . 17 Regional Anesthesia . . . . . . . . . . . . . . . . . . . . . 19 Definition of Regional Anesthesia Preparation for Regional Anesthesia Epidural and Spinal Anesthesia Peripheral Nerve Blocks
Toronto Notes 2011
Anesthesia A1
A2 Anesthesia
Anesthesia Basics/Pre-Operative Assessment
Toronto Notes 2011
Anesthesia Basics 6 A’s of General Anesthesia 1. Anesthesia 2. Anxiolysis 3. Amnesia 4. Areflexia (muscle relaxation not always required) 5. Autonomic Stability 6. Analgesia Types of Anesthesia • general general anesthesia total IV anesthesia (TIVA) • regional spinal, epidural peripheral nerve block IV regional • local local infiltration topical • sedation monitored anesthesia care • note that different types of anesthesia can be combined (e.g. general + regional)
Pre-Operative Assessment • to identify the patient’s medical and surgical issues; to allow for the arrangement of further investigations, consultations and treatments for patients not yet optimized; and to plan anesthetic techniques
History and Physical History • indication for surgery • surgical/anesthetic Hx: previous anesthetics/complications, previous intubations, medications, drug allergies • PMHx CNS: seizures, stroke, raised intracranial pressure (ICP), spinal disease CVS: coronary artery disease (CAD), myocardial infarction (MI), congestive heart failure (CHF), hypertension (HTN), valvular disease, dysrhythmias, peripheral vascular disease (PVD), conditions requiring endocarditis prophylaxis, exercise tolerance, CCS class, NYHA class (see Cardiology and Cardiovascular Surgery, C33 for NYHA classification) respiratory: smoking, asthma, chronic obstructive pulmonary disease (COPD), recent upper respiratory tract infection (URTI), sleep apnea GI: gastroesophageal reflux disease (GERD), liver disease renal: insufficiency, dialysis, CKD hematologic: anemia, coagulopathies, blood dyscrasias MSK: conditions associated with difficult intubations – arthritides (e.g. rheumatoid arthritis), cervical tumours, cervical infections/abscess, trauma to cervical spine, Down syndrome, scleroderma, obesity, conditions affecting neuromuscular junction (e.g. myasthenia gravis) endocrine: diabetes, thyroid, adrenal disorders other: morbid obesity, pregnancy, ethanol/other drug use • FHx: malignant hyperthermia, atypical cholinesterase (pseudocholinesterase), other abnormal drug/anesthetic reactions Physical Examination • oropharynx and airway assessment to determine the likelihood of difficult intubation • ability to assume “sniffing position” – upper cervical spine extension, lower cervical spine flexion (assesses likelihood of difficult intubation) • no single test is specific or sensitive – all aid in determining the ease of intubation Mallampati Classification (Figure 1) thyromental distance (the distance of the lower mandible in the midline from the mentum to the thyroid notch) with the adult patient’s neck fully extended, 50 y.o.)
Echocardiogram
CHF, cardiomyopathy, valvular pathology, limited cardiac reserve, stroke of unknown etiology
Chest radiograph
Cardiac or pulmonary disease, malignancy, age >60
Guidelines to the Practice of Anesthesia, Revised 2006. Supplement to the Canadian Journal of Anesthesia, Vol 53(12), Dec. 2006. Reproduced with permission © Canadian Anesthesiologists’ Society.
Fasting Guidelines Fasting Guidelines Prior to Surgery (Canadian Anesthesiologists’ Society) • 8 hours after a meal that includes meat, fried or fatty foods • 6 hours after a light meal (such as toast, crackers and clear fluid) or after ingestion of infant formula or nonhuman milk • 4 hours after ingestion of breast milk or jello • 2 hours after clear fluids (water, black coffee, tea, carbonated beverages, juice without pulp)
Impact of Anesthesia Management Characteristics on Severe Morbidity and Mortality Anesthesiology 2005; 102(2):257-258 Study: Case-control study of patients undergoing anesthesia. Patients: 803 cases and 883 controls were analyzed among a cohort of 869,483 patients undergoing anesthesia between 1995-1997. Cases were defined as patients who either remained comatose or died within 24 hours of receiving anesthesia. Controls were defined as patients who neither remained comatose nor died within 24 hours of receiving anesthesia. Intervention: General, regional, or combined anesthesia to patients undergoing a surgical procedure. Main Outcome: coma or death within 24 hours of receiving anesthesia Results: The incidence of 24-hour postoperative death was 8.8 per 10,000 anesthetics (95% CI, 8.2–9.5) and the incidence of coma was 0.5 (95% CI, 0.3-0.6). Anesthesia management risk factors that were associated with a decreased risk of morbidity and mortality were: equipment check with protocol and documentation, directly available anesthesiologist with no change during anesthesia, 2 persons present at emergence of anesthesia, reversal of muscle relaxation, and postoperative pain medication.
A4 Anesthesia
Pre-Operative Assessment/Pre-Operative Optimization
Toronto Notes 2011
American Society of Anesthesiology (ASA) Classification • common classification of physical status at the time of surgery • a gross predictor of overall outcome, NOT used as stratification for anesthetic risk (mortality rates) • ASA 1: a healthy, fit patient • ASA 2: a patient with mild systemic disease, e.g. controlled Type 2 diabetes, controlled essential HTN, obesity, smoker • ASA 3: a patient with severe systemic disease that limits activity, e.g. stable CAD, COPD, DM, obesity • ASA 4: a patient with incapacitating disease that is a constant threat to life, e.g. unstable CAD, renal failure, acute respiratory failure • ASA 5: a moribund patient not expected to survive 24 hours without surgery, e.g. ruptured abdominal aortic aneurysm (AAA), head trauma with increased ICP • for emergency operations, add the letter E after classification (e.g. ASA 3E)
Pre-Operative Optimization Risk Assessment 1. Reconcile patient factors with surgical needs and devise a safe and effective anesthetic plan 2. Optimize co-morbidities
Effects of Extended-release Metoprolol Succinate in Patients Undergoing Non-Cardiac Surgery (POISE trial): A Randomised Controlled Trial. Lancet 2008; 371:1839-47 Purpose: To investigate the role of beta-blockers (metoprolol) peri-operatively in patients with known vascular disease, undergoing non-cardiac surgery. Methods: Patients from 190 centres in 23 countries were eligible if they were >45, undergoing noncardiac surgery and were known to have significant vascular disease. Patients were randomised to either the metoprolol group or placebo. Participants received metprolol (or placebo) 100 mg 2-4 hours before surgery and again 6 hours after surgery and following that 200 mg daily for 30 days. The primary endpoint was a composite of cardiovascular death, non-fatal myocardial infarction and non-fatal cardiac arrest. Analyses was by intention to treat. Results: 8351 patients were recruited into the study, with 8331 completing the 30 day course. Use of metoprolol was found to significantly reduce the risk of cardiovascular death, non-fatal MI or non-fatal cardiac arrest vs. placebo (hazard ratio 0.84, p70 >60
Hemorrhage Management RED Rest Elevate the bleeding area above the level of the heart if possible Direct pressure on the bleeding site
Fluid Resuscitation Give bolus until HR decreases, urine output picks up, and patient stabilizes Maintenance: 4-2-1 rule first 10kg: 4cc/kg/hr 10-20kg: 2cc/kg/hr remaining weight: 1cc/kg/hr replace ongoing losses and deficits (assume 10% of body weight)
ER4 Emergency Medicine
Since only 30% of infused isotonic crystalloids remains in intravascular space, you must give 3x estimated blood loss.
Initial Management of Any Patient in Shock ABCs IV fluids Oxygen Monitor (HR, BP, urine, mentation, O2 sat.) Control hemorrhage
Method of Assessing Level of Consciousness AVPU Alert Responds to Verbal stimuli Responds to Painful stimuli Unresponsive
Initial Patient Assessment/Management
Toronto Notes 2011
• transfusion options with packed red blood cells (pRBCs) crossmatched if possible type-specific (provided by most blood banks within 10 minutes) preferred to O-negative un-crossmatched blood if both available O-negative (children and women of child-bearing age) O-positive if no time for cross-match (males/postmenopausal women) anticipate complications with massive transfusions consider replacement of other blood products (plalelets, FFP) after 2-4 units pRBCs • transfusion with fresh frozen plasma (FFP) used for clinical evidence of impaired hemostasis ongoing hemorrhage, PT >1.5x normal range D. DISABILITY • assess level of consciousness by AVPU method (see sidebox) or GCS Glasgow Coma Scale (GCS) • for use in trauma patients with decreased LOC; good indicator of severity of injury and neurosurgical prognosis • may be used for metabolic coma, but less meaningful • most useful if repeated and used for monitoring of trend change in GCS with time is more relevant than the absolute number patient with deteriorating GCS needs immediate attention prognosis based on best post-resuscitation GCS • reported as a 3 part score: Eyes + Verbal + Motor = Total (see Table 3) • if patient intubated, GCS score reported out of 10 + T (T= tubed, i.e. no verbal component) Table 3. Glasgow Coma Scale
Unproven or Harmful Treatments for Hemorrhage Shock • Trendelenberg position • Steroids (used only in spinal cord injury) • MAST garments • Vasopressors
Vasopressin versus Norepinephrine Infusion in Patients with Septic Shock NEJM 2008; 358:877-87 Study: Multicenter, randomized, double-blind trial Patients: 778 patients with septic shock Intervention: Low-dose vasopressin (0.01 to 0.03 U per minute) or norepinephrine (5 to 15 ug per minute) in addition to open-label vasopressors and a minimum of 5ug of norepinephrine. Outcome: Mortality rate 28 days after start of infusions. Results: No significant difference between the vasopressin and the norepinephrine groups at 28days or 90days. However, in patients with less severe septic shock, mortality rate was lower in the vasopressin group.
Foley Contraindications • Blood at urethral meatus • Scrotal hematoma • High-riding prostate on DRE
NG Tube Contraindications • Significant mid-face trauma • Basal skull fracture
Eyes Open Spontaneously
Best Verbal Response
Best Motor Response
4
Answers questions appropriately
5
Obeys commands
6
To voice
3
Confused, disoriented
4
Localizes to pain
5
To pain
2
Inappropriate words
3
Withdraws from pain
4
1
Incomprehensible sounds
2
Decorticate (flexion)
3
No verbal response
1
Decerebrate (extension)
2
No response
1
No response
13-15 = mild injury, 9-12 = moderate injury, ≤8 = severe injury See Table 28 for modified GCS for infants and children
E. EXPOSURE/ENVIRONMENT • undress patient completely and assess entire body for injury; logroll to examine back • digital rectal exam • keep patient warm with a blanket ± radiant heaters; avoid hypothermia • warm IV fluids/blood • keep providers safe (contamination, combative patient)
2. Resuscitation • done simultaneously with primary survey • attend to ABCs (see Table 4) • manage life-threatening problems as they are identified • vital signs q5-15 minutes • ECG, BP and O2 monitors • Foley catheter and nasogastric (NG) tube if indicated • tests and investigations: CBC, electrolytes, BUN, Cr, glucose, amylase, INR/PTT, b-hCG, toxicology screen, cross and type
Toronto Notes 2011
Initial Patient Assessment/Management
Emergency Medicine ER5
Table 4. 2005 AHA CPR Guidelines Step/Action Airway Breaths Foreign-body airway obstruction Compressions Compression landmarks Compression method: push hard and fast and allow for complete recoil Compression depth Compression rate Compressionventilation ratio Compression-only CPR Defibrillation
Adult: >8yrs
Child: 1-8 years Head tilt-chin lift 2 breaths at 1 second/breath Abdominal thrust
Infant: 4-5 minutes after the call
3. Detailed Secondary Survey • done after rapid primary survey problems have been addressed • identifies major injuries or areas of concern • full physical exam and x-rays (C-spine, chest, pelvis – required in blunt trauma, consider T-spine and L-spine) HISTORY • “SAMPLE”: Signs and Symptoms, Allergies, Medications, Past medical history, Last meal, Events related to injury PHYSICAL EXAMINATION Head and Neck • pupils assess equality, size, symmetry, reactivity to light inequality/sluggish suggests local eye problem or lateralizing CNS lesion relative afferent pupillary defect (swinging light test) – optic nerve damage extraocular movements and nystagmus fundoscopy (papilledema, hemorrhages) reactivity/level of consciousness (LOC) reactive pupils + decreased LOC g metabolic or structural cause non-reactive pupils + decreased LOC g structural cause (especially if asymmetric) • palpation of facial bones, scalp Chest • inspect for midline trachea, flail segment: ≥2 rib fractures in ≥2 places; if present look for associated hemothorax, pneumothorax, and contusions • auscultate lung fields • palpate for subcutaneous emphysema Abdomen • assess for peritonitis, abdominal distention, and evidence of intra-abdominal bleeding • FAST (Focused Abdominal Sonogram in Trauma), diagnostic peritoneal lavage (DPL) or CT • rectal exam for GI bleed, high riding prostate and anal tone (best to do during the log roll) • bimanual exam in females as appropriate Musculoskeletal (MSK) • examine all extremities for swelling, deformity, contusion, tenderness, range of motion • check for pulses and sensation in all injured limbs • log roll and palpate thoracic and lumbar spines • palpate iliac crests and pubic symphysis, pelvic stability (lateral, AP, vertical)
Unilateral, Dilated, Non-reactive Pupil, Think: Focal mass lesion Epidural hematoma Subdural hematoma
Non-contrast head CT is the best imaging modality for intracerebral injury.
Signs of Increased Intracranial Pressure (ICP) • Deteriorating LOC (hallmark) • Deteriorating respiratory pattern • Cushing reflex (high BP, low heart rate, irregular respirations) • Lateralizing CNS signs (e.g. cranial nerve palsies, hemiparesis) • Seizures • Papilledema (occurs late) • N/V and H/A
ER6 Emergency Medicine
Initial Patient Assessment/Management/Traumatology
Toronto Notes 2011
Neurological • GCS • full cranial nerve exam • alterations of rate and rhythm of breathing are signs of structural or metabolic abnormalities progressive deterioration of breathing pattern implies a failing CNS • assess spinal cord integrity conscious patient: assess distal sensation and motor unconscious patient: response to painful or noxious stimulus applied to extremities
4. Definitive Care • continue therapy • continue patient evaluations and special investigations • specialty consultations including OR as needed • disposition: home, admission, or transfer to another setting (e.g. OR, ICU)
Ethical Considerations Jehovah’s Witnesses • Capable adults have the right to refuse medical treatment • May refuse whole blood, pRBCs, platelets and plasma even if lifesaving • Should be questioned directly about the use of albumin, immunoglobulins, hemophilic preparations • Do not allow autologous transfusion unless there is uninterrupted extra corporeal circulation • Usually ask for the highest possible quality of care without the use of the above interventions (e.g. crystalloids for volume expansion, attempts at bloodless surgery) • Patient will generally sign hospital forms releasing medical staff from liability • Most legal cases involve children of Jehovah’s Witnesses; if life-saving treatment is refused contacted CAS
Consent to Treatment: Adults • Emergency Rule: consent is not needed when patient is at imminent risk from a serious injury (e.g. severe suffering, loss of limb, vital organ or life) AND obtaining consent is either: a) not possible (e.g. patient is comatose); OR b) would increase risk to the patient (e.g. time delay) assumes that most people would want to be saved in an emergency • any capable and informed patient can refuse treatment or part of treatment, even if it is life-saving consider: is the patient truly capable? Does pain, stress, or psychological distress impair their judgment? • exceptions to the Emergency Rule: treatment cannot be initiated if a competent patient has previously refused the same or similar treatment and there is no evidence to suggest the patient’s wishes have changed an advance directive is available – e.g. do not resuscitate (DNR) order • refusal of help in a suicide situation is not an exception; care must be given • if in doubt, initiate treatment care can be withdrawn if appropriate at a later time or if wishes clarified by family Consent to Treatment: Children • treat immediately if patient is at imminent risk • parents/guardians have the right to make treatment decisions • if parents refuse treatment that is life-saving or will potentially alter the child’s quality of life, Children’s Aid Society (CAS) must be contacted – consent of CAS is needed to treat Other Issues of Consent • need consent for HIV testing, as well as for administration of blood products Duty to Report • law may vary depending on province and/or state gunshot wounds, potential drunken drivers, suspected child abuse, various communicable diseases medical unsuitability to drive
Traumatology • epidemiology leading cause of death in patients 12 ft (3.6 m)
• important to know the mechanism of injury in order to anticipate traumatic injuries • always look for an underlying cause (alcohol, medications, illicit substances, seizure, suicide attempt, medical problem) • always inquire about head injury, loss of consciousness, amnesia, vomiting, headache and seizure activity
Toronto Notes 2011
Traumatology
Emergency Medicine ER7
Motor Vehicle Collision (MVC) • vehicle(s) involved: weight, size, speed, amount of damage • type of crash (to assess location of possible injuries) lateral/T-bone and head-on: head, cervical spine, thoracic, abdominal, pelvic and lower extremity rear-end: hyper-extension of cervical spine (whiplash injury to neck) roll over: energy dissipated, less likely severe injury if victim restrained by seatbelt, however still significant potential morbidity • location of patient in vehicle • use and type of seatbelt lap belt: spine and abdominal injury shoulder belt: look for major vessel injury • ejection of patient from vehicle/entrapment of patient under vehicle • airbag deployment • use of helmet in motorcycle or bicycle collisions Pedestrian-Automobile Impact • high morbidity and mortality • vehicle speed is an important factor • site of impact on car children tend to be run over adults tend to be struck in lower legs, impacted again on car (truncal injury) and thrown to the ground (head injury)
Vehicle vs. Pedestrian Crash In adults look for triad of injuries (Waddle’s triad): 1. Tibia-fibula or femur fracture 2. Truncal injury 3. Craniofacial injury
Falls • 1 storey = 12 feet = 3.6 m • distance of fall: 50% mortality at 4 stories and 95% mortality at 7 stories • position in which patient landed and type of surface • assess for shock, lower extremity, spine and pelvic fractures Gunshot Wounds (GSW) • type of gun handgun injuries: low or medium velocity, extent of injury may be limited to a small area hunting and rifle injuries: high velocity, widespread injury shot gun: widespread tissue destruction • type of ammunition (e.g. hollow point bullets) • range of shot close range: massive tissue destruction, deposition of wadding into wound • characterize route of entry, even or odd number of wounds and site of exit wound (if any) GSW with hypotension: immediate transport to OR hypotension indicates severe blood loss (>2 L blood loss in 70 kg patient is required to produce hypotension) Stab Wounds • route/direction of entry, length of blade • type of penetration (stab, slash, impalement) • victim recollection and witness reports are often inaccurate and may not correlate with depth/ severity of wound • if blade in-situ, DO NOT REMOVE – it may be tamponading bleeding vessel (to be removed in OR)
Cardiac box: sternal notch, nipples and xiphoid process; injuries inside this area should increase suspicion of cardiac injury.
Always completely expose and count the number of wounds.
Head Trauma • see Neurosurgery, NS29 • 60% of trauma admissions have head injuries • 60% of MVC-related deaths are due to head injury Specific Injuries • fractures (diagnosed by CT head, often not visible on x-ray) A. skull fractures vault fractures linear, non-depressed – most common – typically occur over temporal bone, in area of middle meningeal artery (commonest cause of epidural hematoma) depressed – open (associated overlying scalp laceration, torn dura) vs. closed basal skull typically occur through floor of anterior cranial fossa (longitudinal more common than transverse) clinical diagnosis superior as poorly visualized on CT (Battle’s sign, raccoon eyes, CSF rhinorrhea/otorrhea, hemotympanum)
Signs of Basal Skull Fracture Battle’s sign (bruised mastoid process) Hemotympanum Raccoon eyes (periorbital bruising) CSF Rhinorrhea/Otorrhea
ER8 Emergency Medicine
Warning Signs of Severe Head Injury • GCS 24hrs, frequent signs of brainstem dysfunction B. focal injuries contusions intracranial hemorrhage (epidural, subdural, intracerebral) ASSESSMENT OF BRAIN INJURY History • pre-hospital status • mechanism of injury
Canadian CT Head Rule The Lancet 2001; 357:9266;1391-1396 CT Head is only required for patients with minor head injuries with any one of the following: High risk (for neurological intervention) • GCS score 30 min • Dangerous mechanism (pedestrian struck by motor vehicle, occupant ejected from motor vehicle, fall from height >3 feet or five stairs) Minor head injury is defined as witnessed loss of consciousness, definite amnesia, or witnessed disorientation in a patient with a GCS score of 13-15.
Treatment of Increased ICP • Elevate head of bed • Mannitol • Hyperventilate • Paralyzing agents/sedating agents See also Neurosurgery, NS6
Physical Examination • assume C-spine injury until ruled out • vital signs shock (not likely due to isolated brain injury, except in infants) Cushing’s response to increasing ICP (bradycardia, hypertension, irregular respirations) • severity of injury determined by 1. level of consciousness (LOC) GCS ≤8 intubate, any change in score of 3 or more = serious injury 2. pupils: size, anisocoria >1 mm (in patient with altered LOC), response to light 3. lateralizing signs (motor/sensory) may become more subtle with increasing severity of injury • re-assess frequently Investigations • labs: CBC, electrolytes, coags, glucose, tox screen • CT scan (non-contrast) to exclude intracranial mass lesions • C-spine imaging, often with CT head and neck to exclude intracranial mass lesions Management • general ABCs ensure oxygen delivery to brain through intubation and prevent hypercarbia maintain BP treat other injuries, must treat hypotension, hypoxia (both contribute significantly to mortality) • early neurosurgical consultation for acute and subsequent patient management medical seizure treatment/prophylaxis – benzodiazepines, phenytoin, phenobarbital – steroids are of no proven value treat suspected raised ICP g consider if head injury with signs of increased ICP: – raise head of stretcher 20º if patient hemodynamically stable – intubate and hyperventilate (100% O2) to a pCO2 of 30-35 mmHg – mannitol 1g/kg infused as rapidly as possible – consider paralysing meds if agitated/high airway pressures – maintenance of cerebral perfusion pressure is critical surgical Disposition • neurosurgical ICU admission for severe head injuries (HI) • in hemodynamically unstable patient with other injuries, prioritize most life-threatening injuries and maintain cerebral perfusion • for minor head injury not requiring admission, provide 24-hour HI protocol to competent caregiver, follow-up with neurology as even seemingly minor HI may cause lasting deficits
Toronto Notes 2011
Traumatology
Emergency Medicine ER9
Spine and Spinal Cord Trauma • assume cord injury with significant falls (>12 ft), deceleration injuries, blunt trauma to head, neck or back • spinal immobilization (cervical collar, spine board during patient transport only) must be maintained until spinal injury has been ruled out (Figure 2) • vertebral injuries may be present without spinal cord injury; normal neurologic exam does not exclude spinal injury • cord may be injured despite normal C-spine x-ray (SCIWARA = spinal cord injury without radiologic abnormality) • injuries can include: complete/incomplete transection, cord edema, spinal shock
Collar Everyone with at Least One of the Following Criteria • Midline tenderness • Neurological symptoms or signs • Significant distracting injuries • Head injury • Intoxication • Dangerous mechanism • History of altered LOC
History • mechanism of injury, previous deficits, SAMPLE • neck pain, paralysis/weakness, paresthesia Physical Exam • ABCs • abdo: ecchymosis, tenderness • neuro: complete exam, including mental status • spine: maintain neutral position, palpate C-spine for tenderness, step-off; log-roll, then palpate thoracic and lumbar spine; assess rectal tone • extremities: check cap refill, suspect thoracolumbar injury with calcaneal fractures Investigations • labs: CBC, electrolytes, creatinine, glucose, coags, cross and type, tox screen • imaging full C-spine x-ray series for trauma (AP, lateral, odontoid) • thoracolumbar x-rays AP and lateral views indications: patients with C-spine injury unconscious patients (with appropriate mechanism of injury) patients with neurological symptoms or findings patients with deformities that are palpable when patient log-rolled patients with back pain patients with bilateral calcaneal fractures (due to fall from height) – concurrent burst fractures of the lumbar or thoracic spine in 10% (T11-L2) consider CT (for subtle bony injuries), MRI (for soft tissue injuries) if appropriate
Note: Patients with penetrating trauma (especially gunshot and knife wounds) can also have spinal cord injury.
Of the investigations, the lateral C-spine x-ray is the single most important film. 95% of radiologically visible abnormalities are found on this film.
Cauda Equina Syndrome can occur with any spinal cord injury below T10 vertebrae. Look for incontinence, anterior thigh pain, quadriceps weakness, abnormal sacral sensation, decreased rectal tone and variable reflexes.
Suspected C-spine Injury ** based on mechanism of injury (e.g. MVC, fall, sports) History: midline neck pain, numbness or parasthesia, presence of distracting pain, patient head-injured, patient intoxicated, loss of consciousness or past history of spinal mobility disorder Physical exam: posterior neck spasm, tenderness or crepitus, any neurologic deficit or autonomic dysfunction, altered mental state No
C-spine cleared
C-spine cleared Normal
Flexion/extension films
Neck pain
Abnormal
Yes
1. Plain x-rays, 3 views 2. CT scan if: • Inadequate plain film survey • Suspicious plain film findings • To better delineate injuries seen on plain films • Any clinical suspicion of atlanto-axial dislocation • High clinical suspicion of injury despite normal x-ray • To include C1-C3 when head CT is indicated in head trauma
Normal films
Remain immobilized, consult spine service Abnormal
MRI
Abnormal neurological exam
Normal
C-spine cleared
Figure 2. Approach to Clearing the C-spine
Abnormal films
Remain immobilized, consult spine service
The Canadian C-spine Rule versus the NEXUS Low-risk Criteria in Patients with Trauma NEJM 2003; 349(26):2510-8. Purpose: To compare the clinical performance of the Canadian C-Spine Rule (CCR) and the National Emergency X-Radiography Utilization Study (NEXUS) Low-Risk Criteria (NLC). Study: Trauma patients (n=8283) in stable condition were prospectively evaluated by both the CCR and NLC by 394 physicians before radiography. 2% of these patients had a C-spine injury. Results: Compared to the NLC, the CCR was more sensitive (99.4 vs. 90.7%) and more specific (45.1 vs. 36.8%) after exclusion of indeterminate cases. The number of missed patients would be 1 for the CCR and 16 for the NLC. The range of motion was not evaluated in some CCR cases likely because physicians were not comfortable with the procedure and this may slightly lower the sensitivity or specificity of the CCR in practice. Summary: The CCR is superior to the NLC in alert and stable patients with trauma. The use of the CCR can result in lower radiography rates.
ER10 Emergency Medicine
Traumatology
The Canadian C-Spine Rule For Alert (Glasgow Coma Scale Score = 15) and Stable Trauma Patients where Cervical Spine (C-Spine) injury is a concern 1. Any high-risk factor that mandates radiography? Age ≥65 years or Dangerous mechanism* or Paresthesias in extremities No
2. Any low-risk factor that allows safe assessment of range of motion? Simple rear-end MVC† or Sitting position in ED or Ambulatory at any time or Delayed onset of neck pain§ or Absence of midline C-spine tenderness
Yes
No
Radiography
Yes
3. able to actively rotate neck? >5º left and right
Unable
Toronto Notes 2011
Can Clear C-spine if: • no posterior midline cervical tenderness • no evidence of intoxication • oriented to person, place, time and event • no focal neurological deficits • no painful distracting injuries (e.g. long bone #) Management of Cord Injury • immobilize • evaluate ABCs • treat shock (maintain sBP >100 mmHg) • insert NG and Foley catheter • high dose steroids: methylprednisolone 30 mg/kg bolus, then 5.4 mg/kg/hr drip, start within 6-8 hrs of injury (controversial and recently has less support) • complete imaging of spine and consult spine service if available • continually reassess high cord injuries as edema can travel up cord • if cervical cord lesion, watch for respiratory insufficiency low cervical transection (C5-T1) produces abdominal breathing (phrenic innervation of diaphragm still intact) high cervical cord injury (above C4) may require intubation and ventilation • beware of hypotension (neurogenic shock) treatment: warm blanket, Trendelenberg position (occasionally), volume infusion, consider vasopressors Approach to C-Spine X-Rays • 3-view C-spine series is the screening modality of choice 1. lateral C1-T1 ± swimmer’s view (Figure 3, see Table 5 for interpretation) lateral view is best, identifies 90-95% of injuries 2. odontoid view (open mouth or oblique submental view) (see Figure 4) examine the dens for fractures – beware of artifact (horizontal or vertical) caused by the radiological shadow of the teeth overlying the dens – if unable to rule out fracture, repeat view or consider CT or plain film tomography examine lateral aspects of C1 and spacing relative to C2 3. AP view alignment of spinous processes in the midline spacing of spinous processes should be equal check vertebral bodies and facet dislocations
Able
No radiography *Dangerous Mechanism: • Fall from ≥1 meter/5 stairs • Axial load to head e.g. diving • MVC high speed (>100 km/hr), rollover, ejection • Motorized recreational vehicles • Bicycle collision †Simple rear-end MVC excludes: • Pushed into oncoming traffic • Hit by bus/large truck • Rollover • Hit by high-speed vehicle
Supine Oblique Views • rarely used • better visualization of posterior element fractures (lamina, pedicle, facet joint) • good to assess patency of neural foramina • can be used to visualize the C7-T1 junction
§Delayed: Not immediate onset of neck pain JAMA 2001; 286:1841-1848
Table 5. Interpretation of Lateral View: The ABCS A
Adequacy and Alignment • Must see C1 to C7-T1 junction; if not, downward traction of shoulders, swimmer’s view, bilateral supine obliques, or CT scan needed • Lines of contour – in children 100,000 x 106/L • WBC >500 x 106/L, amylase >175 IU
ER14 Emergency Medicine
Traumatology
Toronto Notes 2011
• imaging must be done if equivocal abdominal examination, suspected intra-abdominal injury or distracting injuries multiple trauma patient resulting in unreliable physical exam (altered sensorium, e.g. secondary to drugs, alcohol, head trauma, or distracting injury; spinal cord injury resulting in abdominal anesthesia) unexplained shock/hypotension multiple trauma patients who must undergo general anesthesia for orthopaedic, neurosurgical, or other injuries fractures of lower ribs, pelvis, spine positive FAST Laparotomy is Mandatory if Penetrating Trauma and: • Shock • Peritonitis • Evisceration • Free air in abdomen • Blood in NG tube, Foley catheter, or on rectal exam
“Rule of Thirds” for stab wounds: • 1/3 do not penetrate peritoneal cavity • 1/3 penetrate but are harmless • 1/3 cause injury requiring surgery
Management • general: ABCs, fluid resuscitation and stabilization • surgical: watchful wait vs. laparotomy • solid organ injuries: decision based on hemodynamic stability, not the specific injuries • hemodynamically unstable or persistently high transfusion requirements: laparotomy • hollow organ injuries: laparotomy • even if low suspicion on injury: admit and observe for 24 hours PENETRATING TRAUMA • high risk of gastrointestinal perforation and sepsis • history: size of blade, calibre/distance from gun, route of entry • local wound exploration under direct vision may determine lack of peritoneal penetration (not reliable in inexperienced hands) with the following exceptions: thoracoabdominal region (may cause pneumothorax) back or flanks (muscles too thick) Management • general: ABCs, fluid resuscitation and stabilization • gunshot wounds g always require laparotomy
Genitourinary Tract Injuries • see Urology, U32
Gross hematuria suggests bladder injury.
Etiology • blunt trauma – often associated with pelvic fractures renal contusions (minor injury – parenchymal ecchymoses with intact renal capsule) renal parenchymal tears/laceration: non-communicating (hematoma) vs. communicating (urine extravasation, hematuria) extraperitoneal rupture of bladder from pelvic fracture fragments intraperitoneal rupture of bladder from trauma and full bladder anterior (bulbous) urethral damage with pelvic fractures ureter: rare, at uretero-pelvic junction • penetrating trauma damage to: kidney, bladder, ureter (rare) • acceleration/deceleration injury renal pedicle injury – high mortality rate (laceration and thrombosis of renal artery, renal vein, and their branches) • iatrogenic ureter (from instrumentation) History • mechanism of injury • hematuria (microscopic or gross), blood on underwear • dysuria, urinary retention • history of hypotension
In the case of gross hematuria, the GU system is investigated from distal to proximal (i.e. urethrogram, cystogram, etc.)
Physical Examination • abdominal pain, flank pain, costovertebral angle (CVA) tenderness, upper quadrant mass, perineal lacerations • DRE: sphincter tone, position of prostate, presence of blood • scrotum: ecchymoses, lacerations, testicular disruption, hematomas • bimanual exam, speculum exam • extraperitoneal bladder rupture: pelvic instability, suprapubic tenderness from mass of urine or extravasated blood • intraperitoneal bladder rupture: acute abdomen
Toronto Notes 2011
Traumatology
Emergency Medicine ER15
Investigations • urethra: retrograde urethrography • bladder: urinalysis, CT scan, urethrogram, ± retrograde cystoscopy, ± cystogram (distended bladder + post-void) • ureter: retrograde ureterogram • renal: CT scan (best, if hemodynamically stable), intravenous pyelogram (IVP) Management • urology consult • renal minor injuries – conservative management bedrest, hydration, analgesia, antibiotics major injuries – admit conservative management with frequent reassessments, serial urinalysis, ± reimaging surgical repair (exploration, nephrectomy): e.g. hemodynamically unstable or continuing to bleed >48 h, major urine extravasation, renal pedicle injury, all penetrating wounds and major lacerations, infections, renal artery thrombosis • ureter uretero-uretostomy • bladder extraperitoneal minor rupture: Foley drainage x 10-14 days major rupture: surgical repair intraperitoneal drain abdomen and surgical repair • urethra anterior: conservative, if cannot void g Foley or suprapubic cystostomy and antibiotics posterior: suprapubic cystostomy (avoid catheterization) ± surgical repair
Orthopaedic Injuries • see Orthopaedics (Shoulder, Knee, Wrist, Ankle) Goals of ED Treatment • identify injuries accurately and address potentially life/limb threatening problems appropriately • reduce and immobilize fractures (cast/splint) as appropriate • provide adequate pain relief • arrange proper follow-up if necessary History • use SAMPLE • mechanism of injury may be very important
Description of Fractures SOLARTAT Site Open vs. closed Length Articular Rotation Translation Alignment/Angulation Type (e.g. Salter-Harris, etc.)
Physical Examination • Look (inspection): “SEADS” Swelling, Erythema, Atrophy, Deformity, Skin changes (e.g. bruises) • Feel (palpation): all joints/bones – local tenderness, swelling, warmth, crepitus, joint effusions, subtle deformity • Move: joints affected plus above and below injury – active ROM preferred to passive • Neurovascular status: distal to injury (BEFORE and AFTER reduction) LIFE AND LIMB THREATENING INJURIES (see Table 9) • threat to life is usually due to blood loss (e.g. up to 3 L in pelvic fractures, 1.5 L per long bone fracture) • threat to limb is usually due to interruption of blood supply to distal part of limb or to susceptible part of bone Table 9. Life and Limb Threatening Orthopedic Injuries Life Threatening Injuries
Limb Threatening Injuries
Major pelvic fractures
Fracture/dislocation of ankle (talar AVN)
Traumatic amputations
Crush injuries
Massive long bone injuries (beware of fat emboli)
Compartment syndrome
Vascular injury proximal to knee/elbow
Open fractures Dislocations of knee/hip Fractures above knee/elbow
Reasons for Emergent Orthopaedic Consultation • Compartment syndrome • Irreducible dislocation • Circulatory compromise • Open fracture • Injury requiring surgical repair
ER16 Emergency Medicine
Traumatology
When Dealing with an Open Fracture, Remember “STAND” Splint Tetanus prophylaxis Antibiotic Neurovascular status (before and after) Dressings (to cover wound)
Toronto Notes 2011
Open Fractures • communication between fracture site and external surface of skin – risk of osteomyelitis • remove gross debris, irrigate, cover with sterile dressing – formal irrigation and debridement often done in the OR • control bleeding with pressure (no clamping) • splint • antibiotics (1st generation cephalosporin and aminoglycoside) and tetanus prophylaxis • must secure definitive surgical care within 6-8 hours Vascular Injuries • realign limb/apply longitudinal traction and reassess pulses (e.g. Doppler probe) • surgical consult • direct pressure if external bleeding Compartment Syndrome • increased interstitial pressure in an anatomical “compartment” (forearm, calf) with little room for expansion, resulting in decreased perfusion and potential muscle/nerve necrosis • excessive pain which is worse with passive stretching and refractory to analgesia is the hallmark sign early on; also look for “the 6 Ps” (see side bar) • requires prompt decompression – remove constrictive casts, dressings; fasciotomy may be needed emergently
Vascular injury/compartment syndrome is suggested by “The 6 Ps”: Pulse discrepancies Pallor Paresthesia/hypoesthesia Paralysis Pain (especially when refractory to usual analgesics) Polar (cold)
1
2
Lateral view 3 4
5
6
© Willa Bradshaw 2005
A-P view 1. Dorsal tilt 2. Dorsal displacement 3. Ulnar styloid fracture 4. Radial displacement 5. Radial tilt 6. Shortening
Figure 6. Colles’ Fracture
Ulna
Radius Scaphoid
Lunate
Trapezium
Triquetrum Pisiform Hamate
1
Trapezoid Capitate
5 4 3 2 Metacarpal bones (1-5) © Elisheva Marcus
Figure 7. Carpal Bones
UPPER EXTREMITY INJURIES • anterior shoulder dislocation axillary nerve (lateral aspect of shoulder) and musculocutaneous nerve (extensor aspect of forearm) at risk seen on lateral view: humeral head anterior to glenoid reduce (traction, scapular manipulation), immobilize in internal rotation, repeat x-ray, out-patient follow-up with ortho with forceful injury, look for fracture • Colles’ fracture (Figure 6) distal radius fracture with dorsal displacement from Fall On an Outstretched Hand (FOOSH) AP film: shortening, radial deviation, radial displacement lateral film: dorsal displacement, volar angulation reduce, immobilize with splint, out-patient follow-up with ortho or immediate orthopedic referral if complicated fracture if involvement of articular surface, emergent orthopedic referral • scaphoid fracture (see Figure 7 for review of carpal bones) tenderness in anatomical snuff box, pain on scaphoid tubercle, pain on axial loading of thumb negative x-ray: thumb spica splint, re-x-ray in 1 week ± bone scan positive x-ray: thumb spica splint x 6-8 weeks, re-x-ray in 2 weeks risk of avascular necrosis (AVN) of scaphoid if not immobilized outpatient ortho follow-up LOWER EXTREMITY INJURIES • ankle and foot fractures see Ottawa Ankle and Foot Rules (Figure 8) • knee injuries see Ottawa Knee Rules (Figure 9) • avulsion of the base of 5th metatarsal occurs with inversion injury supportive tensor or below knee walking cast for 3 weeks • calcaneal fracture associated with fall from height associated injuries may involve ankles, knees, hips, pelvis, lumbar spine
Toronto Notes 2011
Traumatology
Emergency Medicine ER17
MALLEOLAR ZONE
A. Posterior edge or tip of lateral malleolus
MIDFOOT ZONE
6 cm
D. Navicular
C. Base of 5th metatarsal
LATERAL VIEW
6 cm
B. Posterior egde or tip of medial malleolus
Reasons for Splinting • Reduces pain • Reduces further damage to vessels and nerves • Reduces risk of inadvertently converting a closed fracture into an open fracture • Facilitates patient transport
MEDIAL VIEW
An ankle radiographic series is required only if there is any pain in malleolar zone and any of these findings: 1. bone tenderness at A or 2. bone tenderness at B or 3. inability to bear weight both immediately and in emergency department A foot radiographic series is required only if there is any pain in midfoot zone and any of these findings: 1. bone tenderness at C or 2. bone tenderness at D or 3. inability to bear weight both immediately and in emergency department
Figure 8. Ottawa Ankle Rules
Adapted from Stiell et. al. JAMA 1994; 271:827-832
A knee x-ray examination is required only for acute injury patients with one or more of: • Age 55 years or older • Tenderness at head of fibula • Isolated tenderness of patella* • Inability to flex to 90º • Inability to bear weight both immediately and in the emergency department (four steps)**
Accuracy of Ottawa Ankle Rules to Exclude Fractures of the Ankle and Mid-Foot: Systematic Review BMJ 2003; 326(7386):417 This systematic review and meta-analysis of 27 studies including 15,581 patients evaluated the sensitivity and specificity of the Ottawa ankle rules for excluding fractures of the ankle and mid-foot. Results: The pooled likelihood ratio of a negative result (obtaining a false negative) among those with a fracture was determined to be 0.08 for both the ankle and mid-foot. Reviewers’ Conclusions: The Ottawa ankle rules provide an accurate instrument for excluding fractures of the ankle and mid-foot with a sensitivity of almost 100% and a specificity of 26%. The use of this instrument can reduce the number of unnecessary radiographs.
*no bony tenderness of knee other than patella **unable to transfer weight twice onto each lower limb regardless of limping
Figure 9. Ottawa Knee Rules
Adapted from: Stiell et. al. JAMA 1997; 278:2075-2079
Wound Management Goals of ED Treatment • identify injuries and stop any active bleeding – direct pressure • manage pain • wound examination and exploration (history and physical) • cleansing ± antibiotic and tetanus prophylaxis • repair and dressing
Acute Treatment of Contusions
Tetanus Prophylaxis • both tetanus toxoid (Td) and immunoglobulin (TIG) are safe (and indicated) in pregnancy
RICE Rest Ice Compression Elevation
Table 10. Guidelines for Tetanus Prophylaxis for Wounds Non Tetanus Prone Wounds
Tetanus Prone Wounds1
Td2
TIG3
Td
TIG
Uncertain or 10 years
Yes
No
Yes
No
3 or more, 5 to 10 years ago
No
No
Yes
No
3 or more, 6 hours old, >1 cm deep, puncture wounds, avulsions, wounds resulting from missiles, crush wounds, burns, frostbite, wounds contaminated with dirt,
feces, soil, or saliva
2 0.5 mL IM tetanus and diphtheria toxoids (Td), adsorbed 3 tetanus immune globulin (TIG), 250 units deep IM
Source: MMWR 2001; 50(20);418-427. MMWR 1991; 40(RR12);1-52.
Bruises • non palpable = ecchymosis • palpable collection (not swelling) = hematoma following blunt trauma • assess for coagulopathy (e.g. liver disease), anticoagulant use
Suture Use and Duration Suture to
Close with nylon or Approx. other nonabsorbable duration suture (days)
Face
6-O
Not Joint
4-O
7
Joint
3-O
10
Scalp Mucous Membrane
5
4-O
7
absorbable (vicryl)
N/A
N.B. Patients on steroid therapy may need sutures in for longer periods of time
Alternatives to Sutures • Tissue glue • Steristrips® • Staples
ER18 Emergency Medicine
Where NOT to use local anesthetic with epinephrine: Ears, Nose, Fingers, Toes and Hose (Penis)
Differential Diagnosis of Cellulitis Necrotizing Fasciitis Gas gangrene Cutaneous anthrax Vaccinia vaccination Insect bite (hypersensitivity) Acute gout DVT Fixed drug eruption Kawasaki’s Pyoderma gangrenosum
Features of Necrotizing Fasciitis Infection ABCDE A – Anaerobic, Aerobic, Adult, Antibiotics refractory B – Bacterial synergistic gangrene, Blood count higher than normal C – Cellulitis, Crepitus, and Coagulopathy D – Dermal gangrene, Delay in presentation almost fatal E – Erythema with spreading Edema
Traumatology
Toronto Notes 2011
Abrasions • partial to full thickness break in skin • management clean thoroughly, ± local anesthetic, with brush to prevent foreign body impregnation (tattooing) antiseptic ointment (Polysporin® or Vaseline®) for 7 days for facial and complex abrasions tetanus prophylaxis (Table 10) Lacerations • see also Plastic Surgery, PL6 • consider every structure deep to a laceration injured until proven otherwise • in hand injury patients, include following in history: handedness, occupation, mechanism of injury, previous history of injury • physical exam think about underlying anatomy examine tendon function actively against resistance and neurovascular status distally clean and explore under local anesthetic; look for partial tendon injuries x-ray or ultrasound wounds if a foreign body is suspected (e.g. shattered glass) and not found when exploring wound (remember: not all foreign bodies are radiopaque), or if suspect intra-articular involvement • management disinfect skin/use sterile techniques irrigate copiously with normal saline analgesia ± anesthesia (Figure 10) maximum dose of lidocaine: 7 mg/kg with epinephrine 5 mg/kg without epinephrine • in children, topical anesthetics such as LET (lidocaine, epinephrine and tetracaine) and in selected cases a short-acting benzodiazepine (midazolam or other agents) for sedation and amnesia are useful • secure hemostasis • evacuate hematomas, debride non-viable tissue, remove hair and remove foreign bodies • ± prophylactic antibiotics • suture unless delayed presentation, a puncture wound, or mammalian bite • take into account patient and wound factors when considering suturing • advise patient when to have sutures removed
Dorsal digital nerves
Palmar digital nerves
Figure 10. Digital Block – Local Anesthesia of Digits
Early wound irrigation and debridement are the most important factors in decreasing infection.
Which Abscesses Need Antibiotics? • Evidence of systemic illness (e.g. cellulitis) • Immunocompromised patient • Patient at risk for endocarditis
Cellulitis • see also Plastic Surgery, PL12 • localized infection of the dermis • bacterial (S. aureus, GAS, H. influenzae, occasionally Pseudomonas spp., MRSA) infection of skin and subcutaneous tissues • look for “rubor, calor, dolor, tumour” (erythema, warmth, pain, swelling) • have high index of suspicion in patients who are immunocompromised (e.g. HIV, DM), vasculopaths, IV drug users • treat with immobilization and elevation of infected area, antibiotics, analgesics, and close follow-up • antibiotics for mild cellulitis: PO cephalexin or cloxacillin • if MRSA: PO clindamycin, doxycycline, TMP-SMX; IV vancomycin if bateremic Abscess • may be associated with a retained foreign body • look for warm, swollen, painful, erythematous fluctuant masses • ensure absence of systemic symptoms and presence of subcutaneous air in simple abscesses • anesthetize locally • treat with incision and drainage ± antibiotics – apply warm compress, give analgesics
© Michael Corrin
Digital arteries
Toronto Notes 2011
Traumatology/Approach to Common ER Presentations
Emergency Medicine ER19
Trauma in Pregnancy • priorities: Airway, Breathing, Circulation Hemodynamic Considerations • near term, inferior vena caval compression in the supine position can decrease cardiac output by 30-40% (see Maternal Physiology, Obstetrics, OB3) use left lateral decubitus (LLD) positioning or hip bolster to alleviate compression and increase blood return if BP is low • BP drops 5-15 mmHg systolic in 2nd trimester, increases to normal by term • HR increases 15-20 beats per minute by 3rd trimester
The best treatment for the fetus is the effective treatment of the mother.
Blood Considerations • physiologic macrocytic anemia of pregnancy (Hb 100-120) • WBC increases to high of 20,000 Shock • pregnant patients may lose 35% of blood volume without typical signs of shock (i.e. tachycardia, hypotension) • the fetus may be in “shock” due to contraction of the uteroplacental circulation • fetal HR changes are an early warning of maternal circulatory compromise Management Differences • place bolster under right hip to stop inferior vena cava compression • fetal monitoring (continuous tocographic monitoring if possible viable fetus >20 weeks) • early obstetrical consult • do not avoid necessary x-rays, but shield as much as posssible • consider need for RhoGAM if mother Rh negative
Approach to Common ER Presentations Abdominal Pain Rule Out Life-Threatening Causes • CVS: MI, aortic dissection, ruptured AAA (tearing pain) • GI: perforated viscus, hepatic/splenic injury, ischemic bowel (diffuse pain) • gynecologic: ectopic pregnancy Additional Differential Diagnosis • GI: appendicitis, diverticulitis, bowel obstruction, hepatitis, cholecystitis, pancreatitis • urinary: cystitis, pyelonephritis, ureteral calculi • genital female: pelvic inflammatory disease (PID)/salpingitis, tubo-ovarian abscess, ovarian torsion, ovarian cyst, endometriosis male: testicular torsion, epididymitis • other: diabetic ketoacidosis (DKA), Herpes Zoster Virus (HZV), intra-abdominal abscess, pneumonia, lead poisoning, porphyria, sickle cell crisis, psychiatric History and Physical Examination • determine onset, course, location and character of pain: PQRST • broad differential, including GU, Gyne, GI, respiratory, and CV systems • recent/remote abdominal trauma/surgeries • general appearance, vitals, ABCs • respiratory, CVS • abdomen and back: CVA tenderness, ecchymoses, stigmata of liver disease, DRE, pelvic exam (females), genital exams (males) • extremities: differential pulses, psoas/obturator sign Investigations • do not delay consultation if patient unstable • CBC, electrolytes, glucose, LFTs, amylase, BUN/creat, U/A, + others if indicated: b-hCG, ECG, troponins • AXR: look for calcifications, free air, gas pattern, air fluid levels • CXR upright: look for pneumoperitoneum (free air under diaphragm) • U/S: biliary tract, ectopic pregnancy, AAA, free fluid • CT: trauma, AAA, pancreatitis, nephro/urolithiasis, appendicitis and diverticulitis
Red Flags • Extremes of age • Unstable vital signs • Fever • Signs/symptoms of shock • Rapid onset severe pain
Abdominal Assessment in all 4 Quadrants DR. GERM Distention Rigidity Guarding Evisceration/Ecchymosis Rebound tenderness Masses
If both AST and ALT elevated, AST > ALT indicates potential alcohol related hepatic diseases ALT > AST indicates viral hepatic pathology If ALP and GGT elevated, think biliary tree
ER20 Emergency Medicine
Old age, pregnancy (T3), and chronic corticosteroid use can blunt peritoneal findings, so have increased suspicion of intrabdominal process in these individuals!
Unstable patients should not be sent for imaging.
Approach to Common ER Presentations
Toronto Notes 2011
Management • NPO, IV, NG tube, analgesics growing evidence that small amounts of narcotic analgesics improve diagnostic accuracy of physical exam of surgical abdomen • consult as necessary: general surgery, vascular, gynecology, etc. Disposition • admission: in addition to a surgical abdomen, admission is sometimes required for workup of abnormal findings on investigation, IV antibiotics, pain control, etc. • discharge: patients with a negative lab and imaging workup who improve clinically during their stay; instruct the patient to return if severe pain, fever, or persistent vomiting develop
Acute Pelvic Pain
All women of childbearing age assumed to be pregnant until proven otherwise.
Gynecological Causes of Pelvic Pain: Ovarian Cyst Dysmenorrhea Mittelshmerz Endometriosis Ovarian Torsion Uterine Fibroids/neoplasm Adnexal Neoplasm PID + Cervicitis
Ultrasound is the preferred imaging modality in the assessment of acute pelvic pain.
Etiology • gynecological 2nd most common gynecological complaint after vaginal bleeding ruptured ovarian cysts – most common cause of pelvic pain, follicular cyst most common type ovarian torsion – rare, 50% will have ovarian mass leiomyomas (uterine fibroids) – especially with torsion of a pedunculated fibroid or in pregnant patient (degeneration) ectopic pregnancy – ruptured/expanding/leaking spontaneous abortion – threatened or incomplete infection – PID, endometritis, tubo-ovarian abscess dysmenorrhea and endometriosis • non-gynecological GI – appendicitis, constipation, bowel obstruction, gastroenteritis, diverticulitis, IBD, IBS GU – cystitis, pyelonephritis, ureteric stone other – porphyria, abdominal angina, aneurysm, hernia, zoster History and Physical Exam • determine onset, course, location and character of the pain • associated symptoms: vaginal bleeding, bowel or bladder symptoms, radiation • vitals • gynecological exam • abdominal exam Investigations • b-hCG for all women of childbearing age • CBC and differential, PTT/INR • pelvic and abdominal U/S – evaluate adnexa, look for free fluid in the pelvis or masses, evaluate thickness of endometrium • doppler flow studies for ovarian torsion Management • general: analgesia, determine if admission and consults needed gynecology consult if history and physical suggestive of serious cause other consults as indicated – general surgery, urology, etc. • specific: ovarian cysts unruptured or ruptured and hemodynamically stable – analgesia and follow-up ruptured with significant hemoperitoneum – may require surgery ovarian torsion – surgical detorsion or removal of ovary uncomplicated leiomyomas, endometriosis and secondary dysmenorrhea can usually be treated on an outpatient basis, discharge with gynecology follow-up PID: requires broad spectrum antibiotics Disposition • patients requiring IV therapy or surgery should be admitted • patients to be discharged should be given clear instructions for appropriate follow-up
Toronto Notes 2011
Approach to Common ER Presentations
Emergency Medicine ER21
Altered Level of Consciousness (LOC) Definitions • altered mental status – collective, non-specific term referring to change in cognitive function, behaviour, or attentiveness • delirium – acute, transient, fluctuating, potentially reversible organic brain disorder presenting as altered LOC and attentiveness (see Psychiatry, PS17) • dementia – insidious, progressive, organic brain disorder with change in memory, judgment, personality and cortical function (see Psychiatry, PS18) • lethargy – state of decreased awareness and alertness (patient may appear wakeful) • stupor – unresponsiveness from which the patient can be aroused • coma – a sleep-like state, not arousable to consciousness (Figure 11) • use the GCS to evaluate LOC (see Initial Patient Assessment and Management, ER2) Coma (GCS ≤8) (Majority)
(Minority)
Toxic/Metabolic
Primary CNS Disease/Trauma Possible Causes of Coma
M– E – T – A – B – O – L – I – C –
Major organ failure Electrolyte/Endocrine Toxins/Temperature Acid disorders Base disorders decreased Oxygen level Lactate Insulin/Infection (sepsis) Cardiac/hyperCalcemia
Bilateral Cerebral Hemispheres (affecting cognition)
Diffuse trauma/ischemia
Diffuse lesion
Brainstem (affecting RAS)
Compression • Supra/infratentorial tumour • Sub/epidural hematoma
Direct • Brainstem infarct or hemorrhage
AEIOU TIPS Acidosis/Alcohol Epilepsy Infection Oxygen (hypoxia)/Opiates Uremia Temperature/Trauma (especially head) Insulin (too little or too much) Psychogenic/Poisoning Stroke
Figure 11. Etiology of Coma
MANAGEMENT OF ALTERED LOC History • obtained from family, friends, police, paramedics, old chart, etc. • onset and progression abrupt onset suggests CNS hemorrhage/ischemia or cardiac cause progression over hours to days suggests progressive CNS lesion or toxic/metabolic cause • preceding events it is essential to determine patient’s baseline LOC preceding deterioration antecedent trauma, seizure activity, fever • past medical history (e.g. similar episode, depression, overdose)
In general, GCS under 8, intubate, but ability to protect airway is primary consideration!
Physical Examination • ABC’s, vitals including temperature, cardiac, chest, respiratory, abdominal exam, and the “five Ns” (see sidebar) • complete neuro exam, in particular examination of the eyes, look for medic alert bracelet
Noggin e.g. raccoon eyes and Battle’s sign (bruising of the mastoid process) appears about 8 hours after trauma
Investigations • rapid blood sugar, CBC, electrolytes, Cr, BUN, LFTs, glucose, serum osmolality, ABGs, coags, troponins, U/A • ECG, CXR, CT head • drug levels of specific toxins if indicated Diagnosis • administer appropriate universal antidotes thiamine 100 mg IV if history of EtOH or patient looks malnourished one ampule D50W IV if low blood sugar on finger-prick naloxone 0.4-2 mg IV or IM if opiate overdose suspected • distinguish between structural and toxic-metabolic coma structural coma pupils, extraocular movements and motor findings, if present, are usually asymmetric look for focal or lateralizing abnormalities toxic-metabolic coma dysfunction at lower levels of the brainstem (e.g. caloric unresponsiveness) respiratory depression in association with an intact upper brainstem (e.g. equal and reactive pupils; see exceptions in Table 11) extraocular movements and motor findings are symmetric or absent
Evaluation of Comatose Patient Five Ns
Neck C-spine, neurogenic shock, nuchal rigidity ENT Otorrhea, rhinorrhea, tongue biting, odour on breath, hemotympanum Needles Inspect for track marks Neurological Concentrate on GCS, respiration, posture, movement, pupils, reflexes
Universal Antidotes DON’T Dextrose Oxygen Naloxone 2 mg Thiamine 100 mg
ER22 Emergency Medicine
Approach to Common ER Presentations
Toronto Notes 2011
• essential to re-examine frequently – status can change rapidly • diagnosis may become apparent only with the passage of time delayed deficit after head trauma suggestive of epidural hematoma (characteristic “lucid interval”) Table 11. Toxic – Metabolic Causes of Fixed Pupils Dilated
Dilated to Normal
Constricted
Anoxia
Hypothermia
Cholinergic agents (e.g. organophosphates)
Anticholinergic agents (e.g. atropine, TCAs)
Barbiturates
Opiates (e.g. heroin), except meperidine
Methanol (rare)
Antipsychotics
Cocaine Opioid withdrawal Amphetamines Hallucinogens
Disposition • readily reversible alteration of LOC: discharge if adequate follow-up care available • ongoing decreased LOC: admit to service based on tentative diagnosis • transfer patient if appropriate level of care not available
Chest Pain Life Threatening causes of Chest Pain PET MAP Pulmonary embolism Esophageal rupture Tamponade MI/angina Aortic dissection Pneumothorax
Imaging is necessary for all suspected aortic dissections, regardless of blood pressure.
Signs and Symptoms of MI PULSE Persistent chest pain Upset stomach Lightheadedness Shortness of breath Excessive sweating
PERC score for PE Age >50 years HR >100 bpm O2 sat on RA 100 Recent immobility or Sx Clinical signs of DVT Alternate Dx less likely than PE Hemoptysis Cancer
+1.5 +1.5 +1.5 +3 +3 +1 +1
Low probability = 0-2 Intermediate probability = 2-6 High probability = >6
Table 12. Common Life Threatening ECG Changes Pathology
ECG Findings
Dysrhythmia a) Torsade de Pointes b) Ventricular tachycardia c) Ventricular flutter d) Ventricular fibrillation
Ventricular complexes in upward-pointing and downward-pointing continuum (250-350 bpm) 6 or more consecutive premature ventricular beats (150-250 bpm) Smooth sine wave pattern of similar amplitude (250-350 bpm) Erratic ECG tracing, no identifiable waves
Conduction a) 2nd degree heart block (Mobitz Type II) b) 3rd degree heart block c) Left bundle branch block
Ischemia a) STEMI Metabolic a) Hyperkalemia b) Hypokalemia Digitalis Toxicity Syndromes a) Brugada b) Wellens c) Long QT Syndrome
PR interval stable, some QRS’s dropped Total AV dissociation, but stable P-P and R-R intervals Prolonged QRS complex (>0.12s) RSR’ in V5 or V6 Monophasic I and V6 May see ST elevation Difficult to interpret, new LBBB is considered STEMI equivalent ST elevation in leads associated with injured area of heart and reciprocal lead changes (depression) Tall T waves P wave flattening QRS complex widening and flattening U waves appear Flattened T waves Gradual downward curve of ST At risk for AV blocks and ventricular irritability RBBB with ST elevation in V1, V2 and V3 Susceptible to deadly arrhythmias, including V. Fib. Marked T wave inversion in V2 and V3 Left anterior descending coronary stenosis QT interval longer than ½ of cardiac cycle Predisposed to ventricular arrhythmia
ACUTE MYOCARDIAL INFARCTION • see Cardiology, C25 Management • immediate stabilization oxygen 4L/min IV access cardiac monitors STAT ECG cardiac enzymes (CK, Troponins) • ASA 162-325 mg chewed • nitroglycerin 0.3 mg SL q5min x 3 (IV for CHF, HTN, unresolved pain) • morphine 2-5 mg IV q5-30min if unresponsive to NTG • metoprolol 5 mg slow IV q5min x 3 if no contraindication (beware in inferior wall AMI)
Important to look for reciprocal changes in STEMI in order to differentiate from pericarditis (diffuse elevations).
Immediate Treatment of Acute MI BEMOAN Beta-Blockade Enoxaparin Morphine Oxygen ASA Nitroglycerin Addition of Clopidogrel to Aspirin and Fibrinolytic Therapy for Myocardial Infarction with STSegment Elevation NEJM 2005; 352(12):1179-1191 Purpose: To assess the benefit of adding clopidogrel to aspirin and fibrinolytic therapy in ST-elevation MI. Study Characteristics: Double-blind, RCT, following intention-to-treat analysis, with 3491 patients and clinical follow-up at 30 days. Participants: Individuals presenting within 12 hours of onset of ST-elevation MI (mean age 57, 80.3% male, 50.3% smokers, 9.1% previous MI). Those presenting after 12 hours, age >75, or with previous CABG were excluded. Intervention: Clopidogrel (300 mg loading dose followed by 75 mg od until day of angiogram) or placebo, in addition to aspirin, a fibrinolytic agent, and heparin when appropriate. Primary Outcome: Composite of occluded infarct-related artery on angiography (Thrombosis in Myocardial Infarction flow grade 0 or 1), or death or recurrent MI prior to angiography. Results: Rates of primary end point were 21.7% in the placebo group and 15.0% in the clopidogrel group (95% CI, 24-47%). Among the individual components of the primary end point, clopidigrel had a significant effect on the rate of an occluded infarct-related artery and the rate of recurrent MI, but no effect on the rate of death from any cause. At 30 days clinical follow-up, there was no difference in rate of death from cardiovascular causes, a significant reduction in the odds of recurrent MI, and a non-significant reduction in recurrent ischemia with need for urgent revascularization. The rates of major bleeding and intracranial hemorrhage were similar between the two groups. Conclusion: Addition of clopidogrel improves the patency rate of infarct-related arteries and reduces ischemic complications, both of which are associated with improved long-term survival after MI. The trial was not powered to detect a survival benefit and none was seen.
ER24 Emergency Medicine
Approach to Common ER Presentations
Toronto Notes 2011
• low molecular weight heparin 1 mg/kg SC bid (30 mg IV STAT post TNK infusion) • thrombolytics or primary percutaneous coronary intervention (PCI) agents include t-PA, r-PA, Streptokinase, and TNK evaluate indications and contraindications prior to use • other – antiarrhythmics, cardioversion, defibrillation, transthoracic pacing, angioplasty • cardiology consult
Epistaxis • see Otolaryngology, OT27 • 90% of nosebleeds stem from the anterior nasal septum (at Kiesselbach’s plexus located in Little’s area) • can be life-threatening Etiology • most commonly caused by trauma (digital, blunt, foreign bodies), but can also be caused by barometric changes, nasal dryness, chemicals (cocaine, Otrivin®), or systemic disease (coagulopathies, hypertension, etc.) Investigations • CBC, PT/PTT (if indicated) • x-ray, CT as needed Thrombocytopenic patients – use resorbable packs to avoid risk of re-bleeding caused by pulling out the removable pack.
Complications of Nasal Packing Hypoxemia Toxic-shock syndrome Aspiration Pharyngeal fibrosis/stenosis Alar/septal necrosis
Treatment • aim is to localize bleeding and achieve hemostasis • first-aid: ABC’s, lean forward, pinch soft part of nose for 20 minutes • assess blood loss: vitals, IV normal saline, cross match 2 units packed RBC if significant • determine site of bleeding: use topical anaesthetic/vasoconstrictor to facilitate; use nasal speculum and good lighting • attempt to control the bleeding first line: Otrivin® or cocaine second line: cauterize with silver nitrate (one side of septum only!) if these fail, or if bleeding is posterior g nasal packing (must monitor for complications) if packing fails, consult ENT Disposition • most patients can be discharged after ensuring vitals are stable, bleeding is controlled and patient has appropriate follow-up • educate patients about prevention (e.g. humidifiers, saline spray, topical ointments, avoiding irritants, managing hypertension) • admission may be required for severe cases
Headache • see also Neurology, N39
Note: up to 5% of patients with subarachnoid hemorrhage have a normal CT scan; if suspect SAH with a negative CT, perform a lumbar puncture.
BEWARE: every headache is serious until proven otherwise.
DDx Subarachnoid Hemorrhage BATS Berry aneurysm Arteriovenous malformation/Adult polycystic kidney disease Trauma Stroke
Etiology • the common common migraine (no aura)/classic migraine (involves aura) gradual onset, unilateral/bilateral, throbbing nausea/vomiting, photo/phonophobia treatment: analgesics, neuroleptics, vasoactive meds tension/muscular headache never during sleep, gradual over 24 hours posterior/occipital increased with stressors treatment: modify stressor, local measures, NSAIDs, tricyclic antidepressants • the deadly subarachnoid hemorrhage (SAH) (see Neurosurgery, NS17) sudden onset, increased with exertion “worst” headache, nausea and vomiting, meningeal signs diagnosis: CT, LP (5-10% of patients with SAH have negative initial CT) – sensitivity of CT decreases with time and is much less sensitive by 48-72 hr management: urgent neurosurgery consult increased ICP worst in morning, supine, or bending down physical exam: neurological deficits, cranial nerve palsies, papilledema diagnosis: CT scan management: consult neurosurgery
Toronto Notes 2011
Approach to Common ER Presentations
meningitis (see Infectious Diseases, ID6) flu-like presentation initially (fever, nausea/vomiting, malaise), meningeal signs, purpuric rash altered level of consciousness and confusion perform CT to rule out increased ICP then do LP for diagnosis treatment: early empiric antibiotics (depending on age group), steroid therapy temporal arteritis (not immediately deadly but causes great morbidity) (see Ophthalmology, OP38) unilateral scalp tenderness, jaw claudication, visual disturbances labs: elevated ESR temporal artery biopsy is gold standard for diagnosis treatment: high-dose steroids immediately if TA suspected
Emergency Medicine ER25
Meningitis Do not delay IV antibiotics for LP.
Disposition • admit if underlying diagnosis is critical or emergent, if there are abnormal neurological findings, if patient is elderly or immunocompromised (atypical presentation), or if pain is refractory to oral medications • most patients can be discharged with appropriate analgesia and follow up with their family physician; instruct patients to return for fever, vomiting, neurologic changes, or increasing pain
Joint Pain • see Rheumatology Rule Out Life-Threatening Causes • septic joint (see Orthopaedics, OR8) Differential Diagnosis • articular pain monoarticular infectious: bacterial, viral, fungal hemarthrosis: trauma/fracture, anticoagulants, bleeding diatheses crystal induced: gout, CPPD, hydroxyapatite inflammatory: seropositive, seronegative neoplasm degenerative: osteoarthritis polyarticular infectious: Lyme disease, bacterial endocarditis, septicemia, gonococcus, viral post-infectious: rheumatic fever, reactive arthritis, enteric infections inflammatory: seropositive, seronegative degenerative: osteoarthritis • non-articular musculoskeletal localized: tendonitis, bursitis, capsulitis, muscle sprain generalized: fibromyalgia, PMR • other neurologic: spinal stenosis/spondylolithesis, degenerative disc disease, cauda equina syndrome, neoplasm, thoracic outlet syndrome, Charcot joint vascular: intermittent claudication History and Physical Examination • determine onset, course, location, character of the pain (OPQRST) and recurrence • determine which joint or joints are involved • associated symptoms: fever, constitutional symptoms, skin lesions, conjunctivitis, urethritis • patterns of joint involvement: polyarticular vs. monoarticular, symmetric vs. asymmetric • inflammatory symptoms: prolonged morning stiffness, stiffness and pain ease through the day, midday fatigue, soft tissue swelling • non-inflammatory symptoms: stiffness short lived after inactivity, short duration stiffness in the morning, pain increases with activity • assess ROM, presence of joint effusion, warmth • watch for: localized joint pain, erythema, warmth, swelling with pain on active ROM, inability to bear weight, fever as these may indicate presence of septic joint Investigations • x-ray, CBC, ESR, CRP, WBC, INR/PTT, blood cultures, urate • joint aspirate g send for: WBC, protein, glucose, Gram stain, crystals
Causes of Joint Pain SOFTER TISSUE Sepsis Osteoarthritis Fractures Tendon/muscle Epiphyseal Referred Tumour Ischemia Seropositive arthritides Seronegative arthritides Urate Extra-articular rheumatism (e.g. polymyalgia)
ER26 Emergency Medicine
Approach to Common ER Presentations
Toronto Notes 2011
Management • septic joint: IV antibiotics ± joint decompression and drainage antibiotics can be started empirically if septic arthritis cannot be ruled out • crystalline synovitis: NSAIDs at high dose, colchicine with in first 24 hours, corticosteroids do not use allopurinol, as it may worsen acute attack • acute polyarthritis: NSAIDs, analgesics (acetaminophen ± opioids), corticosteroids local or systemic hospitalization is required in the presence of (1) significant, concomitant internal organ involvement; (2) signs of bacteremia, including vesiculopustular skin lesions, Roth spots, shaking chills, or splinter hemorrhages; (3) systemic vasculitis; (4) severe pain; (5) severe constitutional symptoms; (6) purulent synovial fluid in one or more joints; or (7) immunosuppression • osteoarthritis: acutely: NSAIDs, acetaminophen • soft tissue pain: allow healing with enforced rest ± immobilization nonpharmacologic treatment: local heat or cold, electrical stimulation, massage pharmacologic: oral analgesics, NSAIDs, muscle relaxants, corticosteroid injections, topical agents
Otalgia Differential Diagnosis (see also Otolaryngology, OT6) • local infections: AOE, AOM, OM with effusion, mastoiditis, myringitis, malignant otitis in diabetics, herpes simplex/zoster, auricular cellulites, external canal abscess others: trauma, neoplasm, foreign body, cerumen impactions, Wegener’s • determine onset, course, location and character of pain • otorrhea, aural fullness, hearing loss, pruritis • Q-tip use, hearing aids, headphones • associated symptoms: fever • observe for otorrhea, palpation of outer ear, otoscope to see bulging erythematous TM, perforation Investigations • consider audiogram if hearing loss Management • debridement and antibiotics for cerumen and infection
Seizures • see Neurology, N8 Definition • paroxysmal alteration of behaviour and/or EEG changes resulting from abnormal, excessive activity of neurons Categories • generalized seizure (consciousness always lost): tonic/clonic, absence, myoclonic, atonic • partial seizure (focal): simple partial, complex partial • causes: trauma, intracranial hemorrhage, structural abnormality, infection, toxins/ drugs, metabolic disturbance (hypo/hyperglycemia, hypo/hypernatremia, hypocalcemia, hypomagnesemia); primary seizure disorder • differential diagnosis: syncope, pseudoseizures, migraines, movement disorder, narcolepsy/ cataplexy, myoclonus History • from patient and bystander: flaccid and unconscious, often with deep rapid breathing • preceding aura, rapid onset, loss of bladder/bowel control, tongue-biting (sides of the tongue) Physical Examination • injuries to head and spine and bony prominences (e.g. elbows), tongue laceration, aspiration, urinary incontinence Min. Workup in an Adult with 1st Time Seizure CBC and diff Electrolytes including Ca, Mg, P04 Head CT
Investigations • known seizure disorder: anticonvulsant levels • Accu-Chek® • first time seizure: CBC, serum glucose, electrolytes, BUN, creatinine, Ca, Mg; consider prolactin, b-hCG, tox screen • initial: CT; x-ray if suspected extremity injuries. Definitive: MRI, EEG
Toronto Notes 2011
Approach to Common ER Presentations
Emergency Medicine ER27
Table 13. Management of Status Epilepticus Time (min)
Steps
0-5
Give oxygen; ensure adequate ventilation Monitor: vital signs, electrocardiography, oximetry Establish IV access; obtain blood samples for glucose level, CBC, electrolytes, toxins, and anticonvulsant levels
6-9
Give 50 mL 50% glucose (preceded by thiamine 100 mg IM in adults)
10-20
IV lorazepam 0.1 mg/kg at 2 mg/min or IV diazepam 0.2 mg/kg at 5 mg/min Diazepam can be repeated if seizures do not stop after 5 min; if diazepam is used to stop the status, then phenytoin should be administered promptly to prevent the recurrence of status
21-60
If status persists, administer 15-20 mg/kg of phenytoin intravenously no faster than 50 mg/min in adults and 1 mg/kg/min in children
>60
If status does not stop after 20 mg/kg of phenytoin, give additional doses of 5 mg/kg to a maximal dose of 30 mg/kg. If status persists, then give 20 mg/kg of phenobarbital IV at 100 mg/min. When phenobarbital is given after a benzodiazepine, ventilatory assistance is usually required If status persists, then give general anaesthesia (e.g., pentobarbital). Vasopressors or fluid volume are usually necessary. Electroencephalogram should be monitored. Neuromuscular blockade may be needed.
If administering phenytoin, patient must be on a cardiac monitor as arrhythmias and/or hypotension may occur.
Adapted form: Cecil’s Essentials of Medicine, 7th edition, Table 125-7. Used with permission.
Disposition • the decision to admit or discharge should be based on the underlying disease process identified if a patient has returned to baseline function and is neurologically intact, then consider discharge with outpatient follow-up • first-time seizure patients being discharged should be referred to a neurologist for follow-up • admitted patients should generally have a neurology consult • patient should not drive until medically cleared (local regulations vary) complete notification form to appropriate authority re: ability to drive • warn regarding other safety concerns (e.g. no swimming, bathing children alone, etc.)
Shortness of Breath • see Respirology and Cardiology Etiology • categorized into one of two groups: respiratory or cardiovascular • respiratory system dyspnea: discomfort related to disorders of the central controller (brain), the ventilatory pump (ventilatory muscles, peripheral nerves), and the gas exchanger (alveoli and pulmonary capillaries) • cardiovascular system dyspnea: cardiac diseases (acute ischemia, heart failure, systolic dysfunction, valvular disorders, pericardial diseases), anemia, and deconditioning History/Physical • acute SOB is often due to a relatively limited number of conditions. Associated symptoms and signs are key to the appropriate diagnosis substernal chest pain with cardiac ischemia fever, cough and sputum with respiratory infections urticaria with anaphylaxis wheezing with acute bronchospasm • dyspnea may be the sole complaint and the physical examination may reveal few abnormalities (e.g. pulmonary embolism, pneumothorax) • chest tightness may be indicative of bronchospasm • a sensation of rapid, shallow breathing may correspond to interstitial disease • a sense of heavy breathing is typical of deconditioning • vitals including pulse oximetry wheeze (airway) vs. crackles (parenchymal), JVP, and murmurs Investigations • CBC + differential (hematocrit to exclude anemia), electrolytes, consider ABG • CXR (hyperinflation and bullous disease suggestive of obstructive lung disease, or changes in interstitial markings consistent with inflammation, infection or interstitial fluid) • serial cardiac enzymes and ECG if considering cardiac source • CT chest usually is not indicated in the initial evaluation of patients with dyspnea, but can be valuable in patients with interstitial lung disease, occult emphysema, or chronic thromboembolic disease (PE) Disposition • the history and physical examination lead to accurate diagnoses in patients with dyspnea in approximately two-thirds of cases; the decision to admit or discharge should be based on the underlying disease process identified consideration for intubation should be early in CO2 retainers (e.g. COPD) • if the decision to discharge is chosen, provide appropriate discharge instructions to return in case of returning/worsening SOB
Causes of Acute Dsypnea Cardiovascular: acute MI, CHF, cardiac tamponade. Respiratory: bronchospasm, pulmonary embolism, pneumothorax, infection (bronchitis, pneumonia), upper airway obstruction (aspiration, anaphylaxis).
Contraindication to 100% Oxygen CO2 retainers (e.g. COPD).
ER28 Emergency Medicine
Approach to Common ER Presentations
Toronto Notes 2011
Syncope 5 Types of Syncope 1. Vasomotor 2. Cardiac 3. CNS 4. Metabolic 5. Psychogenic
Causes of Syncope by System HEAD, HEART, VeSSELS Hypoxia/Hypoglycemia Epilepsy Anxiety Dysfunctional brainstem Heart attack Embolism (PE) Aortic obstruction Rhythm disturbance Tachycardia Vasovagal Situational Subclavian steal ENT (glossopharyngeal neuralgia) Low systemic vascular resistance Sensitive carotid sinus
Definition • sudden, transient loss of consciousness and postural tone with spontaneous recovery • usually caused by generalized cerebral or reticular activating system hypoperfusion Etiology • cardiogenic: arrhythmia, outflow obstruction (e.g. PE, tamponade, tension pneumothorax, pulmonary HTN), MI, valvular disease • non-cardiogenic: peripheral vascular (hypovolemia), vaso-vagal, cerebrovascular disorders, CNS, metabolic disturbances (e.g. EtOH intoxication) History • gather details from witnesses, and clarify patient’s experience (e.g. dizziness, ataxia, or true syncope) • distinguish between syncope and seizure (see Neurology, N9) some patients may have myoclonic jerks with syncope – NOT a seizure signs and symptoms during presyncope, syncope and postsyncope past medical history, drugs think anatomically in differential; pump (heart), blood (quality), vessels, brain • sudden loss of consciousness with no warning or prodrome is cardiogenic until proven otherwise Physical Examination • postural BP and HR • cardiovascular, respiratory and neuro exam • physical findings in the elderly patient who falls (I HATE FALLING): Inflammation of joints (or joint deformity) Hypotension (orthostatic blood pressure changes) Auditory and visual abnormalities Tremor (Parkinson’s disease or other causes of tremor) Equilibrium (balance) problem Foot problems Arrhythmia, heart block or valvular disease Leg-length discrepancy Lack of conditioning (generalized weakness) Illness Nutrition (poor; weight loss) Gait disturbance Investigations • ECG (tachycardia, bradycardia, blocks, WPW, long QT interval), bedside glucose • as indicated: CBC, electrolytes, BUN, creatinine, ABGs, Troponin, Ca, Mg, b-hCG • consider drug screen Management • ABCs, IV, O2, monitor • examine for signs of trauma caused by syncopal episode • cardiogenic syncope: admit to medicine/cardiology • non-cardiogenic syncope: discharge with follow-up as indicated by cause
Which Patients with Syncope should be Admitted? Those at risk of complications: • Older than 60-70 years • Significant cardiac risk factors • Recurrent syncope • Serious underlying illness
Disposition • decision to admit is based on etiology • most patients will be discharged • on discharge, instruct patient to follow up with family physician educate re: avoiding orthostatic or situational syncope patients with recurrent syncope should avoid high-risk activities (e.g. driving)
Sexual Assault Legally required to report sexual assault if victim is 50 years HR >100 bpm O2 sat on RA 6
Investigations (Figures 12-14) • ECG and CXR are useful to look for other causes (e.g. ACS, pneumonia) • D-dimer is only useful if it is negative in low risk patients • Ultrasound has high sensitivity and specificity for proximal clot but only 73% sensitivity for DVT below the knee • CT angiography has high sensitivity and specificity for PE, may also suggest other etiology • V/Q scan useful when CT angio not available, or patient unable to tolerate IV contrast (e.g. renal failure, allergy) Management of DVT/PE • LMWH unless patient also has renal failure dalteparin 200 IU/kg SC q24h or enoxaparin 1.5 mg/kg SC q24h • warfarin started at same time as LMWH (5 mg PO daily initially) • LMWH discontinued when INR has been therapeutic (2-3) for 2 consecutive days early ambulation with analgesia is safe if appropriately anticoagulated • IVC filter or surgical thrombectomy considered if anticoagulation is contraindicated • consider thrombolysis if extensive DVT or PE causing hemodynamic compromise often can be treated as outpatient, may require analgesia for chest pain (narcotic or NSAID) • admit if hemodynamically unstable, require supplemental O2, major comorbidities, lack of sufficient social supports, unable to ambulate, need invasive therapy • long term anticoagulation if reversible risk factor: 3-6 months of warfarin idiopathic VTE: may need longer term warfarin (5 yrs or more)
D-dimer is only useful if it is negative. Negative predictive value >99%.
Suspected (symptomatic) acute DVT Compression U/S
50% of patients with symptomatic proximal DVT will develop PE, often within days to weeks of the event.
Normal
Inconclusive or inadequate study
DVT present
Repeat U/S in 5 days
Venography or MRI
Treatment
DVT present
DVT absent
DVT present
Negative for DVT
Treatment
No treatment
Treatment
No treatment
Figure 12. Approach to Suspected DVT
Toronto Notes 2011
Medical Emergencies Determine need to investigate via PERC score
Positive ≥1/8
Wells criteria
Low
Emergency Medicine ER35
Negative 0/8
PE excluded
Moderate/High
D-dimer assay 500 ng/mL
Spiral CT pulmonary angiogram (CT-PA)
PE excluded Negative
Positive
PE excluded
PE confirmed
Figure 13. Approach to Suspected PE Determine clinical probability of PE via Well’s (high, moderate or low) V/Q scan Normal V/Q scan and any clinical probability of PE
Low probability V/Q scan and low clinical probability of PE
High probability V/Q scan and high clinical probability of PE
All other combinations of V/Q scan results and clinical probability of PE
PE excluded
PE excluded
PE confirmed
Pulmonary angiogram or serial lower extremity venous ultrasounds
Clinical Criteria to Prevent Unnecessary Diagnostic Testing in Emergency Department Patients with Suspected Pulmonary Embolism J Thromb Haemost 2004; 2(8):1247-55. Purpose: To develop pulmonary embolism (PE) ruleout criteria (PERC) that can be used at the bedside, and prevents overtesting for PERC. Also, to prevent over-testing for PE, which includes the D-dimer test that frequently results in false positives. Study: 21 variables were collected prospectively from 3148 ER patients evaluated for possible PE to develop rule-out criteria. The application of the developed rules was investigated in 1427 low-risk patients and 382 very-low risk patients. Results: Eight variables were included in a block rule (Age 120, aim to reduce dBP by 20% in the first 24 hrs treatment may be initiated in the ED followed by prompt admission to ICU for continuous BP monitoring re-administer sympathetic blocker if due to withdrawal (e.g. clonidine, propranolol) Hypertensive Urgency • definition: severely elevated blood pressure (usually sBP >180, dBP >115) with no evidence of end-organ damage • most commonly due to non-adherence with medications • treatment: gradually reduce pressure over 24-48 hours to a level appropriate for the patient • goal is to differentiate hypertensive emergencies from hypertensive urgencies History and Physical Examination • prior hypertensive crises • antihypertensive medications prescribed, adherence, and BP control • MAOIs, substance use, use of stimulants or withdrawal from sedatives including EtOH • blood pressure measurement in all limbs • fundoscopic exam (hemorrhages, papilledema, etc.), gross motor examination Investigations • CBC, electrolytes, BUN, creatinine, urinalysis • peripheral blood smear – to detect microangiopathic hemolytic anemia • CXR – if SOB or chest pain • ECG, troponins, CK – if chest pain • CT head – if neurological findings or severe headache Treatment of Hypertensive Emergencies • see Table 16
Signs of Fluid Depletion Increased heart rate Postural changes in vital signs Decreased urine output (normal: 0.5 cc/kg/hr) Hypertensive Decreased skin turgor Sunken eyes Dry mucous membranes Decreased capillary refill
HELLP Syndrome (seen only in preeclampsia/eclampsia) Hemolytic anemia Elevated Liver enzymes Low Platelet count
Catecholamine Induced Hypertensive Emergencies Avoid use of non-selective β-blockers as they inhibit β-mediated vasodilation and leave α-adrenergic vasoconstriction unopposed.
Signs/Symptoms of CNS Hypertensive Emergency N/V Seizure Headache or altered mental status Cushing response
Evidence of End-Organ Damage CNS: headache, focal neurological signs, seizures CVS: angina, CHF, back pain (aortic dissection) Renal: hematuria, oliguria Eyes: papilledema, retinal hemorrhages
With CNS manifestations of severe hypertension, it is often difficult to differentiate causal relationships [i.e. hypertension could be secondary to primary cerebral event (Cushing effect)].
ER38 Emergency Medicine
Medical Emergencies
Toronto Notes 2011
Table 16. Most Commonly Used Agents for the Treatment of Hypertensive Crisis Drug Most commonly used agents for hypertensive crisis are labetalol and nitroprusside.
Drugs that Increase Adrenergic Stimulation MAOIs TCAs Amphetamines Cocaine
Dosage
Onset of Action
Duration Adverse of Action Effects*
Special Indications
0.25-10 µg/kg/min
Immediate 3-5 min
N/V, muscle twitching, sweating, cyanide intoxication, coronary steal syndrome
Most hypertensive emergencies (esp CHF, aortic dissection) Use in combination with b-blockers (e.g. esmolol) in aortic dissection Caution with high ICP and azotemia
15-30 min
40 min
Tachycardia, headache, flushing, local phlebitis (e.g. encephalopathy, RF, eclampsia, sympathetic crisis)
Most hypertensive emergencies Caution with acute CHF
Most hypertensive emergencies Caution with glaucoma
VASODILATORS Sodium Nitroprusside (vascular smooth muscle dilator) 1st line
Nicardipine (CCB) 2 mg IV bolus, then 4 mg/kg/hr IV
Fenoldopam Mesylate (dopamine receptor antagonist)
0.05-0.1 µmg/kg/min IV 1st degree
ADRENERGIC INHIBITORS Labetalol
20 mg IV bolus q10 min 5-10 min or 0.5-2 mg/min
3-6 hr
Esmolol
250-500 µg/kg/min 1-2 min 1 min, then 50 µg/kg/min for 4 min; repeat
10-20 min Hypotension, nausea, bronchospasm
Aortic dissection, acute MI SVT dysrhythmias, perioperative HTN Avoid in acute CHF, HB >1st degree
Phentolamine
5-15 mg q5-15 min
3-10 min
Catecholamine excess (e.g. pheo)
1-2 min
Tachycardia, headache, flushing
*Hypotension may occur with all of these agents If patient presents within 4.5 hours of onset of disabling neurological deficits greater than 60 minutes with no signs of resolution, they may be candidate for thrombolysis. Do brief assessment and order stat CT head. Exclusion Criteria for tPA: Suspected subarachnoid hemorrhage Previous intracranial hemorrhage Cerebral infarct or severe head injury within the past 3 months Recent pericarditis Major surgery within the past 14 days GI or urinary hemorrhage within the past 21 days Recent lumbar puncture or arterial puncture at noncompressible site Patient is pregnant BP=185 mmHg systolic, or =110 mmHg diastolic Bleeding diathesis Prolonged PTT (more than 40 seconds) or INR >1.4 Platelet count leg weakness) and hypoesthesia, ipsilateral hemianopsia, gaze preference to side of lesion ± agnosia, receptive/expressive aphasia
Posterior Cerebral Artery
Affects vision and thought Homonymous hemianopsia, cortical blindness, visual agnosia, altered mental status, impaired memory
Vertebrobasilar Artery
Wide variety of CN, cerebellar and brainstem deficits: vertigo, nystagmus, diplopia, visual field deficits, dysphagia, dysarthria, facial hypoesthesia, syncope, ataxia Loss of pain and temperature sensation ipsilateral face and contralateral body
Investigations • CBC, electrolytes, blood glucose, coagulation studies, ± cardiac biomarkers, ± toxicology screen • non-contrast CT head: look for hemorrhage, ischemia • ECG ± echocardiogram: rule out atrial fibrillation, acute MI as source of emboli other imaging: carotid dopplers, CTA, MRA as appropriate Management • quickly determine if patient is eligible for thrombolysis (need acute onset less than 4.5 hours from drug administration time AND compatible physical findings AND normal CT with no bleed) – not much time to do all of this (often requires designated stroke team) • ABCs with RSI if GCS ≤8, rapidly decreasing GCS, or inadequate airway protection reflexes • IV ± cardiac monitoring judge fluid rate carefully to avoid overhydration (cerebral edema) as well as underhydration (underperfusion of the ischemic penumbra) • BP control: only treat severe hypertension (sBP >200, dBP >120, mean arterial BP >140) or hypertension associated with hemorrhagic stroke transformation, cardiac ischemia, aortic dissection, or renal damage; use IV nitroprusside or labetalol • cerebral edema control: hyperventilation, mannitol to decrease ICP if necessary • consult neurosurgery, neurology as indicated Medications • acute ischemic stroke: thrombolytics (rt-PA, e.g. alteplase) if within 3 hours of symptom onset with no evidence of hemorrhage on CT scan • antiplatelet agents: prevent recurrent stroke or stroke after TIAs, e.g. aspirin (1st-line); clopidogrel, ticlopidine (2nd-line)
Differentiation of UMN Disease versus LMN Disease Category
UMN Disease
LMN Disease
Muscular deficit Reflexes Tone Fasciculations Atrophy
Muscle groups Increased Increased Absent Absent/minimal
Individual muscles Decreased/absent Decreased Present Present
7 Causes of Emboli from the Heart Atrial Fib MI Endocarditis Valvular disease Dilated cardiomyopathy Left heart myxoma Prosthetic Valves
Causes of Acute Ataxia UNABLE TO STAND Underlying weakness (mimic ataxia) Nutritional neuropathy (vitamin B12 deficiency) Arteritis/vasculitis Basilar migraine Labyrinthitis/vestibular neuronitis Encephalitis/infection Trauma (post-concussive) Other (rare genetic or metabolic disease) Stroke (ischemia or hemorrhage) Toxins (drugs, toluene, mercury) Alcohol Neoplasm/paraneoplastic syndrome Demyelination (Miller Fisher, Guillain Barré, MS)
Gynecologic/Urologic Emergencies Vaginal Bleed • see Gynecology, GY6 and Obstetrics, OB20 Etiology • pregnant patient 1st/2nd trimester pregnancy: ectopic pregnancy, abortion (threatened, incomplete, complete, missed, inevitable, septic), molar pregnancy 2nd/3rd trimester pregnancy: placenta previa, placental abruption, premature rupture of membranes, preterm labour either: trauma, bleeding cervical polyp • postpartum postpartum hemorrhage, uterine inversion, retained placental tissue, endometritis • non-pregnant patients dysfunctional uterine bleeding, uterine fibroids, pelvic tumours, trauma, endometriosis, PID, exogenous hormones
Vaginal bleeding can be life threatening. Always start with ABCs and ensure your patient is stable.
ER40 Emergency Medicine
Gynecologic/Urologic Emergencies
Toronto Notes 2011
History • menstrual history, sexual activity, contraception, history of PID • pregnancy details • determine amount of blood • urinary, GI symptoms Physical Examination • look for signs of hypovolemia • pelvic examination – NOT if suspected placenta previa (ultrasound first) • speculum exam if pregnant use sterile speculum • bimanual examination if pregnant use sterile gloves if patient is near term with possible rupture of membranes and without other indications defer bimanual examination (infection risk)
Need b-hCG ≥1200 to see interuterine changes on transvaginal U/S.
Classifying miscarriage (abortion): Missed – non-viable intrauterine pregnancy Threatened – viable intrauterine pregnancy with os closed Inevitable – os closed, no products of conception passed Incomplete – products of conception partially expelled Complete – products of conception completely expelled Septic – any of above with presence of infection (usually incomplete) Recurrent – >3 spontaneous abortions (recurrent pregnancy loss)
Vaginal bleeding (and its underlying causes) can be a very emotionally taxing presentation for patients. Ensure appropriate support is provided.
Investigations • b-hCG test for all patients with child-bearing potential • CBC, blood and Rh type, quantitative b-hCG, PTT, INR • type and cross if significant blood loss • 1st/2nd trimester/non-pregnant ultrasound (U/S) – intrauterine pregnancy, ectopic pregnancy, traumatic injury, foreign body must correlate U/S findings with b-hCG if U/S is non-diagnostic (transvaginal ultrasound will not see gestation in uterus if b-hCG 1000-2000 IU: further work-up and/or gynecology consult abortions: if complete, discharge if stable; for all others, acquire gynecology consult • 2nd/3rd trimester pregnancy placenta previa or placental abruption: obstetrics consult for possible admission • postpartum uterine inversion: replace uterus immediately, may require operative management postpartum hemorrhage: extraction of placenta if retained, hysterectomy if uncontrolled bleeding retained tissue: D&C endometritis: IV antibiotics • non-pregnant dysfunctional uterine bleeding (prolonged or heavy flow ± breakthrough bleeding and without ovulation, a diagnosis of exclusion) 35-40 years of age: uterine sampling necessary prior to initiation of hormonal treatment to rule out endometrial cancer, U/S for any masses felt on exam structural abnormalities: fibroids or uterine tumours may require excision for diagnosis/ treatment, U/S for workup of other pelvic masses, Pap smear/biopsy for cervical lesions Disposition • the decision to admit or discharge should be based on the stability of the patient, as well as the nature of the underlying cause; consult gynecology for admitted patients • if patient can be safely discharged, ensure follow up with family physician or gynecologist instruct patient to return to emergency for increased bleeding, presyncope
Toronto Notes 2011
Gynecologic/Urologic Emergencies
Emergency Medicine ER41
Pregnant Patient in the ER Table 18. Complications of Pregnancy Trimester
Fetal
Maternal
First 1-14 wks
Pregnancy failure • Spontaneous abortion • Fetal demise • Gestational trophoblastic disease
Ectopic pregnancy Anemia Hyperemesis gravidarum UTI/pyelonephritis
Second 15-27 wks
Disorders of fetal growth • IUGR • Oligo/polyhydramnios
Gestational diabetes mellitus Rh incompatibility UTI/pyelonephritis
Third 28-40 wks
Vasa previa
Preterm labour/PPROM Preeclampsia/eclampsia Placenta previa Placental abruption Uterine rupture DVT
Nephrolithiasis (Renal Colic) • see Urology, U15 Epidemiology and Risk Factors • 10% of population (twice as common in males) • recurrence 50% at 5 yrs • peak incidence 30-50 years of age • 75% of stones 5 mm, or if patient has signs of obstruction or infection • a-blocker helpful to increase stone passage in select cases Disposition • most patients can be discharged • ensure patient is stable, has adequate analgesia, and is able to tolerate oral medications • may advise hydration, calcium supplementation, and limitation of protein, sodium, oxalate and alcohol intake
Indications for Admission to Hospital • Intractable pain • Fever (suggests infection) • Single kidney with ureteral obstruction • Bilateral obstructing stones • Intractable vomiting • Compromised renal function
ER42 Emergency Medicine
Ophthalmologic Emergencies
Toronto Notes 2011
Ophthalmologic Emergencies Ophthalmologic Foreign Body and Corneal Abrasion • see also Ophthalmology, OP17
ALWAYS assess visual acuity in both eyes when a patient presents to the ER with an ophthalmologic complaint.
Any etiology of red eyes may also present with blurred vision.
Other Ophthalmologic Emergencies (See Ophthalmology, OP5) Infectious – Red eye (Table 19), endophthalmitis, orbital cellulitis Trauma – retinal detachment, globe rupture, orbital blow-out fractures, chemical burns Autoimmune – Giant cell arteritis
Contraindications to Pupil Dilation • Shallow anterior chamber • Iris-supported lens implant • Potential neurological abnormality requiring pupillary evaluation • Caution with CV disease – mydriatics can cause tachycardia
History • patient may complain of pain, tearing, itching, redness, photophobia, foreign body sensation • elicit history of potential trauma to eye • mechanism of foreign body insertion – if high velocity injury suspected (welding, metal grinding, metal striking metal), must obtain orbital x-rays or ultrasound to exclude presence of intraocular metallic foreign body • see Table 19 for important considerations of red eye in the emergency department Table 19. Differential Diagnosis of Red Eye in the Emergency Department Symptom
Possible Serious Etiology
Light Sensitivity
Iritis, keratitis, abrasion, ulcer
Unilateral
Above + herpes simplex, acute angle closure glaucoma
Significant pain
Above + scleritis
White spot on cornea
Corneal ulcer
Blurred vision
All of the above
Non-reactive pupil
Acute glaucoma, iritis
Copious discharge
Gonococcal conjunctivitis
Physical Examination • visual acuity in both eyes with best corrected vision • pupils, extraocular movement, external ocular structures • fundoscopy • tonometry – measurement of intraocular pressure (with Tonopen) normal pressure: 10-20 mmHg, glaucoma associated with increased pressures • slit lamp exam: start with unaffected eye, perform a systematic examination: lids, lashes, lacrimal apparatus, conjunctiva, sclera, cornea, anterior chamber, iris, lens, vitreous proparacaine anaesthetic drops may ease examination look for rust ring with metallic foreign body, corneal edema, anterior chamber cells/flare may use fluoroscein dye which stains de-epithelialized cornea green when viewing with cobalt blue filter Management • copious irrigation with saline for any foreign body • remove foreign body under slit lamp exam with cotton swab or sterile needle • antibiotic drops qid until healed • patching may not improve healing or comfort – do not patch contact lens wearers • limit use of topical anesthetic to examination only • consider tetanus prophylaxis • ophthalmology consult if globe penetration suspected Initial Management of other Opthalmologic Emergencies • ruptured globe – stabilize any foreign body, shield eye with no pressure, elevate head of bed to 30°, tetanus prophylaxis, IV antibiotics, NPO, analgesic, antiemetic, sedation prn • retinal artery occlusion – globe massage, paper bag breathing, carbogen inhalation (95% oxygen, 5% carbon dioxide) • chemical burn – immediate copious irrigation, may consider topical anaesthetic drops to facilitate irrigation • acute angle-closure glaucoma – IV or PO acetazolamide, topical pilocarpine and timolol • preseptal cellulitis (2o to superficial trauma) – topical or systemic Abx • orbital cellulitis (2o to sinusitis) – admit, IV antibiotics, blood cultures, CT Disposition • most patients can be discharged with outpatient ophthalmology follow-up • admit patients requiring emergent ophthalmologic procedures or IV antibiotics
Toronto Notes 2011
Dermatologic Emergencies
Dermatologic Emergencies Life Threatening Dermatoses Rash Characteristics • DIFFUSE RASH staphylococcal scalded skin syndrome (SSSS) caused by an exotoxin from infecting strain of coagulase-positive S. aureus mostly occurs in children prodrome: fever, irritability, malaise and skin tenderness sudden onset of diffuse erythema: skin is red, warm, and very tender flaccid bullae that are difficult to see, then desquamate in large sheets Nikolsky’s sign: gentle lateral stroking of skin causes epidermis to separate toxic epidermal necrolysis (TEN) see Dermatology, D22 caused by drugs (e.g. phenytoin, sulfas, penicillins and NSAIDs), bone marrow transplantation, blood product transfusions usually occurs in adults diffuse erythema followed by necrosis severe mucous membrane blistering entire epidermis desquamation high mortality (>50%) toxic shock syndrome (TSS) see Infectious Diseases, ID27 caused by superantigen from S. aureus or GAS activating T-cell and cytokines patient often presents with onset of shock and multi-organ failure, fever diffuse erythematous macular rash at least 3 organ systems involved: CNS, respiratory, GI, muscular, mucous membranes, renal, liver, hematologic, skin (necrotizing fasciitis, gangrene) • VESICOBULLOUS LESIONS erythema multiforme (EM) see Dermatology, D22 immunologic reaction to herpes simplex viral prodrome 1-14 days before rash “target lesion”: central gray bulla or wheal surrounded by concentric rings of erythema and normal skin Stevens-Johnson syndrome (SJS) see Dermatology, D22 related to drugs such as antiepileptics and biologic agents, e.g. infliximab EM with constitutional symptoms and mucous membrane involvement (milder mucous membrane involvement than TEN) • DISCRETE LESIONS pyoderma gangrenosum often associated with immunocompromised patients (HIV, leukemia or lymphoma) with Gram-negative sepsis often occurs in arms, hands, feet, or perineal region usually begins as painless macule/vesicle g pustule/bulla on red/blue base g sloughing, leaving a gangrenous ulcer disseminated gonococcal infection (DGI) see Dermatology, D32 fever, skin lesions (pustules/vesicles on erythematous base ~5 mm in diameter), arthritis (joint swelling and tenderness), septic arthritis (in larger joints, e.g. knees, ankles and elbows) most commonly in gonococcus positive women during menstruation or pregnancy skin lesions usually appear in extremities and resolve quickly (10 yrs or incomplete primary series Snake Bites • history, physical exam, investigations and initial management similar to mammalian bites • additional management issues snake bites are rarely fatal but proper precautions must be taken supportive management, observe for compartment syndrome, analgesia, tetanus prophylaxis contact regional Poison Control Centre for consultation constriction band should be placed proximal to bite observe for signs and symptoms of envenomation 15min-2hrs after bite (pain, sweating, edema, chills, weakness, numbness, tingling, HR changes, faintness, ecchymosis, N/V); if no envenomation then remove band and monitor closely for 24 hrs if envenomation present, administer antivenom Insect Bites • Bee Stings 5 types of reactions to stings (local, large local, systemic, toxic, unusual) history and physical exam key to diagnosis; no lab test will confirm investigations: CBC, electrolytes, BUN, creatinine, glucose, ABGs, ECG ABC management, epinephrine 0.1 mg IV over 5 minutes if shock, antihistamines, cimetidine 300mg IV/IM/PO, steroids, b-agonists for SOB/wheezing 3 mg in 5 mL NS via nebulizer, local site management • West Nile Virus (see Infectious Diseases, ID28) severity: asymptomatic 80%, flu-like symptoms 20%, encephalitis 12 hrs old • Hand or foot wounds • Wounds near joints Patient Factors • Immunocompromised • Age >50 years • Prosthetic joints or valves
Consider Admission if: • Moderate to severe infections • Infections in immunocompromised patients • Not responding to oral Rx • Penetrating injuries to tendons, joints, CNS • Open fractures
ER48 Emergency Medicine
Environmental Injuries/Toxicology
Toronto Notes 2011
Near Drowning • most common in children 50 mmHg, or pO2 2 suggests alcohol as the cause as well as elevated GGT with acute ingestion
Common Deficiencies • Thiamine • Niacin • Folate • Glycogen • Magnesium • Potassium
ER50 Emergency Medicine
Toxicology
Toronto Notes 2011
• peritonitis/spontaneous bacterial peritonitis occasionally accompanies cirrhosis leukocytosis, fever, generalized abdominal pain/tenderness paracentesis for diagnosis (common pathogens: E. coli, Klebsiella, Strep) • GI bleeds most commonly gastritis or ulcers, even if patient known to have varices consider Mallory-Weiss tear secondary to retching often complicated by underlying coagulopathies minor – treat with antacids severe or recurrent – endoscopy Miscellaneous Problems • rhabdomyolysis presents as acute weakness associated with muscle tenderness usually occurs after prolonged immobilization increased creatinine kinase (CK), hyperkalemia myoglobinuria – may lead to acute renal failure treatment: IV fluids, forced diuresis (mannitol 20% 15 mg/kg IV over 30 min) • increased infections – due to impaired host defenses, immunocompromise, poor living conditions Disposition • before patient leaves ED ensure stable vital signs can walk unassisted fully oriented • offer social services to find shelter or detox program • ensure patient can obtain any medications prescribed and can complete any necessary follow-up
Approach to the Overdose Patient
Suspect Overdose when: • Altered level of consciousness/coma • Young patient with life-threatening arrhythmia • Trauma patient • Bizarre or puzzling clinical presentation
History • who? age, weight, underlying medical problems, medications • what? substance and how much • when? time since exposure determines prognosis and need for decontamination, symptoms since • how? route • why? intention, suicidality Physical Examination • focus on: ABCs, LOC/GCS, vitals, pupils Principles of Toxicology • 4 principles to consider with all ingestions I. resuscitation (ABCs) II. screening (toxidrome? clinical clues?) III. decrease absorption of drug IV. increase elimination of drug
ABCs of Toxicology • basic axiom of care is symptomatic and supportive treatment • address underlying problem only once patient is stable A Airway (consider stabilizing the C-spine) B Breathing C Circulation D1 Drugs ACLS as necessary to resuscitate the patient universal antidotes D2 Draw bloods D3 Decontamination (decrease absorption) E Expose (look for specific toxidromes)/Examine the Patient F Full vitals, ECG monitor, Foley, x-rays, etc. G Give specific antidotes, treatments Go back and reassess Call poison information centre Obtain corroborative history from family, bystanders
Toronto Notes 2011
Toxicology
Emergency Medicine ER51
D1 – Universal Antidotes • treatments that will not harm patients and may be essential Oxygen • do not deprive a hypoxic patient of oxygen no matter what the antecedent medical history (i.e. even COPD with CO2 retention) • if depression of hypoxic drive, intubate and ventilate • exception: paraquat or diquat (herbicides) inhalation or ingestion (oxygen radicals increase morbidity) Glucose • give to any patient presenting with altered LOC • measure blood glucose prior to glucose administration if possible • adults: 0.5-1.0 g/kg (1-2 mL/kg) IV of D50W • children: 0.25 g/kg (2-4 mL/kg) IV of D25W Thiamine (Vitamin B1) • 100 mg IV/IM to all patients with IV/PO glucose • a necessary cofactor for glucose metabolism, but do not delay glucose if thiamine unavailable • to prevent Wernicke-Korsakoff syndrome • must assume all undifferentiated comatose patients are at risk Naloxone (central µ-receptor competitive antagonist, shorter t1/2 than naltrexone) • antidote for opioids: administration is both diagnostic and therapeutic (1 min onset of action) • used for the undifferentiated comatose patient • loading dose adults 2 mg initial bolus IV/IM/SL/SC or via ETT (ETT dose = 2-2.5x IV dose) if no response after 2-3 minutes, increase dose by 2 mg increments until a response or to max 10 mg known chronic user, suspicious history, or evidence of track marks, give 0.01 mg/kg child 0.01 mg/kg initial bolus IV/IO/ETT 0.1 mg/kg if no response and narcotic still suspected to max of 10 mg • maintenance dose may be required because half-life of naloxone (30-80 mins) is much shorter than many narcotics hourly infusion rate at 2/3 of initial dose that produced patient arousal
D2 – Draw Bloods • essential tests (see Table 24) CBC, electrolytes, BUN/creatinine, glucose, INR/PTT, osmolality ABGs, measure O2 sat acetylsalicylic acid (ASA), acetaminophen, EtOH levels • potentially useful tests drug levels – this is NOT a serum drug screen Ca, Mg, PO4 protein, albumin, lactate, ketones, liver enzymes, CK – depending on drug and clinical presentation Serum Drug Levels • treat the patient, not the drug level • negative tox screen does not rule out a toxic ingestion – signifies only that the specific drugs tested were not detectable in the specimen • specific drugs available on general screen vary by institution; check before ordering • urine screens also available (qualitative only)
Populations at Risk for Thiamine Deficiency • Alcoholics • Anorexics • Hyperemesis of pregnancy • Malnutrition states
Administration of naloxone can cause opiate withdrawal in chronic users. Minor withdrawal may present as lacrimation, rhinorrhea, diaphoresis, yawning, piloerection, HTN, and tachycardia. Severe withdrawal may present as hot and cold flashes, arthralgias, myalgias, N/V, and abdominal cramps.
ER52 Emergency Medicine
Toxicology
Toronto Notes 2011
Table 23. Toxic Gaps (see also Nephrology)
Plasma Osmolar Gap (POG) = (2 Na + glucose + urea) – plasma osmolarity Normal POG 140 mg/L or May be only sign of acetaminophen poisoning 1000 µmol/L 4 hours after ingestion)
D3 – Decontamination and Enhanced Elimination Ocular Decontamination • saline irrigation to neutralize pH; alkali exposure requires ophthalmology consult Dermal Decontamination (wear protective gear) • remove clothing, brush off toxic agents, irrigate all external surfaces
Substances NOT Adsorbed by Activated Charcoal • Lithium • Iron • Alcohols • Lead • Caustics
Gastrointestinal Decontamination • single dose activated charcoal (SDAC) (see Table 27 for drug toxidromes that are treated with charcoal) adsorption of drug/toxin to AC prevents availability contraindications: caustics, SBO, perforation dose: 10g/g drug ingested or 1g/kg body weight odourless, tasteless, prepared as slurry with H2O • whole bowel irrigation 500 mL (child) to 2000 mL (adult) of polyethylene glycol solution/hour by mouth until clear effluent per rectum start slow (500 mL in an adult) and aim to increase rate hourly as tolerated indications awake, alert, can be nursed upright delayed release product drug/toxin not bound to charcoal drug packages (if any evidence of breakage g emergency surgery) recent toxin ingestion
Toronto Notes 2011
Toxicology
contraindications evidence of ileus, perforation, or obstruction • surgical removal in extreme cases indicated for drugs that are toxic, form concretions, or cannot be removed by conventional means • no evidence for the use of cathartics (or ipecac) EXTRA-CORPOREAL DRUG REMOVAL (ECDR) Urine Alkalinization • may be used for: ASA, methotrexate, phenobarbital, chlorpropamide • weakly acidic substances can be trapped in alkali urine (pH >7.5) to increase elimination Multidose Activated Charcoal (MDAC) • may be used for: carbamazepine, phenobarbital, quinine, theophylline • for toxins which undergo enterohepatic recirculation • removes drug that has already been absorbed by drawing it back into GI tract • various regimens: 12.5 g (1/4 bottle) PO q1h or 25 g (1/2 bottle) PO q2h until non-toxic Hemodialysis • indications/criteria for hemodialysis toxins that have high water solubility, low protein binding, low molecular weight, adequate concentration gradient, small volume of distribution (Vd) or rapid plasma equilibration removal of toxin will cause clinical improvement advantage is shown over other modes of therapy predicted that drug or metabolite will have toxic effects impairment of normal routes of elimination (cardiac, renal, or hepatic) clinical deterioration despite maximal medical support • useful for the following blood toxins: methanol ethylene glycol salicylates lithium phenobarbital: 430-650 mmol/L chloral hydrate (g trichloroethanol): >200 mg/kg • others include theophylline, carbamazepine, valproate, methotrexate
E – Examine the Patient • vital signs (including temperature), skin (needle tracks, colour), mucous membranes, pupils, odours and CNS • head-to-toe survey including C-spine signs of trauma, seizures (incontinence, “tongue biting”, etc.), infection (meningismus), chronic alcohol/drug abuse (track marks, nasal septum erosion) • mental status Table 25. Specific Toxidromes Toxidrome
Overdose Signs and Symptoms
Examples of Drugs
Anticholinergics
Hyperthermia Dilated pupils Dry skin Vasodilation Agitation/hallucinations Ileus Urinary retention Tachycardia
Antidepressants (e.g. TCAs) Cyclobenzaprine (Flexeril®) Carbamazepine Antihistamines (e.g. diphenhydramine) Antiparkinsonians Antipsychotics Antispasmotics Belladonna alkaloids (e.g. atropine)
Cholinergics
“DUMBELS” Diaphoresis, Diarrhea, Decreased blood pressure Urination Miosis Bronchospasm, Bronchorrhea, Bradycardia Emesis, Excitation of skeletal muscle Lacrimation Salivation, Seizures
Natural plants: mushrooms, trumpet flower Anticholinesterases: physostigmine, Insecticides (organophosphates, carbamates) Nerve gases
Extrapyramidal
Dysphonia, dysphagia Rigidity and tremor Motor restlessness, crawling sensation (akathisia) Constant movements (dyskinesia) Dystonia (muscle spasms, laryngospasm, trismus, oculogyric crisis, torticollis)
Major tranquilizers Antipsychotics
“Hot as a hare” “Blind as a bat” “Dry as a bone” “Red as a beet” “Mad as a hatter” “The bowel and bladder lose their tone and the heart goes on alone”
Emergency Medicine ER53
ER54 Emergency Medicine
Toxicology
Toronto Notes 2011
Table 25. Specific Toxidromes (continued) Toxidrome
Overdose Signs and Symptoms
Examples of Drugs
Hemoglobin Derangements
Increased respiratory rate Decreased level of consciousness Seizures Cyanosis unresponsive to O2 Lactic acidosis
Carbon monoxide poisoning (carboxyhemoglobin) Drug ingestion (methemoglobin, sulfmethemoglobin)
Narcotics, Sedatives/Hypnotics, EtOH
Hypothermia Hypotension Respiratory depression Dilated or constricted pupils (pinpoint in opiate OD) CNS depression
EtOH Benzodiazepines Opiates (morphine, heroin, etc.) Barbiturates GHB
Sympathomimetics
Increased temperature CNS excitation (including seizures) Tachycardia, hypertension Nausea and vomiting Diaphoresis Dilated pupils
Amphetamines, caffeine, cocaine, LSD, PCP Ephedrine and other decongestants Thyroid hormone Sedatives, EtOH withdrawal
Serotonin Syndrome
Mental status changes, autonomic hyperactivity, neuromuscular abnormalities, hyperthermia, diarrhea, HTN
MAOI, TCA, SSRI, opiate analgesics Cough medicine, weight reduction medications
Note: ASA poisoning and hypoglycemia mimic sympathomimetic toxidrome
G – Give Specific Antidotes and Treatments Urine Alkalinization Treatment for ASA Overdose • urine pH >7.5 • fluid resuscitate first, then 3 amps NaHCO3/litre of D5W @ 1.5 x maintenance • add 20-40 mEq KCl/litre if patient is able to urinate Table 26. Protocol for Warfarin Overdose INR
Management
20.0
FFP 10-15 mL/kg, Vitamin K 10 mg IV over 10 min, increase Vitamin K dosing (q4h) if needed
Table 27. Specific Antidotes and Treatments – call local poison information centre for specific doses and treatment recommendations Toxin
Treatment
Considerations
Acetaminophen
Decontaminate (activated charcoal) N-acetylcysteine
Often clinically silent; evidence of liver/renal damage delayed >24 hrs Toxic dose >200 mg/kg (>7.5 g adult) Monitor drug level immediately and 4 hrs post-ingestion; also liver enzymes, INR, PTT, BUN, Cr Hypoglycemia, metabolic acidosis, encephalopathy g poor prognosis
ASA
Decontaminate (activated charcoal) Alkalinize urine; want urine pH >7.5
Monitor serum pH and drug levels closely Monitor K level; may require supplement for urine alkalinization Hemodialysis may be needed if intractable metabolic acidosis, very high levels, or end-organ damage (i.e. unable to diurese)
Anticholinergics
Decontaminate (activated charcoal) Supportive care
Special antidotes available. Consult Poison Information Centre (PIC)
Benzodiazepines
Decontaminate (activated charcoal) Supportive care
β-blockers
Decontaminate (activated charcoal) Consider glucagon or high dose insulin euglycemia therapy (HDIE)
Consult PIC
Calcium Channel Blockers
CaCl2 1-4 g of 10% sol’n IV if hypotensive Atropine or isoproterenol if severe Other: HDIE inotropes or aggressive supportive therapy
Order ECG, electrolytes (especially Ca, Mg, Na, K), glucagon may help (2-5 mg)
Cyanide
Cyanide antidote kit or hydroxycobalamin
Toronto Notes 2011
Toxicology
Table 27. Specific Antidotes and Treatments – call local poison information centre for specific doses and treatment recommendations (continued) Toxin
Treatment
Considerations
Digoxin
Decontaminate (activated charcoal) Digoxin-specific Ab fragments 10-20 vials IV if acute; 3-6 if chronic 1 vial (40 mg) neutralizes 0.5 mg of toxin
Use for life-threatening arrhythmias unresponsive to conventional therapy, 6 hr serum digoxin >19 nmol/L, initial K >5 mM, ingestion >10 mg (adult) / >4 mg (child) Common arrhythmias include VFib, VTach, and conduction blocks
Acute Dystonic Rxn Benztropine: 1-2 mg IM/IV then 2 mg PO x 3 days OR Diphenhydramine 1-2 mg/kg IV, then 25 mg PO qid x 3 days
Benztropine (Cogentin®) has euphoric effect and potential for abuse
Heparin
Protamine sulfate 25-50 mg IV
For unfractionated heparin overdose only
Insulin/ Oral Hypoglycemic
Glucose IV/PO/NG tube Glucagon: 1-2 mg IM (if no access to glucose)
Glyburide carries highest risk of hypoglycemia among oral agents Consider octreotide for oral hypoglycemics (50-100 µg SC q6h) in these cases; consult local PIC
Ethanol
Thiamine 100 mg IM/IV Manage airway and circulatory support
Hypoglycemia very common in children Mouthwash = 70% EtOH; perfumes and colognes = 40-60% EtOH Order serum EtOH level and glucose level; treat glucose level appropriately
Ethylene Glycol/ Methanol
Ethanol (10%) 10 ml/kg over 30 min, then 1.5 ml/h or fomepizole (4-methylpyrazole) 15 mg/kg IV load over 30 min, then 10 mg/kg q12h
CBC, electrolytes, glucose, ethanol level Consider hemodialysis
CO Poisoning
See ER46
Opioids
See ER51
TCAs
Aggressive supportive care NaHC03 bolus for wide QRS/seizures
Flumazenil antidote contraindicated in combined TCA and benzodiazepine overdose Also consider cardiac and hypotension support, gastric decontamination, seizure control Intralipid therapy (consult local PIC)
MDMA
Decontaminate (activated charcoal), supportive care
Monitor CK; treat rhabdomyolysis with high flow fluids
Cocaine
Decontaminate (activated charcoal) if oral Aggressive supportive care
b-blockers are contraindicated in acute cocaine toxicity
Disposition from the Emergency Department • methanol, ethylene glycol delayed onset, admit and watch clinical and biochemical markers • TCAs prolonged/delayed cardiotoxicity warrants admission to monitored (ICU) bed if asymptomatic and no clinical signs of intoxication: 6 hour ED observation adequate with proper decontamination and no ECG abnormalities sinus tachycardia alone (most common finding) with history of OD warrants observation in ED • hydrocarbons/smoke inhalation pneumonitis may lag 6-8 hours consider observation for repeated clinical and radiographic examination • ASA, acetaminophen if borderline level, get second level 2-4 hours after first for ASA must have at least 2 levels going down before discharge (3 levels minimum) • oral hypoglycemics admit all patients for minimum 24 hours if hypoglycemic observe asymptomatic patient for at least 8 hours Psychiatric Consultation • once patient medically cleared, arrange psychiatric intervention if required • beware – suicidal ideation may not be expressed
Emergency Medicine ER55
ER56 Emergency Medicine
Psychiatric Emergencies
Toronto Notes 2011
Psychiatric Emergencies Approach to Common Psychiatric Presentations • see Psychiatry • before seeing patient, ensure your own safety; have security/police available if necessary
Key Functions of Emergency Psychiatric Assessment 1. Is the patient medically stable? 2. Rule out medical cause 3. Is psychiatric consult needed? 4. Are there safety issues (SI, HI)? 5. Is patient certifiable? (must demonstrate risk [present/past test] and apparent mental illness [future test])
History • safety assess suicidality: suicidal ideation, intent, plan, lethal means, past attempts assess homicidality: access to weapons, intended victim, history of violence command hallucinations • identify current stressors and coping strategies • mood symptoms: manic, depressive • anxiety: panic attacks, generalized anxiety, phobias, OCD, PTSD • psychotic symptoms: delusions, hallucinations, disorganized speech, disorganized or catatonic behaviour, negative symptoms (affective flattening, alogia, avolition) • substance use history: most recent use, amount, previous withdrawal reactions • past psychiatric history, medications, adherence with medications • medical history: obtain collateral if available Physical • complete physical exam focusing on: vitals, neurological exam, signs of head trauma, signs of drug toxicity, signs of metabolic disorder • mental status exam: general appearance, behavior, cooperation, speech, mood and affect, thought content and form, perceptual disturbances, cognition (including MMSE if indicated), judgment, insight, reliability Investigations • investigations vary with: age, established psychiatric diagnosis vs. first presentation, history and physical suggestive of organic cause • as indicated: blood glucose, urine and serum toxicology screen, pregnancy test, electrolytes, TSH, AST/ALT, bilirubin, serum creatinine, BUN, osmolality • blood levels of psychiatric medications • CT head if suspect neurological etiology • LP if indicated
Acute Psychosis Features that suggest Organic Etiology Age >40 years old Babbling (incoherent speech or speech difficulties) Concerning vital signs Disorientation Emotional lability Fluctuating course Global impairment of cognitive function Headaches Immodesty Just started (sudden onset) K Loss of consciousness Movement abnormalities (tremor, ataxia, psychomotor retardation) Neurological findings (focal) Other abnormalities on physical exam Perceptions (visual hallucinations)
Differential Diagnosis • primary psychotic disorder (e.g. schizophrenia) • secondary to medical condition (e.g. delirium) • drugs: substance intoxication or withdrawal, medications (e.g. steroids, anticholinergics) • infectious (CNS) • metabolic (hypoglycemic, hepatic, renal, thyroid) • structural (hemorrhage, neoplasm) Management • violence prevention remain calm, empathic and reassuring ensure safety of staff and patients, have extra staff and/or security on hand patients demonstrating escalating agitation or overt violent behavior may require physical restraint and/or chemical tranquilization (see Violent Patient, ER57) • treat agitation: whenever possible, offer medication to patients as opposed to administering with force (helps calm and engage patient) benzodiazepines – lorazepam 2 mg PO, IM or SL antipsychotics – olanzapine 5 mg PO, haloperidol 5 mg PO/IM • treat underlying medical condition • psychiatry or Crisis Intervention Team consult
Toronto Notes 2011
Psychiatric Emergencies/Common Pediatric ER Presentations
Emergency Medicine ER57
Suicidal Patient Epidemiology • attempted suicide F>M, completed suicide M>F • second leading cause of death in people 45 years old Depression Previous attempts Ethanol use Rational thinking loss Suicide in family Organized plan No spouse, no support system Serious illness
Differential Diagnosis • rule out lethal organic cause (e.g. EtOH, drugs, and head injuries) Prevention • be aware and look for prodromal signs of violence: anxiety, restlessness, defensiveness, verbal attacks • try to de-escalate the situation: address the patient’s anger, empathize Restraints • pharmacological often necessary – may mask clinical findings and impair exam haloperidol 5-10 mg IM (be prepared for dystonic reactions, especially with multiple doses of neuroleptics over a short period) + lorazepam 2 mg IM/IV look for signs of anticholinergic overdose first (see Table 25) benzodiazepines best option if suspected substance-induced violence • physical present option to patient in firm but non-hostile manner sufficient people to carry it out safely restrain supine or on side; preferably 4-point restraints, never less than 2-points (opposite arm and leg) suction and airway support available in case of vomiting • once restrained, search person/clothing for drugs and weapons
Common Pediatric ER Presentations Modified Coma Score Table 28. Modified GCS Modified GCS for Infants Eye Opening 4 – spontaneously 3 – to speech 2 – to pain 1 – no response
Verbal Response 5 – coos, babbles 4 – irritable cry 3 – cries to pain 2 – moans to pain 1 – no response
Motor Response 6 – normal, spontaneous movement 5 – withdraws to touch 4 – withdraws to pain 3 – decorticate flexion 2 – decerebrate extension 1 – no response
Modified GCS for Children 40 if preschool age, >30 if school age), retractions, tracheal tug see Pediatrics Table 1 for age specific vital signs • pulsus paradoxus • wheezing, grunting, vomiting Table 29. Stridorous Upper Airway Diseases: Diagnosis Feature
Croup
Bacterial Tracheitis
Epiglottitis1
Age Range (yrs)
0.5-4
5-10
2-8
Prodrome
Days
Hrs to days
Minutes to hrs
Temperature
Low grade
High
High
Radiography
Steeple sign
Exudates in trachea
Thumb sign
Etiology
Parainfluenza
S. aureus/GAS
H. flu type b
Barky Cough
Yes
Yes
No
Drooling
Yes
No
Yes
Appear Toxic
No
Yes
Yes
Intubation? ICU?
No
Yes
Yes
Antibiotics
No
Yes
Yes
NOTE:
No oral exam
1Now rare with Hib vaccine in common use
Admission Criteria for Croup • Stridor at rest or significant respiratory distress • Relapse after 2 doses of epinephrine or incomplete response • Co-morbid respiratory or underlying condition
Admission Criteria for Asthma • Respiratory distress 6 hrs after steroids • Ventolin required > q3h • Need for supplemental oxygen • Consider if previous ICU admission
• management of croup (laryngotracheitis caused by parainfluenza viruses) humidified O2 should not be given (no evidence for efficacy) racemic epinephrine q1h x 3 doses, observe for ‘rebound effects’ dexamethasone x 1 dose consider bacterial tracheitis/epiglottitis if unresponsive to croup therapy • management of bacterial tracheitis start croup therapy, but may have poor response usually require intubation, ENT consult, ICU start antibiotics (e.g. cloxacillin), pending C&S • management of epiglottitis 4 D’s: drooling, dyspnea, dysphagia, dysphonia + tripod sitting do not examine oropharynx or agitate patient immediate anaesthesia, ENT call – intubate then IV fluids, Abx, blood cultures • management of asthma supplemental O2 if sats 5 yrs), meningococcus
• cefuroxime • ceftriaxone or cefotaxime, if risk of meningitis • vancomycin, if penicillin/cephalosporin-resistant pneumococci
S. pneumoniae, H. influenzae type b, M. Catarrhalis
• amoxicillin 80-90 mg/kg per day
OTITIS MEDIA 1st line 2nd line
• amoxicillin-clavutanate (Clavulin®)
Treatment failure
• high dose Clavulin® or cefuroxime or ceftriaxone
STREP PHARYNGITIS Group A beta-hemolytic Streptococcus
• penicillin/amoxicillin or erythromycin (pencillin allergy)
UTI E. coli, Proteus, H. influenzae, Pseudomonas, S. saprophyticus Enterococcus, GBS
• amoxicillin/ampicillin or • trimethoprim-sulfamethoxazole
Viral, S. pneumoniae, C. trachomatis, B. pertussis, S. aureus, H. influenzae
• cefuroxime ± macrolide (erythromycin) or ampicillin ± macrolide
3 months5 years
Viral, S. pneumoniae, S. aureus, H. influenzae, Mycoplasma pneumoniae
• ampicillin/amoxicillin or cefuroxime
>5 years
As above
• ampicillin/amoxicillin + macrolide or cefuroxime + macrolide
PNEUMONIA 1-3 months
Child Abuse and Neglect
Presentation of Neglect • Failure to thrive, developmental delay • Inadequate or dirty clothing, poor hygiene • Child exhibits poor attachment to parents, no stranger anxiety
• see also Pediatrics • obligation to report any suspected/known case of child abuse or neglect to CAS yourself (do not delegate) • document injuries • consider skeletal survey x-rays, ophtho consult, CT head • injury patterns associated with child abuse head injuries: torn frenulum, dental injuries, bilateral black eyes, traumatic hair loss, diffuse severe CNS injury, retinal hemorrhage Shaken Baby Syndrome: diffuse brain injury, subdural/subarachnoid hemorrhage, retinal hemorrhage, minimal/no evidence of external trauma, associated bony fractures skin injuries: bites, bruises/burns in shape of an object, glove/stocking distribution of burns, bruises of various ages, bruises in protected areas bone injuries: rib fractures without major trauma, femur fractures age 27 kg/m2) family history of premature coronary artery disease clinical signs of hyperlipidemia (xanthelasma, xanthoma, arcus corneae) evidence of atherosclerosis rheumatoid arthritis, systemic lupus erythematosis, psoriasis HIV infection on highly active antiretroviral therapy chronic kidney disease (estimated GFR 65: Visual acuity (Snellen sight chart) (B) Hearing impairment (inquiry, whispered voice test, audioscope) (B) First degree relative with melanoma: Full body skin exam (B)
Toronto Notes 2011
Periodic Health Examination/Health Promotion and Counselling
Family Medicine FM3
Table 1. Periodic Health Exam (continued) TESTS
THERAPY
General Population
Special Population
• Multiphase screening with the Hemoccult test (adults age >50 q1-2yrs) (A) • Sigmoidoscopy (adults >50) (frequency not established) (B) • Bone mineral density: if at risk (1 major or 2 minor criteria) • Fasting lipid profile (C): • Women age >50 or post-menopausal; earlier if at risk • Men age >40; earlier if at risk (optimal frequency unknown, at least q5yrs) • Fasting blood glucose: age >40 q3yrs (or sooner and more frequently if risk factors present) • Syphilis screen if at risk (D) • Men: PSA testing screening guidelines not estabilshed (I) • Women: Mammography (women age 50-69) q1-2yrs (A) Pap smear annually (women age 18-69 if ever sexually active, start after sexual debut); q3yrs after 2 normal results (more frequently if concerns)
Pediatrics: Routine hemoglobin for high risk infants (B) Blood lead screening of high risk infants (B) Diabetics: Urine dipstick (A) Fundoscopy (B) TB high risk groups: Mantoux skin testing (A) STI high risk groups: Voluntary HIV antibody screening (A) Gonorrhea screening (A) Chlamydia screening in women (B) FAP: Sigmoidoscopy and genetic testing (B) HNPCC: Colonoscopy (B)
• Folic acid supplementation to women of child-bearing age (A) • Varicella vaccine for children age 1-12 and susceptible adolescents/adults (A) • Rubella vaccine for all non-pregnant women of child-bearing age (B) • Pharmacologic treatment of hypertension with dBP >90 mmHg (adults age 21-64, elderly specific subgroups) (A) • Tetanus vaccine: routine booster q10yrs if had 1o series (A) • Pertussis vaccine: routine booster of acellular vaccine once during adulthood (can be given as dTap)
Pediatrics: Routine immunizations (A) Hepatitis B immunization (A) Influenza high risk groups: Outreach strategies for vaccination (A) annual immunization (B), now recommended for all TB high risk groups: INH prophylaxis for household contacts or skin test converters (B) INH prophylaxis for high risk sub-groups (B) Immunocompetent/age ≥65/COPD: Pneumococcal vaccine (A)
Classification of recommendation in brackets. See sidebar on FM2. Reference: Canadian Task Force on Preventative Health Care, 2005.
Health Promotion and Counselling • health promotion is the most effective preventative strategy • 40-70% of productive life lost annually is preventable • there are several effective ways to promote healthy behavioural change, such as discussions appropriate to a patient’s present stage of change
Motivational Strategies for Behavioural Change Table 2. Motivational Strategies for Behavioural Change Patient’s Stage of Change Physician’s Aim Pre-contemplation
Contemplation Preparation
Action
Maintenance
Relapse
Encourage patient to consider the possibility of change Assess readiness for change Increase patient’s awareness of the problem and its risks Understand patient’s ambivalence and encourage change Build confidence and gain commitment to change Explore options and choose course most appropriate to patient Identify high-risk situations and develop strategies to prevent relapse Continue to strengthen confidence and commitment Help patients design rewards for success Develop strategies to prevent relapse Support and reinforce convictions towards long-term change Help patient maintain motivation Review identifying high-risk situations and strategies for preventing relapse Help patient view relapse as a learning experience Provide support appropriate to present level of readiness post-relapse
Adapted from Hunt P. Motivating Change. Nursing Standard 2001; 16(2):45-52, 54-55.
Physician’s Plan Raise issue in a sensitive manner Offer (not impose) a neutral exchange of information to avoid resistance Offer opportunity to discuss pros and cons of change, using reflective listening Offer realistic options for change and opportunity to discuss inevitable difficulties
Offer positive reinforcement and explore ways of coping with obstacles Encourage self-rewards to positively reinforce change Discuss progress and signs of impending relapse Offer a non-judgmental discussion about circumstances surrounding relapse and how to avoid relapse in the future Reassess patient’s readiness to change
When Ordering Fasting Bloodwork • Results are valid only if obtained with ≥12 hours of fasting. • Remember, “fasting” means no food, no drinks (except small quantities of water), no gum, no smoking. • Prescription medications are okay unless otherwise specified.
Guidelines Advisory Committee (GAC) Recommendations for Breast Cancer Screening For women aged 40-69 years, there is fair evidence to recommend that routine teaching of breast self-examination (BSE) be excluded from the PHE. Research shows fair evidence of no benefit to BSE and good evidence of harm.
FM4 Family Medicine
Health Promotion and Counselling
Toronto Notes 2011
Nutrition
Vegetables 50%
Meat & Alternatives 25%
Grain Products 25%
Figure 1. Plate Layout
General Population • Canada’s Food Guide is appropriate for individuals >2 years old • counsel on variety, portion size, and plate layout (see Figure 1) Table 3. Canada’s Food Guide 2007 Recommendations for Adults Food Group
Servings/day
Choose More Often
Grain products Vegetables and fruit Milk products
6-8 7-10 2-3 Children 2-8 years: 2 Youth 9-18 years: 3-4 Pregnant/breastfeeding: 3-4 2-3
Whole grain and enriched grain products Dark green vegetables, orange vegetables and fruit Lower-fat dairy products
Meat and alternatives
Lean meat, poultry, fish, peas, beans, lentils
Cardiovascular Disease Prevention Handy Serving Size Comparisons • 3 oz meat, fish, poultry g palm of hand • 1 cup dairy (milk/yogurt) g size of fist • Bread/grains g one slice, palm of hand • ½ cup rice/pasta g one hand cupped • 1 cup of fruit/vegetables g two cupped hands • 1 oz cheese g full length of thumb • 1 tsp oil/butter g tip of thumb • Nuts/chips/snacks g palm covered
Table 4. Dietary Guidelines for Reducing Risk of Cardiovascular Disease in General Population Food Item
Recommendations
Effects
Fat
Fat intake 65 reported experiences of emotional or financial abuse • older adults who live with someone are more likely to be abused than those who live alone • 2/3 of reported abuse cases involved family members, most often adult children followed by spouses • older females are more likely to be abused than older males • men are more likely than women to be victimized by an adult child (45% vs. 35%) • women are more likely than men to experience violence at the hands of a spouse (30% vs. 19%) (Statistics Canada, 2004) • reasons for under-reporting: fear, shame, cognitive impairment, language/cultural barriers, and social and geographic isolation Screening • insufficient evidence to include or exclude as part of the periodic health examination, but recommended that physicians be alert for indicators of abuse and institute measures to prevent further abuse • general questions such as “Do you feel safe at home?” and move into more specific questions about different kinds of abuse Presentation • signs that an older adult is being abused may include: depression, fear, anxiety, passivity, unexplained injuries, dehydration, malnutrition, poor hygiene, rashes, pressure sores, and over-sedation/inappropriate medication use Management • gather information from all sources (e.g. family members, health care providers, neighbours) • perform a thorough physical examination • ensure immediate safety and devise a plan for follow-up • additional steps depend on whether the patient accepts intervention and whether they are capable of making decisions about their care • interventions may include use of protective and legal services, senior resource nurses, elder abuse intervention teams and senior support groups
Risk Factors • Female • Older age (age 80 and older) • Physical and mental frailty
FM28 Family Medicine
Common Presenting Problems
Toronto Notes 2011
Dyspepsia • see Gastroenterology, G6 Definition and Clinical Features • defined as epigastric pain or discomfort • can be associated with fullness, belching, bloating, heartburn, food intolerance, nausea or vomiting Epidemiology • annual incidence 1-2%, prevalence 20-40% Etiology • common: functional, peptic ulcer disease, gastroesophageal reflux disease, gastritis • others: cholelithiasis, irritable bowel disease, esophageal or gastric cancer, pancreatitis, pancreatic cancer, Zollinger-Ellison syndrome, and abdominal angina History • symptoms may not be useful in finding cause • association with food, anorexia, nausea, vomiting, NSAID use • symptoms suggestive of underlying pathology: weight loss, dysphagia, persistent vomiting, gastrointestinal bleeding (hematemesis, hematochezia) Investigations and Management • empiric therapy: H2 receptor blockers, proton pump inhibitors • testing for H. pylori: serology, urea breath test • upper endoscopy (preferred), upper GI series
Dyspnea • see Respirology, R2 and Emergency Medicine, ER27 DDX of Dyspnea • Pulmonary embolism • Deconditioning • Foreign body aspiration • DKA • Anemia • Asthma • Pneumothorax
History and Physical • cough, sputum, hemoptysis, wheezing, chest pain, palpitations, dizziness, edema • asthma, allergy, eczema, ASA/NSAID sensitivity, nasal polyps • constitutional symptoms • smoking, recreational drugs, medications • occupational exposure, environmental exposure (e.g. pets, allergens, smoke) • travel and birth place • FHx of atopy • previous CXR or PFTs • exam: vitals, respiratory, precordial, HEENT, signs of anemia/liver failure/heart failure Investigations • CXR, ECG • PFTs, ABG acutely if indicated Management • ABC’s: send to Emergency Department if in severe respiratory distress • depends on cause
Dysuria • see Urology, U4
Risk Factors for Complicated Urinary Tract Infection • Male sex • Pregnancy • Recent urinary tract instrumentation • Functional or anatomic abnormality of the urinary tract • Chronic renal disease • Diabetes • Immunosuppression • Indwelling catheter
Definition • the sensation of pain, burning or discomfort on urination Epidemiology • in adulthood, more common in women than men • approximately 25% of women report one episode of acute dysuria per year • most common in women 25-54 years of age and in those who are sexually active • in men, dysuria becomes more prevalent with increasing age Etiology • infectious most common cause presents as cystitis, urethritis, pyelonephritis, vaginitis or prostatitis • non-infectious hormonal conditions (postmenopausal hypoestrogenism), obstruction (BPH, urethral strictures), neoplasms, allergic reactions, chemicals, foreign bodies, trauma
Toronto Notes 2011
Common Presenting Problems
Table 20. Etiology, Signs and Symptoms of Dysuria Infection
Etiology
Signs and Symptoms
UTI/Cystitis
E. coli, S. saprophyticus, Proteus mirabilis, Enterobacter, Klebsiella, Pseudomonas
Internal dysuria throughout micturition, frequency, urgency, incontinence, hematuria, nocturia, back pain, suprapubic discomfort, low grade fever (rare)
Urethritis
C. trachomatis, N. gonorrhea, Trichomonas, Candida, herpes
Initial dysuria, urethral/vaginal discharge, history of STI
Vaginitis
Candida, Gardnerella, Trichomonas, C. trachomatis, atrophic, herpes, lichen sclerosis
External dysuria/pain, vaginal discharge, irritation, dyspareunia, abnormal vaginal bleeding
Prostatitis
E. coli, C. trachomatis, S. saprophyticus, Proteus mirabilis, Enterobacter, Klebsiella, Pseudomonas
Dysuria, fever, chills, urgency, frequency, tender prostate
Pyelonephritis
E. coli, S. saprophyticus, Proteus mirabilis, Enterobacter, Klebsiella, Pseudomonas
Internal dysuria, fever, chills, flank pain radiating to groin, CVA tenderness, nausea or vomiting
Investigations • no investigations necessary when history and physical consistent with uncomplicated UTI – treat empirically (urinalysis can be performed when indicated by dipstick or microscopy) • radiologic studies and other diagnostic tests if atypical presentation • urinalysis/urine R&M: pyuria, bacteriuria, hematuria • urine C&S • if vaginal/urethral discharge present: wet mount, Gram stain, KOH test, vaginal pH, culture for yeast and Trichomonas • endocervical or urethral swab for N. gonorrheae and C. trachomatis • renal U/S ± voiding cystourethrogram (VCUG) in children with recurrent UTI Management • UTI/cystitis pregnant women with bacteriuria (2-7%) must be treated even if asymptomatic, due to risk of preterm labour; need to follow with monthly urine cultures and retreat if still infected patients with recurrent UTIs (>3 per year), should be considered for prophylactic antibiotics if complicated UTI, patients require longer courses of broader spectrum antibiotics • urethritis when swab is positive for chlamydia or gonorrhea must report to Public Health all patients should return 4-7 days after completion of therapy for clinical evaluation
Epistaxis • see Otolaryngology, OT27 Table 21. Characteristics of Anterior vs. Posterior Bleeds Anterior (90%) Location/ Origin Little’s Area/Kiesselbach’s Plexus Age
2-10, 50-80
Common Cause Trauma (digital, fracture, foreign body), dry air, cool climate, post URTI, nasal dryness, chemical (nasal sprays, cocaine), tumour
Posterior (10%) Woodruff’s Plexus/Sphenopalatine Artery Usually >50 Systemic: hepatic disease, primary/secondary bleeding disorder, medications (ASA, NSAIDs, warfarin), HTN, atherosclerosis
Treatment
Conservative: Emergency: ENT/ER consult for posterior packing • Position: upright leaning forward with direct digital with an intranasal balloon/Foley catheter pressure over soft part of nostril for >10 min Embolization/surgery (“pinch” up to cartilage) • Humidifier in bedroom, nasal saline sprays, bacitriacin or Vaseline® application to Little’s area • Silver nitrate • Gelfoam/Hemostat • Nasal packing with Vaseline® gauze, nasal catheter or sponge • Cotton soaked in vasoconstrictor (oxymetazoline 0.5%) and topical anesthetic (4% lidocaine) placed in anterior nasal cavity with direct pressure for >10 min • Investigations: CBC, Hct, cross & type, INR, PTT (only if severe), CT/nasopharyngoscopy if suspected tumour
Prognosis
Usually stops with >10 min of pressure to nose
Copious bleed, often swallowed and vomited May lead to hypovolemic shock if not treated promptly
Family Medicine FM29 Does this Woman have an Acute Uncomplicated Urinary Tract Infection? JAMA 2002; 287:2701-2710 Purpose: To review the accuracy and precision of history taking and physical examination for diagnosing UTI in women. Study Characteristics: Systematic review of 9 studies looking at the accuracy or precision of history or physical examination in diagnosing uncomplicated UTI. Participants: Healthy women. Infants, children or adolescents, pregnant women, nursing home patients, and patients with complicated UTI were excluded. Main Outcomes: Precision and accuracy of history taking and physical exam. Results: No studies examined precision as an outcome. Four symptoms and one sign significantly increased the probability of UTI: dysuria, frequency, hematuria, back pain, and CVA tenderness. Four symptoms and one sign significantly decreased the probability of UTI: absence of dysuria, absence of back pain, a history of vaginal discharge, a history of vaginal irritation, and vaginal discharge on examination. Conclusions: Women who present with 1 or more symptoms of UTI have a probability of infection approaching 50%, effectively ruling in infection. Additional historical elements, physical examination, and urinalysis is unable to lower the post-test probability of UTI to a level where it can be ruled out. Additional testing, such as culture, should be pursued.
Prevention of UTIs • Maintain good hydration (especially with cranberry juice) • Wipe urethra from front to back to avoid contamination of the urethra with feces from the rectum • Avoid feminine hygiene sprays and scented douches • Empty bladder immediately before and after intercourse
FM30 Family Medicine
Common Presenting Problems
Toronto Notes 2011
Erectile Dysfunction (ED) • see Urology, U30 Definition • consistent or recurrent inability to attain and/or maintain penile erection sufficient for sexual performance of ≥3 months duration Epidemiology • ~20% of men aged 40; ~50% of men aged 70 Etiology • organic: vascular (90%) (arterial insufficiency, atherosclerosis), endocrine (low testosterone, diabetes), anatomic (structural abnormality e.g. Peyronie’s), neurologic (post-op, DM), medications (clonidine, antihypertensives, psychotropics) • psychogenic (10%) Table 22. Differentiation Between Organic and Psychogenic Erectile Dysfunction Characteristic
Organic
Psychogenic
Onset
Gradual
Acute
Circumstances
Global
Situational
Course
Constant
Varying
Non-coital erection
Poor
Rigid
Psychosexual problem
Secondary
Long history
Partner problem
Secondary
At onset
Anxiety and fear
Secondary
Primary
Walsh: Campbell’s Urology, 8th ed. Table 46-4.
Modifiable Risk Factors and Erectile Dysfunction: Can Lifestyle Changes Modify Risk? Urology 2000; 56:302-306 Study: A prospective cohort study designed to examine whether changes in smoking, heavy alcohol consumption, sedentary lifestyle, and obesity are associated with the risk of ED in men aged 40-70. Results: Obesity was associated with ED (P=0.006), with baseline obesity conferring higher risk regardless of subsequent weight loss. Level of physical activity was associated with ED (P=0.01): those initiating physical activity or remaining active had a lower risk of ED, while those who remained sedentary had a higher risk. As compared to their sedentary peers, those who initiated exercise in midlife had a 70% reduced ED rate. Changes in smoking or alcohol intake were not associated with ED (P>0.3). Conclusion: Although making lifestyle changes in midlife may be too late to reverse the effects of smoking, obesity, and alcohol consumption on ED, initiating physical activity in midlife may in fact reduce ED relative to peers who remain sedentary. Adopting a healthy lifestyle early in life may be the best approach to reducing the risk of developing ED in later years.
History • comprehensive sexual, medical and psychosocial history • time course last satisfactory erection gradual or sudden onset attempts at sexual activity • quantify presence of morning or night time erections stiffness (scale of 1-10) ability to initiate and maintain an erection with sexual stimulation erection stiffness during sex (scale of 1-10) • qualify partner or situation specific loss of erection before penetration or climax degree of concentration required to maintain an erection percentage of sexual attempts satisfactory to patient and/or his partner significant bends in penis or pain with erection difficulty with specific positions impact on quality of life and relationship Investigations • hypothalamic-pituitary-gonadal axis evaluation: testosterone (free + total), prolactin, LH • risk factor evaluation: fasting glucose, HbA1c, lipid profile • others: TSH, CBC, urinalysis • specialized testing psychological and/or psychiatric consultation in-depth psychosexual and relationship evaluation nocturnal penile tumescence and rigidity (NPTR) assessment vascular diagnostics (e.g. doppler studies, angiography) Management Table 23. Management of Erectile Dysfunction Nonpharmacologic
Pharmacologic
Surgical
Lifestyle changes (alcohol, smoking, exercise)
Oral agents
Implants
Relationship/sexual counselling
Suppository (MUSE: male urethral suppository for erection)
Vascular repair
Vacuum devices
Injections
Realignment
Toronto Notes 2011
Common Presenting Problems
Family Medicine FM31
• pharmacologic treatment phosphodiesterase type 5 inhibitors (see Table 24) alpha adrenergic blockers (e.g. yohimbine) serotonin antagonist and reuptake inhibitor (e.g. trazodone) testosterone – currently only indicated in patients presenting with hypogonadism and testosterone deficiency (note: breast/prostate cancer are absolute contraindications) Table 24. Phosphodiesterase Type 5 Inhibitors Examples
Dosing (1 dose/day)
Specifics
sildenafil (Viagra®)
Side Effects
Contraindications
25-100 mg/dose
Take 0.5-4 hr prior to intercourse Flushing, headache, Not to be used in patients May last 24 hours indigestion taking nitrates
tadalafil (Cialis®)
5-20 mg/dose
Effects may last 36 hours
As above
As above
vardenafil (Levitra®)
2.5-20 mg/dose
Take 1 hr prior to intercourse
As above
As above
Fatigue Epidemiology • 25% of office visits to family physicians peaks in ages 20-40 women 3-4x > men • 50% have associated psychological complaints/problems, especially if 24 hours • exclusion criteria: medical conditions that may explain the fatigue, certain psychiatric disorders (depression with psychotic or melancholic features, schizophrenia, eating disorders), substance abuse, severe obesity (BMI >45) Exercise Therapy for Chronic Fatigue Cochrane Depression, Anxiety, and Neurosis Group. Cochrane Database of Systematic Reviews 2004; Issue 3 Purpose: To determine the effectiveness of exercise therapy for Chronic Fatigue Syndrome (CFS). Methods: Systematic review of 5 RCTs with 336 patients of all ages with a clinical diagnosis of CFS. Interventions: Exercise therapy alone was compared with treatment as usual (or relaxation and flexibility), pharmacotherapy (fluoxetine), or exercise therapy combined with either pharmacotherapy or patient education. Results: At 12 weeks, patients undergoing exercise therapy were less fatigued than controls (SMD -0.77; 95% CI, -1.26 to -0.28). Physical functioning was also significantly improved, but there were more dropouts with exercise therapy. Compared with fluoxetine, patients receiving exercise therapy were less fatigued (WMD -1.24; 95% CI, -5.31 to 2.83). Patients receiving combination therapy with exercise therapy and either fluoxetine or patient education, did better than those on monotherapy. Conclusions: Patients may benefit from exercise therapy. Combination therapy with either fluoxetine or education may offer additional benefit. Further high quality trials are needed.
Epidemiology • F>>M, Caucasians > other groups, majority in their 30s • CFS found in 37.2°C (AM), 37.7°C (PM) History • fever peak temperature, thermometer, route time of day response to antipyretics • systemic symptoms weight loss, fatigue, rash, arthralgia
Toronto Notes 2011
Common Presenting Problems
• symptoms of possible source UTI/pyelonephritis: dysuria, foul-smelling urine, incontinence, frequency, hematuria, flank pain pneumonia: cough, pleuritic chest pain URTI: cough, coryza, ear pain meningitis: headache, confusion, stiff neck, rash osteomyelitis: bone pain skin: purulent discharge PID: discharge, dyspareunia gastroenteritis: abdominal pain, diarrhea, blood per rectum, vomit medications DVT: swollen legs, pain in calf, shortness of breath, pleuritic chest pain history of cancer/family history of cancer • infectious contacts travel history, camping, daycare, contact with TB, foodborne, animals Investigations • CBC & differential, blood culture, urine culture, urinalysis • stool O&P, Gram stain, culture • CXR, TB skin test, sputum culture • LP Management • general: sponge bath, light clothing • acetaminophen/ibuprofen as needed • treat underlying cause
Joint Pain • see Rheumatology, RH3 Differential Diagnosis Table 27. Differential Diagnosis of Joint Pain Non-Articular
Articular
Localized
Generalized
Inflammatory
Degenerative
Bursitis Tendonitis Capsulitis
Fibromyalgia Polymyalgia rheumatica
Seropositive • Rheumatoid arthritis • Systemic lupus erythematosus • Scleroderma • Polymyositis • Sjogren’s syndrome
Primary • Familial Heberden’s node • Inflammatory osteoarthritis • Regional hip or knee
Seronegative • Ankylosing spondylitis • Inflammatory bowel disease • Psoriatic arthritis • Reactive arthritis
Secondary • Metabolic • Hemophiliac • Neuropathic • Traumatic
Crystal • Gout • Pseudogout • Milwaukee shoulder, calcific periarthritis Infectious • Gonococcal • Non-gonococcal
History • number of joints involved – monoarticular, oligoarticular, polyarticular • pattern of joints involved – symmetrical vs. asymmetrical, large vs. small joints, axial skeleton • relation to activity (inflammatory better with activity, degenerative worse) • relation to rest (inflammatory worse with rest, degenerative better) • morning stiffness >30 minutes (inflammatory) • soft tissue swelling, erythema (inflammatory) • onset – acute vs. chronic (>6 weeks) • trauma, infection, medications (steroids, diuretics) • FHx of arthritis • co-morbidities: diabetes mellitus (carpal tunnel syndrome), renal insufficiency (gout), psoriasis (psoriatic arthritis), myeloma (low back pain), osteoporosis (fracture), obesity (OA) • constitutional symptoms (neoplasm)
Family Medicine FM33
FM34 Family Medicine
Common Presenting Problems
Toronto Notes 2011
• systemic features fever (SLE, infection) rash (SLE, psoriatic arthritis) nail abnormalities (psoriatic, reactive arthritis) myalgias (fibromyagia, myopathy) weakness (polymyositis, neuropathy) GI symptoms (scleroderma, IBD) GU symptoms (reactive arthritis, gonoccocemia) Physical Exam • vitals • specific joint exams • systemic features (skin, nails, eyes, hands) Investigations • CBC + differential, ESR, CRP, RF, ANA, HLA-B27, serum uric acid, calcium • urinalysis • tissue cultures • x-ray • joint aspirate for cell count + differential, culture, Gram stain, microscopy
Headache • see Neurology, N39 Primary Headaches Table 28. Primary Headaches Migraine Epidemiology 12% of adults F>M 20% with aura 80% without aura 5-72 hrs Duration
Pain Headache Red Flags Sudden onset of severe headache Worst headache ever New headache after age 50 Headache present on awakening Impaired mental status Fever Neck stiffness Seizures Focal neurologic deficits Jaw claudication Scalp tenderness Worse with exercise, sexual activity or valsalva
Classically unilateral and pulsatile, but 40% are bilateral, moderate-severe intensity, nausea/vomiting, photo/phonophobia
Numerous (e.g. food, sleep disturbance, stress, hormonal, fatigue, weather, high altitude) Aggravated by physical activity Treatment of 1st line: acetaminophen, ASA, ± caffeine Acute 2nd line: NSAIDs Headache 3rd line: 5HT agonists ± antiemetic Prophylactic 1st line: beta-blockers 2nd line: TCAs Therapy 3rd line: anticonvulsants Triggers
Tension-type
Cluster
Caffeine Withdrawal
38% of adults, can be episodic or chronic
>F
~50% of people drinking >2.5 cups/d
May occur as isolated incident or daily, duration is variable Mild to moderate pain, bilateral, fronto-occipital or generalized pain, band-like pain, ± contracted neck/ scalp muscles, associated with little disability
120 with minimal or no target-organ damage • hypertensive emergency high BP + acute target-organ damage Etiology • essential (primary) hypertension (>90%) undetermined cause • secondary hypertension (10%) • watch for labile, “white coat” hypertension (office-induced elevated BP) Predisposing Factors • family history • obesity (especially abdominal) • alcohol consumption • stress • sedentary lifestyle • smoking • male gender • age >30 • excessive salt intake/fatty diet • African American ancestry • dyslipidemia Table 29. Causes of Secondary Hypertension
Causes of Secondary Hypertension ABCDE Apnea, Aldosteronism Bruits, Bad kidneys Coarctation, Cushing’s, Catecholamines, Calcemia Drugs Endocrine disease
Keys to Grade of Recommendations for Hypertension Diagnosis and Treatment Grade A
High levels of internal validity and statistical precision
B/C
Lower levels of internal validity and statistical precision
D
Expert opinion
Obstructive Sleep Apnea
Common cause
Renal
Renovascular HTN Renal parenchymal disease, glomerulonephritis, pyelonephritis, polycystic kidney
Endocrine
1º hyperaldosteronism Pheochromocytoma Cushing’s syndrome Hyperthyroidism/hyperparathyroidism Hypercalcemia of any cause
Vascular
Coarctation of the aorta Renal artery stenosis
Drug-induced
Estrogens MAOIs Cocaine
Steroids Lithium Amphetamines
NSAIDs Decongestants Alcohol
Investigations • for all patients with hypertension (D) CBC, electrolytes, Cr, fasting glucose and lipid profile, 12-lead ECG, urinalysis • for specific patient subgroups (D) DM or renal disease: urinary protein excretion increasing Cr OR history of renal disease or proteinuria OR HTN resistant to 3 meds OR presence of abdominal bruit: renal ultrasound, captopril renal scan, MRA/CTA (B) if suspected endocrine cause: plasma aldosterone, plasma renin (D) if suspected pheochromocytoma: 24h urine for metanephrines and creatinine (C) echocardiogram for left ventricular dysfunction assessment if indicated (C)
Toronto Notes 2011
Common Presenting Problems
Diagnosis HYPERTENSION VISIT 1 Yes
Hypertensive Urgency/Emergency
Diagnosis of HTN
No
sBP ≥140 and/or dBP ≥90 + 2 more readings during same visit + Search for target organ damage Review Medical Record and
Global Cardiovascular Risk • Age • Male gender • Postmenopausal • Smoking • High cholesterol • DM/Impaired glucose tolerance • Family history (age 8 units), rate of bleeding and hemodynamic stability intractable despite medical management (endoscopy) • procedures:
oversewing of ulcer, pyloroplasty vagotomy rarely done now due to H. pylori eradication
Complications of Surgery • retained antrum • fistula (gastrocolic/gastrojejunal) • dumping syndrome, postvagotomy diarrhea, afferent loop syndrome (see Complications of Gastric Surgery, GS19)
Gastric Carcinoma Epidemiology • male:female = 3:2 • incidence for adenocarcinoma = 10 per 100,000, incidence highest in Asia (Japan 80 times higher than in U.S.) • most common age group = 50-59 years • incidence has decreased by 2/3 in past 50 years
Clinical Features • clinical suspicion: ulcer fails to heal lesion on greater curvature of stomach or cardia • asymptomatic, insidious or late onset of symptoms: postprandial abdominal fullness, vague epigastric pain anorexia, weight loss burping, nausea, vomiting, dyspepsia, dysphagia hepatomegaly, epigastric mass (25%) hematemesis, fecal occult blood, melena, iron-deficiency anemia • signs of metastatic disease: Virchow’s node – left supraclavicular node Blumer’s shelf – mass in pouch of Douglas Krukenberg tumour – metastases to ovary Sister Mary Joseph node – umbilical metastases Irish’s node – left axillary nodes • metastasis: liver, lung, brain Investigations • OGD and biopsy • chest/abdo/pelvis CT • CT for metastatic work-up (see Table 4)
(I=1 limb) – distal gastrectomy + gastrojejunostomy
Billroth II
(II=2 limbs) – distal gastrectomy + gastrojejunostomy
Ligament of Treitz
Roux-en-Y
Ligament of Treitz
Figure 8. Billroth I and Billroth II with Roux-en-Y Reconstruction (gastrojejunostomy)
© Sonya Amin 2003
Risk Factors • H. pylori, causing chronic atrophic gastritis • hereditary nonpolyposis colorectal cancer (HNPCC) • smoking, alcohol, smoked food, nitrosamines • pernicious anemia associated with achlorhydria and chronic atrophic gastritis • gastric adenomatous polyps • previous partial gastrectomy (>10 years post-gastrectomy) • hypertrophic gastropathy • blood type A
Billroth I
GS18 General Surgery
Stomach and Duodenum
Toronto Notes 2011
Table 4. Staging of Gastric Carcinoma Stage
Criteria
Prognosis (5-year survival)
I
Mucosa and submucosa
70%
II
Extension to muscularis propria
30%
III
Extension to regional nodes
10%
IV
Distant metastases or involvement of continuous structures
0%
Treatment • adenocarcinoma: proximal lesions: total gastrectomy and esophagojejunostomy – Roux-en-Y (see Figure 8) distal lesions: distal gastrectomy: wide margins, en bloc removal of omentum and lymph nodes palliation: gastric resection to decrease bleeding and relieve obstruction, enables the patient to eat radiation therapy studies are showing larger role for chemotherapy • lymphoma: chemotherapy ± radiation, surgery in limited cases (perforation, bleeding, obsruction)
Gastric Sarcoma Gastrointestinal Stromal Tumour (GIST) • most common mesenchymal neoplasm of GI tract • derived from interstitial cells of Cajal (cells associated with Auerbach’s plexus that have autonomous pacemaker function co-ordinate peristalsis throughout the GI tract) • 75-80% associated with tyrosine kinase (c-KIT) mutations • most common in stomach (50%), and proximal small intestine (25%), but can occur anywhere along GI tract • typically present with vague abdominal mass, feeling of abdominal fullness, or with secondary symptoms of bleeding and anemia • often discovered incidentally on CT, laparotomy or endoscopy Risk Factors • Carney’s Triad: GISTs, paraganglioma, and pulmonary chondroma • Type IA neurofibromatosis
Bariatric (Weight Loss) Surgery for Obesity is considered when Other Treatments have Failed Benefits • Greater weight loss in patients with BMI >30 at 2 years • Reduction in co-morbidities (Type II diabetes, hypertension and medication use) • Improvement in quality of life at 2 years (physical function, physical role, general health, vitality and emotional role) Risks • Complications: leaks, hernias, infection, pulmonary embolism, postoperative mortality • Side effects specific to type of procedure (i.e. vomiting, dumping syndrome, food intolerance) • Cholecystitis occurs as a result of rapid weight loss Colquitt JL, Picot J, Lovemen E, Clegg Aj. Surgery for Obesity (Cochrane Review). In: The Cochrane Library, Issue 2, 2009. Chichester, UK: John Wiley & Sons, Ltd.
Management • surgical resection if >2 cm; follow with serial endoscopy if 10 cm) mitotic activity (worse if >5 mitotic figures or 50/hpf) degree of nuclear pleomorphism location: with identical sizes, extra-gastric location has a higher risk of progression than GISTs in the stomach mets to liver, omentum, peritoneum; nodal mets rare
Bariatric Surgery • weight reduction surgery for morbid obesity • indications: BMI >40 or BMI >35 with related comorbidity (e.g. DM, CAD) • requires multidisciplinary evaluation and follow-up
Toronto Notes 2011
Stomach and Duodenum
Surgical Options • malabsorptive/restrictive: laparoscopic Roux-en-Y gastric bypass (most common) staple off small gastric pouch (restrictive) with Roux-en-Y limb to pouch (malabsorptive) with dumping syndrome physiology most effective, higher complication rates • restrictive: laparoscopic adjustable gastric banding silicone band around fundus creates pouch, adjustable through port under skin laparoscopic vertical banded gastroplasty vertical stapled small gastric pouch with placement of silastic ring band • malabsorptive: biliopancreatic diversion with duodenal switch gastrectomy, enteroenterostomy, duodenal division closure and duodenoenterostomy Complications • perioperative mortality ~1% (anastomotic leak with peritoneal signs, PE) • obstruction at enteroenterostomy (see Complications of Gastric Surgery, below) • staple line dehiscence • dumping syndrome • cholethiasis due to rapid weight loss (20-30%) • band abscess (if long-term)
Complications of Gastric Surgery
General Surgery GS19
Liver Stomach
Gallbladder Pancreas
Bile juice
A. Alkaline Reflux Gastritis Liver Stomach
Gallbladder Pancreas
Obstruction
B. Afferent Loop Syndrome
• most resolve within 1 year (see Figure 9) Alkaline Reflux Gastritis (see Figure 9A) • duodenal contents (bilious) reflux into stomach causing gastritis ± esophagitis • treatment: medical: H2-blocker, metoclopramide, cholestyramine (bile acid sequestrant) surgical: conversion of Billroth I or II to Roux-en-Y Afferent Loop Syndrome (see Figure 9B) • accumulation of bile and pancreatic secretions causes intermittent mechanical obstruction and distention of afferent limb • clinical features: early postprandial distention, RUQ pain, nausea, bilious vomiting, anemia • treatment: surgery (conversion to Roux-en-Y increases afferent loop drainage) Dumping Syndrome (see Figure 9C) • early – 15 minutes post-prandial: etiology: hyperosmotic chyme released into small bowel (fluid accumulation and jejunal distention) clinical features: post-prandial symptoms epigastric fullness or pain, emesis, nausea, diarrhea, palpitations, dizziness, tachycardia, diaphoresis treatment: small multiple low carbohydrate, low fat and high protein meals and avoidance of liquids with meals last resort is interposition of antiperistaltic jejunal loop between stomach and small bowel to delay gastric emptying • late – 3 hours post-prandial: etiology: large glucose load leads to large insulin release and hypoglycemia treatment: small snack 2 hours after meals
Liver Stomach Food Flow
Gallbladder Pancreas Small Intestine
C. Dumping Syndrome Liver Food Flow Stomach
Gallbladder Pancreas Chyme Bacteria
D. Blind Loop Syndrome Liver Cut CN X Stomach
Blind-Loop Syndrome (see Figure 9D) • bacterial overgrowth of colonic Gram-negative bacteria in afferent limb • clinical features: anemia/weakness, diarrhea, malnutrition, abdominal pain and hypocalcemia • treatment: broad-spectrum antibiotics, surgery (conversion to Billroth I) Postvagotomy Diarrhea (see Figure 9E) • up to 25% • bile salts in colon inhibit water resorption • treatment: medical (cholestyramine), surgical (reversed interposition jejunal segment)
H 2O
Gallblader Pancreas
Colon
E. Postvagotomy Diarrhea © Wensi Sheng 2010
Figure 9. Complications of Gastric Surgery
GS20 General Surgery
Small Intestine
Toronto Notes 2011
Small Intestine Meckel’s Diverticulum • remnant of the embryonic vitelline duct on antimesenteric border of ileum • heterotopic – several types of mucosa including gastric, pancreatic, colonic • most common true diverticulum of GI tract
Rule of 2s for Meckel’s Diverticulum • 2% of the population • Symptomatic in 2% of cases • Found within 2 feet (10-90 cm) of the ileocecal (IC) valve • 2 inches in length • Often present by 2 years of age
Clinical Features • 2% symptomatic • GI bleed, small bowel obstruction (SBO), diverticulitis (mimics appendicitis) • painless bleeding – ulceration caused by ectopic gastric mucosa 50% of patients with this presentation are indirect inguinal > direct inguinal intense pain followed by tenderness intestinal obstruction, gangrenous bowel, sepsis surgical emergency DO NOT attempt to manually reduce hernia if septic or if contents of hernial sac gangrenous Treatment • surgical treatment (herniorrhaphy) is only to prevent strangulation and evisceration or for cosmesis or symptoms; if asymptomatic can delay surgery • repair may be done open or laparascopic and may use mesh for tension-free closure • most repairs are now done with a plug in the hernial defect and a patch over it or patch alone • observation is acceptable for small asymptomatic inguinal hernias Postoperative Complications • recurrence (15-20%): risk factors: recurrent hernia, age >50, smoking, BMI >25, poor pre-op functional status (ASA ≥3 – see Anaesthesia, A4), associated medical conditions: type II DM, hyperlipidemia, immunosuppression, any comorbid conditions increasing intra-abdominal pressure less common with mesh/"tension-free" repair • scrotal hematoma (3%): painful scrotal swelling from compromised venous return of testes deep bleeding – may enter retroperitoneal space and not be initially apparent difficulty voiding • nerve entrapment: ilioinguinal genital branch of genitofemoral (in spermatic cord) • stenosis/occlusion of femoral vein: acute leg swelling • ischemic colitis
Toronto Notes 2011
Hernia/Bowel Obstruction
General Surgery GS23
Groin Hernias Table 5. Groin Hernias Direct Inguinal
Indirect Inguinal
Epidemiology
1% of all men
Most common hernia in men and women Affects mostly females Males > females
Femoral
Etiology
Acquired weakness of Congenital persistence of processus transversalis fascia vaginalis in 20% of adults “Wear and tear” Increased intra-abdominal pressure
Pregnancy – weakness of pelvic floor musculature Increased intra-abdominal pressure
Anatomy
Through Hesselbach’s triangle Medial to inferior epigastric artery Usually does not descend into scrotal sac
Originates in deep inguinal ring Lateral to inferior epigastric artery Often descends into scrotal sac (or labia majora)
Into femoral canal, below inguinal ligament but may override it Medial to femoral vein within femoral canal
Treatment
Surgical repair
Surgical repair
Surgical repair
Prognosis
3-4% risk of recurrence
3 cm) + no colonic gas
Air-fluid levels “Picture frame” appearance Proximal distention + distal decompression No small bowel air if competent ileocecal valve Coffee bean sign
Air throughout small bowel and colon
Fossa ovalis
Femoral hernia
© Jason Sharpe 2003
Definition • partial or complete blockage of the bowel resulting in failure of intestinal contents to pass through lumen
Figure 12. Schematic of Inguinal (Direct and Indirect) and Femoral Hernias
GS24 General Surgery
Bowel Obstruction
Toronto Notes 2011
Complications (of total obstruction) • strangulating obstruction (10% of bowel obstructions) = surgical emergency: cramping pain turns to continuous ache, hematemesis, melena (if infarction) fever, leukocytosis, tachycardia peritoneal signs, early shock see also Intestinal Ischemia, GS27 • other: perforation: secondary to ischemia and luminal distention septicemia hypovolemia (due to third spacing)
Increased Risk of Perforation with Distention as seen on Abdo Imaging Small bowel ≥3 cm Distal colon ≥6 cm Proximal colon ≥9 cm Cecum ≥12 cm
Investigations • radiological: upright CXR or left lateral decubitus (LLD) to rule out free air, usually seen under the right hemidiaphragm abdominal x-ray (3 views) to determine SBO vs. LBO vs. ileus (see Table 7) if ischemic bowel look for: free air, pneumatosis, thickened bowel wall, air in portal vein, dilated small and large bowels, thickened or hoselike haustra (normally fingerlike projections) other: CT provides information on level of obstruction, severity, cause upper GI series/small bowel series for SBO (if no cause apparent, i.e. no hernias, no previous surgeries) if suspect LBO, consider a rectal water-soluble (Gastrografin® for PO/PR; Hypaque® for IV) enema rather than barium enema (can thicken and cause complete obstruction) may consider ultrasound or MRI in pregnant patients • laboratory: may be normal early in disease course BUN, creatinine, hematocrit (hemoconcentration) to assess degree of dehydration fluid, electrolyte abnormalities amylase elevated metabolic alkalosis due to frequent emesis if strangulation: leukocytosis with left shift, lactic acidosis, elevated LDH (late signs) Treatment • stabilize vitals, fluid and electrolyte resuscitation (with normal saline/Ringer’s first, then with added potassium after fluid deficits are corrected) • NG tube to relieve vomiting, prevent aspiration and decompress small bowel by prevention of further distention by swallowed air • Foley catheter to monitor in/outs
Small Bowel Obstruction (SBO) Etiology Table 8. Common Causes of SBO Top 3 Causes of SBO (in order)
Intraluminal
Intramural
Extramural
ABC 1. Adhesions 2. Bulge (hernias) 3. Cancer (neoplasms)
Intussusception Gallstones
Crohn’s Radiation stricture Adenocarcinoma
Adhesions Incarcerated hernia Peritoneal carcinomatosis
Treatment • consider whether complete or partial obstruction, ongoing or impending strangulation, location and cause: SBO with history of abdo/pelvic surgery g conservative management (likely to resolve) g surgery if no resolution in 48-72 hrs or complications complete SBO, strangulation g urgent surgery after stabilizing patient trial of medical management may be indicated in Crohn’s, recurrent SBO, carcinomatosis special case: early postoperative SBO (within 30 days of abdominal surgery) – prolonged trial of conservative therapy is appropriate, surgery is reserved for complications such as strangulation Prognosis • mortality: non-strangulating 36 hours), ischemic = up to 50%
Toronto Notes 2011
Bowel Obstruction/Pseudo-Obstruction
General Surgery GS25
Large Bowel Obstruction (LBO) Etiology Table 9. Common Causes of LBO Intraluminal
Intramural
Extramural
Constipation
Adenocarcinoma Diverticulitis IBD stricture Radiation stricture
Volvulus
Clinical Features (unique to LBO) • open loop (10-20%) (safe): incompetent ileocecal valve allows relief of colonic pressure as contents reflux into ileum, therefore clinical presentation similar to SBO • closed loop (80-90%) (dangerous): competent ileocecal valve, resulting in proximal and distal occlusions massive colonic distention g increased pressure in cecum bowel wall ischemia g necrosis g perforation Treatment • surgical correction of obstruction (usually requires resection + temporary diverting colostomy) • volvulus requires sigmoidoscopic or endoscopic decompression followed by operative reduction if unsuccessful if successful, consider sigmoid resection on same admission Prognosis • overall mortality: 10% • cecal perforation + feculent peritonitis: 20% mortality
Pseudo-Obstruction Definition • condition with symptoms of intestinal blockage without any physical signs of blockage Differential Diagnosis • acute: toxic megacolon, trauma, postoperative, neurologic disease, retroperitoneal disease • chronic: neurologic disease (enteric, central, peripheral nervous system), scleroderma
Toxic Megacolon Pathogenesis • extension of inflammation into smooth muscle layer causing paralysis • damage to myenteric plexus and electrolyte abnormalities are not consistently found Etiology • inflammatory bowel disease (ulcerative colitis > Crohn’s Disease) • infectious colitis: bacterial (C. difficile, Salmonella, Shigella, Campylobacter), viral (cytomegalovirus), parasitic (E. histolytica) • volvulus, diverticulitis, ischemic colitis, obstructing colon cancer are rare causes Clinical Features • infectious colitis usually present for >1 week before colonic dilatation • diarrhea ± blood (but improvement of diarrhea may portend onset of megacolon) • abdominal distention, tenderness, ± local/general peritoneal signs (suggest perforation) • triggers: hypokalemia, constipating agents (opioids, antidepressants, loperamide, anticholinergics), barium enema, colonoscopy Diagnostic Criteria • must have both colitis and systemic manifestations for diagnosis • radiologic evidence of dilated colon • three of: fever, HR >120, WBC >10.5, anemia • one of: fluid and electrolyte disturbances, hypotension, altered LOC
Top 3 Causes of LBO (in order) 1. Cancer 2. Diverticulitis 3. Volvulus
In a patient with clinical LBO consider impending perforation when: • Cecum ≥12 cm in diameter • Tenderness present over cecum
GS26 General Surgery
Pseudo-Obstruction
Toronto Notes 2011
Investigations • CBC (leukocytosis with left shift, anemia from bloody diarrhea), electrolytes, elevated CRP, ESR • metabolic alkalosis (volume contraction and hypokalemia) and hypoalbuminemia are late findings • AXR: dilated colon >6 cm (right > transverse > left), loss of haustra • CT: useful to assess underlying disease
Use caution when giving antidiarrheals, especially with bloody diarrhea.
Treatment • NPO, NG tube, stop constipating agents, correct fluid and electrolyte abnormalities, transfusion • serial AXRs • broad-spectrum antibiotics (reduce sepsis, anticipate perforation) • aggressive treatment of underlying disease (e.g. steroids in IBD, metronidazole for C. difficile) • indications for surgery (50% improve on medical management): worsening or persisting toxicity or dilation after 48-72 hrs severe hemorrhage, perforation • procedure: subtotal colectomy + end ileostomy with 2nd operation for re-anastomosis Prognosis • average 25-30% mortality
Paralytic Ileus Pathogenesis • temporary paralysis of the myenteric plexus Associations • postoperative, intra-abdominal sepsis, medications (opiates, anesthetics, psychotropics), electrolyte disturbances (Na, K, Ca), C. difficile, inactivity Treatment • NG decompression, NPO, fluid resuscitation, correct causative abnormalities (e.g. sepsis, medications, electrolytes), consider TPN for prolonged ileus • post-op: gastric and small bowel motility returns by 24-48 hrs, colonic motility by 3-5 d • current interest in novel therapies such as gum chewing and pharmacologic therapy (opioid antagonists, neostigmine)
Ogilvie’s Syndrome • acute pseudo-obstruction • distention of colon without mechanical obstruction in distal colon • arises in bedridden patients with serious extraintestinal illness or trauma • exact mechanism unknown, likely autonomic motor dysregulation g possibly sympathetic deprivation to colon, unopposed parasympathetic tone, and interruption of sacral parasympathetic tone to distal bowel • first presents with abdominal distention (>90%) ± tenderness • later symptoms mimic true obstruction Associations • most common: trauma, infection, cardiac (MI, CHF) • disability (long term debilitation, chronic disease, bed-bound nursing home patients, paraplegia), drugs (narcotic use, laxative abuse, polypharmacy), other (recent orthopaedic or neurosurgery, post-partum, hypokalemia, retroperitoneal hematoma, diffuse carcinomatosis) Investigations • AXR: cecal dilatation – if diameter ≥12 cm, increased risk of perforation Treatment • treat underlying cause • NPO, NG tube • decompression: rectal tube, colonoscopy, neostigmine (cholinergic drug), surgical decompression (ostomy/resection) uncommon • surgery (extremely rare): if perforation, ischemia or failure of conservative management Prognosis • most resolve with conservative management
Toronto Notes 2011
Intestinal Ischemia/Appendix
General Surgery GS27
Intestinal Ischemia Etiology • acute: arterial: occlusive: thrombotic, embolic, extrinsic compression (e.g. strangulating hernia) non-occlusive: mesenteric vasoconstriction 2o to systemic hypoperfusion (preserves supply to vital organs) trauma/dissection venous thrombosis (prevents venous outflow): consider hypercoagulable state, deep vein thrombosis (DVT) • chronic: usually due to atherosclerotic disease – look for CVD risk factors Clinical Features • acute: severe abdominal pain out of proportion to physical findings, vomiting, bloody diarrhea, bloating, minimal peritoneal signs early in course, hypotension, shock, sepsis • chronic: postprandial pain, fear of eating, weight loss • common sites: superior mesenteric artery (SMA) supplied territory, “watershed” areas of colon – splenic flexure, left colon, sigmoid colon
Pain “out of keeping with physical findings” is the hallmark of early intestinal ischemia.
An acute abdomen + metabolic acidosis is bowel ischemia until proven otherwise.
Investigations • labs: leukocytosis (non-specific), lactic acidosis (late finding) amylase, LDH, CK, ALP can be used to observe progress hypercoagulability workup if suspect venous thrombosis • AXR: portal venous gas, intestinal pneumatosis, free air if perforation • contrast CT: thickened bowel wall, luminal dilatation, SMA or SMV thrombus, mesenteric/ portal venous gas, pneumatosis • CT angiography is the gold standard for acute arterial ischemia Treatment • fluid resuscitation, NPO, prophylactic broad-spectrum antibiotics • exploratory laparotomy • angiogram, embolectomy/thrombectomy, bypass/graft, mesenteric endarterectomy, anticoagulation therapy • segmental resection of necrotic intestine: assess extent of viability; if extent of bowel viability is uncertain, a second look laparotomy 12-24 hrs later is mandatory
Appendix Appendicitis Epidemiology • 6% of population, M>F • 80% between 5-35 years of age Pathogenesis • luminal obstruction g bacterial overgrowth g inflammation/swelling g increased pressure g localized ischemia g gangrene/perforation g localized abscess (walled off by omentum) or peritonitis • etiology: children or young adult: hyperplasia of lymphoid follicles, initiated by infection adult: fibrosis/stricture, fecolith, obstructing neoplasm other causes: parasites, foreign body Clinical Features • most reliable feature is progression of signs and symptoms • low grade fever (38ºC), rises if perforation • abdominal pain then anorexia, nausea and vomiting • classic pattern: pain initially periumbilical; constant, dull, poorly localized, then well localized pain over McBurney’s point due to progression of disease from visceral irritation (causing referred pain from structures of the embryonic midgut, including the appendix) to irritation of parietal structures McBurney’s sign
Psoas major muscle Retrocecal (75%) Paracolic Iliacus muscle
Ileal/sub-ileal (4-5 days), consider radiologic drainage + antibiotics x 14 d + interval appendectomy in 6 weeks • appendectomy: laparoscopic vs. open (see sidebar) complications: spillage of bowel contents, pelvic abscess, enterocutaneous fistula perioperative antibiotics: ampicillin + gentamicin + metronidazole (antibiotics x 24 h only if non-perforated) other choices: 2nd/3rd generation cephalosporin for aerobic gut organisms Prognosis • morbidity/mortality 0.6% if uncomplicated, 5% if perforated
Tumours of the Appendix CARCINOID TUMOURS (most common type) • see Tumours of Small Intestines: Carcinoid, GS21 ADENOCARCINOMA • 50% present as acute appendicitis • spreads rapidly to lymph nodes, ovaries, and peritoneal surfaces • treatment: right hemicolectomy OTHER • malignant mucinous cystadenocarcinoma
Inflammatory Bowel Disease (IBD) • see Gastroenterology, G19 Principles of Surgical Management • can alleviate symptoms, address complications, improve quality of life • conserve bowel – resect as little as possible to avoid short gut syndrome • perioperative management: optimize medical status: may require TPN (especially if >7 days NPO) and bowel rest hold immunosuppressive therapy pre-op, provide pre-op stress dose of corticosteroid if patient had recent steroid therapy deep vein thrombosis (DVT) prophylaxis: heparin (IBD patients at increased risk of thromboembolic events)
Toronto Notes 2011
Inflammatory Bowel Disease (IBD)
General Surgery GS29
Crohn’s Disease Treatment • surgery is NOT curative, but over lifetime ~70% of Crohn’s patients will have surgery • indications for surgical management: failure of medical management SBO (due to stricture/inflammation): indication in 50% of surgical cases abscess, fistula (enterocolic, vesicular, vaginal, cutaneous abscess), quality of life, perforation, hemorrhage, chronic disability, failure to thrive (children), perianal disease • surgical procedures: resection and anastomosis/stoma if active or subacute inflammation, perforation, fistula resection margin only has to be free of gross disease (microscopic disease irrelevant to prognosis) stricturoplasty – widens lumen in chronically scarred bowel – relieves obstruction without resecting bowel (contraindicated in acute inflammation) Complications of Treatment • short gut syndrome (diarrhea, steatorrhea, malnutrition) • fistulas • gallstones (if terminal ileum resected, decreased bile salt resorption g increased cholesterol precipitation) • kidney stones (loss of calcium in diarrhea g increased oxalate absorption and hyperoxaluria g stones) Prognosis • recurrence rate at 10 years: ileocolic (25-50%), small bowel (50%), colonic (40-50%) • re-operation at 5 years: primary resection (20%), bypass (50%), stricturoplasty (10% at 1 year) • 80-85% of patients who need surgery lead normal lives • mortality: 15% at 30 years
Ulcerative Colitis Treatment • indications for surgical management: failure of medical management (including inability to taper steroids) complications: hemorrhage, obstruction, perforation, toxic megacolon (emergency), failure to thrive (children) reduce cancer risk (1-2% risk per year after 10 years of disease) • surgical procedures: proctocolectomy and ileal pouch-anal anastomosis (IPAA) ± rectal mucosectomy (operation of choice) proctocolectomy with permanent end ileostomy (if not a candidate for ileoanal procedures) colectomy and IPAA ± rectal mucosectomy in emergency: total colectomy and ileostomy with Hartmann closure of the rectum, rectal preservation Complications of Treatment • early: bowel obstruction, transient urinary dysfunction, dehydration (high stoma output), anastomotic leak • late: stricture, anal fistula/abscess, pouchitis, poor anorectal function, reduced fertility Prognosis • mortality: 5% over 10 years • total proctocolectomy will completely eliminate risk of cancer • perforation of the colon is the leading cause of death from ulcerative colitis
Crohn’s 3 Major Patterns • Ileocecal 40% (RLQ pain, fever, weight loss) • Small intestine 30% (especially terminal ileum) • Colon 25% (diarrhea)
Findings in Crohn’s • “Cobblestoning” on mucosal surface due to edema and linear ulcerations • “Skip lesions”: normal mucosa in between • “Creeping fat”: mesentery infiltrated by fat • Granulomas: 25-30% • Barium enema: “lead-pipe appearance”
Diverticular Disease
Toronto Notes 2011
Diverticular Disease Definitions • diverticulum – abnormal sac or pouch protruding from the wall of a hollow organ • diverticulosis – presence of multiple false diverticuli • diverticulitis – inflammation of diverticuli • right sided (true) diverticuli = contains all layers (congenital) (see Figure 14) • left sided (false) diverticuli = contains only mucosal and submucosal layers (acquired) TRUE DIVERTICULUM (full wall thickness)
FALSE DIVERTICULUM (mucosal herniations)
Mucosa Circular muscle
Antimesenteric tenia
Mesocolon Mesenteric tenia
Figure 14. Diverticular Disease – Cross-Sections of True and False Diverticuli
Diverticulosis Epidemiology • 35-50% of general population, M=F • increased incidence in 5th to 8th decades of life • 95% involve sigmoid colon (site of highest pressure) • higher incidence in Western countries, related to low fibre diet Pathogenesis • risk factors: low-fibre diet (increases gut transit time and intraluminal pressure) muscle wall weakness from aging and illness (e.g. Ehler-Danlos, Marfan’s) possible genetic component • high intraluminal pressures cause outpouching to occur at area of greatest weakness: most commonly at the site of penetrating vessels at antimesenteric tenia, therefore increased risk of hemorrhage Clinical Features • uncomplicated diverticulosis: asymptomatic (70-80%) • episodic LLQ abdominal pain, bloating, flatulence, constipation, diarrhea • absence of fever/leukocytosis • no physical exam findings or poorly localized LLQ tenderness • complications: diverticulitis (15-20%) bleeding (5-15%): PAINLESS rectal bleeding, 2/3 of massive lower GI bleeds Treatment • uncomplicated diverticulosis: high fibre, education • diverticular bleed: initially work up and treat as any lower GI bleed if hemorrhage does not stop, resect involved region
Diverticulitis (“left sided appendicitis”) Definition • infection or perforation of a diverticulum Pathogenesis • erosion of the wall by increased intraluminal pressure (or inspissated food particles) g microperforation/macroperforation g inflammation and focal necrosis • usually mild inflammation with perforation walled off by pericolic fat • sigmoid colon most often involved
© Sonya Amin 2003
GS30 General Surgery
Toronto Notes 2011
Diverticular Disease
General Surgery GS31
Clinical Features • severity ranges from mild inflammation to feculent peritonitis • LLQ pain/tenderness, present for several days before admission • alternating constipation and diarrhea, urinary symptoms (dysuria if inflammation adjacent to bladder) • palpable mass if phlegmon or abscess, nausea, vomiting • low-grade fever, mild leukocytosis • occult or gross blood in stool less common • generalized tenderness suggests macroperforation and peritonitis • complications: abscess – on physical exam may find palpable abdominal mass fistula – colovesical (most common), coloenteric, colovaginal, colocutaneous obstruction – due to scarring from repeated inflammation macroperforation g peritonitis (feculent vs. purulent) recurrent attacks RARELY lead to peritonitis
Treatment • admit, NPO, fluid resuscitation, NG + suction, IV antibiotics covering B. fragilis (e.g. ciprofloxacin, metronidazole) • indications for surgery: unstable patient with peritonitis Hinchey stage 2-4 (see Table 10) after 1 attack if: (a) immunosuppressed, (b) abscess needing percutaneous drainage consider after 2 or more attacks, recent trend is toward conservative management of recurrent mild/moderate attacks • complications: generalized peritonitis, free air, abscess fistula, obstruction, hemorrhage, inability to rule out colon cancer on endoscopy, or failure of medical management • surgical procedures: Hartmann procedure: resection + colostomy and rectal stump g colostomy reversal in 3-6 months (see Figure 15) resection + primary anastomosis (± pre-op bowel prep or on-table lavage): controversial (anastomosis of inflamed tissues = increased risk of anastomotic leakage) Prognosis • 13-30% recurrence after 1st attack, 30-50% after 2nd attack Table 10. Hinchey Staging and Treatment for Diverticulitis Hinchey Stage
Description
Acute treatment
1
Phlegmon / small pericolic abscess
Medical
2
Large abscess / fistula
Abscess drainage, resection ± primary anastomosis
3
Purulent peritonitis (ruptured abscess)
Hartmann procedure, sometimes primary anastomosis
4
Feculent peritonitis
Hartmann procedure
Area of diverticulitis and inflammation
Colostomy Resection of diseased area and closure of distal rectal stump
Anastomosis in approximately 3 months
Figure 15. Hartmann Procedure
© Myra Rudakewich
Investigations • AXR, upright CXR: localized diverticulitis (ileus, thickened wall, SBO, partial colonic obstruction) free air may be seen in 30% with perforation and generalized peritonitis • CT scan (optimal method of investigation) : 97% sensitive, very useful for assessment of severity and prognosis very helpful in localizing an abscess • Hypaque® (water soluble) enema – SAFE (under low pressure): saw-tooth pattern (colonic spasm) may show site of perforation, abscess cavities or sinus tracts, fistulas • barium enema: contraindicated during an acute attack: risk of chemical peritonitis (because of perforation) • sigmoidoscopy/colonoscopy: not during an acute attack, only done on an elective basis take biopsies to rule out other diagnoses (polyps, malignancy)
GS32 General Surgery
Colorectal Neoplasms
Toronto Notes 2011
Colorectal Neoplasms Colorectal Screening Guidelines Men and Women
Symptomatic
Diagnostic Work-up
Asymptomatic regardless of age but positive family history
HNPCC or FAP Genetic counseling and special screening HNPCC Colonoscopy every 1-2 years Begins at age 20 or 10 years younger than the earliest case in the family, whichever comes first FAP Sigmoidoscopy annually Begin at age 10-12 AAPC Colonoscopy annually Begin at age 16-18
One first-degree relative with cancer or adenomatous polyp at age 60 or Two or more second-degree relatives with polyps or cancer
Colonoscopy every 5 years
One second-degree relative or third-degree relative affected Average risk screening Begin at age 50
Polyps found at colonoscopy 1-2 tubular adenomas 2 adenomas: colonoscopy in 3 years Incomplete examination, numerous polyps, advanced adenoma, malignant or large sessile adenoma: colonoscopy after a short interval based on clinical judgment
Average risk screening Begin at age 40
Begin at age 40 or 10 years younger than the earliest case of polyp or cancer in the family, whichever comes first
Figure 16. Approach to Higher Risk Screening
AAPC (attenuated adenomatous polyposis) FAP (familial adenomatous polyposis); HNPCC (hereditary nonpolyposis colorectal cancer); First degree relatives: parents, siblings, children; 2nd degree relatives: grandparents, aunts, uncles; 3rd degree relatives: great grandparents or cousins. Printed with permission Canadian Journal of Gastroenterology. Reference: Volume 18 No. 2, February 2004.
Colorectal Polyps Bowel lumen Bowel wall
Definition • polyp: small mucosal outgrowth into the lumen of the colon or rectum • sessile (flat) or pedunculated (on a stalk) (see Figure 17) Epidemiology • 30% of population have polyps by age 50, 40% by age 60, 50% by age 70
Sessile polyp
Table 11. Characteristics of Tubular vs. Villous Polyps Tubular
Pedunculated polyp
© Janice Wong 2003
Figure 17. Sessile and Pedunculated Polyps
Villous
Incidence
Common (60% to 80%)
Less common (10%)
Size
Small (2 cm)
Attachment
Pedunculated
Sessile
Malignant Potential
Lower
Higher
Distribution
Even
Left-sided predominance
Clinical Features • 50% in the rectosigmoid region, 50% are multiple • usually asymptomatic, but may have rectal bleeding, change in bowel habit, mucous per rectum • usually detected during routine endoscopy or familial/high risk screening Pathology • non-neoplastic: hyperplastic – most common non-neoplastic polyp pseudopolyps – inflammatory, associated with IBD, no malignant potential
Toronto Notes 2011
Colorectal Neoplasms
General Surgery GS33
• neoplastic: hamartomas: juvenile polyps (large bowel), Peutz-Jegher syndrome (small bowel) malignant risk due to associated adenomas (large bowel) low malignant potential g most spontaneously regress or autoamputate adenomas – premalignant, often carcinoma in situ: some may contain invasive carcinoma (“malignant polyp” – 3-9%): invasion into muscularis malignant potential: villous > tubulovillous > tubular (see Table 11) Investigations • flexible sigmoidoscope can reach 60% of polyps in men and 35% of polyps in women; if polyps detected, proceed to colonoscopy for examination of entire bowel and biopsy • colonoscopy still the gold standard Treatment • indications: symptoms, malignancy or risk of malignancy (i.e. adenomatous polyps) • endoscopic removal of entire growth • surgical resection for those invading into muscularis (high risk of malignancy) and those too large to remove endoscopically • follow-up endoscopy 1 year later, then every 3-5 years
Familial Colon Cancer Syndromes FAMILIAL ADENOMATOUS POLYPOSIS (FAP) Pathogenesis • autosomal dominant (AD) inheritance, mutation in adenomatous polyposis coli (APC) gene on chromosome 5q Clinical Features • hundreds to thousands of colorectal adenomas usually by age 20 (by 40’s in attenuated FAP) • extracolonic manifestations: carcinoma of duodenum, bile duct, pancreas, stomach, thyroid, adrenal, small bowel congenital hypertrophy of retinal pigment epithelium presents early in life in 2/3 of patients • virtually 100% lifetime risk of colon cancer (because of number of polyps) • variants: Gardner’s syndrome: FAP + extraintestinal lesions (sebaceous cysts, osteomas, desmoid tumours) Turcot’s syndrome: FAP + CNS tumours (glioblastoma multiforme) Investigations • genetic testing (80-95% sensitive, 99-100% specific) (see sidebar) • if no polyposis found: annual flexible sigmoidoscopy from puberty to age 50, then routine screening • if polyposis found: annual colonoscopy and consider surgery (see Figure 16) Treatment • surgery indicated by age 17-20 • total proctocolectomy with ileostomy OR total colectomy with ileorectal anastomosis • doxorubicin-based chemotherapy • NSAIDS for intra-abdominal desmoids HEREDITARY NON-POLYPOSIS COLORECTAL CANCER (HNPCC) Pathogenesis • AD inheritance, mutation in a DNA mismatch repair gene resulting in genomic instability and subsequent mutations Clinical Features • early age of onset, right > left colon, synchronous and metachronous lesions • mean age of cancer presentation is 44 years, lifetime risk 70-80% (M>F) Lynch syndrome I: hereditary site-specific colon cancer Lynch syndrome II: cancer family syndrome – high rates of extracolonic tumours (endometrial, ovarian, hepatobiliary, small bowel) Diagnosis • diagnosis is clinical – based on Amsterdam Criteria: at least 3 relatives with colorectal cancer or HNPCC related CA 2 or more generations involved, and 1 must be 1st degree relative of the other 2 1 case must be diagnosed before 50 years old FAP is excluded
Referral Criteria for Genetic Screening for APC • To confirm the diagnosis of FAP (in patients with ≥100 colorectal adenomas) • To provide pre-symptomatic testing for indviduals at risk for FAP (1st degree relatives who are ≥10 years old) • To confirm the diagnosis of attenuated FAP (in patients with ≥20 colorectal adenomas)
GS34 General Surgery
Revised Bethesda Criteria – Refer for Genetic Screening for HNPCC • Individuals with cancer in families that meet the Amsterdam Criteria • Patients with two HNPCC-related cancers, including synchronous and metachronous colorectal cancer or associated extracolonic cancers (endometrial, ovarian, gastric, hepatobiliary, small bowel, or transitional cell carcinoma of the renal pelvis or ureter). • Patients with colorectal cancer and a first degree relative with colorectal cancer and/or HNPCCrelated extracolonic cancer and/or a colorectal adenoma with one of the cancers diagnosed before age 45, and the adenoma diagnosed before age 40. • Patients with right-sided colorectal cancer having an undifferentiated pattern (solid/cribriform) on histopathologic diagnosis before age 45. • Patients with signet-ring cell type colorectal cancer diagnosed before age 45. • Patients with adenomas diagnosed before age 40.
Screening for Colorectal Cancer (asymptomatic, no history of UC, polyps, or CRC) • Average risk individuals, at age 50 (incl. those with 2 relatives with CRC/ adenoma, one being a 1st degree relative): • Start screening 10 years prior to the age of the relative’s with the earliest onset of carcinoma • FAP genetic testing +ve: • Yearly sigmoidoscopy starting at puberty (“B” recommendation) • HNPCC genetic testing +ve: • Yearly colonoscopy starting at age 20 (“B” recommendation)
Elderly persons who present with iron-deficiency anemia should be investigated for colon cancer.
Colorectal Neoplasms
Toronto Notes 2011
Investigations • genetic testing (80% sensitive) – colonoscopy mandatory even if negative refer for genetic screening individuals who fulfill EITHER the Amsterdam Criteria (as above) OR the revised Bethesda Criteria (see sidebar) • colonoscopy (starting age 20) anually • surveillance for extracolonic lesions Treatment • total colectomy and ileorectal anastomosis with yearly proctoscopy
Colorectal Carcinoma (CRC) Epidemiology • 3rd most common cancer (after lung, prostate/breast), 2nd most common cause of cancer death Risk Factors • most patients have no specific risk factors • FAP, HNPCC, family history of CRC • adenomatous polyps (especially if >1 cm, villous, multiple) • age >50 (dominant risk factor in sporadic cases), mean age is 70 • IBD (especially UC: risk is 1-2%/yr if UC >10 yrs) • previous colorectal cancer (also gonadal or breast) • diet (increased fat, red meat, decreased fibre) and smoking • diabetes mellitus (insulin is a growth factor for colonic mucosal cells) and acromegaly Screening Tools • digital rectal exam (DRE): most common exam, but not recommended as a screening tool • fecal occult blood test (FOBT): proper test requires 3 samples of stool collected at 3 different times recommended annually by the World Health Organization (WHO) results in 16-33% reduction in mortality in RCTs Minnesota Colon Cancer Study: RCT showed that annual FOBT can decrease mortality rate by 1/3 in patients 50-80 years old • sigmoidoscopy: can identify 30-60% of lesions sigmoidoscopy + FOBT misses 24% of colonic neoplasms • colonoscopy: can remove or biopsy lesions during procedure can identify proximal lesions missed by sigmoidoscopy used as follow-up to other tests if lesions found disadvantages: expensive, not always available, poor compliance, requires sedation, risk of perforation (0.2%) • virtual colonoscopy (CT colonography): 91% sensitive, 17% false positive rate • air contrast barium enema (ACBE): 50% sensitive for large (>1 cm) adenomas, 39% for polyps Pathogenesis • adenoma-carcinoma sequence; rarely arise de novo Clinical Features (see Table 12) • often asymptomatic • hematochezia/melena, abdominal pain, change in bowel habits • others: weakness, anemia, weight loss, palpable mass, obstruction • 3-5% have synchronous lesions • spread: direct extension, lymphatic, hematogenous (liver most common, lung, rarely bone and brain) peritoneal seeding: ovary, Blumer’s shelf (pelvic cul-de-sac) intraluminal Table 12. Clinical Presentation of CRC Right Colon
Left Colon
Rectum
Frequency
25%
35%
30%
Pathology
Exophytic lesions with occult bleeding
Annular, invasive lesions
Ulcerating
Symptoms
Weight loss, weakness, rarely obstruction
Constipation ± overflow (alternating bowel patterns), abdominal pain, decreased stool caliber, rectal bleeding
Obstruction, tenesmus, rectal bleeding
Signs
Fe-deficiency anemia, RLQ mass (10%)
BRBPR, LBO
Palpable mass on DRE, BRBPR
Toronto Notes 2011
Colorectal Neoplasms
General Surgery GS35
Investigations • colonoscopy (best), look for synchronous lesions; alternative: air contrast barium enema (“apple core” lesion) + sigmoidoscopy • if a patient is FOBT +ve, has microcytic anemia or has a change in bowel habits, do colonoscopy • metastatic workup: CXR, abdominal CT/ultrasound • bone scan, CT head only if lesions suspected • labs: CBC, urinalysis, liver function tests, CEA (before surgery for baseline) • staging (see Table 13 and sidebar) • rectal cancer: pelvic MRI or endorectal ultrasound to determine T and N stage
Staging for CRC I T1,2 N0M0 II T3,4 N0M0 III TxN+M0 IV TxNxM1
Table 13. TNM Classification System for Staging of Colorectal Carcinoma
Prognosis for CRC
Primary Tumour (T)
Regional Lymph Nodes (N)
Distant Metastasis (M)
Stage
5 yr survival % >90
T0 No primary tumour found
N0 No regional node involvement
M0 No distant metastasis
T1N0M0
Tis Carcinoma in situ
N1 Metastasis in 1-3 pericolic nodes
M1 Distant metastasis
T2N0M0
85
T1 Invasion into submucosa
N2 Metastasis in 4 or more pericolic nodes
T3N0M0
70-80
T2 Invasion into muscularis propria
N3 Metastasis in any nodes along the course of named vascular trunks
TXN1M0
35-65
TXNXM1
5
T3 Invasion through muscularis and into serosa T4 Invasion into adjacent structures or organs
Case Finding for Colorectal Cancer (symptomatic or history of UC, polyps, or CRC) • surveillance (when polyps are found): colonoscopy within 3 years after initial finding • patients with past CRC: colonoscopy every 3-5 years, or more frequently • IBD: some recommend colonoscopy every 1-2 years after 8 years of disease (especially UC) Follow-Up • intensive follow up improves overall survival in low risk patients • currently there are no data suggesting optimal follow-up • combination of periodic CT chest/abdo/pelvis, CEA and colonoscopy is recommended • carcinogenic embryonic antigen (CEA): to monitor for initial response to treatment, and to assess for recurrence q3 months (not a screening test)
Removed
© Geoffrey Cheung & Shelley Chen 2010
Treatment • surgery (indicated in potentially curable or symptomatic cases – not usually in stage IV) curative: wide resection of lesion (5 cm margins) with nodes and mesentery palliative: if distant spread, then local control for hemorrhage or obstruction 80% of recurrences occur within 2 years of resection improved survival if metastasis consists of solitary hepatic mass that is resected colectomy: most patients get primary anastomosis [e.g. hemicolectomy, low anterior resection (LAR)] (see Figure 18) if cancer is low in rectum, patient may require an abdominal perineal resection (APR) with a permanent end colostomy, especially if lesion involves the sphincter complex complications: anastomotic leak or stricture, recurrent disease, pelvic abscess, enterocutaneous fistula • radiotherapy and chemotherapy: chemotherapy (5-FU based regimens): for patients with node-positive disease radiation: for patients with node-positive or transmural rectal cancer (pre ± post-op), not effective as 1o treatment of colon cancer adjuvant therapy: chemotherapy (colon) and radiation (rectum) palliative chemotherapy/radiation therapy for improvement in symptoms and survival neoadjuvant chemoradiation for T3 or N1 rectal cancer
Figure 18. Low Anterior Resection (LAR) Combined-modality Treatment for Resectable Metastatic Colorectal Cancer to the Liver: Surgical Resection of Hepatic Hetastases in Combination with Continuous Infusion of Chemotherapy – An Intergroup Study J Clin Oncol 2002; 20(6):1499-505 Background: Metastatic spread of colorectal cancer commonly targets the liver, and long-term outcome studies of surgical resection of hepatic metastases have shown high rates of treatment failure. Arterial chemotherapy regimens targeted to the liver represent a promising adjuvant treatment to reduce recurrence rates. Methods: Patients with 1-3 resectable liver metastases were randomized preoperatively to receive no further intervention (45 patients, control group) or postoperative floxuridine and fluorouracil (30 patients). Results: 4-year recurrence-free survival rates were 25% for the control group and 46% for the chemotherapy group (P=0.04), with liver recurrence-free rates of 43% and 67% respectively (P =0.03). Conclusions: Adjuvant intra-arterial and intravenous chemotherapy shows promise in preventing hepatic recurrence after surgical resection of colorectal cancer hepatic metastases.
APR removes distal sigmoid colon, rectum and anus, permanent end colostomy required. LAR removes distal sigmoid and rectum with anastomosis of distal colon to anus.
GS36 General Surgery
Other Conditions of the Large Intestine
Toronto Notes 2011
Other Conditions of the Large Intestine Angiodysplasia Definition • vascular anomaly: focal submucosal venous dilatation and tortuosity Clinical Features • most frequently in right colon of patients >60 years old • bleeding typically intermittent (melena, anemia, guaiac positive stools) Investigations • endoscopy (cherry red spots, branching pattern from central vessel) • angiography (slow filling/early emptying mesenteric vein, vascular tuft) • RBC technetium-99 scan • barium enema is contraindicated (obscures other x-rays, i.e. angiogram) Treatment • none if asymptomatic • cautery, right hemicolectomy, embolization, vasopressin infusion, sclerotherapy, band ligation, laser, octreotide, and rarely segmental resection if other treatments fail
Volvulus
Cecal Volvulus AXR: Central cleft of “coffee bean” sign points to RLQ.
Sigmoid Volvulus AXR: Central cleft of “coffee bean” sign points to LLQ. Barium enema: “ace of spades” or “birds beak” sign.
Gastric Volvulus Brochardt’s Triad Epigastric distention Failure to pass NG tube Emesis followed by inability to vomit Treatment: exploratory laparotomy to untwist and gastropexy
Definition • rotation of segment of bowel about its mesenteric axis • sigmoid (70%), cecum (30%) Risk Factors • age (50% of patients >70 yrs: stretching/elongation of bowel with age is a predisposing factor) • high fibre diet (can cause elongated/redundant colon), chronic constipation, laxative abuse, pregnancy, bedridden, institutionalized (less frequent evacuation of bowels) • congenitally hypermobile cecum Clinical Features • symptoms due to bowel obstruction (GS23) or intestinal ischemia (GS27) Investigations • AXR: “omega”, “bent inner-tube”, “coffee-bean” signs (see sidebar) • barium/gastrograffin enema: “ace of spades” (or “bird’s beak”) appearance due to funnel-like luminal tapering of lower segment towards volvulus • sigmoidoscopy or colonoscopy as appropriate • CT Treatment • initial supportive management with fluid, electrolyte resuscitation • cecum: nonsurgical may attempt colonoscopic detorsion and decompression surgical: right colectomy + ileotransverse colonic anastomosis • sigmoid: nonsurgical decompression by flexible sigmoidoscopy and insertion of rectal tube past obstruction subsequent elective surgery recommended (50-70% recurrence) surgical: Hartmann procedure (if urgent) indications: strangulation, perforation or unsuccessful endoscopic decompression
Toronto Notes 2011
Fistula/Ostomies
General Surgery GS37
Fistula Definition • abnormal communication between two epithelialized surfaces (e.g. enterocutaneous, colovesical, aortoenteric, entero-enteric) Etiology • foreign object erosion (e.g. gallstone, graft) • infection, IBD (especially Crohn’s), diverticular disease • iatrogenic/surgery (e.g. postoperative anastomotic leak) • congenital, trauma • neoplastic
Why Fistulae Stay Open FRIENDO Foreign body Radiation Infection Epithelialization Neoplasm Distal obstruction (most common) Others: increased flow; steroids (may inhibit closure, usually will not maintain fistula)
Investigations • contrast radiography (fistulogram) • sonogram • CT scan • measure amount of drainage from fistula Treatment • fluid resuscitation, manage electrolytes • bowel rest – NPO • drain any abscesses/control sepsis • nutrition – elemental/low residue, TPN • decrease secretion – octreotide/somatostatin/omeprazole • skin care (for enterocutaneous fistula) • surgical intervention – dependent upon etiology (for non-closing fistulas); uncertainty of diagnosis
Ostomies Definition • an opening of the GI tract onto the surface of the abdomen wall • types (see Figure 19): colostomy vs. ileostomy, temporary vs. permanent, continent vs. incontinent, end vs. loop, ileoconduit end (Brooke) ileostomy: for incontinent, continuous drainage in patients requiring total colectomy Koch ileostomy: for continent, manual drainage – rarely used
Colostomy/Ileostomy • Connection of proximal limb of colon or ileum to abdominal wall skin Mucous Fistula • Connection of distal limb of colon to abdominal wall skin
Complications (10%) • obstruction: herniation, stenosis (skin and abdominal wall), adhesive bands, volvulus • peri-ileostomy abscess and fistula • skin irritation • prolapse or retraction • diarrhea (excessive output)
Ileoconduit • Connection of colon to ureter proximally + abdominal wall distally to drain urine
Ileostomy
Figure 19. Ostomies
Colostomy
End Colostomy
© Geoffrey Cheung 2010
Removed
GS38 General Surgery
Anorectum
Toronto Notes 2011
Anorectum Hemorrhoids Always rule out more serious causes (e.g. colon CA) in a person with hemorrhoids and rectal bleeding.
Etiology • vascular and connective tissue complexes form a plexus of dilated veins (cushion) internal: superior hemorrhoidal veins, above dentate line, portal circulation external: inferior hemorrhoidal veins, below dentate line, systemic circulation
Internal hemorrhoid
Inferior rectal vein
Dentate line External hemorrhoid
Figure 20. Hemorrhoids
© Shelley Wall 2003
Middle rectal vein
Risk Factors • increased intra-abdominal pressure: chronic constipation, pregnancy, obesity, portal hypertension, heavy lifting Clinical Features and Treatment • internal hemorrhoids (see Figure 20): engorged vascular cushions usually at 3, 7, 11 o’clock positions (patient in lithotomy position) painless rectal bleeding, anemia, prolapse, mucus discharge, pruritus, burning pain, rectal fullness: 1st degree: bleed but do not prolapse through the anus – treatment: high fibre/bulk diet, sitz baths, steroid cream, parmoxine (Anusol®), rubber band ligation, sclerotherapy, photocoagulation 2nd degree: prolapse with straining, spontaneous reduction – treatment: rubber band ligation, photocoagulation 3rd degree: prolapse requiring manual reduction – treatment: same as 2nd degree, but may require closed hemorrhoidectomy 4th degree: permanently prolapsed, cannot be manually reduced – treatment: closed hemorrhoidectomy • external hemorrhoids (see Figure 20): dilated venules usually mildly symptomatic pain after bowel movement, associated with poor hygiene medical treatment: dietary fibre, stool softeners, steroid cream (short course), parmoxine (Anusol®), avoid prolonged straining thrombosed hemorrhoids are very painful: resolve within 2 weeks, may leave excess skin = perianal skin tag treatment: consider surgical decompression within first 48 hours of thrombosis, otherwise medical treatment
Anal Fissures Definition • tear of anal canal below dentate line (very sensitive squamous epithelium) • 90% posterior midline, 10% anterior midline • if off midline: consider IBD, STIs, TB, leukemia or anal carcinoma • repetitive injury cycle after first tear: sphincter spasm occurs preventing edges from healing and leads to further tearing ischemia may ensue and contribute to chronicity Etiology • large, hard stools and irritant diarrheal stools • tightening of anal canal secondary to nervousness/pain • others: habitual use of cathartics, childbirth Clinical Features • acute fissure: very painful bright red bleeding especially after bowel movement treatment is conservative: stool softeners, sitz baths • chronic fissure: triad: fissure, sentinel skin tags, hypertrophied papillae treatment: stool softeners, bulking agents, sitz baths topical nitroglycerin or nifedipine – increases local blood flow, promoting healing and relieves sphincter spasm surgery (most effective) – lateral internal sphincterotomy; objective is to relieve sphincter spasm g increases blood flow and promotes healing; but 5% chance of fecal incontinence therefore not commonly done alternative treatment: botulinum toxin – inhibits release of acetylcholine (ACh), reducing sphincter spasm
Toronto Notes 2011
Anorectum
General Surgery GS39
Anorectal Abscess Definition • infection in one or more of the anal spaces (see Figure 21) • usually bacterial infection of blocked anal gland at the dentate line E. coli, Proteus, Streptococci, Staphylococci, Bacteroides, anaerobes
Supralevator space Supralevator abscess Levator muscle Column of Morgagni Internal sphincter Deep external sphincter Intersphincteric abscess (origin)
External sphincter Perianal abscess Normal
Inflammed
© Cynthia Yoon 2003
Ischiorectal abscess
Figure 21. Different Types of Perianal Abscesses
Clinical Features • throbbing pain that may worsen with straining and ambulation • abscess can spread vertically downward (perianal), vertically upward (supralevator) or horizontally (ischiorectal) • tender perianal/rectal mass on exam Treatment • incision and drainage curative in 50% of cases 50% develop anorectal fistulas • may require antibiotics if diabetic, heart murmur or cellulitis
Fistula-In-Ano Definition • connection between two epithelialized surfaces, one must be the rectum or anus • an inflammatory tract with internal os at dentate line, external os on skin Etiology • see Fistula, GS37 • same perirectal process as anal abscess therefore usually associated with abscess • other causes: post-op, trauma, anal fissure, malignancy, radiation proctitis Clinical Features • intermittent or constant purulent discharge from perianal opening • pain • palpable cord-like tract Treatment • identification: internal opening: Goodsall’s rule (see Figure 22): – a fistula with an external opening anterior to the transverse anal line will have its internal opening at relatively the same position (e.g. external opening at 2 o’clock = internal opening at 2 o’clock) whereas all external openings posterior to the line will tend to have their internal openings in the midline fistulous tract: probing or fistulography under anesthesia
Anterior Secondary opening Primary opening in crypt
Transverse anal line
Posterior
Figure 22. Goodsall’s Rule
GS40 General Surgery
Anorectum
Toronto Notes 2011
• surgery: fistulotomy: unroof tract from external to internal opening, allow drainage low lying fistula (does not involve external sphincter) g primary fistulotomy high lying fistula (involves external sphincter) g staged fistulotomy with Seton suture placed through tract: promotes drainage promotes fibrosis and decreases incidence of incontinence delineates anatomy usually done to spare muscle cutting Postoperative • sitz baths, irrigation and packing to ensure healing proceeds from inside to outside Complications • recurrence • rarely fecal incontinence
Pilonidal Disease Definition • acute abscess or chronic draining sinus in sacrococcygeal area Epidemiology • occurs most frequently in young men age 15-40 yrs Etiology • obstruction of the hair follicles in this area g formation of cysts, sinuses or abscesses Clinical Features • asymptomatic until acutely infected, then pain/tenderness, purulent discharge Treatment • acute abscess: incision and drainage wound packed open 40% develop chronic pilonidal sinuses • chronic disease: pilonidal cystotomy excision of sinus tract and cyst ± marsupialization (cyst edge sewn to surrounding tissue to leave sinus tract open)
hemorrhoids
Rectal Prolapse Definition • protrusion of full thickness of rectum through anus Epidemiology • extremes of ages – 5th decade • 85% women Etiology • lengthened attachment of rectum secondary to constant straining • 3 types: I – false/mucosal: redundant rectal mucosa, radial furrows II – incomplete: rectal intussusception without sliding hernia III – true/complete (most common) (see Figure 23): protrusion of entire rectal wall through anal orifice with herniation of pelvic peritoneum/ cul-de-sac circular furrows
True rectal prolapse
External hemorrhoids
Figure 23. Rectal Prolapse (true vs. false)
© Katie McCormack
Risk Factors • gynecological surgery • chronic neurologic/psychiatric disorders affecting motility Clinical Features • extrusion of mass with increased intra-abdominal pressure: straining, coughing, laughing, Valsalva • difficulty in bowel regulation: tenesmus, constipation, fecal incontinence • permanently extruded rectum with excoriation, ulceration and constant soiling • may be associated with urinary incontinence or uterine prolapse
Toronto Notes 2011
Anorectum/Liver
General Surgery GS41
Treatment • Types I and II (false/mucosal/incomplete): conservative – gentle replacement of prolapsed area, especially in children hemorrhoidectomy with excision of redundant mucosa, mostly in adults • Type III (true/complete): conservative – reduce if possible surgery – abdominal, perineal, transsacral approaches
Anal Neoplasms ANAL CANAL Squamous Cell Carcinoma (SCC) of Anal Canal (above dentate line) • most common tumour of anal canal (75%) • anus prone to human papilloma virus (HPV) infection, therefore at risk for anal squamous intraepithelial lesions (ASIL) high grade squamous intraepithelial lesion (HSIL) and low grade squamous intraepithelial lesion (LSIL) terminology used • clinical features: anal pain, bleeding, mass, ulceration • treatment: chemotherapy ± radiation ± surgery • prognosis: 80% 5-year survival Malignant Melanoma (MM) of Anal Canal • 3rd most common site for primary MM after skin, eyes • aggressive, distant metastases common at time of diagnosis • treatment: early radical surgery • prognosis: 5 cm), due to risk of malignancy and spontaneous rupture/ hemorrhage Focal Nodular Hyperplasia • pathogenesis: thought to be due to local ischemia and tissue regeneration • risk factors: female, middle age • clinical features: asymptomatic, rarely grows or bleeds, no malignant potential • investigations: central stellate scar on CT scan, technetium-99 scan is helpful • treatment: may be difficult to distinguish from adenoma (malignant potential) g often resected MALIGNANT LIVER NEOPLASMS Primary • usually hepatocellular carcinoma (HCC)/hepatoma • others include angiosarcoma, hepatoblastoma, hemangioendothelioma • epidemiology: uncommon in North America, but represents 20-25% of all carcinomas in Asia and Africa • risk factors: chronic liver inflammation: chronic hepatitis B (inherently oncogenic) and C, cirrhosis (especially macronodular), hemochromatosis, a1-anti-trypsin meds: OCPs (3x increased risk), steroids smoking, alcohol chemical carcinogens (aflatoxin, vinyl chloride – associated with angiosarcoma) • clinical features: RUQ discomfort, right shoulder pain jaundice, weakness, weight loss, ± fever hepatomegaly, bruit, rub ascites with blood (sudden intra-abdominal hemorrhage) paraneoplastic syndromes – e.g. Cushing’s syndrome, hypoglycemia metastasis: lung, bone, brain, peritoneal seeding • investigations: elevated ALP, bilirubin, and a-fetoprotein (80% of patients) U/S (poorly-defined margins with internal echos), triphasic CT (enhancement on arterial phase and washout on portal venous phase), MRI, CT or MRI angiography biopsy • treatment: cirrhosis is a relative contraindication to tumour resection due to decreased hepatic reserve surgical: resection (10% of patients have resectable tumours) liver transplant (if cirrhosis plus solitary nodule 3.5 g/dL
30-35 3.0-3.5
80% of infections ampicillin + gentamicin OR Cipro® + Flagyl® • cholecystectomy: early (within 72 hrs) vs. delayed (after 6 weeks) equal morbidity and mortality early cholecystectomy preferred: shorter hospitalization and recovery time emergent OR indicated if high risk, e.g. emphysematous, diabetic patient laparoscopic is standard of care (convert to open for complications or difficult case) laparoscopic: reduced risk of wound infections, shorter hospital stay, reduced post-op pain, increased risk of bile duct injury • intra-operative cholangiography (IOC): indications: clarify bile duct anatomy, obstructive jaundice, history of biliary pancreatitis, small stones in gallbladder with a wide cystic duct (>15 mm), single faceted stone in gallbladder, bilirubin >137 µmol/L • percutaneous cholecystostomy tube: critically ill or if general anesthetic contraindicated
Toronto Notes 2011
Biliary Tract
General Surgery GS47
Acalculous Cholecystitis Definition • acute or chronic cholecystitis in the absence of stones Pathogenesis • typically due to gallbladder stasis sludge forms in gallbladder Risk Factors • DM, immunosuppression, ICU admission, trauma patient, TPN, sepsis Clinical Features • see Acute Cholecystitis, GS46 • occurs in 20% of cases of acute cholecystitis Investigations • U/S: shows sludge in gallbladder, other U/S features of cholecystitis (see Acute Cholecystitis, GS46) • CT or HIDA scan Treatment • cholecystectomy • if patient unstable cholecystostomy
Choledocholithiasis Definition • stones in common bile duct (CBD) Clinical Features • 50% asymptomatic • often have history of biliary colic • tenderness in RUQ or epigastrium • acholic stool, dark urine, fluctuating jaundice • primary vs. secondary stones: primary: formed in bile duct, indicates bile duct pathology (e.g. benign biliary stricture, sclerosing cholangitis, choledochal cyst) secondary: formed in gallbladder (85% of cases in U.S.) Investigations • CBC: usually normal; leukocytosis suggests cholangitis • LFTs: increased bilirubin, ALP • amylase/lipase: to rule out gallstone pancreatitis • U/S: intra/extra-hepatic duct dilatation • ERCP, PTC • MRCP (90% sensitive, almost 100% specific, not therapeutic) Complications • cholangitis, pancreatitis, biliary stricture and biliary cirrhosis Treatment • if no evidence of cholangitis: treat with ERCP for CBD stone extraction possibly followed by elective cholecystectomy in 25% of patients
Acute Cholangitis Pathogenesis • obstruction of CBD leading to biliary stasis, bacterial overgrowth, suppuration and biliary sepsis Etiology • choledocholithiasis (60%), stricture, neoplasm (pancreatic or biliary), extrinsic compression (pancreatic pseudocyst or pancreatitis), instrumentation of bile ducts (PTC, ERCP), biliary stent • organisms: E. coli, Klebsiella, Pseudomonas, Enterococcus, B. fragilis, Proteus Clinical Features • Charcot’s triad – fever, RUQ pain, jaundice • Reynolds’ pentad – fever, RUQ pain, jaundice, shock, confusion • may have nausea, vomiting, abdominal distention, ileus, acholic stools, tea-coloured urine Investigations • CBC: elevated WBC + left shift • may have positive blood cultures • LFTs: obstructive picture (elevated ALP and conjugated bilirubin, mild increase in AST, ALT) • amylase/lipase: rule out pancreatitis • U/S: intra/extra-hepatic duct dilatation
Charcot’s Triad Fever, RUQ pain, jaundice.
Reynolds’ Pentad Fever, RUQ pain, jaundice, shock, confusion.
GS48 General Surgery
Common Bacteria in Biliary Tract KEEPS Klebsiella Enterococcus E. Coli, Enterobacter Proteus, Pseudomonas Serratia
Biliary Tract
Toronto Notes 2011
Treatment • initial: NPO, fluid and electrolyte resuscitation, ± NG tube, IV antibiotics • decompression: ERCP + sphincterotomy – diagnostic and therapeutic PTC with catheter drainage – if ERCP not available or unsuccessful laparotomy with CBD exploration and T-tube placement if above fails • all patients should also have a cholecystectomy, unless contraindicated Prognosis • suppurative cholangitis – mortality rate: 50%
Gallstone Ileus Pathogenesis • repeated inflammation causing a cholecystoenteric fistula (usually duodenal) g large gallstone enters the gut and impacts at or near the ileocecal valve, causing a true bowel obstruction (note: ileus is a misnomer in this context)
Bouveret’s Syndrome Gastric outlet/duodenal obstruction caused by a large gallstone passing through a cholecystogastric or cholecystoduodenal fistula.
Clinical Features • crampy abdominal pain, nausea, vomiting (see Bowel Obstruction, GS23) Investigations • AXR: dilated small intestine, air fluid levels, may reveal radiopaque gallstone, air in biliary tree (40%) • CT: biliary tract air, obstruction, gallstone in intestine Treatment • fluid resuscitation, NG decompression • surgery: enterotomy and removal of stone, inspect small and large bowel for additional proximal stones • fistula usually closes spontaneously • elective cholecystectomy after recovery if patient experiences gallbladder symptoms
Carcinoma of the Gallbladder Risk Factors • chronic symptomatic gallstones (70% of cases), old age, female, gallbladder polyps, porcelain gallbladder Clinical Features • majority are adenocarcinoma • may be incidental finding on elective cholecystectomy (~1% of elective cholecystectomies) • many patients are asymptomatic until late • local: vague RUQ pain, ± palpable RUQ mass • systemic: jaundice (50%) due to invasion of CBD or compression of CBD by pericholedochal nodes, weight loss, malaise, anorexia • early local extension to liver, may extend to stomach, duodenum • early metastasis common to liver, lung, bone Investigations • U/S: mural thickening, calcification, loss of interface between gallbladder and liver, fixed mass • endoscopic U/S (EUS): good for distinguishing carcinomas from other diagnoses such as polyps, good for staging, allows sampling of bile for cytology • abdominal CT: polypoid mass, mural thickening, liver invasion, nodal involvement, distant mets Treatment • if carcinoma of the gallbladder is suspected preoperatively, an open cholecystectomy should be done to avoid tumour seeding of trocar sites • confined to mucosa (rare) – cholecystectomy • beyond mucosa – cholecystectomy, en bloc wedge resection of 3-5 cm underlying liver, dissection of hepatoduodenal lymph nodes Prognosis • poor 5-year survival (10%) as gallbladder carcinoma is often detected late
Toronto Notes 2011
Biliary Tract/Pancreas
General Surgery GS49
Cholangiocarcinoma Definition • malignancy of extra intrahepatic bile ducts Risk Factors • age 50-70, gallstones, ulcerative colitis, primary sclerosing cholangitis, choledochal cyst, Clonorchis sinensis infection (liver fluke) Clinical Features • majority are adenocarcinoma • gradual signs of biliary obstruction: jaundice, pruritis, dark urine, pale stool • anorexia, weight loss, RUQ pain, Courvoisier’s sign (if CBD obstructed), hepatomegaly • early metastases are uncommon, but commonly tumour grows into portal vein or hepatic artery • Klatskin tumour – cholangiocarcinoma located at bifurcation of common hepatic duct Investigations • LFTs show obstructive picture • U/S, CT: bile ducts usually dilated, but not necessarily • ERCP or PTC: to determine resectability, for biopsies • CXR, bone scan: for metastatic workup Treatment • generally palliative • if resectable: biliary drainage and wide excision margin upper third lesions: duct resection + Roux-en-Y hepaticojejunostomy, ± liver resection middle third lesions (uncommon): duct resection + Roux-en-Y hepaticojejunostomy lower third lesions: Whipple procedure • unresectable lesions: stent or choledochojejunostomy (surgical bypass)
Obstructive jaundice is the most common presenting symptom for cholangiocarcinoma.
Courvoisier’s Sign Palpable, nontender distended gallbladder due to CBD obstruction. Present in 33% of patients with pancreatic carcinoma. The distended gallbladder could not be due to acute cholecystitis or stone disease because the gallbladder would actually be scarred and smaller, not larger.
Prognosis • radiotherapy useful for additional palliation, chemotherapy may be helpful • the more proximal to the liver, the worse the prognosis • overall 5-year survival – 15%
Pancreas Acute Pancreatitis • see Gastroenterology, G48 GALLSTONE PANCREATITIS Pathogenesis • obstruction of pancreatic duct by large or small gallstones and biliary sludge • backup of pancreatic enzymes can cause autodigestion of the pancreas
Ranson’s Criteria
Clinical Features (pancreatitis of any etiology) • pain (epigastric pain radiating to back), nausea, vomiting, ileus, peritoneal signs, jaundice, fever • Inglefinger’s sign: pain worse when supine, better when sitting forward • rarely may have coexistent cholangitis or pancreatic necrosis • Ranson’s criteria for determining prognosis of acute pancreatitis (see sidebar)
A. At admission 1. Age >55 years 2. WBC >16 x 109/L 3. Glucose >11 mmol/L 4. LDH ≥350 IU/L 5. AST >250 IU/L
Investigations • high amylase (higher than alcoholic pancreatitis), lipase, high liver enzymes, leukocytosis • U/S may show multiple stones (may have passed spontaneously), edematous pancreas • CXR, AXR, CT (if severe to evaluate for complications)
B. During initial 48 hours 1. Hct drop >10% 2. BUN rise >1.8 mmol/L 3. Arterial PO2 4 mmol/L 5. Calcium 6 L
Treatment • supportive • NPO, hydration, analgesia and antibiotics for severe cases of necrotizing pancreatitis or signs of sepsis • stone often passes spontaneously (~90%); usually no surgical management in uncomplicated acute pancreatitis • cholecystectomy during same admission after acute attack has subsided (25-60% recurrence if no surgery)
C. Interpretation ≥2 – difficult course ≥3 – high mortality
GS50 General Surgery
Pancreas
Toronto Notes 2011
• may need urgent ERCP + sphincterotomy if failure of conservative management (no benefit has been shown for early ERCP + sphincterotomy if no obstructive jaundice is present) • surgical indications in acute pancreatitis (rare): debridement and drain placement for necrotizing pancreatitis if refractory to medical management, if septic or in ICU without other sources of sepsis Complications • pseudocyst (collection of pancreatic secretions >4 weeks old surrounded by a defined wall of granulation tissue) • abscess/infection, necrosis • splenic/mesenteric/portal vessel thrombosis or rupture • pancreatic ascites/pancreatic pleural fluid effusion • diabetes • ARDS/sepsis/multiorgan failure • coagulopathy/DIC • encephalopathy • severe hypocalcemia
Chronic Pancreatitis • see also Gastroenterology, G50
The hallmark of chronic pancreatitis is epigastric pain radiating to the back.
Surgical Treatment • treatment is generally medical • indications for surgery: failure of medical treatment debilitating abdominal pain pseudocyst complications: persistence, hemorrhage, infection, rupture CBD obstruction (e.g. strictures), duodenal obstruction pancreatic fistula, variceal hemorrhage secondary to splenic vein obstruction rule out pancreatic cancer anatomical abnormality causing recurrent pancreatitis • pre-op CT and/or ERCP are mandatory to delineate anatomy • surgical options: drainage procedures – only effective if ductal system is dilated endoscopic duct decompression Puestow procedure (longitudinal pancreatojejunostomy) – improves pain in 80% of patients pancreatectomy – best option in absence of dilated duct proximal disease – Whipple procedure (pancreatoduodenectomy): pain relief in 80% distal disease – distal pancreatectomy ± Roux-en-Y pancreatojejunostomy total pancreatectomy – refractory disease nerve ablation: celiac plexus block – lasting benefit in 30% patients, much less invasive • pseudocyst (most resolve spontaneously with pancreatic rest): cyst wall must be mature (4-6 weeks) internal drainage (preferred): Roux-en-Y cyst-jejunostomy or cyst-gastrostomy external drainage: may require second operation to treat pancreatic fistula consider biopsy of cyst wall to rule out cystadenocarcinoma
Pancreatic Cancer Epidemiology • fourth most common cause of cancer-related mortality in both men and women in Canada in 2007 (Canadian Cancer Society) • male:female = 1.7:1, average age: 50-70 Risk Factors • increased age • smoking – 2-5x increased risk, most clearly established risk factor • high fat/low fibre diets, heavy alcohol use • DM, chronic pancreatitis • chemicals: betanaphthylamine, benzidine • African descent
Toronto Notes 2011
Pancreas
Clinical Features • head of the pancreas (70%): weight loss, obstructive jaundice, vague constant mid-epigastric pain (often worse at night, may radiate to back) painless jaundice (occurs more often with peri-ampullary), Courvoisier’s sign (see sidebar GS49) palpable tumour mass g generally incurable • body or tail of pancreas (30%): tends to present later and usually inoperable weight loss, vague mid-epigastric pain 300 µmol/L • U/S, contrast CT (also evaluates metastasis and resectability), ERCP Pathology • ductal adenocarcinoma – most common type (75-80%); exocrine pancreas • intraductal papillary mucinous neoplasm (IPMN) • other: mucinous cystic neoplasm (MCN), acinar cell carcinoma, islet-cell (insulinoma, gastrinoma, VIPoma, glucagonoma, somatostatinoma) Treatment • resectable (20% of pancreatic cancer) no involvement of liver, peritoneum or vasculature (hepatic artery, SMA, SMV, portal vein, IVC, aorta), no distant metastasis Whipple procedure (pancreatoduodenectomy) for cure – 5% mortality (Figure 26) distal pancreatectomy ± splenectomy, lymphadenectomy if carcinoma of midbody and tail of pancreas • non-resectable (palliative g relieve pain, obstruction) most body/tail tumours are not resectable (due to late presentation) relieve biliary/duodenal obstruction with endoscopic stenting or double bypass procedure (choledochoenterostomy + gastroenterostomy) chemotherapy (gemcitabine), radiotherapy – only slightly increase survival
Whipple Procedure (Pancreaticoduodenectomy) 1. Removal Choledochectomy Cholecystectomy Duodenectomy Distal pancreatectomy ± Distal gastrectomy 2. New Connections Hepaticojejunostomy (connect common hepatic duct to jejunum post cholecystectomy) Pancreaticojejunostomy (connect distal pancreas remnant) Gastrojejunostomy
Prognosis • most important prognostic indicators are lymph node status, size >3 cm, perineural invasion (invasion of tumour into microscopic nerves of pancreas) • overall 5 year survival is 1% • average survival – 6 months if unresected, 12-18 months with curative resection Left and right hepatic ducts
Common hepatic duct Stomach
Liver
Gastrojejunostomy
Gallbladder Tail of pancreas Cystic duct Pancreas
Common bile duct Ampulla of vater
Pancreatic duct
Duodenum
Hepaticjejunostomy Pancreaticojejunostomy
Resected portion
Figure 26. Schematic of Whipple Resection, Showing the Resected Components
© Caitlin O’Connell 2009
Jejunum
GS52 General Surgery
Spleen/Breast
Toronto Notes 2011
Spleen Splenic Trauma • typically from blunt trauma (especially in people with splenomegaly) • most common intra-abdominal organ injury in blunt trauma • may have Kehr’s sign: left shoulder pain due to diaphragmatic irritation from splenic rupture Treatment • in stable patients – extended bed rest with serial hematocrit levels, close monitoring • hemostatic control • splenic artery embolization • splenorrhaphy (suture of spleen) – if patient hemodynamically stable, patient has stopped bleeding and laceration does not involve hilum • partial splenectomy • total splenectomy if patient unstable or high-grade injury
Splenectomy Indications • splenic trauma (most common reason for splenectomy), hereditary spherocytosis, primary hypersplenism, chronic immune thrombocytopenia purpura (ITP), splenic vein thrombosis causing esophageal varices, splenic abscess, thrombotic thrombocytopenia purpura (TTP), non-Hodgkin’s lymphoma, primary splenic tumour (rare) • does not benefit all thrombocytopenic states (e.g. infection, most malignancies involving the bone marrow, drugs/toxins) • probability of cure of ITP by splenectomy is 60-70%, may be predicted by response to IVIg Complications • short-term: atelectasis of left lower lung, bleeding, infection injury to surrounding structures (e.g. gastric wall, tail of pancreas) post-op thrombocytosis, leukocytosis subphrenic abscess • long-term: post-splenectomy sepsis (encapsulated organisms): 4% of splenectomized patients 50% mortality pre-op prophylaxis with vaccinations (pneumococcal, H. influenzae and meningococcus) liberal use of penicillin especially in children 40 years old: mammography every 3 years no strong evidence for avoidance of xanthine-containing products (coffee, tea, chocolate, cola) analgesia (ibuprofen, ASA) for severe symptoms: OCP, danazol, bromocriptine PROLIFERATIVE LESIONS – No Atypia Fibroadenoma • most common benign breast tumour in women under age 30 • risk of subsequent breast cancer is increased only if fibroadenoma is complex, there is adjacent atypia or a strong family history of breast cancer • clinical features: nodules: smooth, rubbery, discrete, well-circumscribed, non-tender, mobile, hormone dependent unlike cysts, needle aspiration yields no fluid • investigations: core or excisional biopsy required ultrasound and FNA alone cannot differentiate fibroadenoma from phyllodes tumour • treatment: generally conservative: serial observation consider excision if size 2-3 cm and rapidly growing on serial ultrasound, if symptomatic or patient preference Intraductal Papilloma • solitary intraductal benign polyp • present as nipple discharge (most common cause of spontaneous, unilateral bloody nipple discharge), breast mass, nodule on U/S • can harbour areas of atypia or DCIS • treatment: excision of involved duct to ensure no atypia Ductal Hyperplasia Without Atypia • increased number of cells within the ductal space • cells retain benign cytology • no treatment required • slightly increased cancer risk if moderate or florid hyperplasia PROLIFERATIVE LESIONS – With Atypia Atypical Hyperplasias • can involve ducts (ductal hyperplasia with atypia) or lobules (lobular hyperplasia with atypia) • cells lose apical-basal orientation • increased risk of breast cancer • diagnosis: core or excisional biopsy • treatment: complete resection, risk modification (avoid exogenous hormones), close follow-up OTHER LESIONS Fat Necrosis • uncommon, result of trauma (may be minor, positive history in only 50%), after breast surgery (i.e. reduction) • firm, ill-defined mass with skin or nipple retraction, ± tenderness • regress spontaneously, but complete imaging ± biopsy to rule out carcinoma
DDx for Breast Mass • Breast Ca • Fibrocystic changes • Fibroadenoma • Fat necrosis • Papilloma/papillomatosis • Galactocele • Duct ectasia • Ductal/lobular hyperplasia • Sclerosing adenosis • Lipoma • Neurofibroma • Granulomatous mastitis (e.g. TB, Wegener’s, sarcoidosis) • Abscess • Silicone implant
GS54 General Surgery
Breast
Toronto Notes 2011
Mammary Duct Ectasia • obstruction of a subareolar duct leading to duct dilation, inflammation, and fibrosis • may presesnt with nipple discharge, bluish mass under nipple, local pain • risk of secondary infection (abscess, mastitis) • resolves spontaneously Montgomery Tubercle • Montgomery tubercles aka Morgagni tubercles are papular projections at the edge of the areola • obstruction of these glands can lead to inflammation or cystic collections (cyst of Montgomery aka retroareolar cyst) • if signs of secondary infection, start treatment for mastitis • resolves spontaneously in weeks to years Abscess • lactational (see Obstetrics, OB50) vs. periductal/subareolar • unilateral localized pain, tenderness, erythema, subareolar mass, nipple discharge, nipple inversion • rule out inflammatory carcinoma, as indicated • treatment: initially broad-spectrum antibiotics and I&D, if persistent total duct excision (definitive) • if mass does not resolve: fine needle aspiration (FNA) to exclude cancer, U/S to assess for presence of abscess
Breast Cancer Gender followed by age are the two greatest risk factors for breast cancer.
Epidemiology • 2nd leading cause of cancer mortality in women (1st is lung cancer) • 1/9 women in Canada will be diagnosed with breast cancer in their lifetime • 1/27 women in Canada will die from breast cancer Risk Factors • gender (99% female) • age (80% >40 years old) • most important risk factors are prior history of breast cancer and/or prior breast biopsy (regardless of pathology) • 1st degree relative with breast cancer (greater risk if relative was premenopausal) • increased risk with high breast density, nulliparity, first pregnancy >30 years old, menarche 55 years old • decreased risk with lactation, early menopause, early childbirth • radiation exposure (e.g. Mantle radiation for Hodgkin’s disease) • >5 years HRT
Any palpable dominant breast mass requires further investigation.
Diagnostic mammography is indicated in all patients, even in women liver > brain • treatment is palliative: hormone therapy, chemotherapy, radiation
General Surgery GS57
Breast conserving surgery can be offered to most women with stage I/ II disease.
Twenty-year Follow-up of a Randomized Study comparing Breast-conserving Surgery with Radical Mastectomy for Early Breast Cancer Veronesi U, et al. NEJM 2002; 16:1227-32 Background: Women enrolled in a randomized trial to compare the efficacy of radical mastectomy (RM) with that of breast-conserving surgery (BCS) were followed over a 20-year period for long-term outcomes including disease recurrence and survival. Methods: From 1973-1980, 701 women with breast cancers measuring > cyst, carcinoma, pheochromocytoma, neuroblastoma • metastasis to adrenal gland from: lung > breast, colon, lymphoma, melanoma, kidney • peak incidence of carcinoma: females ages 50-60, risk decreases with increasing age and male gender Investigations • MRI, CT: size >6 cm is best predictor of primary adrenal carcinoma (92% are >6 cm) • functional studies: pheochromocytoma: 24 hour urine epinephrine, norepinephrine, metanephrine, normetanephrine, VMA (vanillylmandelic acid) Cushing's: 24 hour urine cortisol or 1 mg overnight dexamethasone suppression test aldosteronoma: electrolytes, aldosterone: renin level, saline suppression test if appropriate adrenal androgens: 17-OH progesterone, DHEAS • FNA biopsy: if suspect metastasis to adrenal (must exclude pheochromocytoma first) indicated if history of cancer or patient is smoker • iodocholesterol scintigraphy: may distinguish benign vs. malignant disease Treatment • functional tumour: resect • non-functioning tumour: >6 cm: resect 3-6 cm: MRI (T2 density, shape, margins), more likely to resect in females and if 65 years old and ~50% of people >80 years old fall each year approximately 20% of falls require medical attention 5% of falls lead to hospitalization 5-10% with serious injuries (e.g. hip fracture, head injury, laceration) 1-2% of falls associated with hip fracture 15% die in hospital, 33% 1-year mortality between 25-75% do not recover to previous level of ADL function mortality increases with age (171/100,000 in men >85 years old) and type of injury (25% with hip fracture die within 6 months) Complete History & Physical Exam
Specific Tests Get Up & Go test Chair Stand Romberg test, Pull test 20 foot walk with 360º turns
Intervention Resistance training Quadriceps strengthening
Poor Balance Positive Romberg test Positive Pull test Poor vision
Medication Toxicity Alcohol Anticholinergics Anticonvulsants Antihypertensives Digoxin Nitrates Sedatives
Hypotension Orthostatic Postprandial
Intervention Balance training Widen support base (i.e. shoes, cane, walker) Correct vision
Intervention Medication review Reduce or eliminate nitrates, benzodiazepines, neuroleptics, antihypertensives (if possible)
Intervention Medication review Behaviour changes (separate meals and medications, exercise) Volume maintenance (compression stockings, salt intake)
Interventions Appropriate for All Patients with Falls Evaluation and correction of loose rugs, cords, poor lighting, movable furniture, bathtubs, thresholds, clutter
Figure 2. Approach to Falls in the Elderly
I HATE FALLING Inflammation of joints Hypotension (orthostatic changes) Auditory and visual abnormalities Tremor Equilibrium (balance) problem Foot Problems Arrhythmia, heart block or valvular disease Leg-length discrepancy Lack of conditioning (generalized weakness) Illness Nutrition Gait disturbance Fuller, G. Falls in the elderly. Am Fam Phys 2001; 61(7): 2159-2172.
Drugs That May Increase the Risk of Falling Sedative-hypnotic and anxiolytic drugs (especially long-acting benzodiazepines) Tricyclic antidepressants Major tranquilizers (phenothiazines and butyrophenones) Antihypertensive drugs Cardiac medications Corticosteroids Nonsteroidal anti-inflammatory drugs Anticholinergic drugs Hypoglycemic agents Alcohol Fuller, G. Falls in the elderly. Am Fam Phys 2001; 61(7): 2159-2172.
Identification of Precipitating Activity
Weakness of Leg Extension Impaired chair stand Slow gait Poor stair climbing
Key Physical Findings in the Elderly Patient Who Falls or Nearly Falls
Adapted from: Ganz, DA, Bao Y, Shekelle PE, Rubenstein LZ. Will my patient fall? JAMA 2007; 297: 77-86. Tinetti ME, Baker DI, McAvay G, et al. A multifactorial intervention to reduce the risk of falling among elderly people living in the community. NEJM 1994; 331(13):821-827.
Will My Patient Fall? JAMA 2007; 297:77-86 Purpose: To identify the prognostic value of risk factors for future falls among older patients. Study Selection: Prospective cohort studies of risk factors for falls that performed a multivariate analysis of such factors. Results: Clinically identifiable risk factors were identified across 6 domains: orthostatic hypotension, visual impairment, impairment of gait or balance, medication use, limitations in basic or instrumental activities of daily living and cognitive impairment. Eighteen studies met inclusion criteria and provided a multivariate analysis including at least 1 of the risk factor domains. The estimated pretest probability of falling at least once in any given year for individuals 65 years and older was 27% (95% confidence interval, 19%-36%). Patients who have fallen in the past year are more likely to fall again [likelihood ratio range, 2.3-2.8]. The most consistent predictors of future falls are clinically detected abnormalities of gait or balance (likelihood ratio range, 1.7-2.4). Visual impairment, medication variables, decreased activities of daily living and impaired cognition did not consistently predict falls across studies. Orthostatic hypotension did not predict falls after controlling for other factors. Conclusions: Screening for risk of falling during the clinical examination begins with determining if the patient has fallen in the past year. For patients who have not previously fallen, screening consists of an assessment of gait and balance. Patients who have fallen or who have a gait or balance problem are at higher risk of future falls.
GM6 Geriatric Medicine
Fall Prevention Tips 1. Improve lighting, especially on stairs 2. Caution while adjusting to new bifocal prescription (poor depth perception) 3. Siderails in bathtubs 4. Railings on steps 5. Connect patient to lifeline button signaling systems 6. Remove loose mats or carpets, telephone cords and other tripping hazards 7. Recommend support hose for varicose veins and swelling of ankles Goldlist B, Turpic I, Borins M. (1997). Essential Geriatrics: Managing 6 conditions: Patient Care Canada. 8(9).
Medication as a Risk Factor for Falls: Critical Systematic Review J Gerontol A Biol Sci Med Sci 2007; 62(10):1172-81 Purpose: To review all original articles systematically examining medication use as a risk factor for falls or fall-related fractures in people aged more than 60 years. Study Selection: Studies investigating “falls” or “accidental falls” and “pharmaceutical preparations” or specific groups of drugs were included. Studies not meeting the age criterion, not controlled with nonusers of target medicines or nonfallers, or with no clear definition of target medication were excluded. Results: Twenty-eight observational studies and one randomized controlled trial met the inclusion criteria. The outcome measure was a fall in 22 studies and a fracture in 7 studies. The main group of drugs associated with an increased risk of falling was psychotropics: benzodiazepines, antidepressants, and antipsychotics. Antiepileptics and drugs that lower blood pressure were weakly associated with falls. Conclusions: Central nervous system drugs, especially psychotropics, seem to be associated with an increased risk of falls. The quality of observational studies needs to be improved, as many appear to lack even a clear definition of a fall, target medicines, or prospective follow-up. Many drugs commonly used by older persons are not systematically studied as risk factors for falls.
Interventions for Preventing Falls in Older People Living in the Community Cochrane Database Syst Rev 2009; 2:CD007146 Study: Cochrane systematic review. 11 RCT and quasi-RCT trials. Population: 55,303 Patients over 60 or clearly elderly, senior, or older and living in the community. Intervention: Any intervention to prevent falls. Primary Outcomes: Rate of falls and number of fallers. Results: Exercise is effective in reducing risk and rate of falls. Multifactorial assessment with a multi-professional team reduces rates of falls but not risk of falls. Limited evidence shows that environmental assessments and interventions may not reduce risk or rate of falls. Vitamin D does not appear to be effective in reducing rate of falls in all patients. Cardiac pacing in patients with carotid sinus hypersensivity and history of syncope or falls reduces rate of falls. Home-based physiotherapy does not benefit patients with Parkinson’s or stroke related mobility problems. Conclusions: Exercise interventions reduce risk of falls in the elderly. Additional research is required to confirm and elaborate the contexts in which other interventions are effective.
Differential Diagnoses of Common Presentations
Toronto Notes 2011
Etiology • commonly multifactorial • extrinsic environmental (e.g. home layout, lighting, stairs, footwear), accidental, abuse medications/substances (e.g. alcohol) month after hospital discharge, acute illness, exacerbation of chronic illness • intrinsic orthostasis/syncope age-related changes and diseases associated with aging: musculoskeletal (arthritis, muscle weakness), sensory (visual, proprioceptive, vestibular), cognitive (depression, dementia, delirium, anxiety), cardiovascular (CAD, arrhythmia, MI, low BP), neurologic (stroke, decreased LOC, gait disturbances/ataxia), metabolic (glucose, electrolytes) Investigations • directed by history and physical • CBC, electrolytes, BUN, creatinine, glucose, Ca, TSH, B12, urinalysis, cardiac enzymes, ECG, CT head Prevention • multidisciplinary, multifactorial, health and environment risk factor screening and intervention programs in the community • program of muscle strengthening, balance retraining and group exercise programs (e.g. tai chi) • home hazard assessment and modification (e.g. remove rugs, add shower bars, etc.) • withdrawal of psychotropic medication • cardiac pacing for those with cardio-inhibitory carotid sinus hypersensitivity • optimize eyesight and footwear
Fecal Incontinence Epidemiology • second leading cause of nursing home placement Etiology • commonly multifactorial • pelvic floor intact neurologic conditions – age-related, neuropathy, multiple sclerosis, stroke, dementia tumour/trauma (e.g. brain, spinal cord, cauda equina) overflow (e.g. encopresis, impaction) diarrheal conditions • pelvic floor affected trauma/surgery nerve/sphincter damage malformation, anorectal Risk Factors • prior vaginal delivery • anorectal surgery • pelvic radiation • diabetes • neurologic disease • diarrheal conditions Investigations (if cause not apparent from history and physical) • stool studies • endorectal ultrasound • colonoscopy, sigmoidoscopy, anoscopy • anorectal manometry/functional testing Management • diet/bulking agent if stool is liquid or loose • disimpaction • anti-diarrheal agents • regular defecation program in patients with dementia • counsel about biofeedback therapy (retraining of pelvic floor muscles)
Gait Disorders • see Neurology, N36
Toronto Notes 2011
Differential Diagnoses of Common Presentations
Hazards of Hospitalization Table 4. Recommendations for Sequelae of Hospitalization in Older Patients Sequelae
Recommendations
Malnutrition
No dietary restrictions (except diabetes), assistance, dentures if necessary, eating out of bed
Urinary incontinence
Medication review, remove environmental barriers, discontinue use of catheter
Depression
Routine screening
Adverse drug event
Medication review
Confusion/delirium
Orientation, visual and hearing aids, volume repletion, noise reduction, early mobilization, medication review, remove restraints
Pressure ulcers
Low-resistance mattress, daily inspection, repositioning every 2 hours
Infection
Early mobilization, remove unnecessary IV lines, catheters, NG tubes
Falls
Appropriate footwear, assistive devices, early mobilization, remove restraints, medication review
Hypotension/dehydration
Early recognition and repletion
Diminished aerobic capacity/loss Early mobilization of muscle strength/contractures Decreased respiratory function
Incentive spirometry, physiotherapy
Hypertension • see Family Medicine, FM35 • 60-80% of elderly (>65 years old) have hypertension 60% of these have isolated systolic HTN • non-pharmacologic treatments are first-line, then thiazide monotherapy is recommended • add ACEI/ARB if also atherosclerosis, DM, CHF or chronic kidney disease • add beta-blockers if also angina or CHF • target BP: sBP 40 years are at higher risk of endometrial cancer must do endometrial biopsy in all women presenting with postmenopausal bleeding to exclude endometrial cancer • D&C: not for treatment; diagnosis only (usually with hysteroscopy)
Gynecology GY13
GY14 Gynecology Dysfunctional Uterine Bleeding Abnormal bleeding not attributable to organic (anatomic/systemic) disease. DUB is a diagnosis of exclusion. Anovulatory AUB often used synonymously with DUB.
Disorders of Menstruation
Toronto Notes 2011
Treatment • treat underlying disorders: if anatomic lesions and systemic disease have been ruled out, consider dysfunctional uterine bleeding (DUB) • medical mild DUB (see sidebar) NSAIDs anti-fibrinolytic (e.g. Cyklokapron®) at time of menses combined OCP progestins (Provera®) on first 10-14 days of each month if oligomenorrheic Mirena® IUD danazol acute, severe DUB replace fluid losses, consider admission medical treatment a) estrogen (Premarin®) 25 mg IV q4h x 24h with Gravol® 50 mg IV/PO q4h or b) Ovral® 1 tab PO q4h x 24h with Gravol® 50 mg IV/PO q4h – taper Ovral®: 1 tab tid x 2d g bid x 2d g OD after (a) or (b), maintain patient on monophasic OCP for next several months or consider alternative medical treatment clomiphene citrate consider in patients who are anovulatory and who wish to get pregnant • surgical endometrial ablation; consider pretreatment with danazol or GnRH agonists if finished childbearing repeat procedure may be required if symptom recurrence hysterectomy: definitive treatment
Dysmenorrhea Primary Dysmenorrhea Menstrual pain in absence of organic disease. Secondary Dysmenorrhea Menstrual pain due to organic disease.
Etiology • see Differential Diagnoses of Common Presentations, GY6 Table 3. Comparison of Primary and Secondary Dysmenorrhea Primary Dysmenorrhea
Secondary Dysmenorrhea
Features
Menstrual pain in absence of organic disease Begins 6 months-2 years after menarche (once ovulatory cycles established)
Menstrual pain due to organic disease Usually begins in women who are in their 20s, worsens with age May improve temporarily after childbirth
Signs and Symptoms
Colicky pain in abdomen, radiating to the lower back, labia, Associated dyspareunia, abnormal bleeding, and inner thighs beginning hours before onset of bleeding infertility and persisting for hours or days (48-72 h) Associated symptoms: nausea, vomiting, altered bowel habits, headaches, fatigue [prostaglandin (PG)-associated]
Diagnosis
Associated dyspareunia, abnormal bleeding, infertility Rule out underlying pelvic pathology and confirm cyclic nature of pain
Bimanual exam: uterine or adnexal tenderness, fixed uterine retroflexion, uterosacral nodularity, pelvic mass, or enlarged irregular uterus U/S, laparoscopy and hysteroscopy may be necessary to establish the diagnosis
Treatment
PG synthetase inhibitors (e.g. Anaprox®) • Should be started before onset of pain OCP: suppress ovulation/reduce menstrual flow
Treat underlying cause
Toronto Notes 2011
Endometriosis
Gynecology GY15
Endometriosis Etiology • not fully understood • proposed mechanisms (combination likely involved) retrograde menstruation (Sampson’s theory) seeding of endometrial cells by transtubal regurgitation during menstruation endometrial cells most often found in dependent sites of the pelvis immunologic theory: altered immunity may limit clearance of transplanted endometrial cells from pelvic cavity (may be due to decreased NK cell activity) metaplasia of coelomic epithelium undefined endogenous biochemical factor may induce undifferentiated peritoneal cells to develop into endometrial tissue extrapelvic disease may be due to abherrant vascular or lymphatic dissemination of cells e.g. ovarian endometriosis may be due to direct lymphatic flow from uterus to ovaries Epidemiology • incidence: 15-30% of pre-menopausal women • mean age at presentation: 25-30 years • regresses after menopause Risk Factors • family history (7-10 fold increased risk if affected 1st degree relative) • obstructive anomalies of the genital tract (earlier onset) • nulliparity • age >25 years
Endometriosis The presence of endometrial tissue (glands and stroma) outside of the uterine cavity.
Differential Diagnosis 1. Chronic PID, recurrent acute salpingitis 2. Hemorrhagic corpus luteum 3. Benign/malignant ovarian neoplasm 4. Ectopic pregnancy
Endometrioma = endometriotic cyst on surface of ovary.
Sites of Occurrence • ovaries: 60% patients have ovarian involvement • broad ligament, vesicoperitoneal fold • peritoneal surface of the cul-de-sac, uterosacral ligaments • rectosigmoid colon, appendix • rarely may occur in sites outside abdomen/pelvis, including lungs Clinical Features • may be asymptomatic • history menstrual symptoms cyclic symptoms due to growth and bleeding of ectopic endometrium, usually precede menses (24-48 hrs) and continue throughout and after flow secondary dysmenorrhea sacral backache with menses pain may eventually become chronic, worsening perimenstrually premenstrual and postmenstrual spotting deep dyspareunia infertility 30-40% of patients with endometriosis will be infertile 15-30% of those who are infertile will have endometriosis bowel and bladder symptoms frequency, dysuria, hematuria diarrhea, constipation, hematochezia, dyschezia • physical tender nodularity of uterine ligaments and cul-de-sac felt on rectovaginal exam fixed retroversion of uterus firm, fixed adnexal mass (endometrioma) physical findings not present in adolescent population Investigations • definitive diagnosis requires: direct visualization of lesions typical of endometriosis at laparoscopy biopsy and histologic exam of specimens (2 or more of: endometrial epithelium, glands, stroma, hemosiderin-laden macrophages) • laparoscopy mulberry spots: dark blue or brownish-black implants on the uterosacral ligaments, cul-desac or anywhere in the pelvis endometrioma: “chocolate” cysts on the ovaries “powder-burn” lesions on the peritoneal surface early white lesions and clear blebs peritoneal “pockets” • CA-125 may be elevated in patients with endometriosis
There may be little correlation between the extent of endometriosis and symptomatology.
Classic Triad of Endometriosis • Dysmenorrhea • Dyspareunia (cul de sac, uterosacral ligament) • Dyschezia (uterosacral ligament, cul-de-sac, rectosigmoid attachment)
A sharp, firm, and exquisitely tender “barb” on the uterosacral ligament is a classic feature of endometriosis.
GY16 Gynecology
Endometriosis is classified according to a scoring system standardized by the American Society for Reproductive Medicine. Score is based on location and extent of disease.
Recurrence Rates Medical therapy: 30-50% Conservative surgery: 14-40%
Endometriosis/Adenomyosis/Leiomyomata (Fibroids)
Toronto Notes 2011
Treatment • depends on certainty of the diagnosis, severity of symptoms, extent of disease, desire for future fertility and impact to GI/GU systems (e.g. intestinal obstruction) • medical NSAIDs – e.g. naproxen sodium (Anaprox®) pseudopregnancy cyclic/continuous estrogen-progestin (OCP) medroxyprogesterone (Depo-Provera®) pseudomenopause 2nd line: only short-term (35 years old; found in 20-40% of hysterectomy specimens • mean age at presentation: 40-50 years old (older age group than seen in endometriosis) • adenomyosis is a common histologic finding in asymptomatic patients Clinical Features • often asymptomatic • menorrhagia, secondary dysmenorrhea, pelvic discomfort • dyspareunia, dyschezia • uterus symmetrically bulky, usually 35 years • more common, larger and occur at earlier age in black women • common indication for major surgery in females • minimal malignant potential (1:1000) • typically regress after menopause; enlarging fibroids in a postmenopausal woman should prompt consideration of malignancy
Toronto Notes 2011
Leiomyomata (Fibroids)
Pathogenesis • estrogen stimulates monoclonal smooth muscle proliferation; progesterone stimulates production of proteins that inhibit apoptosis • degenerative changes (occur when tumour outgrows blood supply) hyaline degeneration (most common degenerative change) cystic degeneration (from breakdown of hyaline) red/carneous degeneration (hemorrhage into tumour, may occur in pregnancy) fatty degeneration calcification sarcomatous degeneration (rare) parasitic myoma: tumour becomes attached to another organ (typically omentum or small bowel mesentery), develops new blood supply and loses connection to uterus Clinical Features • majority asymptomatic (60%), often discovered as incidental finding on pelvic exam or U/S • abnormal uterine bleeding (30%): dysmenorrhea, menorrhagia • pressure/bulk symptoms (20-50%) pelvic pressure/heaviness increased abdominal girth urinary frequency and urgency acute urinary retention (extremely rare but surgical emergency!) constipation, bloating (rare) • acute pelvic pain fibroid degeneration fibroid torsion (pedunculated subserosal) • infertility (submucosal), recurrent pregnancy loss • pregnancy complications (potential enlargement and increased pain, obstructed labour, difficult C-section) Investigations • bimanual exam: uterus asymmetrically enlarged, usually mobile • CBC: anemia • ultrasound: to confirm diagnosis and assess location of fibroids • sonohysterogram: useful for differentiating endometrial polyps from submucosal fibroids • endometrial biopsy to rule out uterine cancer for abnormal uterine bleeding (especially if age >40 years) • occasionally MRI is used for pre-op planning (e.g. before myomectomy) Treatment • only if symptomatic, rapidly enlarging or menorrhagia • treat anemia if present • conservative approach (watch and wait) symptoms absent or minimal fibroids 40 yrs – always do endometrial biopsy to rule out cancer.
GY18 Gynecology
Contraception
Toronto Notes 2011
Contraception • see Family Medicine, FM19 Table 4. Classification of Contraceptive Methods Counselling the Adolescent about Contraception More than 90% of adolescent pregnancies are unintended and approximately 50% of all pregnancies occur within the first 6 months of initiating sexual activity. In addition, 85% of sexually active women become pregnant within 1 year if no contraception is used and even some of the least effective contraceptive methods markedly decrease the risk of pregnancy. Rimsza, ME. Counselling the Adolescent about Contraception. Pediatr Rev 2003; 24;162.
Risk of Thromboembolism in Women Taking Ethinylestradiol/Drospirenone and Other Oral Contraceptives. Obstet Gynecol 2007; 110(3):587-93. Purpose: To compare the association between thromboembolism and the use of ethyinylestradiol/ drospirenone (ie. Yasmin) or other other oral contraceptives. Study: Cohort study used a health insurer database and followed patients for an average of 7.6 months. Patients: 22,429 women who had initiated ethyinylestradiol/drospirenone were matched to 44,858 who had initiated other oral contraceptives between the age of 10 and 59 years. Main Outcome: Thromboembolic events. Results: The incidence rate of thromboembolism was similar between women intiating ethyinylestradiol/drospirenone (1.3 per 1000 woman-years) compared to initiating other OCPs (1.4 per 1000 woman-years) (RR 0.9l; 95% CI 0.5 – 1.6). Specific events, including deep vein thrombosis and pulmonary embolism also occurred with similar frequencies. Summary: The risk of thromboembolism is similar in ethyinylestradiol/drospirenone compared to other oral contraceptives.
Suppression of Ovarian Activity with a Drospirenon-Containing Oral Contraceptive in a 24/4 Regimen Contraception 2008; 78:16-25 Study: Double-blind randomized. Patients: Women aged 18-35 years post-ovulation or had a follicular diameter >15 mm before day 23 during a pre-treatment cycle. Intervention: Drospirenone 3mg plus ethinylestradiol 20 mcg administered in 24/4 regimen vs. 21/7 regimen. Outcome: Suppression of ovarian activity (Hoogland score). Results: Women on a 24/4 regimen had greater and more consistent ovarian suppression than the 21/7 group. 87.8% in the 24/4 group had no ovarian activity vs. 56% in the 21/7 group.
Irregular breakthrough bleeding often occurs in the first few months after starting OCP. Usually resolves after three cycles.
Type
Effectiveness (perfect use, typical use)
Physiological Withdrawal/coitus interruptus Rhythm method/calendar/mucous/symptothermal Lactational amenorrhea Chance – no method used Abstinence of all sexual activity
77.0% 98.0%, 76.0% 98% (first 6 months postpartum) 10.0% 100.0%
Barrier Methods Condom alone Spermicide alone Sponge – Parous – Nuliparous Diaphragm with spermicide Female condom Cervical cap – Parous – Nuliparous
98.0%, 85.0% 82.0%, 71.0% 80.0%, 68.0% 91.0%, 84.0% 94.0%, 84.0% 95.0%, 79.0% 74.0%, 68.0% 91.0%, 84.0%
Hormonal OCP Nuvaring Transdermal (Ortho Evra®) Depo-Provera® Progestin-only pill (Micronor®) Mirena® IUD
99.7%, 92.0% 99.7%, 92.0% 99.7%, 92.0% 99.7%, 97.0% 90-99% 99.9%
Copper IUD
99.3%
Surgical Tubal ligation Vasectomy
99.65% 99.9%
Emergency Postcoital Contraception (EPC) Yuzpe method “Plan B” levonorgestrel only Postcoital IUD
98% (within 24 hours) 98% (within 24 hours) 99.9%
Effectiveness: percentage of women reporting no pregnancy after 1 year of use.
Hormonal Methods Combined Oral Contraceptive Pills (OCP) • most contain low dose ethinyl estradiol (20-35 µg) plus progestin (norethinedrone, norgestrel, levonorgestrel, desogestrel, norgestimate, drospirenone) • failure rate (0.3% to 8%) depending on compliance • monophasic or triphasic formulations (varying amount of progestin throughout cycle) Transdermal (Ortho Evra®) • continuous release of 6 mg norelgestromin and 0.60 mg ethinyl estradiol into bloodstream • applied to lower abdomen, back, upper arm, buttocks, NOT breast • worn for 3 consecutive weeks (changed every week) with 1 week off to allow menstruation • as effective as OCP in preventing pregnancy (>99% with perfect use) • may be less effective in women >90 kg body weight • may not be covered by drug plans Contraceptive Ring (Nuvaring®) • thin flexible plastic ring; releases etonogestrel 120 µg/d and estradiol 15 µg/d • works for 3 weeks then removed for 1 week to allow menstruation • as effective as OCP in preventing pregnancy (98%) • avoids first pass effect • side effects: vaginal infection/irritation, vaginal discharge • may have better cycle control, i.e. decreased breakthrough bleeding Starting Hormonal Contraceptives • thorough history and physical examination including blood pressure and breast exam • follow-up visit 6 weeks after hormonal contraceptives prescribed • pelvic exam can be delayed until a subsequent visit
Toronto Notes 2011
Contraception
Gynecology GY19
Table 5. Combined Estrogen and Progestin Contraceptive Methods Mechanism of Action
Advantages
Side Effects
Contraindications
• Ovulatory suppression through inhibition of LH and FSH • Decidualization of endometrium • Thickening of cervical mucus resulting in decreased sperm penetration
• Highly effective, reversible • Cycle regulation • Decreased dysmenorrhea and menorrhagia (less anemia) • Decreased benign breast disease and ovarian cyst development • Decreased risk of ovarian and endometrial cancer • Increased cervical mucus which may lower risk of STIs • Decreased PMS symptoms • Improved acne • Osteoporosis protection (possibly)
Estrogen-related • Nausea • Breast changes (tenderness, enlargement) • Fluid retention/bloating/edema • Weight gain (rare) • Migraine, headaches • Thromboembolic events • Liver adenoma (rare) • Breakthrough bleeding (low estradiol levels)
Absolute • Known/suspected pregnancy • Undiagnosed abnormal vaginal bleeding • Prior thromboembolic events, thromboembolic disorder (Factor V Leiden mutation; protein C, S or antithrombin III deficiency), active thrombophlebitis • Cerebrovascular or coronary artery disease • Estrogen-dependent tumours (breast, uterus) • Impaired liver function associated with acute liver disease • Congenital hypertriglyceridemia • Smoker age >35 years • Migraines with focal neurological symptoms (excluding aura) • Uncontrolled hypertension
Progestin-related • Amenorrhea/breakthrough bleeding • Headaches • Breast tenderness • Increased appetite • Decreased libido • Mood changes • Hypertension • Acne/oily skin* • Hirsutism*
Relative • Migraines – non-focal with aura 24 hour culture ICSI (intracytoplasmic sperm injection) IUI (intrauterine insemination) ± oocyte or sperm donors IVM (in vitro maturation)
Male Factors • see Urology, U34
Normal Semen Analysis (WHO criteria) • Must be obtained after 48-72 hours of abstinence 1. Volume 2-5 cc 2. Count >20 million/cc 3. Motility >50% forward progression 4. Morphology >30% normal 5. Absence of pyospermia, hyperviscosity, agglutination NB: does not assess sperm function
Toronto Notes 2011
Etiology • varicocele (>40%) • idiopathic (>20%) • obstruction (~15%) • cryptorchidism (~8%) • immunologic (~3%) Investigations • semen analysis and culture • post-coital (Huhner) test: rarely done
Toronto Notes 2011
Polycystic Ovarian Syndrome (PCOS)
Polycystic Ovarian Syndrome (PCOS) • also called chronic ovarian androgenism Etiology Insulin estrogen
FSH secretion + LH secretion
Anovulation
peripheral conversion to estrogen
ovarian secretion of androgens
Oligomenorrhea
Obesity
Hirsutism
Infertility
Gynecology GY23
Clinical Signs of Endocrine Imbalance • Menstrual disorder/amenorrhea (80%) • Infertility (74%) • Hirsutism (69%) • Obesity (49%) • Impaired glucose tolerance (35%) • Diabetes Mellitus (10%)
Polycystic Ovarian Syndrome – HAIR-AN Hirsutism, HyperAndrogenism, Infertility, Insulin Resistance, Acanthosis Nigricans
Figure 12. Pathophysiology of Polycystic Ovarian Syndrome
Diagnosis • 2 of 3 to make diagnosis 1. oligomenorrhea/irregular menses for 6 months 2. clinical or lab evidence of hyperandrogenism 3. polycystic ovaries on U/S Clinical Features • average age 15-35 years at presentation • abnormal/irregular uterine bleeding, hirsutism, infertility, obesity, virilization • insulin resistance occurs in both lean and obese patients • acanthosis nigricans: browning of skin folds in intertriginous zones (indicative of insulin resistance) • family history of diabetes Investigations • goal of investigations is to identify hyperandrogenism or chronic anovulation and rule out specific pituitary or adrenal disease as the cause • labs prolactin, 17-hydroxyprogesterone, free testosterone, DHEA-S, TSH, free T4, androstenedione, sex hormone binding globulin (SHBG) LH:FSH >2:1; LH is chronically high with FSH midrange or low (low sensitivity and specificity) increased DHEAS, androstenedione and free testosterone (most sensitive), decreased SHBG • transvaginal ultrasound: polycystic-appearing ovaries (“string of pearls”) • tests for insulin resistance or glucose tolerance fasting glucose:insulin ratio 30 are genital subtypes • HPV types 6 and 11 are classically associated with anogenital warts/condylomata acuminata • HPV types 16 and 18 are the most oncogenic (classically associated with cervical HSIL) • types 16, 18, 31, 33, 35, 36, 45 (and others) associated with increased incidence of cervical and vulvar intraepithelial hyperplasia and carcinoma Clinical Features • latent infection no visible lesions, asymptomatic only detected by DNA hybridization tests • subclinical infection visible lesion found during colposcopy or on Pap test • clinical infection visible wart-like lesion without magnification hyperkeratotic, verrucous or flat, macular lesions vulvar edema Investigations • cytology (see Cervical Screening (Pap Test), GY43) koilocytosis – nuclear enlargement and atypia with perinuclear halo • biopsy of lesions at colposcopy • detection of HPV DNA subtype using nucleic acid probes not routinely done but can be done in presence of abnormal Pap test to guide treatment Treatment • patient administered: podofilox 0.5% solution or gel bid x 3 days in a row (4 days off) then repeat x 4 weeks imiquimod (Aldara®) 5% cream 3x/wk qhs x 16wks • provider administered: cryotherapy with liquid nitrogen: repeat q1-2wks podophyllin resin in tincture of benzoin: weekly trichloroacetic acid (TCA) or bichloroacetic acid weekly (80-90%); safe in pregnancy surgical removal/laser intralesional interferon Prevention • HPV types 6, 11, 16, 18 – preventable with Gardasil® (quadrivalent HPV recombinant vaccine) • cannot be prevented by using condoms
Genital Warts During Pregnancy • Condyloma tend to get larger in pregnancy and should be treated early (consider excision). • C-section only if obstruction of birth canal or risk of extensive bleeding. • Do not use imiquimod, podophyllin or podofilox.
Human Rights in Health Equity: Cervical Cancer and HPV Vaccines Erdman, JN. American Journal of Law & Medicine. 2009 • While cervical cancer rates have drastically fallen in developed countries due to effective prevention and treatment, socially disadvantaged women within these countries remain disproportionately more likely to develop and die of cervical cancer. • In most developing countries, in contrast, cervical cancer rates have risen or remained unchanged. • Must recognize that cervical cancer disparities between race groups, urban and rural residence, and high and low wealth status are attributed to disparate screening and vaccination coverage. • Programs are implemented without sufficient attention to conditions that render screening less effective or inaccessible to disadvantaged social groups including: lack of information, undervaluing of preventive care, opportunistic delivery in limited health care settings, sexual health stigma and related privacy concerns.
GY28 Gynecology
Gynecological Infections
Toronto Notes 2011
HERPES SIMPLEX VIRUS OF VULVA (HSV) Etiology • 90% are HSV-2, 10% are HSV-1
Classically... HSV I – disease above the belt (oral) HSV II – disease below the belt (genital)
Clinical Features • may be asymptomatic • initial symptoms: present 2-21 days following contact • prodromal symptoms: tingling, burning, pruritus • multiple, painful, shallow ulcerations with small vesicles appear 7-10 days after initial infection (absent in many infected persons); lesions are infectious • inguinal lymphadenopathy, malaise, and fever often with first infection • dysuria and urinary retention if urethral mucosa affected • recurrent infections: less severe, less frequent and shorter in duration (especially with HSV-1) Investigations • viral culture preferred in patients with ulcer present – decreased sensitivity as lesions heal • cytologic smear multinucleated giant cells, acidophilic intranuclear inclusion bodies • type specific serologic tests for antibodies to HSV-1 and HSV-2 (not available routinely in Canada) • HSV DNA PCR
HSV Infections During Pregnancy • Antiviral suppression of women with first episode or history of HSV infections from 36 weeks GA on. • C-section should be performed on women who have active genital lesions at time of delivery. • Treatment: acyclovir 400 mg PO tid.
Treatment • first episode acyclovir 400 mg PO tid x 7-10d, or famciclovir 250 mg PO tid x 7-10d, or valacyclovir 1 g PO bid x 7-10d • recurrent episode acyclovir 400 mg PO tid x 3-5d, or famciclovir 125 mg PO bid x 3-5d, or valacyclovir 500 mg PO bid x 3d • daily suppressive therapy consider if 6-8 recurrences per year acyclovir 400 mg PO bid, or famciclovir 250 mg bid, or valacyclovir 0.5-1 g PO OD • severe disease consider IV therapy acyclovir 5-10 mg/kg IV q8h x 5-7d • education regarding transmission • avoid contact from onset of prodrome until lesions have cleared • use barrier contraception SYPHILIS Etiology • Treponema pallidum
Epidemiology of Genital Ulcers HSV 70-80% 1º syphilis 5% Chancroid 38.3ºC lower abdominal pain and tenderness abnormal discharge: cervical or vaginal • uncommon nausea and vomiting dysuria AUB • chronic disease (often due to chlamydia) constant pelvic pain dyspareunia palpable mass very difficult to treat, may require surgery Investigations • bloodwork b-hCG (must rule out ectopic pregnancy), CBC, blood cultures if suspect septicemia • urine R&M • speculum exam, bimanual exam vaginal swab for Gram stain, C&S cervical cultures for N. gonorrheae, C. trachomatis endometrial biopsy will give definitive diagnosis (rarely done) • ultrasound may be normal free fluid in cul-de-sac pelvic or tubo-ovarian abscess hydrosalpinx (dilated fallopian tube) • laparoscopy (gold standard) for definitive diagnosis: may miss subtle inflammation of tubes or endometritis Treatment • must treat with polymicrobial coverage • inpatient if: moderate to severe illness atypical infection adnexal mass, tubo-ovarian or pelvic abscess unable to tolerate oral antibiotics or failed oral therapy immunocompromised pregnant adolescent – first episode surgical emergency cannot be excluded (e.g. ovarian torsion) PID is secondary to instrumentation recommended treatment cefoxitin 2 g IV q6h (no longer available in U.S.A.) or cefotetan 2 g IV q12h + doxycycline 100 mg IV/PO q12h or clindamycin 900 mg IV q8h + gentamicin 2 mg/kg IV loading dose then gentamicin 1.5 mg/kg q8h maintenance dose continue IV antibiotics for 24 hours after symptoms have improved then doxycycline 100 mg PO bid to complete 14 days percutaneous drainage of abscess under U/S guidance when no response to treatment, laparoscopic drainage if failure, treatment is surgical (salpingectomy, TAH/BSO) • outpatient if: typical findings mild to moderate illness oral antibiotics tolerated compliance ensured follow-up within 48-72 hours (to ensure symptoms not worsening) recommended treatment ofloxacin 400 mg PO bid x 14d or levofloxacin 500 mg PO bid x 14d ± metronidazole 500 mg PO bid x 14d (if suspect abscess) ceftriaxone 250 mg IM x 1 + doxycycline 100 mg PO bid x 14d or cefoxitin 2 g IM x 1 + probenecid 1 g PO + doxycyline 100 mg PO bid ± metronidazole 500 mg PO bid x 14d consider removing IUD after a minimum of 24 hours of treatment reportable disease treat partners consider re-testing for C. trachomatis and N. gonorrheae 4-6 weeks after treatment if documented infection
Toronto Notes 2011
Gynecological Infections/Sexuality and Sexual Dysfunction
Complications of Untreated PID • chronic pelvic pain • abscess, peritonitis • adhesion formation • ectopic pregnancy • infertility 1 episode of PID g 13% infertility 2 episodes of PID g 36% infertility • bacteremia • septic arthritis, endocarditis
Gynecology GY31
PID Complications I FACE PID Infertility Fitz-Hugh-Curtis syndrome Abscesses Chronic pelvic pain Ectopic pregnancy Peritonitis Intestinal obstruction Disseminated infection (sepsis, endocarditis, arthritis, meningitis)
Toxic Shock Syndrome • see Infectious Diseases, ID27 Risk Factors • tampon use • diaphragm, cervical cap or sponge use (prolonged use, i.e. >24 hours) • wound infections • post-partum infections • early recognition and treatment of syndrome is imperative as incorrect diagnosis can be fatal Clinical Presentation • sudden high fever • sore throat, headache, diarrhea • erythroderma • signs of multisystem organ failure • refractory hypotension • exfoliation of palmar and plantar surfaces of the hands and feet 1-2 weeks after onset of illness Treatment • remove potential sources of infection (foreign objects and wound debris) • debride necrotic tissues • adequate hydration • penicillinase-resistant antibiotics, e.g. cloxacillin • steroid use controversial but if started within 72 hours, may reduce severity of symptoms and duration of fever
Surgical Infections Post-Operative Infections in Gynecological Surgery • pelvic cellulitis common post hysterectomy, affects vaginal vault erythema, induration, tenderness, discharge involving vaginal cuff treat if fever and leukocytosis with broad spectrum antibiotics, i.e. clindamycin and gentamicin drain if excessive purulence or large mass can result in intra-abdominal and pelvic abscess • see General Surgery, Post-Operative Fever, GS7
Sexuality and Sexual Dysfunction SEXUAL RESPONSE 1. desire: energy that allows an individual to initiate or respond to sexual stimulation 2. arousal: physical and emotional stimulation leading to breast and genital vasodilation and clitoral engorgement 3. orgasm: physical and emotional stimulation is maximized, allowing the individual to relinquish their sense of control 4. resolution: most of the congestion and tension resolves within seconds, complete resolution may take up to 60 minutes SEXUAL DYSFUNCTION Etiology • intrapsychic: patient’s life experiences, value system • relationship/interpersonal issues • physical/organic
Toxic Shock Syndrome Multiple organ system failure due to S. aureus exotoxin (rare condition).
GY32 Gynecology Dyspareunia Cycle Painful intercourse (initially due to organic etiology) 2o vaginismus
Fear of pain with intercourse
Anxiety with or without sexual response
Kegel Exercises Regular contraction and relaxation to strengthen pelvic floor muscles. Reverse Kegel Exercises 1 second contraction then 5 seconds of relaxation.
Sexuality and Sexual Dysfunction/Menopause
Toronto Notes 2011
Classification • lack of desire (60-70% of women) • lack of arousal • anorgasmia (5-10%) primary anorgasmia: never before achieved orgasm under any circumstances secondary anorgasmia: was able to achieve orgasms before but now unable • dyspareunia (3-6%) – painful intercourse, superficial or deep vaginismus (15%) vulvodynia vaginal atrophy vulvar vestibulitis: associated with history of frequent yeast infections PID Treatment • lack of desire – assess factors, rule out organic causes, relationship therapy, sensate focus exercises • anorgasmia – self-exploration/pleasuring, relationship therapy if needed, bridging techniques (different sexual positions, clitoral stimulation during intercourse) • dyspareunia Kegel and reverse Kegel exercises dilator treatment comfort with self-exam psychotherapy, other behavioural techniques female on top position – allows for control of speed and duration vestibulitis – remove local irritants, change in contraceptive methods, and dietary changes (increased citrate, decreased oxalate), vestibulectomy (rare) vulvodynia – local moisturization, cold compresses, systemic nerve blocking therapy (amitriptyline, neurontin), topical anesthetic, estrogen cream
Menopause • see Family Medicine, FM41 • Menopause: occurrence of last spontaneous menstrual period, resulting from loss of ovarian function (loss of oocyte response to gonadotropins). • “Being in menopause”: lack of menses for 1 yr. • Perimenopause: period of time surrounding menopause (2-8 yrs preceding + 1 yr after last menses) characterized by fluctuating hormone levels, irregular menstrual cycles, and symptom onset.
• 85% of women experience hot flashes • 20-30% seek medical attention • 10% are unable to work
• Osteoporosis is the single most important health hazard associated with menopause. • Cardiovascular disease is the leading cause of death post-menopause.
• Increased risk of breast cancer (RR 1.3) is associated with HRT use. • All women taking HRT should have periodic surveillance and counselling regarding its benefits and risks.
Definitions • types of menopause physiological; average age 51 years (follicular atresia) premature ovarian failure; before age 40 (autoimmune disorder, infection, Turner’s syndrome) iatrogenic (surgical/radiation/chemotherapy) Clinical Features • associated with estrogen deficiency vasomotor instability (tends to dissipate with time) hot flushes/flashes, night sweats, sleep disturbances, formication, nausea, palpitations urogenital atrophy involving vagina, urethra, bladder dyspareunia, vaginal itching, vaginal dryness, bleeding, urinary frequency, urgency, incontinence skeletal osteoporosis, joint and muscle pain, back pain skin and soft tissue decreased breast size, skin thinning/loss of elasticity psychological mood disturbance, irritability, fatigue, decreased libido, memory loss Investigations • increased levels of FSH (>35 IU/L) on day 3 of cycle (if still cycling) and LH (FSH>LH) • decreased levels of estradiol (later) Treatment • goal is for individual symptom management vasomotor instability HRT (first line), clonidine, SSRI, Effexor®, gabapentin, propranolol vaginal atrophy local estrogen – cream (Premarin®), vaginal suppository (VagiFem®), ring (Estring®) lubricants (Replens®) urogenital health lifestyle changes (weight loss, bladder re-training), local estrogen replacement, surgery
Toronto Notes 2011
Menopause
osteoporosis 1000-1500 mg calcium daily, 800-1000 IU vitamin D, weight-bearing exercise, quit smoking bisphosphonates (e.g. alendronate) selective estrogen receptor modifiers (SERMs): raloxifene (Evista®) – mimics estrogen effects on bone, avoids estrogen-like action on breast and uterine cancer; does not help hot flashes HRT: second-line treatment (unless for vasomotor instability as well) decreased libido vaginal lubrication, counselling, androgen replacement (testosterone cream) cardiovascular disease management of cardiovascular risk factors mood and memory antidepressants (first line), HRT (augments effect) alternative choices (not evidence-based, safety not established) black cohosh, phytoestrogens, St. John’s wort, gingko biloba, valerian, evening primrose oil, ginseng, Don Quai
Hormone Replacement Therapy (HRT) • see Family Medicine, FM41 • primary indication is treatment of menopausal symptoms (vasomotor instability) • keep doses low (e.g. 0.3 mg Premarin®) and duration of treatment short ( Asian or African women) collagen disorders
Toronto Notes 2011
Urogynecology
Gynecology GY35
GENERAL CONSERVATIVE TREATMENT (for pelvic relaxation/prolapse and urinary incontinence) • Kegel exercises • local vaginal estrogen therapy • vaginal pessary Table 12. Pelvic Prolapse Type
Clinical Features
Treatment
Uterine Prolapse (Protrusion of cervix and uterus into vagina)
• Groin/back pain (stretching of uterosacral ligaments) • Feeling of heaviness/pressure in the pelvis • Worse with standing, lifting • Worse at the end of the day • Relieved by lying down • Ulceration/bleeding (particularly if hypoestrogenic) • ± urinary incontinence
• See General Conservative Treatment, above • Vaginal hysterectomy ± surgical prevention of vault prolapse • Consider additional surgical procedures if urinary incontinence, cystocele, rectocele, and/or enterocele are present
• See General Conservative Treatment, above • Sacralcolpopexy (vaginal vault suspension), sacrospinous fixation, or uterosacral ligament suspension
Vault Prolapse (Protrusion of apex of vaginal vault into vagina, post-hysterectomy) Cystocele (Protrusion of bladder into the anterior vaginal wall)
• Frequency, urgency, nocturia • See General Conservative Treatment, above • Stress incontinence • Anterior colporrhaphy (“anterior repair”) • Incomplete bladder emptying ± associated • Consider additional/alternative surgical procedure if increased incidence of urinary tract documented urinary stress incontinence infections – may lead to renal impairment
Rectocele (Protrusion of rectum into posterior vaginal wall)
• Straining/digitation to evacuate stool • Constipation
• See General Conservative Treatment, above • Also laxatives and stool softeners • Posterior colporrhaphy (“posterior repair”), plication of endopelvic fascia and perineal muscles approximated in midline to support rectum and perineum (can result in dyspareunia) • Similar to hernia repair • Contents reduced, neck of peritoneal sac ligated, uterosacral ligaments, and levator ani muscles approximated
Sacrum
Small intestine
Utero-sacral ligaments Uterus Rectum
Rectocele
Figure 14. Pelvic Prolapse
Bladder Vaginal Canal Urethra
Cystocele
Uterine Prolapse
The only true hernia of the pelvis is an ENTEROCELE because peritoneum herniates with the small bowel.
© Lori Waters 2008
Enterocele (Prolapse of small bowel in upper posterior vaginal wall)
Grading of Pelvic Organ Prolapse • 0 = no descent during straining • 1 = distal portion of prolapse >1cm above level of hymen • 2 = distal portion of prolapse ≤1cm above or below level of hymen • 3 = distal portion of prolapse >1cm below level of hymen but without complete vaginal eversion • 4 = complete eversion of total length of lower genital tract • Procidentia: failure of genital supports and complete protrusion of uterus through the vagina
Enterocele
GY36 Gynecology
Urogynecology/Gynecological Oncology
Toronto Notes 2011
Urinary Incontinence • see Urology, U5 Stress Incontinence Involuntary loss of urine with increased intra-abdominal pressure (coughing, laughing, sneezing, walking, running).
The gold standard diagnostic test for urinary incontinence is multichannel urodynamics. A large proportion of cases are correctly diagnosed from clinical history alone and this can be supplemented with patient urinary and intake diaries. Health Technol Assess 2006; 10(6):1-132.
Urge Incontinence Urine loss associated with an abrupt, sudden urge to void.
Rule Out Neurological Causes of Urge Incontinence Multiple sclerosis Slipped disc Diabetes mellitus
STRESS INCONTINENCE Risk Factors for Stress Incontinence in Women • pelvic prolapse • pelvic surgery • vaginal delivery • hypoestrogenic state (post-menopause) • age • smoking • neurological/pulmonary disease Treatment • see General Conservative Treatment, GY35 • surgical tension-free vaginal tape (TVT), tension-free obturator tape (TOT), prosthetic/fascial slings or retropubic bladder suspension (Burch or Marshall-Marchetti-Krantz procedures) URGE INCONTINENCE Definition • urine loss associated with an abrupt, sudden urge to void • “overactive bladder” • diagnosed based on symptoms Etiology • idiopathic (90%) • detrusor muscle overactivity (“detrusor instability”) Associated Symptoms • frequency, urgency, nocturia, leakage Treatment • behaviour modification (reduce caffeine/liquid, smoking cessation, regular voiding schedule) • Kegel exercises • medications anticholinergics – oxybutinin (Ditropan®), tolterodine (Detrol®) tricyclic antidepressants – imipramine
Gynecological Oncology Uterus ENDOMETRIAL CARCINOMA
Incidence of Malignant Gynecological Lesions in North America endometrium > ovary > cervix > vulva > vagina > fallopian tube
Epidemiology • most common gynecological malignancy in North America (40%); 4th most common cancer in women • 2-3% of women develop endometrial carcinoma during lifetime • mean age is 60 years • majority are diagnosed in early stage due to detection of symptoms • 85-90% 5-year survival for stage I disease • overall 5-year survival for all stages is 70-80% Classification • Type I – endometrioid adenocarcinoma (~80% of cases) • Type II – serous, clear cell carcinomas (~15% of cases)
Toronto Notes 2011
Gynecological Oncology
Risk Factors • Type I: excess estrogen (estrogen unopposed by progesterone) obesity PCOS unbalanced HRT (balanced HRT is actually protective) nulliparity late menopause estrogen-producing ovarian tumours (e.g. granulosa cell tumours) HNPCC (hereditary non-polyposis colorectal cancer)/Lynch II syndrome tamoxifen • Type II: not estrogen related possibly tamoxifen Clinical Features • Type I: postmenopausal bleeding in majority, abnormal uterine bleeding in majority of affected pre-menopausal women (menorrhagia, intermenstrual bleeding) • Type II: may not present with bleeding in early stage, more likely to present with advanced stage disease with symptoms like ovarian cancer (i.e. bloating, bowel dysfunction, pelvic pressure)
Gynecology GY37
Risk Factors for Endometrial Cancer COLD NUT Cancer (ovarian, breast, colon) Obesity Late menopause Diabetes mellitus Nulliparity Unopposed estrogen: PCOS, anovulation, HRT Tamoxifen: chronic use
Postmenopausal bleeding = endometrial cancer until proven otherwise. 95% present with vaginal bleeding.
Table 13. FIGO Staging of Endometrial Cancer Stage
Description
Stage
Description
I IA IB II
Confined to corpus No or less than half myometrial invasion Invades through ≥ one half of myometrium Tumour invades cervical stroma, but does not extend beyond uterus* Local and/or regional spread of the tumour Invasion of serosa, corpus uteri ± adnexae Vaginal ± parametrial involvment Metastasis to pelvic ± para-aortic LNs Positive pelvic LN Positive para-aortic LN ± positive pelvic LNs
IV IVA IVB
Invasion of bladder ± bowel mucosa ± distant metastases Invasion of bladder ± bowel mucosa Distant mets, including intra-abdominal mets ± inguinal LNs
III IIIA IIIB IIIC IIIC1 IIIC2
An endometrial thickness of 5 mm or more is considered abnormal in a postmenopausal woman with vaginal bleeding.
True Pelvis Area of pelvis between pelvic inlet and outlet, i.e. it does not include the abdominal contents in the pelvis found above the pelvic inlet.
FIGO: International Federation of Gynecology and Obstetrics *Note: endocervical glandular involvement is now considered as Stage I (previously Stage II)
Investigations • endometrial sampling: office endometrial biopsy D&C ± hysteroscopy • ± pelvic ultrasound (in women where adequate endometrial sampling not feasible without invasive methods) not acceptable as alternative to pelvic exam or endometrial sampling to rule out cancer
Prognostic Factors 1. Grade (histological differentiation) 2. Vascular, lymphatic involvement 3. Progesterone-receptor levels 4. Myometrial invasion
Spread • most common is direct extension • lymphatic spread to pelvic and para-aortic nodes • transtubal dissemination to peritoneal cavity • hematogenous spread (usually to lungs, liver) Treatment • surgical: hysterectomy/bilateral salpingo-oophorectomy (BSO) and pelvic washings ± pelvic and para-aortic node dissection ± omentectomy goals: diagnosis, staging, treatment, defining optimal adjuvant treatment laparoscopic approach associated with improved quality of life (optimal for most patients) • adjuvant radiotherapy (for improved local control in patients at risk for local recurrence) and adjuvant chemotherapy (in patients at risk for distant recurrence or with metastatic disease) – based on presence of poor prognostic factors in definitive pathology • chemotherapy often used for recurrent disease (especially if high grade or aggressive histology) • hormonal therapy: progestins can be used for recurrent disease (especially if low grade) UTERINE SARCOMA • rare: 2-6% of all uterine malignancies • arise from stromal components (endometrial stroma, mesenchymal or myometrial tissues) • behave more aggressively and are associated with poorer prognosis than endometrial carcinoma; 5-year survival – 35% • vaginal bleeding is most common presenting symptom
Uterine Sarcoma – Symptoms BAD-P Bleeding Abdominal distention Foul smelling vaginal Discharge Pelvic Pressure
GY38 Gynecology
Gynecological Oncology
Toronto Notes 2011
1. Mixed Müllerian Mesodermal Tumour (Carcinosarcoma) • most common type of uterine sarcoma (43%) • both epithelial and sarcomatous malignant elements are present • tend to form bulky polypoid masses that often fill the uterine cavity and extend into or through the endocervical canal – often have extrauterine disease at presentation Treatment • usually treated as “very high grade endometrial carcinoma” since behaviour and treatment similar (i.e. surgical staging, adjuvant chemotherapy and radiation)
A rapidly enlarging uterus, especially in a postmenopausal woman, should prompt consideration of leiomyosarcoma.
2. Leiomyosarcoma • account for one third of uterine sarcomas • when occurs, often coexists with benign leiomyomata (fibroids) • 50% of time, leiomyosarcomata arise within a fibroid (“sarcomatous degeneration”) • average age of presentation is 55 years but may present in pre-menopause • histologic distinction from leiomyoma increased mitotic count (>10 mitoses/10 high power fields) tumour necrosis cellular atypia • often diagnosed postoperatively after uterus removed for presumed fibroids Clinical Features • “rapidly” enlarging fibroids in a pre-menopausal woman • enlarging fibroids in a postmenopausal woman Treatment • hysterectomy/BSO usually without node dissection due to high propensity for vascular spread (i.e. liver/lung metastases) • adjuvant chemotherapy may be used if tumour has spread beyond uterus, for palliation • radiation therapy does not improve local control or survival • poor outcome overall, even for early stage disease 3. Endometrial Stromal Sarcoma • usually presents in perimenopausal or postmenopausal women with abnormal uterine bleeding • diagnosed by histology of endometrial biopsy or D&C Treatment • hysterectomy/BSO (ALWAYS remove ovaries as ovarian hormones may stimulate growth) • adjuvant therapy based on stage and histologic features (hormones and/or radiation) • hormonal therapy (progestins) may be used for metastatic disease in low grade ESS
Ovary Ovarian Tumour Markers Epithelial cell – CA-125 Stromal Granulosa cell – inhibin Sertoli-Leydig – androgens Germ cell Dysgerminoma – LDH Yolk sac – AFP Choriocarcinoma – beta-hCG Immature Teratoma – none Embryonal cell – AFP + beta-hCG
BENIGN OVARIAN TUMOURS • see Table 14 • most are asymptomatic • usually enlarge slowly, if at all • may rupture or undergo torsion, causing pain • pain associated with torsion of an adnexal mass usually originates in the iliac fossa and radiates to the flank • peritoneal irritation may result from an infarcted tumour – rare MALIGNANT OVARIAN TUMOURS • see Table 14 Epidemiology • lifetime risk 1.4% (1/70) • in women >50 years, more than 50% of ovarian tumours are malignant • causes more deaths in North America than all other gynecologic malignancies combined • 4th leading cause of cancer death in women • 65% epithelial; 35% non-epithelial • 5-10% of epithelial ovarian cancers are related to hereditary predisposition
Toronto Notes 2011
Gynecological Oncology
Risk Factors (for epithelial ovarian cancers) • nulliparity • early menarche/late menopause • age • family history of breast, colon, endometrial, ovarian cancer • race: Caucasian Protective Factors (for epithelial ovarian cancers) • OCP: likely due to ovulation suppression (significant reduction in risk even after 1 year of use) • pregnancy/breastfeeding • tubal ligation (recently questioned) • hysterectomy (without removal of ovaries) • bilateral salpingo-oophorectomy (prophylactic surgery performed for this reason in women with known high risk – i.e. BRCA mutation carriers) Clinical Features • most women with epithelial ovarian cancer present with advanced stage disease since often “asymptomatic” until disseminated disease vague non-specific symptoms associated with early stage disease • when present, non-specific symptoms may include: vague abdominal symptoms (nausea, bloating, dyspepsia, anorexia, early satiety) symptoms of mass effect increased abdominal girth – from ascites or tumour itself urinary frequency constipation fluid wave – signs of ascites postmenopausal bleeding; irregular menses if pre-menopausal (rare) Low Malignant Potential (also called “Borderline”) Tumours • pregnancy, OCP and breastfeeding are found to be protective factors • ~15% of all epithelial ovarian tumours • tumour cells display malignant characteristics histologically, but no invasion is identified • able to metastasize, but not commonly • treated primarily with surgery (BSO/omental biopsy ± hysterectomy) NO proven benefit of chemotherapy • generally slow growing, excellent prognosis 5-year survival >99% recurrences tend to occur late, may be associated with low grade serous carcinoma
Gynecology GY39
Risk/Protective Factors for Epithelial Ovarian Cancer NO CHILD Nulliparity OCP, breast-feeding, tubal ligation, hysterectomy (protective) Caucasian Family History Increasing age (>40) Late menopause Delayed child-bearing
Any adnexal mass in postmenopausal women should be considered malignant until proven otherwise.
Most (70%) epithelial ovarian cancers present at stage III disease.
Diagnosis requires surgical pathology.
Table 14. Ovarian Tumours Type
Description
Presentation
Ultrasound/Cytology
Treatment Symptomatic or suspicious masses warrant surgical exploration Otherwise if 200: Gynecologic Oncology referral is recommended
Causes of Elevated CA-125 • Age influences reliability of test as a tumour marker • 50% sensitivity in early stage ovarian cancer (poor) – therefore not good for screening MALIGNANT • Gyne: ovary, uterus • Non-Gyne: pancreas, stomach, colon, rectum NON MALIGNANT • Gyne: benign ovarian neoplasm, endometriosis, pregnancy, fibroids, PID • Non-Gyne: cirrhosis, pancreatitis, renal failure
CA-125 is indicated for monitoring response to treatment.
Malignant Ovarian Tumour Prognosis 5-year Survival • Stage I: 75-95% • Stage II: 60-75% • Stage III: 23-41% • Stage IV: 11%
FIGO: International Federation of Gynecology and Obstetrics
Cervix
Gland opening
External os
New squamocolumnar junction Original squamocolumnar junction
Figure 15. The Cervix
© Ayalah Hutchins
or m tio n a
n
sf
ne
Tra
MALIGNANT CERVICAL LESIONS • majority are squamous cell carcinomas (95%), adenocarcinomas increasing (5%), rare subtypes include small cell, adenosquamous • 8,000 deaths annually in North America • annual Pap test reduces a woman’s chance of dying from cervical cancer from 0.4% to 0.05% • average age: 52 years old
Original squamous epithelium Squamous metaplasia Columnar epithelium
zo
BENIGN CERVICAL LESIONS • Nabothian cyst/inclusion cyst no treatment required • endocervical polyps treatment is polypectomy (office procedure)
GY42 Gynecology
Cervical cancer is caused by HPV infection.
Systematic Review of RCTs for HPV Vaccination Prophylactic Vaccination Against Human Papillomavirus Infection in Women: A Systematic Review of Randomized Controlled Trials Rambout L, Hopkins L, Fung Kee Fung M, et al. CMAJ 2007; 177 Purpose: To assess the effectiveness of HPV vaccination for preventing HPV infection and precancerous cervical lesions. Study: Systematic review of studies of prophylactic HPV vaccination. Data Sources: MEDLINE, EMBASE, Cochrane Central Registry of Controlled Trials, and the Cochrane Library. Patients: Of 457 studies, nine were included in the review (six of these were RCTs). A total of 40,323 females were enrolled. All participants had received HPV vaccinations that included coverage of the HPV 16 strain. Main outcomes: Frequency of high-grade cervical lesions, persistent HPV infection, low-grade cervical lesions, external genital lesions, adverse events, and death. Results: HPV vaccination was associated with a reduction in the frequency of high-grade cervical lesions caused by vaccine-type HPV strains compared with control groups. The HPV vaccination was also found to be efficacious in reducing persistent HPV infection, low-grade lesions, and genital warts. Conclusion: Prophylactic vaccination of women between 15-25 years not previously infected with vaccine-type HPV strains, has been found to be efficacious in preventing HPV infection and precancerous cervical lesions.
Liquid-based Cytologic Smear vs. Conventional Pap Smear Am J Obstet Gynecol 2001; 185(2):308-17 Purpose: To assess the cytologic diagnosis and sample adequacy of liquid-based cervical cytologic smear (ThinPrep) versus conventional Papanicolaou smear. Study: Systematic review of prospective trials comparing ThinPrep and conventional Pap smears. Data sources: MEDLINE, PubMed, Silver Platter were searched for literature published in English between January 1990 and April 2000. Selection criteria included split-sample (SS) and direct-to-vial (DV) (case-cohort) studies. Patients: 25 studies met the selection criteria (n=533,039 women; 221,864 in ThinPrep group; 378,659 in conventional smear group; 67,484 in both groups) Main outcomes: (i) Frequency of diagnoses of ASCUS, LSIL, HSIL. (ii) Adequacy of sample collection (contains squamous cells, endocervical cells, and possibly metaplastic cells). Results: Liquid-based smears (ThinPrep) had significantly improved cytologic diagnosis of LSIL (OR = 1.27 to 2.15) and diagnosis of HSIL (OR = 2.26), but no difference in rate of diagnosis of ASCUS (OR = 1.03). Liquid-based pap smear also resulted in improved sample adequacy (OR = 1.64 to 2.11). Conclusion: Liquid-based cytologic smears resulted in better diagnosis of cervical premalignant lesions (HSIL and LSIL) and improved sample adequacy, compared to conventional Pap smears.
Gynecological Oncology
Toronto Notes 2011
Etiology • at birth, vagina is lined with squamous epithelium; columnar epithelium lines only the endocervix and the central area of the ectocervix (original squamocolumnar junction) • during puberty, estrogen stimulates eversion of a single columnar layer (ectopy), thus exposing it to the acidic pH of the vagina, leading to metaplasia (change of exposed epithelium from squamous to columnar) a new squamocolumnar junction forms as a result • the transformation zone (TZ ) is the area located between the original and the current squamocolumnar junction (Figure 15) • the majority of dysplasias and cancers arise in the TZ of the cervix • must have active metaplasia in presence of inducing agent (HPV) to get dysplasia • dysplasia g carcinoma in situ (CIS) g invasion • slow process (~10 years on average) • growth is by local extension • metastasis occurs late Risk Factors • HPV infection see Sexually Transmitted Infections, GY26 high risk of neoplasia associated with types 16, 18 low risk of neoplasia associated with types 6, 11 >99% of cervical cancers contain one of the high risk HPV types • smoking • high risk behaviours (risk factors for HPV infection) multiple partners other STIs (HSV, trichomonas) early age first intercourse high risk male partner • poor screening uptake is the most important risk factor for cervical cancer in Canada • at-risk groups include: immigrant Canadians First Nations Canadians geographically isolated Canadians sex-trade workers low socioeconomic status Prevention: Quadrivalent HPV Recombinant Vaccine (Gardasil®) • currently indicated for females 9 to 26 years of age for prevention of diseases caused by HPV types 6, 11, 16 and 18 (genital warts, cervical, vulvar and vaginal dysplasias and cancers) • for optimal benefit of vaccination, should be administered before onset of sexual activity (i.e. before exposure to virus) • administered IM at time 0, 2 and 6 months, may be given at the same time as Hep B or other vaccines using a different injection site • not for treatment of active infections • most women will not be infected with all four types of the virus at the same time, therefore vaccine is still indicated for sexually active females or those with a history of previous HPV infection or HPV-related disease • conception should be avoided until 30 days after last dose of vaccination • side effects: pain, swelling, erythema, low grade fever • contraindications: pregnant women and women who are nursing (limited data) Clinical Features • squamous cell carcinoma (SCC) exophytic, fungating tumour • adenocarcinoma endophytic, with barrel-shaped cervix • early asymptomatic discharge: initially watery, becoming brown or red post-coital bleeding • late 80-90% present with bleeding: either post-coital, postmenopausal or irregular bleeding pelvic or back pain (extension of tumour to pelvic walls) bladder/bowel symptoms • signs friable, raised, reddened or ulcerated area visible on cervix
Toronto Notes 2011
Gynecological Oncology
Gynecology GY43
Cervical Screening Guidelines (Pap Test) • endocervical and exocervical cell sampling (aim is to sample the TZ) • best identifies squamous cell abnormalities, less reliable for glandular abnormalities false positives 5-10%, false negatives 10-40% (for single test) false negative rate 50% for existing cervical cancer • all women: start annual screening at age 21, or 3 years after onset of vaginal intercourse women ≥30 years: if 3 normal Paps in a row, and no previous abnormal Paps, can get screened every 2-3 years (if adequate recall mechanism in place) women ≥70 years: if 3 normal Paps in a row and no abnormal Paps in last 10 years, can discontinue screening (if remain at low risk) • pregnant women and women who have sex with women should follow the routine cervical screening regimen • women who have had a hysterectomy: total: discontinue screening if hysterectomy was for benign disease and no history of cervical dysplasia or HPV infection subtotal: continue screening according to guidelines • exceptions to guidelines: immunocompromised (transplant, steroids, DES exposure) HIV and high risk previously unscreened patients Table 16. Cytological Classification Bethesda Grading System
Classic System/ Cervical Intraepithelial Neoplasia (CIN) Grading System
Within normal limits
Normal
Infection
Inflammatory atypia (organism)
The Bethesda Classification System is based on cytological results of a Pap test that permits the examination of cells but not tissue structure. The diagnosis of cervical intraepithelial neoplasia (CIN) or cervical carcinoma requires a tissue sample, obtained by biopsy of suspicious lesions (done during colposcopy), to make a histologic diagnosis.
Reactive and reparative changes Squamous cell abnormalities Atypical squamous cells of undetermined significance (ASCUS)
Squamous atypia of uncertain significance
Atypical squamous cells, cannot exclude HSIL (ASC-H) Low grade squamous intraepithelial lesion (LSIL)
HPV atypia or mild dysplasia (CIN I)
High grade squamous intraepithelial lesion (HSIL)
Moderate dysplasia (CIN II) Severe dysplasia (CIN III) Carcinoma in situ (CIS)
Squamous cell carcinoma (SCC)
Squamous cell carcinoma (SCC)
Glandular cell abnormalities Atypical glandular cells of undetermined significance (AGUS)
Glandular atypia of uncertain significance
Endocervical adenocarcinoma
Adenocarcinoma
Endometrial adenocarcinoma Extrauterine adenocarcinoma Adenocarcinoma, not otherwise specified (NOS) Normal
Inadequate sample
Repeat cytology in 1-3 yrs
Repeat cytology in 3 mos
ASCUS
ASC-H
Women 3 mm deep, not >7 mm wide 3-5 mm deep; not >7 mm wide Clinically visible lesion confined to cervix, or microscopic lesion >IA Clinically visible lesion ≤4 mm in greatest dimension Clinically visible lesion >4 mm in greatest dimension
II IIA IIA1 IIA2 IIB
Beyond uterus but not to the pelvic wall or lower 1/3 of vagina No obvious parametrial involvement Clinically visible lesion ≤4 mm in greatest dimension Clinically visible lesion >4 mm in greatest dimension Obvious parametrial involvement
III IIIA IIIB
Extends to pelvic wall, and/or involves lower 1/3 of vagina and/or causes hydronephrosis or non-functioning kidney Involves lower 1/3 vagina but no extension into pelvic side wall Involves lower 1/3 vagina and extends into pelvic side wall and/or hydronephrosis or non-functioning kidney
IV IVA IVB
Carcinoma has extended beyond true pelvis or has involved (biopsy proven) the mucosa of the bladder or rectum Spread of the growth to adjacent organs Distant metastases
Table 18. Treatment of Patients with Cervical Dysplasia and Cervical Cancer Treatment CIN I (LSIL)
Observe with regular cytology (every 6 months) Many lesions will regress or disappear (60%) Colposcopy if positive on 2 consecutive smears Lesions which progress should have area excised by either LEEP, laser, cryotherapy or cone biopsy (with LEEP, tissues obtained for histological evaluation)
CIN II and CIN III (HSIL)
Colposcopy referral Ablation or excision therapy: LEEP, laser, cryotherapy, cone excision, cautery Hysterectomy – only if no desire for future childbearing
Stage IA1 (=microinvasive SCC if: 4 cm), II, III, IV
Concurrent chemoradiation therapy
Abnormal Pap Tests in Pregnancy • incidence – 1/2,200 • Pap test at all initial prenatal visits if abnormal Pap or suspicious lesion, refer to colposcopy • if diagnostic conization required, should be deferred until second trimester (T2) to minimize risk of pregnancy loss • if invasive cancer ruled out, management of dysplasia deferred until after completion of pregnancy (may deliver vaginally)
Toronto Notes 2011
Gynecological Oncology
Gynecology GY45
• if invasive cancer present, management depends on prognostic factors, degree of fetal maturity, and patient wishes general recommendations in T1: consider pregnancy termination, management with either radical surgery (hysterectomy vs. trachelectomy if desires future fertility) or concurrent chemoradiation therapy recommendations in T2/T3: delay of therapy until viable fetus and C/S for delivery with concurrent radical surgery or subsequent concurrent chemoradiation therapy
Vulva BENIGN VULVAR LESIONS Non-Neoplastic Disorders of Vulvar Epithelium • biopsy is necessary to make diagnosis and/or rule out malignancy • hyperplastic dystrophy (squamous cell hyperplasia) surface thickened and hyperkeratotic pruritus most common symptom typically postmenopausal women treatment: 1% fluorinated corticosteroid ointment bid for 6 weeks • lichen sclerosis subepithelial fat becomes diminished, labia become thin and atrophic, membrane-like epithelium, labial fusion pruritus, dyspareunia, burning ‘figure of 8’ distribution most common in postmenopausal women but can occur at any age treatment: ultrapotent topical steroid 0.05% clobetasol x 2-4wks then taper down • mixed dystrophy (lichen sclerosis with epithelial hyperplasia) hyperkeratotic areas with areas of thin, shiny epithelium treatment: fluorinated corticosteroid ointment Tumours • papillary hidradenoma, nevus, fibroma, hemangioma MALIGNANT VULVAR LESIONS Epidemiology • 5% of genital tract malignancies • 90% squamous cell carcinoma; remainder melanomas, basal cell carcinoma, Paget’s disease, Bartholin’s gland carcinoma Type I disease: HPV-related disease (50-70%) more likely in younger women 90% of vulval intraepithelial neoplasia (VIN) contain HPV DNA (usually types 16, 18) Type II disease: not HPV-related, associated with current or previous vulvar dystrophy usually postmenopausal women Risk Factors • HPV infection (see above) • VIN (vulvar intraepithelial neoplasia): precancerous change which presents as multicentric white or pigmented plaques on vulva (may only be visible at colposcopy) progression to cancer rarely occurs with appropriate management treatment: local excision (i.e. superficial vulvectomy ± split thickness skin grafting to cover defects [if required]) vs. ablative therapy (i.e. laser, cauterization) vs. local immunotherapy (imiquimod) Clinical Features • many patients asymptomatic at diagnosis (many also deny or minimize symptoms) • most lesions occur on the labia majora, followed by the labia minora (less commonly on the clitoris or perineum) • localized pruritus or lesion most common • less common: raised red, white or pigmented plaque, ulcer, bleeding, discharge, pain, dysuria • patterns of spread local groin lymph nodes (usually inguinal g pelvic nodes) hematogenous Investigations • physical examination • ± colposcopy • ALWAYS biopsy any suspicious lesion
Any suspicious lesion of the vulva should be biopsied.
GY46 Gynecology
Gynecological Oncology
Toronto Notes 2011
Table 19. FIGO Staging Classification and Treatment of Vulvar Cancer Stage Description
Treatment
0
Intraepithelial neoplasia (VIN), carcinoma in situ
Local excision/superficial vulvectomy Laser ablation Local immunotherapy (imiquimod)
I IA
Tumour confined to vulva Radical local excision + groin node dissection if >1 mm invasion ≤2 cm lesion, confined to vulva, perineum ± stromal Sentinel node dissection acceptable if lesion 2 mm lesion or stromal invasion >1 mm, no LNs, confined to vulva or perineum
IB II
Tumour any size with adjacent extension to 1/3 lower Individualized urethra, 1/3 lower vagina or anus Radical surgical excision ± chemoradiation Negative inguino-femoral LNs
III IIIA IIIB IIIC
II plus positive inguino-femoral LNs 1 LN met (≥5 mm) or 1-2 LN mets (3 LNs mets (4 cm associated with poorer prognosis • toxicities of therapy common surgical site infection lymphedema radiation fibrosis, cystitis, proctitis • overall 5-year survival rate: 79%
Vagina BENIGN VAGINAL LESIONS • inclusion cysts cysts form at site of abnormal healing of laceration (e.g. episiotomy) no treatment required • endometriosis dark lesions that tend to bleed at time of menses treatment is excision • Gartner’s duct cysts remnants of Wolffian duct, seen along side of cervix treatment conservative unless symptomatic • urethral diverticulum can lead to recurrent urethral infection, dyspareunia surgical correction if symptomatic MALIGNANT VAGINAL LESIONS Risk Factors • associated with HPV infection (analogous to cervical cancer) • increased incidence in patients with prior history of cervical and vulvar cancer Investigations • cytology significant false negative rate for existing malignancy (i.e. if gross lesion present, biopsy!) • colposcopy • Schiller test (normal squamous epithelium takes up Lugol’s iodine) • biopsy, partial vaginectomy (wide local excision for diagnosis) • rule out disease on cervix, vulva, or anus (most vaginal cancers are actually metastatic from one of these sites) • staging (see Table 20)
Toronto Notes 2011
Gynecological Oncology
Gynecology GY47
VAIN (Vaginal Intra-Epithelial Neoplasia) • grades: analogous to cervical dysplasia • treatment must rule out invasive cancer via biopsies and colposcopy prior to conservative treatment laser ablation vs. surgical excision vs. local immunotherapy (e.g. imiquimod) Squamous Cell Carcinoma (SCC) • 80-90% of vaginal cancer • 2% of gynecological malignancies • most common site is upper 1/3 of posterior wall of vagina • 5-year survival – 42% • clinical features asymptomatic painless discharge and bleeding vaginal discharge (often foul-smelling) vaginal bleeding especially during/post-coitus urinary and/or rectal symptoms 2º to compression • treatment usually concurrent chemoradiation therapy for 1o vaginal cancer consider radical hysterectomy/upper vaginectomy if early stage lesion and young patient Adenocarcinoma • most are metastatic, usually from the cervix, endometrium, ovary, or colon • most primaries are clear cell adenocarcinomas • 2 types: non-DES and DES syndrome • management as for SCC Diethylstilbestrol (DES) Syndrome • fetal exposure to DES (due to maternal use) predisposes to cervical or vaginal clear cell carcinoma, occurs in 30-95% of exposed females • if exposed, 6 cm (50%) – pre-eclampsia (27%) – hyperemesis gravidarum (26%) – hyperthyroidism (7%) – beta-hCG >100,000 mIU/mL – no fetal heart detected • partial (or incomplete) mole hydropic villi and focal trophoblastic hyperplasia are associated with fetus or fetal parts often triploid (XXY, XYY, XXX) with chromosome complement from both parents usually related to single ovum fertilized by two sperm low risk of progression to malignant sequelae (100,000 excessive uterine size prominent theca-lutein cysts Treatment • suction D&C with sharp curettage and oxytocin • Rhogam® if Rh negative • consider hysterectomy (if patient no longer desires fertility) • prophylactic chemotherapy of no proven benefit • chemotherapy for GTN if develops after evacuation Follow-up • contraception required to avoid pregnancy during entire follow-up period • serial beta-hCGs (as tumour marker) every week until negative x 3 (usually takes several weeks), then monthly for 6-12 months – prior to trying to conceive again • increase or plateau of beta-hCG indicates GTN g patient needs chemotherapy
Toronto Notes 2011
Gynecological Oncology
GTN (MALIGNANT GTD) • invasive mole or persistent GTN diagnosis made by rising or plateau in beta-hCG, development of metastases following treatment of documented molar pregnancy (see sidebar) histology: molar tissue from D&C metastases are rare (4%) • choriocarcinoma often present with symptoms from metastases highly anaplastic, highly vascular no chorionic villi, elements of syncytiotrophoblast and cytotrophoblast may follow molar pregnancy, abortion, ectopic, or normal pregnancy • placental-site trophoblastic tumour rare aggressive form of GTN abnormal growth of intermediate trophoblastic cells low beta-hCG, production of human placental lactogen (hPL), relatively insensitive to chemotherapy CLASSIFICATION of GTN • non-metastatic ~15% of patients after molar evacuation may present with abnormal bleeding all have rising or plateau of beta-hCG negative metastases on staging investigations • metastatic 4% patients after treatment of complete molar pregnancy metastasis more common with choriocarcinoma which tends toward early vascular invasion and widespread dissemination if signs or symptoms suggest hematogenous spread, don’t biopsy (they bleed) lungs (80%): cough, hemoptysis, CXR lesion(s) vagina (30%): vaginal bleeding, “blue lesions” on speculum exam pelvis (20%): rectal bleeding (if invades bowel), U/S lesion(s) liver (10%): elevated LFTs, U/S or CT findings brain (10%): headaches, dizziness, seizure (symptoms of space-occupying lesion), CT/MRI findings highly vascular tumour g bleeding g anemia all have rising or plateau of beta-hCG classification of metastatic GTN divided into good prognosis and bad prognosis features of bad prognosis – long duration (>4 months from antecedent pregnancy) – high pre-treatment beta-hCG titre: >100,000 IU/24h urine or >40,000 mIU/mL of blood – brain or liver metastases – prior chemotherapy – metastatic disease following term pregnancy good prognosis characterized by the absence of each of these features Investigations – For Staging • history and physical • bloodwork: CBC, electrolytes, creatinine, beta-hCG, TSH, LFTs • imaging: CXR, U/S pelvis, CT abdo/pelvis, CT brain • if suspect brain metastasis but CT brain negative, consider lumbar puncture for CSF beta-hCG ratio of plasma beta-hCG:CSF beta-hCG 6 month use
Endometriosis 1° menorrhagia/DUB
S/E: weight gain, acne, mild hirsutism, hepatic dysfunction C/I: pregnancy, undiagnosed vaginal bleeding, breastfeeding, severely impaired renal/hepatic/ cardiac function, porphyria, genital neoplasia D/I: warfarin, carbamazepine, cyclosporine, tacrolimus, anti-hypertensives
doxycycline
Tetracycline derivative; inhibit protein synthesis
100 mg PO bid x ≥7d
Chlamydia, gonococcal infection, syphilis
S/E: GI upset, hepatotoxicity C/I: pregnancy, severe hepatic dysfunction D/I: warfarin, digoxin
fluconazole (Diflucan®)
Antifungal; disrupt fungal cell membrane
150 mg PO x 1 dose
Vulvovaginal candidiasis unresponsive to clotrimazole
S/E: headache, rash, nausea, vomiting, abdo pain, diarrhea D/I: terfenadine, cisapride, astemizole, hydrochlorothiazide, phenytoin, warfarin, rifampin
leuprolide (Lupron®)
Synthetic GnRH analog Induces reversible hypoestrogenic state
3.75 mg IM q1month or 11.25 mg IM q3months Usually ≤6 months, check bone density if >6 months
Endometriosis Leiomyomata DUB Precocious puberty
S/E: hot flashes, sweats, headache, vaginitis, reduction in bone density C/I: pregnancy, undiagnosed vaginal bleeding, breastfeeding
menotropin (Pergonal®)
Human Gonadotropin with FSH and LH effects; induce ovulation and stimulate ovarian follicle development
75-150 U of FSH and LH IM qd x 7-12d, Infertility then 10,000 U hCG one day after last dose
S/E: bloating, irritation at injection site, abdo/pelvic pain, headache, nausea and vomiting C/I: primary ovarian failure, intracranial lesion (e.g. pituitary tumour), uncontrolled thyroid/adrenal dysfunction, ovarian cyst (not PCOS), pregnancy
metronidazole (Flagyl®)
Bactericidal; forms toxic metabolites which damage bacterial DNA
2 g PO x 1 dose or 500 mg PO bid x 7d Bacterial vaginosis, trichomonas vaginitis
S/E: headache, dizziness, nausea, vomiting, diarrhea, disulfiram-like reaction (flushing, tachycardia, nausea and vomiting) C/I: pregnancy (1st trimester) D/I: cisapride, warfarin, cimetidine, lithium, alcohol
Toronto Notes 2011
Common Medications
Gynecology GY51
Table 22. Common Medications (continued) Drug Name (Brand Name)
Action
oxybutinin (Ditropan®)
Indications
Side Effects (S/E), Contraindications (C/I), Drug Interactions (D/I)
Anticholinergic – relaxes 5 mg PO bid-tid bladder smooth muscle, inhibits involuntary detrusor contraction
Overactive bladder (urge incontinence)
S/E: dry mouth/eyes, constipation, palpitations, urinary retention C/I: glaucoma, GI ileus, severe colitis, obstructive uropathy, use with caution if impaired hepatic/renal function
tolterodine (Detrol®)
Anticholinergic
1-2 mg PO bid
Overactive bladder (urge incontinence)
S/E: anaphylaxis, psychosis, tachycardia, dry mouth/eyes, headache, constipation, urinary retention, chest pain C/I: glaucoma, gastric/urinary retention, use with caution if impaired hepatic/renal function
tranexamic acid (Cyklokapron®)
Anti-fibrinolytic, reversibly inhibits plasminogen activation
1-1.5 g tid-qid for first Menorrhagia 4 days of cycle Ophthalmic check if used for several weeks
S/E: nausea, vomiting, diarrhea, dizziness, rare cases of thrombosis C/I: thromboembolic disease, acquired disturbances of colour vision, subarachnoid hemorrhage, age 35 years, migraines with focal neurological symptoms (excluding aura), uncontrolled hypertension Relative – non focal migraines with aura 20% (normal is 60 years old Clinical Features • insidious onset • fatigue, weakness, pallor, infections, bruising, epistaxis • rarely: weight loss, fever, hepatosplenomegaly • infections and bleeding out of proportion with peripheral blood counts Investigations • diagnosed by anemia ± thrombocytopenia ± neutropenia bone marrow hypercellularity with tri-lineage dysplastic changes (dysmyelopoiesis, dyserythropoiesis, dysthrombopoiesis) • CBC and peripheral blood film RBC: usually macrocytic with oval shaped red cells (macro-ovalocytes), decreased reticulocyte count WBC: decreased granulocytes and abnormal morphology (e.g. bilobed or unsegmented nuclei = Pelger abnormality) platelets: thrombocytopenia, abnormalities of size and cytoplasm (e.g. giant hypogranular platelets)
Toronto Notes 2011
Myeloid Malignancies/Myeloproliferative Neoplasms (MPNs)
• bone marrow aspirate and biopsy with cytogenetic analysis required for definitive diagnosis bone marrow – normocellular/hypercellular (but 10% hypocellular), often with micromegakaryocytes; 10% have marrow fibrosis may see ring sideroblasts in varying proportion cytogenetics – partial or total loss of chromosomes 5, 7, Y, or trisomy 8 Prognosis • the MDS International Prognostic Scoring System (IPSS) uses 3 factors to estimate mean survival: the percentage of bone marrow blasts, the karyotype and the number of cytopenias based on the calculated score, a patient’s MDS is categorized as “low”, “intermediate 1”, “intermediate 2”, or “high” with a mean survival of 0.4, 3.5, 1.2 and 5.7 years respectively Treatment • low risk of transformation to acute leukemia (IPSS low and intermediate 1) supportive care: RBC and platelet transfusion, antibiotics, antifungals erythropoietin SC weekly may be effective in reducing transfusion requirements hematopoietic growth factors (G-CSF, GM-CSF) may decrease risk of infection • high risk of transformation to acute leukemia (IPSS intermediate 2 and high) supportive care stem cell transplantation AML-type chemotherapy epigenetic therapy: DNA methyltransferase inhibitors (e.g. 5-Azacytidine), histone deacetylase inhibitors angiogenesis inhibitors arsenic trioxide farnesyl transferase inhibitors
Hematology H37 Use of Epoetin and Darbepoetin in Patients with Cancer Blood 2008 ;111:25-41 Clinical practice guideline update by American Societies of Hematology and Clinical Oncology (2007) Recommendations: 1) Initiate an erythropoiesis-stimulating agent (ESA) when hemoglobin (Hb) is near or below 100 g/L (10 g/dL) in patients with chemotherapyassociated anemia to decrease the need for transfusions. 2) Same as #1 for patients with low-risk myelodysplasia. 3) Follow the package insert for dose initiation and modification. 4) Discontinue ESAs when patient not responding to treatment beyond 6 to 8 weeks. 5) Monitor iron stores and supplement iron intake for ESA-treated patients when necessary. 6) Use ESAs cautiously with chemotherapy or in patients with an elevated risk for thromoembolic complications. 7) ESA should not be used for patients with cancer who are not receiving chemotherapy, as it increases thromboembolic risks and lowers survival rate.
Myeloproliferative Neoplasms (MPNs) Definition • clonal myeloid stem cell abnormalities leading to overproduction of one or more cell lines (leading to abnormalities in erythrocytes, platelets and other cells of myeloid lineage) Basophilia is uncommon in other medical conditions.
Epidemiology • mainly middle-aged and older patients (peak 60-80 years) Prognosis • may develop marrow fibrosis with time • all disorders may progress to AML Table 20. Chronic Myeloproliferative Disorders PV
CML
IMF
ET
Hct
ÓÓ
Ô/N
Ô
N
WBC
Ó
ÓÓ
Ó/Ô
N
Plt
Ó
Ó/Ô
Ó/Ô
ÓÓÓ
Marrow fibrosis
±
±
+++
±
Splenomegaly
+
+++
+++
+
Hepatomegaly
+
+
++
–
Genetic Association
JAK2 mut. (95%)
Bcr-Abl mut. (90+%)
JAK2 mut. (~50%)
JAK2 mut. (~50%)
PV = polycythemia vera CML = chronic myeloid leukemia IMF = idiopathic myelofibrosis ET = essential thrombocythemia
H38 Hematology
Myeloproliferative Neoplasms (MPNs)
Toronto Notes 2011
Polycythemia Rubra Vera (PRV) Erythromelalgia is a pathognomonic microvascular thrombotic complication in PRV and ET.
Definition • stem cell disorder characterized by elevated RBC mass (erythrocytosis) accompanied by increased white cell and platelet production Clinical Features • those secondary to high red cell mass and hyperviscosity (see Polycythemia, H5) • bleeding complications: epistaxis, gingival bleeding, ecchymoses and GI bleeding due to platelet abnormalities • thrombotic complications: DVT, PE, thrombophlebitis, increased incidence of stroke, MI due to increased blood viscosity, increased platelet number and/or activity • erythromelalgia (burning pain in hands and feet) associated with platelets >400 x 109/L pathognomonic microvascular thrombotic complication in PRV and ET • pruritus, especially after warm bath or shower (40%) due to cutaneous mast cell degranulation and histamine release • epigastric distress, PUD due to increased histamine from tissue basophils, alterations in gastric mucosal blood flow due to increased blood viscosity • gout (hyperuricemia) due to increased cell turnover • characteristic physical findings plethora (ruddy complexion) of face (70%), palms splenomegaly (70%), hepatomegaly (40%)
Efficacy and Safety of Low-dose Aspirin in Polycythemia Vera NEJM 2004; 350:114-24 Study: Double-blind, placebo-controlled, randomized trial. Participants: 518 patients with polycythemia vera with no clear indication for or contraindication to aspirin therapy. Intervention: Patients received either low-dose aspirin 100 mg daily (n=253) or placebo (n=265) and were followed for up to 5 years. Primary Outcome: Cumulative rate of (I) nonfatal MI, nonfatal stroke, or death from cardiovascular causes and the cumulative rate of (II) the previous 3 plus pulmonary embolism and major venous thrombosis. Results: Primary outcomes (I) and (II) were reduced with treatment compared to placebo (RR 0.41; P=0.09 and RR 0.4; P=0.03, respectively). There were no differences in overall or cardiovascular mortality and major bleeding episodes. Conclusion: Low-dose aspirin can safely prevent thrombotic complications in patients with polycythemia vera.
Investigations • see Polycythemia, H5 • must rule out secondary polycythemia • diagnosis (WHO) involves two required (*) A criteria + 1 other A or 2 B criteria A Criteria (Major) elevated red cell mass* (>25% above mean predicted value) no cause of 2° erythrocytosis * (i.e. arterial PO2 >92%) palpable splenomegaly clonal genetic abnormality other than bcr-abl fusion gene endogenous erythroid colony formation in vitro B Criteria (Minor) thrombocytosis (>400 x 109/L) leukocytosis (>12 x 109/L) bone marrow biopsy revealing panmyelosis with erythroid and megakaryocytic proliferation low serum EPO level • JAK2 mutation identified in most cases Treatment • phlebotomy to keep hematocrit 65, prior thrombosis or symptoms), 32P (age >80 or lifespan 90% those who do NOT achieve CCR on imatinib – 6 year overall survival (OS) of 66% • acute phase (blast crisis – usually within 3-5 years) 2/3 develop a picture similar to AML unresponsive to remission induction 1/3 develop a picture similar to ALL remission induction (return to chronic phase) achievable
Imatinib Compared with Interferon and Lowdose Cytarabine for Newly Diagnosed Chronicphase Chronic Myeloid Leukemia NEJM 2003; 348:994-1004 Study: Randomized, open-label, multicenter trial. Patients: 1106 patients with newly diagnosed chronic-phase chronic myeloid leukemia (CML). Intervention: Imatinib (553 patients) or interferonalfa plus low-dose cytarabine (553 patients). Outcome: Patients were evaluated for hematologic and cytogenetic responses, toxic effects, and rates of progression. Results: After a median follow-up of 19 months, the estimated rate of a major cytogenetic response (0 to 35% of cells in metaphase positive for the Philadelphia chromosome) at 18 months was 87.1% (95%CI, 84.1 to 90.0) in the imatinib group and 34.7% (95% CI, 29.3 to 40.0) in the group given interferon-alfa plus cytarabine (p600 x 109/L bone marrow biopsy – proliferation of megakaryocyte lineage with enlarged, mature megakaryocytes acquired JAK2 mutation • criteria for exclusion: no evidence of PRV, CML, IMF, MDS no bcr-abl fusion gene no evidence of bone marrow collagen or reticulin fibrosis no evidence of reactive thrombocytosis due to inflammation, infection, neoplasm, prior splenectomy Clinical Features • often asymptomatic • vasomotor symptoms (40%) headache (common), dizziness, syncope erythromelalgia (burning pain of hands and feet, dusky colour, usually worse with heat, caused by platelet activation g microvascular thrombosis) • thrombosis (arterial and venous) • bleeding (often GI; associated with platelets >1000 x 109/L) • constitutional symptoms, splenomegaly • pregnancy complications; increased risk of spontaneous abortion • risk of transformation to AML (0.6-5%), myelofibrosis
Etiology of Secondary Thrombocythemia Infection Inflammation (IBD, arthritis) Malignancy Hemorrhage Iron deficiency Hemolytic anemia Post splenectomy Post chemotherapy
Investigations • CBC: increased platelets; may have abnormal platelet aggregation studies • bone marrow hypercellularity, megakaryocytic hyperplasia, giant megakaryocytes • increased K, increased PO4 (2o to release of platelet cytoplasmic contents) • diagnosis: exclude other myeloproliferative disorders and reactive thrombocytosis Treatment • low dose aspirin if previous history of thrombotic event, ≥1 cardiovascular risk factors, older or symptomatic • cytoreductive therapy if thrombosis or thrombotic symptoms: hydroxyurea (HU) (1st line therapy), anagrelide, interferon-alpha, or 32P (age >80 or lifespan 38oC unexplained weight loss (>10% of body weight in 6 months) night sweats
Toronto Notes 2011
Lymphomas
Hematology H43
Table 22. Chromosome Translocations Translocation
Gene Activation
Associated Neoplasm
t(8;14)
c-myc activation
Burkitt’s lymphoma
t(14;18)
bcl-2 activation
Follicular lymphoma
t(9;22)
Philadelphia chromosome (bcr-abl hybrid)
CML, ALL in adults (25% of the time)
t(11;14)
Overexpression protein cyclin D1
Mantle cell lymphoma
Hodgkin’s Lymphoma Definition • malignant proliferation of lymphoid cells with Reed-Sternberg cells (thought to arise from germinal centre B-cells) Epidemiology • bimodal distribution with peaks at 20 years and >50 years • association with Epstein-Barr virus in up to 50% of cases Clinical Features • asymptomatic lymphadenopathy (70%) non-tender, rubbery consistency cervical/supraclavicular (60-80%), axillary (10-20%), inguinal (6-12%) • splenomegaly (50%) ± hepatomegaly • mediastinal mass found on routine CXR, may be symptomatic (cough) rarely may present with SVC syndrome, pleural effusion • systemic symptoms B symptoms (especially in widespread disease; fever in 30%), pruritus • non-specific/paraneoplastic alcohol-induced pain in nodes, nephrotic syndrome • starts at a single site in lymphatic system (node), spreads first to adjacent nodes disease progresses in contiguity with lymphatic system
Hodgkin’s Lymphoma classically presents as a painless, non-tender, firm, rubbery enlargement of superficial lymph nodes, most often in the cervical region.
Investigations • CBC anemia (chronic disease, rarely hemolytic), eosinophilia, leukocytosis, platelets normal or increased early, decreased in advanced disease • biochemistry LFTs (liver involvement) RFTs (prior to initiating chemotherapy) ALP, Ca (bone involvement) ESR, LDH (monitor disease progression) • imaging CXR, CT chest (lymph nodes, mediastinal mass), CT abdomen/pelvis (liver or spleen involvement), gallium scan (assess treatment response) cardiac function assessment – (MUGA or echocardiography) for patients at high risk of pretreatment cardiac disease (age >60, history of HTN, CHF, PUD, CAD, MI, CVA) PFTs – if history of lung disease (COPD, smoking, previous radiation to lung) • excisional lymph node biopsy confirms diagnosis • bone marrow biopsy to assess marrow infiltration (only necessary if B symptoms, stage III or IV, bulky disease or cytopenia) Treatment • stage I-II: chemotherapy (ABVD) followed by involved field radiotherapy (XRT) • stage III-IV: chemotherapy (ABVD, BEACOPP) with XRT for bulky disease • relapse, resistant to therapy: high dose chemotherapy, bone marrow transplant PET scans used to follow response to treatment Complications of Treatment • cardiac disease – secondary to XRT (adriamycin is cardiotoxic) • pulmonary disease – secondary to bleomycin (interstitial pneumonitis) • infertility – recommend sperm banking • secondary malignancy in irradiated field 10 years after treatment non-Hodgkin’s lymphoma • hypothyroidism – post XRT
CHOP = cyclophosphamide, hydroxydoxorubicin (Adriamycin), vincristine (Oncovin), prednisone VAD = vincristine, adriamycin, dexamethasone ABVD = adriamycin, bleomycin, vinblastine, dacarbazine BEACOPP = bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine and prednisone
H44 Hematology
International Prognostic Factors Project 1998 Prognostic Factors FFP 0 84% 1 77% 2 67% 3 60% 4 51% 5-7 42% FFP = freedom from progression at 5 years.
Lymphomas
Toronto Notes 2011
Prognosis • adverse prognostic factors: 1. serum albumin 1 extranodal site based on calculated risk, mean 5 year survival ranges from 26-73% Table 23. Characteristics of Selected Non-Hodgkin’s Lymphomas Follicular Lymphoma
Diffuse Large B-Cell Lymphoma (DLBCL)
Burkitt’s Lymphoma
Mantle Cell Lymphoma
Percentage of NHLs
22-30%
33%
F Pathophysiology • accumulation of neoplastic lymphocytes in blood, bone marrow, lymph nodes and spleen Clinical Features • 25% asymptomatic (incidental finding) • 5-10% present with B symptoms (≥1 of: unintentional weight loss ≥10% of body weight within previous 6 months, fevers >38ºC or night sweats for ≥2 weeks without evidence of infection, extreme fatigue) • lymphadenopathy (50-90%), splenomegaly (25-55%), hepatomegaly (15-25%) • immune dysregulation – autoimmune hemolytic anemia (Coombs positive), immune thrombocytopenic purpura (ITP), hypogammaglobulinemia ± neutropenia • bone marrow failure – late, secondary to marrow involvement by CLL cells
Smudge cells are artifacts of damaged lymphocytes from slide preparation.
Investigations • CBC: absolute lymphocytosis >5 x 109/L • peripheral blood film lymphocytes are small and mature smudge cells • flow cytometry • cytogenetics – FISH • bone marrow aspirate lymphocytes >30% of all nucleated cells infiltration of marrow by lymphocytes in 3 patterns: nodular (10%), interstitial (30%), diffuse (35%, worse prognosis) or mixed (25%) Natural History and Treatment • natural history – indolent but incurable, with slow progression; thus select gentlest treatment that will control symptoms observation if early, stable, asymptomatic intermittent chlorambucil or fludarabine chemotherapy combined with Ritxuimab corticosteroids, IVIG – especially for autoimmune phenomena radiotherapy • small minority present with aggressive disease; usually associated with chromosomal abnormalities (e.g. p53 deletion) • 9 year median survival, but varies greatly
Toronto Notes 2011
Malignant Clonal Proliferations of Mature B Cells
Hematology H47
• prognosis predicted by Rai staging low risk: lymphocytosis in blood and bone marrow only intermediate risk: lymphocytosis with enlarged nodes in any site or splenomegaly, hepatomegaly high risk: lymphocytosis with disease-related anemia ( lambda light chain better prognosis.
Malignant Clonal Proliferations of Mature B Cells
Toronto Notes 2011
Investigations • CBC normocytic anemia, thrombocytopenia, leukopenia rouleaux formation on peripheral film • biochemistry increased Ca, increased ESR, decreased anion gap, increased Cr, albumin, beta2microglobulin (as part of staging), proteinuria (24 hour urine collection) • monoclonal proteins serum protein electrophoresis (SPEP) – demonstrates monoclonal protein spike in serum in 80% (i.e. M protein) urine protein electrophoresis (UPEP) – demonstrates light chains in urine = Bence-Jones Protein (15% only secrete light chains) immunofixation – demonstrates M protein and identifies Ig type; also identifies light chains serum free light chain quantification – kappa and lambda light chains, calculated ratio • bone marrow aspirate and biopsy often focal abnormality, greater than 10% plasma cells, abnormal morphology, clonal plasma cells; send for FISH or cytogenetics (prognostic implications) • skeletal series (x-rays), MRI if symptoms of cord compression presence of lytic lesions and areas at risk of pathologic fracture bone scans are not useful since they detect osteoblast activity • beta-2 microglobulin, LDH and CRP are poor prognosticators Diagnosis • International Myeloma working group criteria 1. serum or urinary monoclonal protein 2. presence of clonal plasma cells in bone marrow or a plasmacytoma 3. presence of end-organ damage related to plasma cell dyscrasia, such as: increased serum Ca lytic bone lesions anemia renal failure Treatment • treatment is non-curative • treatment goals: improvement in quality of life (improve anemia, reverse renal failure, bony pains) prevention of progression and complications increase overall survival • autologous stem cell transplant if 70 years old or transplant-ineligible consider melphalan and prednisone alone if >75 years old melphalan, prednisone and thalidomide or melphalan, prednisone and bortezomib if 65-75 years • dexamethasone and bortezomib if ARF; bortezomib ± dexamethasone in light chain amyloidosis • supportive management: bisphosphonates for those with osteoporosis or lytic bone lesions local XRT for bone pain, spinal cord compression kyphoplasty for vertebral fractures to improve pain relief and regain height treat complications: hydration for hypercalcemia and renal failure, bisphosphonates for severe hypercalcemia, prophylactic antibiotics, erythropoietin for anemia • all patients will relapse; choice of retreatment regimen depends on duration of remission, organ involvement, patient’s comorbidities and preferences Prognosis • Salmon-Durie (Hb, calcium, M protein, radiograph appearance and creatinine) and International Staging System (beta-2 micoglobulin, CRP, chromosome 13 status, serum IL-6, duration of initial plateau phase) used to stage and estimate prognosis • median survival based on stage usually 16-70 months
Toronto Notes 2011
Malignant Clonal Proliferations of Mature B Cells/Complications of Hematologic Malignancies
Monoclonal Gammopathy of Unknown Significance (MGUS) Definition • presence of M protein in serum in absence of any clinical or laboratory evidence of a plasma cell dyscrasia or lymphoproliferative disorders incidence: 0.15% in general population, 5% of people >70 years of age asymptomatic Diagnosis • presence of a serum monoclonal protein (M-protein) at a concentration 20-40 mmol/L) 3. high FENa Table 5. Disorders Associated with SIADH Tumour
Pulmonary
CNS
Drugs
Miscellaneous
Small cell Ca Bronchogenic Ca AdenoCa of pancreas Hodgkin’s disease Thymoma
Pneumonia Lung abscess TB Acute respiratory failure Positive pressure ventilation
Mass lesion Encephalitis Subarachnoid hemorrhage Stroke Head trauma Acute psychosis Acute intermittent porphyria
Antidepressants TCAs SSRIs Antineoplastics Vincristine Cyclophosphamide Other DDAVP Oxytocin Nicotine Carbamazepine Barbiturates Chlorpropamide
Post-op state Pain Severe nausea HIV
Hypernatremia • hypernatremia: serum [Na] >145 mmol/L • too little water relative to total body Na; always a hyperosmolar state • usually due to net water loss, rarely due to hypertonic Na gain • results from problems with water intake (access, thirst) and/or site of increased water loss (renal or extrarenal) • less common than hyponatremia because patients are protected against hypernatremia by thirst and release of ADH Hypernatremia
Non-Hypervolemic
Hypervolemic (rare) • Iatrogenic (hypertonic saline or NaHCO3) • Cushing’s syndrome • Hyperaldosteronism • Treat with salt restriction, diuretics, water replacement • Dialysis if renal failure
Is patient putting out a small volume (500 mL/d) of maximally concentrated (>800 mOsm/kg) urine?
No Is urine osmole excretion rate >750 mOsm/d ?
No Positive renal response to DDAVP 50% increase in urine osmolality?
No • Nephrogenic DI
Yes • Insensible water loss • Respiratory, skin • GI (diarrhea) • Osmotic (lactulose, malabsorption) • Remote renal loss
Yes • Diuretics (loop) • Osmotic diuresis Hyperglycemia Endogenous (urea with excess NG protein feeds)
Yes • Central DI
Figure 8. An Approach to Hypernatremia
Signs and Symptoms • with acute hypernatremia no time for adaptation, therefore more likely to be symptomatic • adaptive response: cells import and generate new osmotically active particles to normalize size • due to brain cell shrinkage: altered mental status, weakness, neuromuscular irritability, focal neurologic deficits, seizures, coma, death • ± polyuria, thirst, signs of hypovolemia Complications • increased risk of vascular rupture resulting in intracranial hemorrhage • rapid correction may lead to cerebral edema due to ongoing brain hyperosmolarity
Toronto Notes 2011
Electrolyte Disorders
Treatment of Hypovolemic Hypernatremia • general measures for all patients give free water (oral or IV) treat underlying cause monitor serum Na frequently to ensure correction is not occurring too rapidly • if evidence of hemodynamic instability, must first correct volume depletion with NS bolus • loss of water is often accompanied by loss of Na but a proportionately larger water loss • in patients with presumed normal total body Na content, use formula to calculate water deficit: H2O deficit = TBW x (serum [Na] - 140) [TBW = 0.6 x wt(kg) for men, 0.5 x wt(kg) for women] 140 • replace free water deficit; “free water” is water without sodium • encourage patient to drink pure water, as oral route is preferred for fluid administration • if unable to replace PO or NG, correct H2O deficit with hypotonic IV solution 1L D5W approximately equals 1 L free water 1L 0.45% NS approximately equals 500 mL free water • use formula (see Hyponatremia, NP11) to estimate expected change in serum Na with 1 L infusate • aim to to lower [Na] by no more than 12 mmol/L in 24 hours (0.5 mmol/L/hr) • must also provide maintenance fluids and replace ongoing losses • rule of thumb: give 2 cc/kg/hour of free water to correct serum [Na] by about 0.5 mmol/L/hour or 12 mmol/L/day Treatment of Hypervolemic Hypernatremia • general measures as above • hypervolemic hypernatremia: remove excess total body Na with diuresis or dialysis (if renal failure present), then replace water deficit using D5W DIABETES INSIPIDUS (DI) • collecting tubule is impermeable to water due to absence of ADH or impaired response to ADH • central defect in release of ADH (central DI) or renal response to ADH (nephrogenic DI) Etiology • central DI: neurosurgery, granulomatous diseases, trauma, vascular events, and malignancy • nephrogenic DI: lithium (most common), hypokalemia, hypercalcemia, and congenital Diagnosis • urine osmolality inappropriately low in patient with hypernatremia (Uosm decrease HCO3, there is a coexisting metabolic alkalosis 4. Calculate osmolar gap osmolar gap = measured osmolality – calculated osmolality calculated osmolality = (2 x Na) + urea + glucose (all units are in mmol/L) normal osmolar gap 10, consider: methanol poisoning, ethylene glycol poisoning, OR another cause of acidosis plus ethanol ingestion Etiology and Pathophysiology 1. Increased PAG Metabolic Acidosis (4 types) 1. Lactic acidosis (2 types) L-lactic acid – Type A: due to tissue hypoperfusion (any cause of shock), ischemic bowel, profound hypoxemia – Type B: failure to metabolize normally produced lactic acid in the liver due to severe liver disease, excessive alcohol intake, thiamine deficiency, or metformin accumulation (metformin interferes with electron transport chain) D-lactic acid: rare syndrome characterized by episodes of encephalopathy and metabolic acidosis, requires carbohydrate malabsorption (e.g. short bowel syndrome), colonic bacteria that produce D-lactic acid, a carbohydrate load, diminished colonic motility and impaired D-lactate metabolism 2. Ketoacidosis diabetic starvation alcoholic (decreased carbohydrate intake and vomiting) 3. Toxins methanol (toxic to brain and retina, can cause blindness and brain death) – metabolized to formic acid ethylene glycol (toxic to brain and kidneys) – metabolized to oxalic acid (envelope shaped crystals in urine) salicylate 4. Advanced renal failure (i.e. serum Cr increased at least 5x above baseline – a very low GFR causes anion retention, and renal disease leads to impaired bicarbonate production)
Useful Equations 1. PAG = [Na] - [Cl] - [HCO3-] (normal range = 10-14) 2. Osmolar Gap = measured osmolality – calculated osmolality (normal 20 mg/mmol, (>180 mg/d); in women, ACR is >28 mg/mmol (>250 mg/d) clinically detectable proteinuria, +ve urine dipstick normal GFR very expanded mesangial matrix • stage 4 increased proteinuria (>500 mg/24hr) decreased GFR 900 mg, serum Creatinine (Cr) 88(Male)/106(Female)265 µmol/L) Intervention: BP control with Irbesartan vs. Amlodipine vs. Placebo, with use of adjuncts (not including ACEs, ARBs, or CCI) as needed. Outcomes: Primary composite endpoint included doubling of serum Cr, ESRD, or death. Secondary composite endpoint included morbidity and mortality from CVD causes. Results: BP control was similar in all three arms. Irbesartan had a relative risk reduction of 20% vs. placebo and 23% vs. amlodipine for the primary end point. The Irbesartan group had a 33% risk reduction vs. placebo and 37% reduction vs. amlodipine for risk of doubling serum Cr. Serum Cr increased more slowly in the Irbesartan group versus placebo or amlodipine. No difference in absolute mortality or secondary end point. Conclusion: Irbesartan conferred significant renoprotective benefits in patients with type 2 diabetes and nephropathy, independent of blood pressure lowering effects.
Renal Outcomes with Telmisartan, Ramipril, or Both in People at High Vascular Risk (ONTARGET Study) Lancet 2008; 372:547-553 Study: Prospective, multicentre, double-blind, randomized controlled trial. Participants: 25,620 patients with median followup of 56 months. Intervention: Patients received either ramipril (10 mg/d; N=8576), telmisartan (80 mg/d; N=8542) or a combination of both drugs (N=8502). Primary Outcome: Composite of dialysis, doubling of creatinine level, and death. Results: The number of outcome events was similar for telmisartan (n=1147) and ramipril (1150; HR 1.00, CI 0.92-1.09), but was increased with combination therapy (1233; HR 1.09, 1.01-1.18, p=0.037). The need for dialysis or doubling of serum creatinine, was similar with telmisartan (189) and ramipril (174; HR 1.09, 0.89-1.34) and more frequent with combination therapy (212; HR 1.24, 1.01-1.51, p=0.038). Estimated GFR declined least with ramipril compared with telmisartan or combination therapy (p60 mL/min: ACEI or ARB – CrCl 30% rise in serum Cr or hyperkalemia, discontinue medication and consider 2nd line agent • consider holding ACEI, ARB and/or diuretic with acute illness and in women before becoming pregnant • consider referral to nephrologist if ACR >60 mg/mmol, eGFR 65 years of age have dementia • common etiologies: 60-80% Alzheimer’s Disease (AD); 10-20% vascular dementia • 400 IU/day associated with excess mortality, level 1 evidence) • symptomatic management low dose neuroleptic trazodone for sleep disturbance antidepressants Prognosis • progressive • mean duration of disease 10 years
Lewy Body Disease (LBD) Definition • progressive cognitive decline interfering with social or occupational function; memory loss may or may not be an early feature • one (possible LBD) or two (probable LBD) of the following: fluctuating cognition with pronounced variation in attention and alertness recurrent visual hallucinations parkinsonism
Neurology N13
N14 Neurology
Behavioural Neurology
Toronto Notes 2011
Etiology and Pathogenesis • Lewy bodies (eosinophilic cytoplasmic inclusions) found in both cortical and subcortical structures Epidemiology • 15-25% of all dementias Signs and Symptoms • fluctuation in cognition with progressive decline • visual hallucinations • parkinsonism • repeated falls • sensitivity to neuroleptic medications (develop rigidity, neuroleptic malignant syndrome, extrapyramidal symptoms) • REM sleep disorder Treatment • acetylcholinesterase inhibitors (e.g. donepezil) Prognosis • typical survival 3-6 years
Frontotemporal Dementia (FTD) Definition • progressive dementia characterized by core symptoms of either disinhibition and emotional lability or of apathy and detachment Etiology and Pathogenesis • gross pathology atrophy of frontal and temporal poles • microscopic pathology Pick bodies (intraneuronal inclusions containing abnormal Tau proteins) Epidemiology • 10% of all dementias Signs and Symptoms • core features • behavioural disorder impairment of personal conduct and of regulation of social interactions decline in personal hygiene and grooming mental rigidity/inflexibility perseverative and stereotyped behaviour • speech and language altered speech output (economy or pressure of speech) echolalia/perseveration • physical signs primitive reflexes (i.e. pout, grasp, palmomental, glabellar) parkinsonism Investigations • MRI/SPECT – frontotemporal atrophy/hypometabolism
Creutzfeldt-Jakob Disease (CJD) Definition • rare degenerative fatal brain disorder Pathophysiology • prion proteins causing alterations in the brain such as spongiform changes, astrocytosis and neuronal loss Epidemiology • rare (1 in a million), peak incidence between 50-70 years old
Toronto Notes 2011
Behavioural Neurology
Neurology N15
Clinical Presentation • sporadic CJD: rapidly progressive dementing illness causing death within months, associated with myoclonus cerebellar ataxia extrapyramidal signs akinetic mutism and cortical blindness sometimes occur fatal within 1 year EEG: triphasic complexes Diagnosis • rule out treatable dementia, neurologic exam, EEG, MRI • only way to confirm diagnosis is brain biopsy/autopsy Types • sporadic CJD: most common form (85%), no risk factors • hereditary CJD: family history or tests positive for genetic mutation (5-10%) • acquired CJD: transmitted via exposure to prion in nervous system tissue (99% of right-handed people have left hemisphere language representation • 70% of left-handed people have left hemisphere language representation, 15% have right hemisphere representation, and 15% have bilateral representation Assessment of Language • assessment of context handedness (writing, drawing, toothbrush, scissors) education level native language learning difficulties • assessment for aphasia 1. spontaneous speech � fluency � paraphasias: semantic (“chair” for “table”), or phonemic (“clable” for “table”) 2. repetition 3. naming 4. comprehension (auditory and reading) 5. writing 6. neologisms
The left hemisphere is dominant for language in almost all right-handed people and 70% of left-handed people.
Broca’s area: posterior inferior frontal lobe
© Enid Hajderi 2011
Definition • an acquired disturbance of language characterized by errors in speech production, writing, comprehension, or reading
Wernicke’s area: posterior aspect of 1st temporal gyrus
Figure 12. Broca’s and Wernicke’s Areas
N16 Neurology
Behavioural Neurology
Toronto Notes 2011
Table 11. Approach to Aphasias Aphasia localizes the lesion to the dominant cerebral hemisphere.
Fluency
Comprehension
Repetition
Naming
Lesion Localization
Broca’s
Non-fluent
Good
Poor
Poor
Posterior inferior frontal lobe
Motor TCA*
Non-fluent
Good
Good
Poor
1. Frontal lobe watershed between MCA and ACA territories 2. White matter lesions deep to (1)
Poor
Combined sensory and motor transcortical
Mixed TCA*
Non-fluent
Poor
Good
Global
Non-fluent
Poor
Poor
Wernicke’s
Fluent
Poor
Poor
Relatively Spared
Posterior superior temporal lobe
Conduction
Fluent
Good
Poor
Poor
Arcuate fasciculus
Sensory TCA*
Fluent
Poor
Good
Relatively Spared
1. Subcortical temporoparietal 2. Temporoparietal watershed between MCA and PCA territories
Anomic
Fluent
Good
Good
Poor
Numerous possible locations
Posterior inferior frontal lobe AND posterior superior temporal lobe
TCA=Transcortical aphasia *Transcortical aphasias are typically associated with cerebral anoxia (e.g. post-MI, CO poisoning, hypotension)
Prognosis • most recovery from stroke-related aphasia occurs in first three months, but may continue for >1 year • with recovery, the type of aphasia may evolve • poor prognosis: global aphasia
Apraxia Definition • inability to perform skilled voluntary motor sequences that cannot be accounted for by weakness, ataxia, sensory loss, impaired comprehension, or inattention Clinicopathological Correlations Table 12. Apraxia Description
Tests
Hemispheres
Ideomotor
Inability to perform skilled learned motor sequences
Blowing out a match; combing one’s hair
Left
Ideational
Inability to sequence actions
Preparing and mailing an envelope
Right and left
Constructional*
Inability to draw or construct
Copying a figure
Right and left
Dressing*
Inability to dress
Dressing
Right
* Refers specifically to the inability to carry out the learned movements involved in construction, drawing, or dressing; not merely the inability to construct, draw, or dress. Many skills aside from praxis are needed to carry out these tasks.
Agnosia Definition • disorder in the recognition of the significance of sensory stimuli in the presence of intact sensation and naming
Toronto Notes 2011
Behaivoural Neurology/Cranial Nerve Deficits
Neurology N17
Clinicopathological Correlations Table 13. Agnosias Description
Lesion
Aperceptive Visual Agnosia
Inability to name or demonstrate the use of an object presented visually 2o to distorted visual perception Recognition by touch remains intact
Bilateral temporo-occipital cortex
Associative Visual Agnosia
Inability to name an object presented visually 2o to Bilateral inferior temporo-occipital junction disconnect between visual cortex and language areas Visual perception is intact as demonstrated by visual matching
Prosopagnosia
Inability to recognize familiar faces in the presence of intact visual perception and intact auditory recognition
Bilateral occipitotemporal areas or right inferior temporo-occipital region
Colour Agnosia
Inability to perceive colour
Bilateral inferior temporo-occipital lesions
Astereognosis
Inability to identify objects by touch
Anterior parietal lobe in the hemisphere opposite the affected hand
Finger Agnosia
Inability to recognize, name, and point to individual fingers
Dominant hemisphere parietal-occipital lesions
Parietal Lobe Lesions Lesions of the dominant parietal lobe are characterized by Gerstmann’s Syndrome: acalculia, agraphia, finger agnosia, and left-right disorientation. Lesions of the non-dominant parietal lobe are characterized by neglect, anosognosia, and cortical sensory loss.
Cranial Nerve Deficits CN I: Anosmia Clinical Features • absence of sense of smell associated with a loss of taste • usually not recognized by patient if it is unilateral
If anosmia is not associated with loss of taste, consider malingering.
Classification • nasal: odours do not reach olfactory receptors because of physical obstruction heavy smoking, chronic rhinitis, sinusitis • olfactory neuroepithelial: destruction of receptors or their axon filaments influenza, herpes simplex, interferon treatment of hepatitis C virus, atrophic rhinitis (leprosy) • central: lesion of olfactory pathway Kallman syndrome, albinism, head injury, cranial surgery, SAH, chronic meningeal inflammation, meningioma, aneurysm, Parkinson’s disease
SR
CN II: Optic Nerve
Common Lesions • midbrain: bilateral with contralateral pyramidal signs ± mydriasis • posterior communicating artery aneurysm: early mydriasis then CNIII palsy • cavernous sinus (internal carotid aneurysm, meningioma, sinus thrombosis) • ischemia of CNIII (DM, temporal arteritis, HTN, atherosclerosis): pupil sparing
SO
LR SO
IR
Figure 13. Diagnostic Positions of Gaze to Isolate Primary Action of Each Muscle
Pupillary constrictor fibres are on the peripheral aspect of CNIII so compression of the nerve leads to mydriasis while infarction (affecting centre of nerve) causes pupillary sparing.
Optic chiasm
IV Internal carotid artery VI V1
© Katerina Tchmoutina 2010
III
V2
Figure 14. Cavernous Sinus
SR
© Sherry H. Lai 2006
Clinical Features • ptosis, resting eye position is “down and out” (depressed and abducted), pupil dilated (mydriasis)
Sphenoid air sinus
IO MR MR
IR
CN III: Oculomotor Nerve
Dura mater
IO
LR
• see Neuro-Ophthalmology, N20
Pituitary gland
Kallmann’s syndrome is a congenital disorder of anosmia and hypogonadotropic hypogonadism.
Lesions involving the cavernous sinus lead to cranial nerve palsies of III, IV, VI, V1 and V2 as well as pain and proptosis.
N18 Neurology
Cranial Nerve Deficits
Toronto Notes 2011
CN IV: Trochlear Nerve CN IV is the only cranial nerve that exits posteriorly and crosses the midline. A CN IV lesion may cause a contralateral deficit.
CN IV is at risk of trauma during neurosurgical procedures involving the midbrain because of its long intracranial course.
Clinical Features • diplopia (with downward and inward gaze), minimized with head tilt to opposite side • patient may complain of difficulty going down stairs or reading Lesions • common: ischemic (DM, HTN), idiopathic, trauma (TBI or surgical), congenital • other: cavernous sinus lesion, orbital fissure (tumour, granuloma)
CN V: Trigeminal Nerve Lesions • trigeminal neuralgia, herpes zoster, cavernous sinus, orbital fissure, trauma, cerebellopontine angle tumours, demyelination, syringobulbia, metastatic infiltration of nerve, ipsilateral brainstem lesion, contralateral parietal lesion
Herpes Zoster of Trigeminal Nerve: typically involves V1 (opthalmic division). Hutchinson’s Sign: tip of nose involvement; predicts corneal involvement.
Trigeminal Neuralgia (Tic Douloureux) • excruciating unilateral paroxysmal shooting “electric” pains in trigeminal root territory usually in V3 distribution ± V1, V2 normal sensory exam • etiologies: idiopathic, compression by tortuous blood vessel (SCA), cerebellopontine angle tumour (5%), multiple sclerosis (5%) • pain lasts seconds/minutes over days/weeks; remits for weeks/months • triggers: touching face, eating, talking, cold wind, shaving, applying make-up • F > M; usually middle-aged and elderly • medical treatment: carbamazepine; narcotics do not help • if medical treatment fails (order increasingly invasive): gamma knife, invasive percutaneous denervation (radiofrequency/glycerol), percutaneous balloon microcompression, microvascular decompression • rule out structural lesion, multiple sclerosis or vascular lesion with MRI
CN VI: Abducens Nerve Clinical Features • inability to abduct the eye on the affected side • patient complains of horizontal diplopia, worse on ipsilateral lateral gaze
CN VI has the longest intracranial course and is vulnerable to increased ICP, creating a false localizing sign.
Common Lesions • pons (infarction, hemorrhage, demyelination) – associated with facial weakness and contralateral pyramidal signs • tentorial orifice (compression, meningioma) – false localizing sign of increased ICP • cavernous sinus (carotid aneurysm, thrombosis) • vascular – may be secondary to DM, HTN, or temporal arteritis • congenital – Duane’s syndrome
CN VII: Facial Nerve Forehead is spared in a UMN CN VII lesion due to bilateral innervation from cerebral hemispheres.
Clinical Features • ipsilateral facial weakness (involuntary and voluntary) • impaired lacrimation, decreased salivation, numbness behind auricle, hyperacusis, taste dysfunction of anterior 2/3 of tongue Investigations • brainstem (LMN) versus cortical (UMN) symptoms and signs help localize lesion Differential Diagnosis • idiopathic = Bell’s Palsy, 80-90% of cases (see Otolaryngology, OT23) • other: temporal bone fracture, EBV, Ramsay-Hunt (HSV), otitis media/mastoiditis, sarcoidosis, DM mononeuropathy, parotid gland disease
Toronto Notes 2011
Cranial Nerve Deficits
A. Facial nerve lesion (Bell’s palsy)
Neurology N19
B. Supranuclear lesion Supranuclear lesion
Motor cortex
X
Internal capsule
Nucleus of CNVII Facial nerve
X
Lesion in facial nerve
Temporal br. Zygomatic br. Mandibular br.
LMN lesion: complete facial paralysis
UMN lesion: forehead sparing
Cervical br.
© June Li 2010
Buccal br.
Figure 15. UMN vs. LMN Facial Nerve Palsy
CN VIII: Vestibulocochlear Nerve • see Otolaryngology, OT12
CN IX: Glossopharyngeal Nerve Clinical Features • sharp paroxysmal pain of posterior pharynx radiating to ear, triggered by swallowing • taste dysfunction in posterior 1/3 of tongue • absent gag reflex (dysphagia) Treatment • carbamazepine or surgical ablation of CN IX
CN X: Vagus Nerve Clinical Features • dysphagia (palatal and pharyngeal weakness) • dysarthria (laryngeal weakness): inability to produce understandable speech due to impaired phonation (laryngeal sound production) and/or resonance (the alteration of sounds in the cavity between the larynx and the lips/nares) secondary to impaired motor control over peripheral speech organs Table 14. Classification of Dysarthria Classification
Characteristics of Speech
Etiologies*
Flaccid (LMN dysarthria or bulbar palsy)
Slurred, indistinct speech Particular difficulty with vibratory “R” Difficulty with lingual consonants produced by tongue and labial consonants produced by lips
Motor neuron (e.g. ALS) Peripheral nerve (e.g. GBS) Neuromuscular junction (e.g. MG) Myopathy (e.g. DM/PM)
Spastic UMN (dysarthria or pseudobulbar palsy)
Slow and monotonous Strained or strangled Harsh Low pitched
Stroke Tumour Demyelination Degeneration
Ataxic
Slow/altered rhythm Improper stress
Cerebellar disease Cerebellar outflow tract disease
When screening for the presence of dysphagia and assessing risk for aspiration, the presence of a gag reflex is insufficient. Rather, the correct screening test is to observe the patient drinking water from a cup and looking for coughing, choking, or “wetness” of voice.
Differential Diagnosis of Lower Cranial Nerve deficits (CN IX, X, XI, XII) Intracranial/skull base: meningioma, neurofibroma, metastases, osteomyelitis, meningitis Brainstem: stroke, demyelination, syringobulbia, poliomyelitis, astrocytoma Neck: trauma, surgery, tumours
Normal swallowing is initiated when the tongue throws a bolus back into the palatal archway – tongue movements are innervated exclusively by CN XII. The bolus stimulates the soft palate to elevate and the bolus is deflected into the oropharynx. Next the pharyngeal constrictors contract, the larynx elevates, and the vocal cords close. Swallowing depends on afferent information via CN V, IX, and X and motor action via CN V, VII, IX, X, and XII. Connections in the nucleus of the tractus solitarius in the medulla, in proximity to the respiratory centre, act as the swallowing centre. Swallowing and breathing are coordinated to prevent aspiration.
N20 Neurology
Cranial Nerve Deficits/Neuro-Ophthalmology
Toronto Notes 2011
Table 14. Classification of Dysarthria (continued) Classification Extrapyramidal
Characteristics of Speech
Etiologies*
Hypokinetic
Low-pitched Monotonous Decrescendo volume
Parkinson’s disease Other causes of parkinsonism (see Movement Disorders)
Hyperkinetic
Choreiform • Prolonged sentence segments intermixed with silences • Variable, improper stress • Bursting quality Dystonic • Slow speaking rate • Prolonged individual phonemes
Huntington’s disease Dystonia musculorum deformans Other hyperkinetic extrapyramidal disorders (see Movement Disorders)
*Abbreviations: ALS – amyotrophic lateral sclerosis; GBS – Guillain-Barré syndrome; MG – myasthenia gravis; DM – dermatomyositis; PM – polymyositis
CN XI: Accessory Nerve CN XI is vulnerable to damage during neck surgery.
Clinical Features • ipsilateral shoulder drop, weakness on turning head to contralateral side
CN XII: Hypoglossal Nerve Clinical Features • tongue deviation toward side of lesion • chronic LMN lesion: ipsilateral tongue atrophy and fasciculations
NEURO-OPHTHALMOLOGY Abnormalities of Vision Acute Visual Loss • ophthalmologic: acute angle closure glaucoma, vitreous hemorrhage, retinal detachment • optic nerve: optic neuritis, anterior ischemic optic neuropathy (arteritic, non-arteritic), compression by space occupying lesion (e.g. aneurysm) • vascular: TIA/amaurosis fugax, central retinal artery or vein occlusion, carotid-cavernous sinus fistula • CNS: stroke, optic tract/chiasm lesion, migraine • infection/inflammation: endophthalmitis
Optic Neuritis • see Optic Disc Edema, N21, Multiple Sclerosis, N49
Anterior Ischemic Optic Neuropathy If you suspect the diagnosis of giant cell arteritis do not wait for biopsy results. Begin treatment immediately!
• see also Optic Disc Edema, N21 • clinical presentation: painless vision loss over hours to days • non-arteritic (NAION): vision loss due to atherosclerosis • arteritic (AION): normally due to giant cell arteritis (see Rheumatology, RH17)
Amaurosis Fugax • see Ophthalmology, OP37 and Stroke section, N44
Central Retinal Vein Occlusion (CRVO) • see Ophthalmology, OP24
Toronto Notes 2011
Neuro-Ophthalmology
Neurology N21
Optic Disc Edema Table 15. Common Causes of Optic Disc Edema Optic Neuritis
Papilledema
AION
CRVO
Age
50
>50
Vision
Rapid progressive central vision loss with Ô acuity and colour vision
Late visual loss
Unilateral acute field defect with Ô colour vision
Unilateral variable vision loss
Symptoms
Pain (esp with eye movement)
Headache, N/V, local neurological deficits
If GCA: headache, scalp Cardiovascular risk factors tenderness, jaw claudication RAPD
Pupil
RAPD
No RAPD
Fundus
Disc swelling if anterior
Pale segmental disc edema, Disc swelling, retinal hemorrhage, no venous retinal dot, flame hemorrhages pulsations
Swollen disc, venous engorgement, retinal hemorrhage
No RAPD
Etiologies
MS, viral
Increased ICP
Giant cell arteritis
Associated with vasculopathy
Treatment
IV (not oral) methylprednisolone
Treat cause
Consider ASA if non-arteritic; optimize risk factors, reduce steroids if arteritic IOP, ± laser
Abbreviations: AION – anterior ischemic optic neuropathy; CRVO – central retinal vein occlusion; RAPD – relative afferent pupillary defect
Optic Disc Atrophy • etiologies: glaucoma, AION, compressive tumour, optic neuritis, Leber’s hereditary optic neuropathy, congenital • presentation: disc pallor, low visual acuity, peripheral vision defect, decreased colour vision • treatment: none (irreversible), aim to prevent
Abnormalities of Visual Field
Anterior Ischemic Optic Neuropathy (AION)
Definitions • monocular scotoma: an area of absent or diminished vision within an otherwise intact visual field • binocular hemianopsia: loss of half of the visual field homonymous: loss of either the right or left half of the visual field in both eyes bitemporal: loss of both temporal visual fields (lesion of chiasm) quadrantanopsia: loss of one quarter of the visual field BITEMPORAL HEMIANOPSIA • chiasmal lesion in children: craniopharyngioma in middle aged: pituitary mass in elderly: meningioma HOMONYMOUS HEMIANOPSIA • retrochiasmal lesion • the more congruent, the more posterior the lesion • check all hemiplegic patients for ipsilateral homonymous hemianopsia (e.g. left hemisphere g right visual field defect)
3
Optic nerve Optic chiasm Optic tract
4
Temporal radiation (Meyer’s loop) Parietal radiation
1
LGB
Calcarine fissure
1
Right anopsia (right optic nerve lesion)
2
Right anopsia and left upper quadrantanopsia (junctional scotoma)
3
Bitemporal hemianopsia (chiasmal lesion)
4
Left homonymous hemianopsia (right optic tract lesion)
5
Left upper quadrantanopsia (right temporal lesion)
6
Left lower quadrantanopsia (right parietal lesion)
2 5 6
LGB – lateral geniculate body
Figure 16. Characteristic Visual Field Defects with Lesions Along the Visual Pathway
© Willa Bradshaw 2005 after Cecil Hahn
Visual Fields Defects
N22 Neurology
Neuro-Ophthalmology
Toronto Notes 2011
Abnormalities of Eye Movements Disorders of Lateral Gaze Etiology • brainstem infarcts • multiple sclerosis • tumours A lesion in a cerebral hemisphere causes eyes to “look away” from the hemiplegia, and to look towards the lesion. A lesion in the brainstem causes the eyes to “look toward” the side of the hemiplegia, and to look away from the lesion.
Pathophysiology • voluntary eye movements are triggered in the frontal eye fields, located anterior to the precentral gyrus, bilaterally in the frontal lobes • each frontal eye field controls voluntary saccades to the contralateral side via connections to the contralateral paramedian pontine reticular formation (PPRF) • a unilateral lesion in one frontal eye field: prevents voluntary saccades to the opposite side, eyes deviate toward the side of the lesion can be overcome with doll’s eye maneuver • a unilateral lesion in the PPRF in the pons: prevents voluntary saccades to the ipsilateral side, eyes deviate away from the lesion cannot be overcome with doll’s eye maneuver • seizure involving a frontal eye field: cause eye deviation towards the opposite side
Internuclear Ophthalmoplegia (INO) FEF
Etiology • MS (most common; see Multiple Sclerosis, N49) • brain stem infarction • neoplasm • AV malformations • Wernicke’s encephalopathy
CN III MLF CN VI R R
L
L Standard (normal)
Right (normal)
Left (abnormal)
Vergence (normal) © Shelley Wall 2003
Figure 17. Internuclear Ophthalmopliegia
Diplopia worse at end of the day suggests myasthenia gravis (e.g. fatiguable).
If only diplopia on extremes of gaze, cover each eye in isolation during extremes of gaze. The covered eye that makes the outermost image disappear is the one with pathology.
Pathophysiology • results from a lesion in medial longitudinal fasciculus (MLF) which disrupts coordination between CN VI nucleus in pons and the contralateral CNIII nucleus in midbrain disrupts conjugate horizontal gaze Clinical Features • on gaze away from the side of the lesion: 1) adduction of ipsilateral eye is impaired; 2) full excursion of contralateral eye in abduction but with monocular abduction nystagmus cannot be overcome by caloric testing accommodation reflex intact • may be bilateral • upbeating nystagmus on upward gaze often present
Diplopia Monocular • mostly due to relatively benign optical problems (refractive error, cataract, functional) Binocular • cranial nerve palsy (see Cranial Nerves, N17) CN III (oculomotor) DM, aneurysm, tumour, trauma isolated CN III palsy with pupil sparing usually due to DM and most will resolve spontaneously in several months isolated CN III palsy with pupil involved usually indicates compressive lesion (especially posterior communicating artery aneurysm) CN IV (trochlear) DM, trauma CN VI (abducens) DM, tumour, trauma, raised ICP (false localizing sign) muscle Graves’ ophthalmopathy • neuromuscular junction myasthenia gravis (MG) (see Myasthenia Gravis, N32) • other orbital trauma, tumour Wernicke’s encephalopathy Miller-Fischer variant of GBS leptomeningial disease
Toronto Notes 2011
Neuro-Ophthalmology
Neurology N23
Nystagmus • definition: rapid, involuntary, small amplitude movements of the eyes that are rhythmic in nature • direction of nystagmus is defined by the rapid component of the eye movement • can be categorized by movement type (pendular, jerking, rotatory, coarse) or as normal vs. pathological
Abnormalities of Pupils Relative Afferent Pupillary Defect (RAPD) (Marcus-Gunn Pupil) • see also Ophthalmology, OP33 Definition • a failure of direct pupillary responses to light, caused by a defect in the visual afferent pathway anterior to the optic chiasm • clinical testing swinging light test swing light from one eye to the other; both pupils should constrict initially when normal side is illuminated, both pupils constrict when damaged side is illuminated, both pupils paradoxically dilate because the damaged eye perceives less light relative to normal eye pupil reacts poorly to light, and better to accommodation • differential diagnosis optic neuritis is the most common cause of RAPD other causes: optic nerve compression, large retinal detachment, central retinal artery/vein occlusion, advanced glaucoma 1 Swinging Light Test Pretectal nucleus (II) Aqueduct
Constriction of both pupils to normal
Edinger-Westphal nucleus (III)
Normal Pupillary Response Direct response Consensual response Oculomotor nerves (III) Constriction of stimulated eye
Constriction of unstimulated eye Optic chiasm
2 Swinging Light Test Pathological eye Normal eye
Constriction of both pupils to normal
Cilliary ganglion 3 Swinging Light Test
Pupils appear to dilate
Rapidly swing light to pathological eye Constrictor muscles of pupil
Figure 18. RAPD
© Katerina Tchmoutina 2010
Optic nerve (II)
N24 Neurology
Neuro-Ophthalmology
Toronto Notes 2011
Horner’s Syndrome
Ciliary ganglion Short ciliary nerves
Sympathetic root of ciliary ganglion
Hypothalamus
Ophthalmic artery Eye
Long ciliary nerve
Trigeminal ganglion
Ophthalmic branch of trigeminal ganglion
Postganglionic Preganglionic
Internal carotid artery
Brainstem
Spinal cord
External carotid artery Superior cervical ganglion Subclavian artery
Lung Thoracic sympathetic trunk
Figure 19. Sympathetic Pathway of Pupillary Dilation
Anisocoria • definition: unequal size of the pupils • see Ophthalmology, OP31
© Camillia Matuk
Horner’s Syndrome • Ptosis • Miosis • Anhydrosis
Definition • a sympathetic defect • clinical features: partial ptosis (drooping eyelid), miosis (constricted pupil), anhydrosis (lack of sweating), and apparent enophthalmos • lesions occur anywhere along the sympathetic pathway on the affected side 1st-order neuron (central): hypothalamus, medulla (brainstem stroke), spinal tumour, MS, intracranial tumours, syringomyelia 2nd-order neuron (preganglionic): apical lung cancer (Pancoast’s tumour), paravertebral mass, carotid artery dissection 3rd-order neuron (postganglionic): cluster headache, cavernous sinus mass, trauma (including surgical) • clinical confirmation with cocaine test: cocaine does not dilate a miotic Horner’s pupil. Cocaine blocks the reuptake of noradrenaline, which dilates a normal pupil • central vs. pre-ganglionic vs. post-ganglionic paredrine (hydroxyamphetamine, stimulates noradrenaline release) will not dilate in a case of post-ganglionic lesion, but will dilate if there is a pre-ganglionic or central lesion no test to differentiate central from pre-ganglionic lesion
Toronto Notes 2011
Movement Disorders
Neurology N25
Movement Disorders Overview of Movement Disorders Table 16. Movement Disorder Definitions Akathisia Asterixis Athetosis Bradykinesia Chorea Dyskinesia Dystonia Freezing Hemiballism Myoclonus Myokimia Tachykinesia Tics Tremor
Subjective restlessness relieved by stereotypic movements (e.g. squirming) Loss of muscle contraction (negative myoclonus) Slow writhing movements, especially distally Slow and/or small amplitude movements Rapid jerky movement that looks semi-purposeful Excessive movements associated with neuroleptics Co-contraction of agonist and antagonists causing sustained twisting movements Episodes of halted motor action, especially during walking Unilateral violent flinging movement Brief muscle group contraction that is either focal, segmental, or generalized Muscle quivering Acceleration of movements Stereotyped repetitive actions due to inner urge; can be suppressed Rhythmic alternating movements
Describing Movement Hyperkinesia: excess of movement (i.e. most movements seen in movement disorders) Hypokinesia: reduction in movement (i.e. bradykinesia and freezing)
Some myoclonus is stimulus sensitive and can be induced by noise, movement, light, visual threat, or pinprick.
Function of the Basal Ganglia • the striatum (caudate and putamen) is the input of the basal ganglia. It receives input from the cortex and thalamus to inhibit the globus pallidus pars interna (GPi) and substantia nigra reticularis (SNr), promoting movement • the GPi and SNr are the output of the basal ganglia. They project fibres to the cortical motor areas via the ventral thalamus (thalamocortical) to prevent excess movement using tonic inhibition (in particular the GPi) • the cerebral cortex initiates movement via excitatory (glutamatergic) projections to the striatum, which then activate two pathways: direct and indirect • indirect: cortex g striatum g GPe g STN g GPi/SNr g thalamus g motor cortex activation of this pathway causes inhibition of the thalamus and ultimately prevents movement • direct: cortex g striatum g GPi/SNr g thalamus g motor cortex activation of this pathway removes the inhibitory effect of the GPi on the thalamus, thereby allowing movement Motor Cortex Premotor Cortex Supplementary Motor Area Glutamate
Glutamate
Putamen Substantia nigra Pars compacta
Dopamine
GABA enkephalin
GABA substance P
dopamine
Pars oralis portion of ventrorateral thalamus
Globus pallidus Pars externa
INDIRECT PATHWAY
GABA
DIRECT PATHWAY
Subthalamic nucleus
Globus pallidus Pars interna Excitatory connections Inhibitory connections
Figure 20. Neural Connections of the Basal Ganglia
GABA Pedunculopontine nucleus
© Susan Park 2009
Glutamate
N26 Neurology
Movement Disorders
Toronto Notes 2011
Genu of corpus callosum Lateral ventricle Caudate nucleus
Putamen Globus pallidus Third ventricle Thalamus Lateral ventricle (trigone) Splenium of corpus callosum
© Lucy Zhang 2011
Figure 21. Horizontal Section of Basal Ganglia In a young patient (90% of essential tremor does not need treatment.
Approach to Movements Disorders 1. Describe the movements. Classify each as hyperkinesias or hypokinesias 2. Name the movements (see Table 16) 3. Consider the differential diagnoses for the movements named Differential Diagnoses 1. Tremor: a. Postural: physiologic, anxiety, sedative/alcohol withdrawal, drug toxicity, heavy metal poisoning, carbon monoxide poisoning, thyrotoxicosis, benign essential tremor, cerebellar, Wilson’s disease benign essential tremor is a common autosomal dominant trait that presents as a bilateral postural tremor of the vertical axis, especially in the upper extremities b. Intention: brainstem lesion, cerebellar lesion, alcohol, anticonvulsants, sedatives, Wilson’s disease c. Resting: Parkinsonism, Wilson’s disease, mercury poisoning Table 17. Approach to Tremors Resting
Postural
Intention
Body Part
Distal UE
UE/head/voice
Anywhere
Characteristics
3-7Hz pill rolling
6-12Hz fine tremor
motor 2. Autonomic: anhydrosis, orthostatic hypotension, impotence, gastroparesis, bowel and bladder dysfunction 3. Mononeuropathy multiplex: nerve infarct or compression 4. Cranial neuropathy: CNIII (pupil sparing) > IV > VI
DDx of Demyelinating Neuropathy GBS, CIDP, paraproteinemia, diptheria, amiodarone, charcot-marie-tooth, storage diseases, pressure palsy predisposition, paraneoplastic.
Axonal neuropathies have decreased amplitude on NCS; demyelinating neuropathies have decreased velocity on NCS.
Ototoxic drugs (e.g. aminoglycosides) should not be given to diabetics. Sensory neuropathy of feet prevent them from adequately compensating for loss of vestibular function.
• monoradiculopathy: dermatomal deficit due to single nerve root lesion due to disc herniation or root compression causing radicular pain • polyradiculopathy: multiple dermatome deficits due to multiple nerve root lesions most common cauda equina syndrome (i.e. lumbosacral roots) • plexopathy: deficit matching distribution of a nerve plexus brachial plexopathy upper (C5-C7): LMN sx of shoulder and upper arm muscles (Erb’s palsy) lower (C8-T1): LMN sx and sensory sx of forearm and hand (Klumpke’s palsy) DDx: trauma, idiopathic neuritis, tumour infiltration, radiation, thoracic outlet syndrome (i.e. cervical rib) lumbosacral plexopathy (rare, especially unilateral) DDx: idiopathic neuritis, infarction (i.e. diabetes), compression • mononeuropathy: single nerve deficit carpal tunnel syndrome (most common): compression of median nerve at wrist symptoms: wrist pain, paresthesia first 3 digits, ± radiation to elbow, worse at night signs: Tinel’s sign, thenar muscle wasting, sensory deficit EMG and NCS: slowing at wrist (both motor and sensory) Bell’s Palsy (most common cranial neuropathy): see Otolaryngology, OT23 other less common mononeuropathies due to entrapment/compression: ulnar (compression at elbow), median (at pronator teres), Saturday night palsy (radial nerve entrapment at spiral groove of humerus), obturator (from childbirth), peroneal (due to crossing legs or surgical positioning), posterior tibial (tarsal canal) • mononeuropathy multiplex: deficit affecting multiple discrete nerves (asymmetric) most commonly due to diabetes • polyneuropathy: symmetrical distal stocking-glove pattern presentation: symmetrical distal sensorimotor deficit affecting longest fibres first (i.e. stocking-glove distribution), hypotonia; progression of dysesthesia early, weakness later most polyneuropathies are due to medical conditions like diabetes, renal disease, substances, toxins, or are hereditary other important etiologies: SLE, HIV, leprosy, alcohol, B12 deficiency, uremia chronic inflammatory demyelinating polyneuropathy (CIDP) chronic relapsing sensorimotor polyneuropathy with increase protein in CSF and demyelination (shown on EMG/NCS) course is fluctuating compared to acute onset of GBS treatment: firstline is prednisone: alternatives are plasmapheresis, IVIg, and asathioprine critical illness polyneuropathy associated with sepsis and multisystem organ failure; severe sensorimotor axonal neuropathy Table 18. Differential Diagnosis of Symmetric Polyneuropathy* Etiology+ Vascular
Infectious
Immune
Mechanism
Course
Modalities
Investigations
PAN
Ischemic
Chronic
S/M
see Rheumatology, RH17
SLE
Ischemic
Chronic
S/M
see Rheumatology, RH9
RA
Ischemic
Chronic
S/M
see Rheumatology, RH6
HIV
Axonal/demyelination
Chronic
S/A
HIV serology
Leprosy
Infiltrative
Chronic
S/A
Leprosy serology Nerve biopsy
Lyme
Axonal/demyelination
Chronic
M
Lyme serology
GBS
Demyelination
Acute
M
LP (Ó protein; no Ó cells)
CIDP
Demyelination
Chronic
S/M
LP (Ó protein)
Toronto Notes 2011
Peripheral Neuropathies
Neurology N31
Table 18. Differential Diagnosis of Symmetric Polyneuropathy* (continued) Etiology+
Mechanism
Course
Modalities
Investigations
Hereditary
HMSN
Axonal/demyelination
Chronic
S/M
Genetic testing
Neoplastic
Paraneoplastic
Axonal/demyelination
Chronic
S/M
Anti-Hu
Myeloma
Axonal/demyelination
Chronic
S/M
SPEP Skeletal bone survey
Lymphoma
Axonal
Chronic
M
SPEP Bone marrow biopsy
Monoclonal gammopathy
Demyelination
Chronic
S/M
SPEP Bone marrow biopsy
EtOH
Axonal
Sub-acute
S/M
GGT
Heavy metals
Axonal
Sub-acute
S/M
Urine heavy metals
Medications
Axonal
Sub-acute
S/M
Drug levels
Diabetes
Ischemic/axonal
Chronic
S/A
Fasting glucose, HbA1C, 2hr OGTT
Toxin
Metabolic
Hypothyroidism
Axonal
Chronic
S/M
TSH, T3, T4
Renal failure
Axonal
Chronic
S/A
Lytes, Cr, BUN
Nutritional
B12 deficiency
Axonal
Sub-acute
S/M
Vitamin B12
Other
Porphyria
Axonal
Sub-acute
M
Urine parphyrins
Amyloid
Axonal
Sub-acute
S
Nerve biopsy
*Abbreviations: GBS – Guillain-Barré Syndrome; PAN – polyarteritis nodosa; SLE– systemic lupus erythromatosus; RA – rheumatoid arthritis; CIDP – chronic inflammatory demyelinating polyradiculoneuropathy; HMSN – hereditary motor sensory neuropathy; SPEP – serum protein electrophoresis; S – sensory; M – motor; A – autonomic +Most common/important etiologies in italics type
Guillain-Barre Syndrome (GBS) • definition: acute rapidly evolving polyneuropathy • risk factors and etiology pathophysiology suspected to be focal inflammation viral/bacterial infections and vaccinations, have been shown to predispose to GBS • signs and symptoms sensory: distal and symmetric paresthesias, loss of proprioception and vibration sense, pain motor: weakness starting distally in legs, areflexia autonomic: blood pressure dysregulation, arrhythmias, bladder dysfunction • investigations CSF: albuminocytological dissociation (high protein, normal WBC) EMG/NCS: conduction block, differential or focal (motor>sensory) slowing, decreased F-wave • subtypes 1. Acute inflammatory demyelinating polyneuropathy (AIDP) 2. Acute motor-sensory axonal neuropathy (AMSAN) 3. Acute motor axonal neuropathy (AMAN) • treatment disease specific: IVIg or plasmapheresis nonpharmacologic: admit and monitor vital signs and vital capacity due to risk of respiratory failure, manage dysautonomia, manage pain • prognosis nadir of symptoms at 2-3 weeks, with resolution at 4-6 weeks 5% mortality (higher if ICU), 7-15% permanent substantial deficits Diagnostic Approach to Peripheral Neuropathies 1. Differentiate: motor vs. sensory vs. autonomic 2. Pattern of Deficit: symmetry, focal vs. diffuse, upper vs. lower limb, cranial nerve involment 3. Tempo: acute to chronic, relapsing remitting vs. constant 4. Good History: PMH, detailed family tree, exposures (e.g. insects, toxins, sex, travel), systemic symptoms 5. Detailed Peripheral Neuro Exam: LMN findings, differentiate between root and peripheral nerves, check cranial nerves, check respiratory status
GBS is a neurological emergency due to risk of imminent respiratory failure.
Miller-Fischer Variant of GBS – Triad 1. Ophthalmoplegia 2. Ataxia 3. Areflexia
IVIg and plasmapheresis lead to more rapid improvement, less intensive care and less ventilation, but do not change mortality or relapse rate.
N32 Neurology
Neuro-oncology/Neuromuscular Junction Diseases
Toronto Notes 2011
Neuro-oncology Paraneoplastic Syndromes Definition • uncommon complication of cancer; often is the presenting complaint Pathophysiology • likely an autoimmune attack on the nervous system by tumour antigens Associated Neoplasms • small cell lung cancer: cerebellar degeneration, encephalitis, opsoclonus-myoclonus, retinopathy, neuropathy, Lambert-Eaton syndrome • breast: cerebellar degeneration, encephalomyelitis, opsoclonus-myoclonus • thymoma: myasthenia gravis • other syndromes: necrotizing myelopathy, motor neuron syndrome, neuropathies, mononeuritis multiplex, polymyositis and dermatomyositis, encephalitis Investigations • antibodies commonly ordered include anti-Hu, anti-Ri and anti-Yo Treatment • unsatisfactory and often palliative. Options to consider are steroids, IVIg, plasmapheresis and treatment of malignancy
Tumours of the Nervous System • see Neurosurgery, NS9
Neuromuscular Junction Diseases Clinical Approach to Disorders of the Neuromuscular Junction Table 19. Common Disorders of the Neuromuscular Junction Myasthenia Gravis
Diseases of the neuromuscular junction typically feature prominent fatiguability.
Lambert-Eaton
Botulism
Ocular/bulbar paresis
+
–
++ (early)
Limb weakness
+
+
+ +
Fatiguability
+
+
Post-exercise enhancement
–
+
+
Reflexes
N
Ô
Ô
ANS anticholinergic Sx
–
+
++
Sensory Sx
–
–
–
Associated conditions Repetitive EMG stimulation
Thymoma
Small cell carcinoma
GI SSx
Ô
Ó (rapid stimulation) Ô (slow stimulation)
Ó (rapid stimulation) Ô (slow stimulation)
Myasthenia Gravis (MG) Etiology and Pathophysiology • damage and blockade of post-synaptic acetylcholine receptors by specific antibodies • 15% of patients with myasthenia gravis have associated thymic neoplasia, 85% have thymic hyperplasia • autoimmune disorder Epidemiology • bimodal age of onset – 20’s (mostly women) and 60’s (mostly men)
Toronto Notes 2011
Neuromuscular Junction Diseases
Signs and Symptoms • see also Table 19 • fatiguability and weakness of skeletal muscles without reflex, sensory, or coordination abnormalities • typically ocular (diplopia/ptosis) g bulbar (dysarthria/dysphagia) g neck flexors/extensors g proximal limbs • respiratory muscle weakness may lead to respiratory failure Investigations • edrophonium (Tensilon®) test – can result in respiratory difficulty so have crash cart nearby assess for improvement over 2 minutes following edrophonium injection • EMG repetitive stimulation g decremental response single fibre electromyography shows increased jitter (80-100% sensitivity) • anti-acetylcholine receptor antibody assay (70-80% sensitivity) • MUSK antibody may be used if seronegative for AChR antibody • CT/MRI to screen for thymoma/thymic hyperplasia Treatment • thymectomy 85% of patients show improvement or remission • symptomatic relief acetylcholinesterase inhibitors (e.g. pyridostigmine) does not affect primary pathologic process g rarely result in control of disease when used alone • immunosuppression steroids are mainstay of treatment – 70-80% remission rate azathioprine, cyclophosphamide and mycophenolate as adjuncts to steroids or as steroid sparing therapy • short-term immunomodulation (for crises) IVIg and plasmapheresis
Neurology N33
Myasthenia Gravis is a neurological emergency due to the risk of imminent respiratory failure!
Tensilon® is a drug that inhibits acetylcholinesterase. It improves muscle function immediately in myasthenia gravis, but not in cholinergic crisis.
2 Clinical Forms of Myasthenia Gravis 1. Ocular (15%) 2. Generalized (85%)
Prognosis • 30% eventual spontaneous remission
Lambert-Eaton Myasthenic Syndrome (LEMS) Etiology and Pathophysiology • downregulation of presynaptic voltage-gated Calcium channels 2o to specific channel binding antibody causing decreased amounts of ACh released into the synaptic cleft • 50-66% are ultimately associated with small cell carcinoma of the lung Signs and Symptoms • weakness of skeletal muscles without sensory or coordination abnormalities • reflexes are diminished or absent, but increase after active muscle contraction • bulbar and ocular muscles affected in 25% • prominent anticholinergic autonomic symptoms (dry mouth > impotence > constipation > blurred vision) Investigations • edrophonium test (see Myasthenia Gravis) g no response • EMG: rapid (> 10Hz) repetitive stimulation g incremental response • screen for malignancy, especially small cell lung cancer • post-exercise facilitation – an incremental response to repetitive stimulation due to presynaptic calcium accumulation Treatment • tumour removal • acetylcholine modulation increased acetylcholine release (3-4 diaminopyridine) decreased acetylcholine degradation (pyridostigmine) • immunomodulation steroids, plasmapheresis, IVIg
Lambert-Eaton myasthenic syndrome can be differentiated from myasthenia gravis, by the phenomenon of postexercise facilitation.
N34 Neurology
Myopathies
Toronto Notes 2011
Myopathies Clinical Approach to Muscle Diseases Table 20. Myopathies Etiology
Key Clinical Features
Key Investigations
Polymyositis
Myalgias Pharyngeal involvement
Ó CK Biopsy: endomesial infiltrates; Necrosis
Dermatomyositis
Myalgias Similar to polymyositis Characteristic rashes Can be paraneoplastic
Ó CK Biopsy: perifascicular atrophy
Sarcoidosis
See Respirology
ACE level Biopsy: granulomas
Inclusion body myositis
Weak quads and deep finger flexors
Ó CK Biopsy: inclusion bodies
Endocrine
Thyroid (Ó or Ô) Cushing’s syndrome Parathyroid (Ó or Ô)
See Endocrinology
TSH, serum cortisol, calcium panel
Toxic
Medication
Medication or toxin history
Toxicology screen
Critical illness myopathy
ICU patient Hx steroids and nondepolarizing paralyzing agents Failure to wean from ventilation
Biopsy: selective loss of thick Myosin filaments
Infectious
Parasitic, bacterial, or viral
Myalgias Inflammatory myopathy
Ó myoglobin
Hereditary Dystrophy
Duchenne
Early onset (Duchenne and Becker)
Biopsy: abnormal dystrophin
Becker
Progressive proximal muscle weakness Calf pseudohypertrophy
Staining
Myotonic dystrophy
Distal myopathy Myotonia Genetic anticipation
Genetic testing
Hereditary Metabolic
McArdle’s
Exercise-related myalgias, cramping, and myoglobuminuria
Ó lactate Ó serum/urinary myoglobin Post-exercise
Hereditary Periodic Paralysis
Periodic paralysis
Episodic weakness Normal between attacks
Ó or Ô K
Hereditary Mitochondrial
MERRF MELAS Kearns Sayre
Ptosis, ophthalmoparesis common Proximal > distal myopathy Exercise intolerance Rhabdomyolysis
Increased lactate Biopsy: ragged red fibres
Inflammatory
Important Information to Know Regarding Myopathies • Weakness: proximal > distal • Pain: myalgias, but no impaired sensation • Myotonia (difficulty with relaxation)
Myopathies are characterized by prominent symmetric proximal weakness and absent sensory changes.
Good Questions to Assess Proximal Weakness • Legs: climbing stairs, stand from sit • Arms: reach above head, wash hair
Common Medications that Cause Myopathy Steroids, statins and antiretrovirals
*Abbreviations: MERRF – mitochondrial encephalomyopathy with ragged red fibers; MELAS – mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes
Polymyositis/Dermatomyositis • see Rheumatology, RH13
Myotonic Dystrophy Etiology and Pathophysiology • unstable trinucleotide repeat in DMK gene (protein kinase) at 19q13.3 • number of repeats correlates with severity of symptoms; autosomal dominant Epidemiology • most common adult muscular dystrophy • prevalence 3-5/100 000 Signs and Symptoms • appearance: ptosis, bifacial weakness, frontal baldness (including women), triangular face giving a drooping/dull appearance
Toronto Notes 2011
Myopathies/Cerebellar Disorders
• physical exam distribution of weakness: distal greater than proximal (in contrast to other myopathic disorders) myotonia: delayed relaxation of muscles after exertion (elicit by tapping on thenar muscles with hammer) cardiac: 90% have conduction defects (1o heart block; atrial arrhythmias) respiratory: hypoventilation 2o to muscle weakness ocular: subcapsular cataracts, retinal degeneration, decreased intraocular pressure • EMG: subclinical myotonia – long runs with declining frequency and amplitude Treatment • no cure • management of myotonia: phenytoin
Duchenne and Becker Muscular Dystrophy • see Pediatrics, P46
Cerebellar Disorders Clinico-Anatomic Correlations • vermis: trunk/gait ataxia • cerebellar lobe (i.e. lateral): tremor, rebound phenomenon, dysarthria, dysdiadochokinesis, nystagamus Symptoms and Signs of Cerebellar Dysfunction • nystagmus: observe on extra-ocular movement testing (most common is gaze-evoked nystagmus) • dysarthria (ataxic dysarthria): abnormal modulation of speech velocity and volume – elicit scanning/telegraphic/slurred speech on spontaneous speech (see Dysarthria, N19) • ataxia: broad-based, uncoordinated, lurching gait • dysmmetria: irregular placement of voluntary limb or ocular movement • dysdiadochokinesis: unable to perform rapid alternating movements (e.g. pronation – supination task) • postural instability: look for truncal ataxia on sitting (titubation = rhythmic rocking of trunk and head); look for difficult tandem gait and broad based gait • intention tremor: elicit on finger-to-nose testing – typically orthogonal to intended movement, and increases as target is approached • hypotonia: decreased resistance to passive muscular extension- occurs immediately after injury to lateral cerebellum • pendular patellar reflex: knee reflex causes pendular motion of leg occurs after injury to cerebellar hemispheres • rebound phenomenon: overcorrection after displacement of a limb (with both arms extended g pushing both will cause one to rebound up if there is lesion on that side)
Wernicke-Korsakoff Syndrome • deficiency of thiamine due to alcohol abuse • acute: apathy, confusion, decreased EOM, ataxia (truncal and gait) without treatment progresses to encephalopathy and ultimately death treatment: thiamine 100 mg • Korsakoff ’s syndrome: progressive decline of both anterograde and retrograde memory • note that alcohol can also cause a cerebellar ataxia separate from thiamine deficiency. The ataxia can be due to cerebellar atrophy or alcohol polyneuropathy
Cerebellar Ataxias Congenital Ataxias • early onset nonprogressive ataxias associated with various syndromes as well as development abnormalities (e.g. Arnold-Chiari malformation, Dandy-Walker cysts) Hereditary Ataxias • autosomal recessive: includes Friedreich’s ataxia, ataxia telangiectasia, vitamin E deficiency Friedreich’s ataxia: prevalence 2/100 000; onset between 8 and 15 years signs: gait and limb ataxia, weakness, areflexia, extensor plantar reflex, impaired proprioception and vibration death in 10-20 years from cardiomyopathy or kyphoscoliotic pulmonary restriction • autosomal dominant: spinocerebellar ataxias (SCAs) of which 30 exist, most are CAG repeats
Neurology N35
N36 Neurology
Cerebellar Disorders/Vertigo/Gait Disturbances/Pain Syndromes
Toronto Notes 2011
Acquired Ataxias • neurodegeneration (e.g. multiple system atrophy) • systemic: alcohol, celiac sprue, hypothyroidism, Wilson’s, thiamine deficiency • toxins: carbon monoxide, heavy metals, lithium, phenytonin, solvents • vascular: infarct, bleed, basilar migraine • autoimmune: MS, Miller-Fischer (GBS) • children: tumours, post-viral
Vertigo • see Otolaryngology, OT12
Gait Disturbances Approach to Gait Disturbances 1. Length of stride • if small paces – look at posture if stooped with no armswing – Parkinsonian gait • look for other signs of extrapyramidal disorders if upright with exaggerated armswing – Marche a petit pas due to diffuse infarction of both cerebral hemispheres (lacunar)
CENTRAL MOTOR SYSTEMS 3 components to the control of gait 1. Pyramidal: main outflow from cortex to spinal cord 2. Extrapyramidal: basal ganglia inhibits excess movements 3. Cerebellum: affects coordination of gait
2. If normal stride length, look at width between feet • if wide-based – ataxia if high stepping and positive Romberg – sensory ataxia loss of joint position sense (+ve Romberg) • if wide based without high stepping – cerebellar ataxia veers to side of the lesion • if scissoring of legs or toe walking – spastic gait bilateral circumduction due to spastic paraparesis from cerebral palsy, multiple sclerosis or cord compression 3. If normal width, look for height of step • if high stepping bilaterally – bilateral foot drop • if feet barely leave ground or disjointed movement – magnetic/apraxic gait frontal lobe pathology due to normal pressure hydrocephalus or cerebrovascular disease 4. If no high stepping, look for stability of pelvis • if rotation of pelvis – waddling gait proximal muscle weakness due to congenital deformity or myopathy 5. If no waddling, look at symmetry • if asymmetric – antalgic gait, deformity or hemiparetic gait antalgic gait is due to pain from an MSK problem hemiparetic gait involves a foot drop and circumduction of spastic leg due to UMN lesion 6. If movement is elaborate and inconsistent, especially when being observed, consider functional gait • rule out an odd gait due to chorea from Huntington’s disease
Pain Syndromes Approach to Pain Syndromes
• Pinprick causes sharpness mediated by Aδ fibers • Pain due to tissue damage is mediated by C fibres
Definitions • Nociceptive pain: pain arising from normal activation of peripheral nociceptors • Neuropathic pain: pain arising from direct injury to neural tissue, bypassing nociceptive pathways • Spontaneous pain: unprovoked burning, shooting, or lancinating pain • Paresthesiae: spontaneous or evoked abnormal nonpainful sensations (e.g. tingling) • Dysesthesiae: spontaneous or evoked pain with inappropriate quality or excessive quantity • Allodynia: a dysesthetic response to a nonnoxious stimulus • Hyperalgesia: an exaggerated pain response to a noxious stimulus
Toronto Notes 2011
Pain Syndromes
Medical Pain Control • primary analgesics: OTCs, opiates • adjuvants: antidepressants (TCAs, SSRIs), anticonvulsants (gabapentin, carbamazepine), baclofen, sympatholytics (phenoxybenzamine), a2-adrenergic agonists (clonidine, pregabalin) Surgical Pain Control • direct delivery: implantable morphine pump • central ablation: stereotactic thalamotomy, spinal tractotomy or dorsal root entry lesion • peripheral ablation: nerve blocks, facet joint denervation • deep brain stimulation (DBS) or dorsal column stimulation
Neuropathic Pain Definition • pain resulting from a disturbance of the central or peripheral nervous system Symptoms and Signs • hyperalgesia/allodynia • subjectively described as – burning, heat/cold, pricking, electric shock, perception of swelling, numbness (i.e. stocking/sock distribution) • can be spontaneous or stimulus evoked • distribution may not fall along classical neuro-anatomical lines Associated Issues • sleep difficulty • anxiety/stress/mood alteration • sexual dysfunction Causes of Neuropathic Pain • peripheral neuropathy systemic disease – diabetes, thyroid disease, renal disease, rheumatoid arthritis nutritional/toxicity – alcoholism, pernicious anemia, chemotherapy infectious – HIV trauma – post surgical, nerve injury • nerve root: post-herpetic neuralgia, cervical and lumbar radiculopathies, tic douloureux (see Trigeminal Nerve, N18), plexopathies • central: MS, post-stroke, phantom limb, spinal cord injury • Complex Regional Pain Syndromes (see N38) • malignancy Treatment • pharmacotherapy: TCA, SNRI, anticonvulsant, long acting opiate, topical lidocaine, capsaicin cream, intrathecal opioid or clonidine, Botox, nerve block • surgical therapies: dorsal column neurostimulator, DBS (thalamus) • other therapies: neuropsychiatry – cognitive behavioural theraphy, psychotherapy rehabilitation – physiotherapy CAM – acupuncture, meditation, massage therapy, TCM
Tic Douloureux (Trigeminal Neuralgia) • see Trigeminal Nerve, N18
Postherpetic Neuralgia (PHN) Definition • pain persisting beyond 3 months in the region of a cutaneous outbreak of herpes zoster Etiology and Pathogenesis • destruction of the sensory ganglion neurons (e.g. dorsal root, trigeminal, or geniculate ganglia) secondary to reactivation of herpes zoster infection Epidemiology • 10-15% of all patients with cutaneous herpes zoster • >80% of herpes zoster infected patients >80 years old
Neurology N37
N38 Neurology
Pain Syndromes
Toronto Notes 2011
Signs and Symptoms • types of pain: constant deep ache or burning, intermittent spontaneous lancinating/jabbing pain, allodynia • distribution: thoracic > trigeminal > cervical > lumbar > sacral Treatment • acute herpes zoster early treatment with antiviral agents (acyclovir; longer-acting famciclovir and valaciclovir more effective) may prevent PHN in patients over 50 years • PHN medical: TCA, pregabalin, gabapentin, opiate, lidocaine patch, intrathecal methylprednisolone surgical: spinal tractotomy, dorsal root entry zone lesion
Complex Regional Pain Syndromes (CRPS) Definitions • CRPS is a pain syndrome characterized by the following 1. presence of an initiating noxious event 2. continuing pain, allodynia, or hyperalgesia with pain disproportionate to inciting event 3. evidence during the course of symptoms of edema, changes in skin blood flow, or abnormal vasomotor activity 4. absence of conditions that would otherwise account for degree of pain and dysfunction Classification • CRPS type I (reflex sympathetic dystrophy): minor injuries of limb or lesions in remote body areas precede onset of symptoms • CRPS type II (causalgia): injury of peripheral nerves precedes the onset of symptoms Signs and Symptoms • stage I (acute) pain: burning or aching disproportionate to initial injury autonomic: edema and temperature inequality • stage II (dystrophic) pain: constant and increased by stimulus to affected part autonomic: osteoporosis, cool hyperhydrotic skin, hair loss, cracked/brittle nails • stage III (atrophic) pain: paroxysmal spread autonomic: thin, shiny skin, thickened fascia with contractures, bony demineralization Investigations • diagnosis is clinical • trial of differential neural blockade may be helpful Treatment • medical: phenoxybenzamine (sympatholytic) • surgical: paravertebral sympathetic ganglion blockade
Thalamic Pain (Dejerine Roussy Syndrome) Definition • hypersensitivity to pain as a result of damage to the thalamus Etiology and Pathogenesis • injury to ventral posterolateral (VPL) and ventral posteromedial (VPM) nuclei of the thalamus ischemic stroke hypertensive vascular hemorrhage Signs and Symptoms • begins with hemianesthesia • then persistent spontaneous burning contralateral to lesion • altered response to light cutaneous and deep painful stimuli Treatment • medical: amitriptyline, anti-convulsants • surgical: stereotactic thalamic stimulation (may increase sensory deficit)
Toronto Notes 2011
Headache
Neurology N39
Headache
The Rational Clinical Examination: Does this Patient with Headache have a Migraine or Need Neuroimaging? JAMA 2006; 296:1274-83
Clinical Approach to Headaches Investigations • good history and physical to rule out serious causes of headache important aspects of neurologic exam: LOC and MSE, pupils (symmetry), fundi (papilledema, retinal hemorrhages), pronator drift, meningismus, deep tendon reflexes and Babinski, gait • indications for a CT new-onset headache, worst headache of life, thunderclap headache, headache with worrisome symptoms (fever, meningismus, altered LOC, focal neurologic deficits, trauma, papilledema, morning headache) • if CT is negative but suspicion of SAH or meningitis, perform a lumbar puncture Table 21. Headaches – Primary Prevalence
Tension-Type
Migraine
Cluster
70%
12%
M
F>M
M>F
Family History
None
+++
+
Location
Bilateral frontal Nuccho-occipital
Unilateral>bilateral Fronto-temporal
Retroorbital
Duration
Minutes – days
Hours – days
10 min-2 hours
Onset/Course
Gradual; worse in PM
Gradual; worse in PM
Daily headache for weeks, months, nocturnal
Quality
Band-like; constant
Throbbing
Constant, aching, stabbing
Severity
Mild-moderate
Moderate-severe
Severe (wakes from sleep)
Provoking
Depression Anxiety Noise Hunger Sleep deprivation
Noise Light Straining Coughing Activity
Light EtOH
Palliating
Rest
Rest
Walking around
Associated Sx
No vomiting No photophobia
Nausea/vomiting Photo/phonophobia Aura
Red watery eye Nasal congestion or rhinorrhea Unilateral Horner’s
Physical Signs
Muscle tension in scalp/neck
Muscle tension in scalp/neck Tender scalp arteries
Red watery eye, rhinorrhea Eyelid droop
Management
Non-pharmacological • Psychological counseling • Physical modalities (e.g. heat, massage) Pharmacological • Simple analgesics • Tricyclic antidepressants
Acute Rx • ASA • NSAIDS • Triptans • Ergotamine Prophylaxis • Propranolol • TCA • Anticonvulsants
Acute Rx • O2 • Sumatriptan (nasal or injection) Prophylaxis • Verapamil • Lithium • Methylsergide • Prednisolone
Table 22. Headaches – Serious Meningeal Irritation
Increased Intracranial Pressure
Temporal Arteritis
Incidence
22, resistant HTN, age >85, Hx AVM or aneurysm.
• Thrombolytic: 1h IV infusion of recombinant tissue plasminogen activator (rt-PA) for ischemic stroke within 3h of stroke onset. Get 24h CT to R/O ICH. Also option of intra-arterial tPA for specific clinical situations • Carotid Endarterectomy: if 50-99% stenosis with low risk of perioperative death or disabling stroke
Blood Pressure Control • do not lower the blood pressure unless the hypertension is severe antihypertensive therapy is withheld for at least 5 days after thromboembolic stroke unless there is acute MI, renal failure, aortic dissection, sBP above 220 mmHg, or dBP above 120 mmHg • acutely elevated BP is necessary to maintain brain perfusion • most patients with an acute cerebral infarct are initially hypertensive and their BP will fall spontaneously within 1-2 days • IV labetalol is usually first line if needed Blood Sugar • avoid hyperglycemia which will increase the infarct size B. OTHER MANAGEMENT ISSUES • prevent complications NPO if swallowing difficulty DVT prophylaxis if limb weakness initiate rehabilitation • therapy (see also Primary and Secondary Prevention below) determine the vascular territory and etiology, then treat accordingly • lower temperature if febrile
Primary and Secondary Prevention Carotid Territory Event • carotid endarterectomy benefits those with symptomatic severe stenosis (70-99%), and is less beneficial for those with symptomatic moderate stenosis (50-69%), see Neurosurgery, NS21 Asymptomatic Carotid Bruit • suggests the presence of atherosclerotic stenosis and signifies increased risk for both cerebral and myocardial infarction • modify risk factors, ± antiplatelet therapy • if stenosis >60%, risk of stroke is 2% per year; carotid endarterectomy reduces the risk of stroke by 1% per year (but 5% risk of complications) Hypertension • primary prevention antihypertensives reduce the risk of ischemic stroke in elderly patient with isolated systolic hypertension (SHEP trial) ramipril 10 mg OD is effective in patients at high risk for cardiovascular disease (HOPEStroke trial) ACEI reduce the risk of stroke beyond their antihypertensive effect • secondary prevention ACEI and thiazide diuretics are useful in patients with a Hx of stroke/TIA (PROGRESS trial)
50% of ICH due to tPA are fatal!
BP must be lowered to sBP 1 month after first attack Dissemination in Space: clinical evidence of 2 or more lesions; or three of [1 gadolinium enhancing or 9 T2 lesions], [1 infratentorial lesion], [1 juxtacortical lesion], [3 periventricular lesions] Features • symptoms in order of frequency: fatigue, depression, numbness, weakness, visual disturbance, bladder dysfunction, spasticity, impaired gait, cognitive disturbance, pain • Lhermitte’s sign: flexion of neck causes electric shock sensation down back into limbs indicating cervical cord lesion • Uhthoff ’s phenomenon: worsening of symptoms (classically optic neuritis) in heat • SPMS: classically weakness of legs in pyramidal distribution paired with cerebellar findings of arm (i.e. intention tremor) • symptoms not commonly found in MS: visual field defects, aphasia, apraxia, progressive hemiparesis Investigations • MRI: demyelinating plaques appear as hyperintense lesions on T2 weighted MRI, with active lesions showing enhancement with gadolinium typical locations: periventricular, corpus callosum, cerebellar peduncles, brainstem, juxta cortical region, and dorslateral spinal cord Dawson’s fingers: periventricular lesions extending superiorly into corpus callosum • CSF: oligoclonal bands in 90%, increased IgG concetration • evoked potentials (visual/auditory/somatosensory): delayed but well-preserved wave forms
Most symptoms in MS are due to cord, brainstem and optic nerve lesions.
N50 Neurology Recombinant Interferon Beta or Glatiramer Acetate for Delaying Conversion of the First Demyelinating Event to Multiple Sclerosis Cochrane Database Syst Rev 2008; 2:CD005278. Study: Cochrane systematic review. 2 RCT and quasi-RCt, double-blinded trials. Population: 1160 patients with fiest neurological event suggestive of demyelination with positive brain MRI (clinically isolated syndromes, CIS). Intervention: Either interferon beta (IFNb) versus placebo. (No appropriate glatiramer acetate trials were found). Primary Outcomes: Proportion of patients converting to clinically definite MS, and adverse effects. Results: A pooled odds ratio (OR) of 0.53 ((5% CI, 0.40-0.71, p50 mmHg in adults • cerebral autoregulation maintains constant CBF by compensating for changes in CPP, unless: high ICP such that CPP 150 mmHg or MAP 20 mmHg • goals: keep ICP 65 mmHg, MAP >90 mmHg General Measures (“ICP HEAD” see sidebar) • elevate head of bed at 30-45º, maintain neck in neutral position g increases intracranial venous outflow • prevent hypotension with fluid and vasopressors, dopamine, norepinephrine prn • ventilate to normocarbia (pCO2 35-40 mmHg) g prevents vasodilatation • oxygen prn to maintain pO2 >60 mmHg g prevents hypoxic brain injury
Toronto Notes 2011
Herniation Syndromes/Hydrocephalus
Neurosurgery NS7
Specific Measures • osmolar diuresis (mannitol 20% IV solution 1-1.5 g/kg, then 0.25 g/kg q6h to serum osmolarity of 315-320) can give rapidly, acts in 30 minutes, must maintain sBP >90 mmHg • sedation (“light” e.g. barbiturates/codeine g “heavy” e.g. fentanyl/MgSO4) • paralysis with vecuronium g reduces sympathetic tone, reduces HTN induced by muscle contraction • hyperventilate to pCO2 30-35 mmHg use for brief periods only – also results in decreased cerebral blood flow (CBF) • drain 3-5 ml CSF via ventricles, assess each situation independently • insert external ventricular drain (if acute) or shunt • corticosteroids g decrease edema over subsequent days around brain tumour, abscess, blood no proven value in head injury or stroke • hypothermia – cool body to 34ºC no proven value in head injury • barbiturate coma induced with pentobarbital to reduce cerebral blood flow and metabolism (10 mg/kg over 30 min, then 1 mg/kg q1h continuous infusion) decreases mortality, but no improvement in neurological outcome • decompressive craniectomy is a last resort
Hydrocephalus 2
3
1
Etiology • obstruction to CSF flow • decreased CSF absorption • increased CSF production (rarely) – e.g. choroid plexus papilloma (0.4-1% of intracranial tumours) Epidemiology • estimated prevalence 1-1.5%; incidence of congenital hydrocephalus ~1-2/1000 live births • hydrocephalus in children, see Pediatric Neurosurgery, NS37 Classification Table 2. Classification of Hydrocephalus Disorder
Definition
Circulation blocked within Obstructive (Non-Communicating) ventricular system proximal to the arachnoid granulations Hydrocephalus
Etiology
Findings on CT/MRI
• Ventricular enlargement proximal Acquired to block • Aqueductal stenosis (adhesions • Periventricular hypodensity following (transependymal migration of CSF infection, hemorrhage) forced into extracellular space) • Intraventricular lesions (tumours • Sulcal effacement e.g. 3rd ventricle colloid cyst, hematoma) • Mass causing tentorial herniation, aqueduct/4th ventricle compression • Others: neurosarcoidosis, abscess/ granulomas, arachnoid cysts Congenital • Aqueductal stenosis, Dandy-Walker malformation, Chiari malformation (see Pediatric Neurosurgery, NS36)
Non-Obstructive (Communicating) Hydrocephalus
CSF absorption blocked at extraventricular site = arachnoid granulations
• Post-infectious (#1 cause)
g meningitis, cysticercosis • Post-hemorrhagic (#2 cause) g SAH, IVH, traumatic • Choroid plexus papilloma (rare, causes increased CSF production) • Idiopathic normal pressure hydrocephalus
• All ventricles dilated
4
5
Persistent ventricular dilatation in the context of normal CSF pressure
• Idiopathic (50%) • Enlarged ventricles without • Others: subarachnoid hemorrhage, increased prominence of cerebral meningitis, trauma, radiationsulci induced
Hydrocephalus Ex Vacuo
Ventricular enlargement resulting from atrophy of surrounding brain tissue
• Normal aging • Alzheimer’s, Creutzfeldt-Jacob Disease
• Enlarged ventricles and sulci • Cerebral atrophy
8
9
6
1. Choroid plexus 2. Lateral ventricles 3. Third ventricle 4. Cerebral aqueduct (of Sylvius) 5. Fourth ventricle 6. Foramen Luschka and Magendie 7. Arachnoid granulations 8. Subarachnoid space 9. Sagittal sinus
Figure 8. The Flow of CSF
CSF produced by choroid plexus, flows to: ventricles foramina of Luschka (lateral) and Magendie (medial) subarachnoid space absorbed by arachnoid villi/granulations into venous sinuses. CSF production = CSF reabsorption = ~500 ml/day in normal adults Normal CSF volume ~150 ml (50% spinal, 50% intracranial 25 ml intraventricular, 50 ml subarachnoid)
NPH Progression
Normal Pressure Hydrocephalus (NPH)
7
AID Ataxia/Apraxia of gait Incontinence Dementia
© Kari Francis 2004
Definition • increased CSF volume
NS8 Neurosurgery
Hydrocephalus
Toronto Notes 2011
Clinical Features (see also Pediatric Neurosurgery, NS36) • acute hydrocephalus signs and symptoms of acute raised ICP (see Elevated ICP, NS5) impaired upward gaze (“sunset eyes”) and/or CN VI palsy • chronic/gradual onset hydrocephalus [i.e. normal pressure hydrocephalus (NPH)] gradual onset of classic triad developing over weeks or months pressure of ventricle on LE motor fibres g gait disturbance (ataxia and apraxia usually initial symptoms) pressure on cortical bowel/bladder centre g urinary incontinence pressure on frontal lobes g dementia CSF pressure within clinically “normal” range, but symptoms abate with CSF shunting Investigations • CT/MRI periventricular lucency suggests raised CSF pressure • ultrasound (through anterior fontanelle in infants) • ICP monitoring (e.g. LP) may be used to investigate NPH, test response to shunting (lumbar tap test) • radionuclide cisternography can test CSF flow and absorption rate (unreliable) Treatment • ventricular drainage • surgical removal of obstruction (if possible) or excision of choroid plexus papilloma • shunts ventriculoperitoneal (VP) – most common ventriculopleural ventriculo-atrial (VA) – not first choice because of increased infections, shunt emboli lumboperitoneal – for communicating hydrocephalus and pseudotumour cerebri • third ventriculostomy (for obstructive hydrocephalus) via ventriculoscopy • LPs [for transient hydrocephalus (e.g. subarachnoid hemorrhage), IVH in premature infants, etc.] Shunt Complications Table 3. Shunt Complications Complication
Etiology
Clinical Features
Investigations
Obstruction (most common)
• Obstruction by choroid plexus • Acute hydrocephalus • Buildup of proteinaceous • Increased ICP accretions, blood, cells (inflammatory or tumour) • Infection • Disconnection or damage
Infection (3-6%)
• S. epidermidis • S. aureus • P. acnes • Gram-negative bacilli
• Fever, N/V, anorexia, irritability • CBC • Meningitis • Blood culture • Peritonitis • Tap shunt for C&S (LP usually NOT • Signs and symptoms of shunt recommended) obstruction • Shunt nephritis (VA shunt)
Overshunting (10% over 6.5 years)
• Slit ventricle syndrome • Collapse of ventricles leading to occlusion of shunt ports by ependymal lining • Secondary craniosynostosis (children)
• Chronic or recurring • CT/MRI headaches often relieved when lying down • Slit-like ventricles on imaging
• “Shunt series” (plain x-rays of entire shunt that only rule-out disconnection, break, tip migration) • CT • Radionuclide “shuntogram”
• Subdural hematoma • Asymptomatic • Collapsing brain tears bridging • Headaches, vomiting, veins (especially common in somnolence NPH patients)
• CT
• Apposition and overlapping of • Abnormal head shape the cranial sutures in an infant following decompression of hydrocephalus
• Clinical • CT
Seizures (5.5% risk in 1st year, 1.1% after 3rd year)
• EEG
• Increased intraperitoneal • Inguinal swelling, discomfort Inguinal Hernia pressure/fluid results in hernia (17% incidence with VP shunt inserted in infancy) becoming apparent ± skin breakdown over hardware
• U/S
Toronto Notes 2011
Benign Intracranial Hypertension/Tumour
Neurosurgery NS9
Benign Intracranial Hypertension (Pseudotumour Cerebri) Definition • raised intracranial pressure and papilledema without evidence of any mass lesion, hydrocephalus, infection or hypertensive encephalopathy (diagnosis of exclusion) Etiology • unknown (majority), but associated with: lateral venous sinus thrombosis habitus/diet: obesity, hyper/hypovitaminosis A endocrine: reproductive age, menstrual irregularities, Addison’s/Cushing’s disease, thyroid irregularities hematological: iron deficiency anemia, polycythemia vera drugs: steroid administration or withdrawal, tetracycline, nalidixic acid, etc. • risk factors overlap with those of venous sinus thrombosis; similar to those for gallstones (“fat, female, fertile, forties”) Epidemiology • incidence ~0.5/100,000 per year • usually in 3rd and 4th decade (F>M) Clinical Features • symptoms and signs of raised ICP (H/A in >90%, pulsatile intracranial noise), but NO decreased LOC or diplopia • decreased visual acuity, papilledema, visual field defect, optic atrophy (key morbidity) • usually self-limited, recurrence is common, chronic in some patients • risk of blindness is not reliably correlated to symptoms or clinical course Investigations • CT: normal • CSF studies: normal • MRI: must look for venous sinus thrombosis Treatment • rule out conditions that cause intracranial hypertension • discontinue offending medications, encourage weight loss, fluid/salt restriction • pharmacotherapy: acetazolamide (decreases CSF production), thiazide diuretic or furosemide • if above fail: serial LPs, shunt • optic nerve sheath decompression (if progressive impairment of visual acuity) • 2-year follow-up with imaging to rule out occult tumour, ophthalmology follow-up
Important features to note on CT and MRI (± contrast enhancement) • Lesions (± edema, necrosis, hemorrhage) • Midline shifts and herniations • Effacement of ventricles and sulci (often ipsilateral), basal cisterns • Single or multiple (multiple implies metastasis)
Primary CNS lymphoma reported in 6-20% of HIV infected patients.
Tumour
Sellar or suprasellar: pituitary adenoma, craniopharyngioma, optic nerve glioma, cyst
Skull base: carcinoma, chordoma, glomus jugulare, osteoma
Figure 9. Tumours
Supratentorial extra-axial: meningioma, cysts
DDx for Ring Enhancing Lesion on CT with Contrast MAGICAL DR Metastases* Abscess* Glioblastoma (high grade astrocytoma)* Infarct Contusion AIDS (toxoplasmosis) Lymphoma Demyelination Resolving hematoma (* 3 most common Dx’s)
Supratentorial intra-axial: astrocytoma, glioblastoma, oligodendrolioma, ganglioma, lymphoma, metastases
Posterior fossa intraaxial: schwannoma, meningioma, cysts, metastases Posterior fossa extra-axial: schwannoma, meningioma, cyst, metastases
© Ryan Kissinger 2010
Ventricular: colloid, choroid process papilloma, ependymoma, germinoma, teratoma, meningioma
Primary Sources of Metastatic Brain Tumours Lung 44% Breast 10% Kidney (RCC) 7% GI 6% Melanoma 3%
NS10 Neurosurgery Management of Single Brain Metastasis: A Practice Guideline Curr Oncol 2007; 14(4):131-43 Patients identified as having single brain metastasis undergo treatment that include whole brain radiation therapy (WBRT), surgical resection and stereotactic radiation surgery (SRS). Given that conflicting evidence has been reported with respect to the best approach to single brain metastases, the Neuro-oncology Disease Site Group of the Cancer Care Ontario Program conducted a systematic review of the evidence and practice guideline. Conclusions: Surgical excision should be considered for patients with good performance status, minimal or no evidence of extracranial disease, and a surgically accessible single brain metastasis amenable to complete excision. Because treatment in cases of single brain metastasis is considered palliative, invasive local treatments must be individualized. To reduce the risk of tumour recurrence for patients who have undergone resection of a single brain metastasis, postoperative WBRT should be considered. As an alternative to surgical resection, WBRT followed by SRS boost should be considered for patients with single brain metastasis. The evidence is insufficient to recommend SRS alone as a single-modality therapy.
Tumour
Toronto Notes 2011
Classification • primary vs. metastatic, intra-axial (parenchymal) vs. extra-axial, supratentorial vs. infratentorial, adult vs. pediatric • benign: non-invasive, but can be devastating due to expansion of mass in fixed volume of skull • malignant: implies rapid growth, invasiveness, but rarely extracranial metastasis • types of intracranial tumours (* = most common) neuroepithelial glial*: astrocytomas, oligodendrogliomas neuronal: ganglion cell tumours, cerebral neurocytomas/neuroblastomas poorly differentiated: glioblastomas, medulloblastomas other: pineal tumours, ependymomas, choroid plexus papillomas meningeal: meningiomas* nerve sheath: schwannoma, neurofibroma blood vessels: hemangioblastomas germ cells: germinomas, teratomas pituitary adenomas* craniopharyngiomas cysts: epidermoid/dermoid cysts, colloid cysts local extension: chordomas, glomus jugulare tumours other: primary CNS lymphomas, metastatic tumours Clinical Features • progressive neurological deficit (70%) – usually motor weakness, ± CN deficits, sensory, cognitive, personality, endocrine deficits (these may localize lesion) • H/A (50%) ± raised ICP (acute or chronic depending on growth rate), H/A classically worse in am but non-specific (likely hypoventilation during sleep causing vasodilatation g increased ICP), may worsen with bending forward/valsalva • N/V (40%) • seizures (25%) • papilledema, vision changes • symptoms suggestive of TIA (ictal, post-ictal, or ischemic 2o to “steal phenomenon”) • rarely presents with hemorrhage • familial syndromes associated with CNS tumours von Hippel-Lindau (hemangioma) tuberous sclerosis (astrocytoma) neurofibromatosis type 1 and 2 (astrocytoma, acoustic neuroma respectively) Li-Fraumeni (astrocytoma) Turcot syndrome (glioblastoma multiforme) multiple endocrine neoplasia type 1 (MEN-1) (pituitary adenoma) Investigations • CT, MRI, stereotactic biopsy (tissue diagnosis), metastatic work-up Treatment • conservative: serial Hx, Px, imaging for slow growing/benign lesions • medical: corticosteroids to reduce cytotoxic cerebral edema, pharmacological (see Pituitary Adenoma, NS13) • surgical: total or partial excision (decompressive, palliative), shunt if hydrocephalus • radiotherapy: conventional fractionated radiotherapy (XRT), stereotactic radiosurgery (Gamma Knife®) • chemotherapy: e.g. alkylating agents (temozolomide) Table 4. Tumour Types: Age, Location Age
Supratentorial
Infratentorial (posterior fossa)
15 years • 80% supratentorial
High grade astrocytoma (12-15%, e.g. GBM) Metastasis (15-30%, includes infratentorial) Meningioma (15-20%) Low grade astrocytoma (8%) Pituitary adenoma (5-8%) Oligodendroglioma (5%) Other: colloid cyst, CNS lymphoma, dermoid/epidermoid cysts
Metastasis Acoustic neuroma (schwannoma) (5-10%) Hemangioblastoma (2%) Meningioma
Toronto Notes 2011
Tumour
Neurosurgery NS11
Metastatic Tumours • most common brain tumour seen clinically • 15-30% of cancer patients present with cerebral metastatic tumours most common sites = lungs, breast other sites = kidney, thyroid, stomach, prostate, testis, melanoma • hematogenous spread most common Location • 80% are hemispheric, often at grey-white matter junction or junction of temporal-parietaloccipital lobes (likely emboli spreading to terminal MCA branches) Investigations • identify primary tumour metastatic work-up (CXR, CT chest/abdo, abdominal U/S, bone scan, mammogram) CT with contrast g round, well-circumscribed, often ring enhancing, ++ edema, often multiple MRI more sensitive, especially for posterior fossa • consider biopsy in unusual cases, or if no primary identified 1. metastatic work-up negative g brain biopsy 2. metastatic work-up positive g biopsy of affected sites other than brain Treatment • medical phenytoin for seizure prophylaxis if patient presents with seizure dexamethasone to reduce edema given with ranitidine chemotherapy (e.g. small cell lung cancer) • radiation stereotactic radiosurgery: for discrete, deep-seated/inoperable tumours multiple lesions: use whole brain radiation therapy (WBRT); consider stereotactic radiosurgery if 10 years, cure if gross total resection
II – Low grade/diffuse
Mass effect, no enhancement
5 years
III – Anaplastic
Complex enhancement
1.5-2 years
IV – Glioblastoma multiforme (GBM)
Necrosis (ring enhancement)
12 months, 10% at 2 years
Clinical Features • epidemiology: most common in 4th-6th decades • sites: cerebral hemispheres >> cerebellum, brainstem, spinal cord • symptoms: recent onset of new/worsening H/A, N/V, seizure, ± focal deficits or symptoms of increased ICP Investigations • CT with contrast: variable appearance depending on grade (see Table 5) • tissue biopsy: WHO grade and histology correlates with prognosis, but 25% chance of sampling error due to tumour heterogeneity
Karnofsky General Cancer Performance Status Scale Rating Criteria (%) 100 No complaints; no evidence of disease 90 Able to carry on normal activity; minor signs or symptoms of disease 80 Normal activity with effort; some signs or symptoms of disease 70 Cares for self; unable to carry on normal activity or to do active work 60 Requires occasional assistance, but is able to care for most of his personal needs 50 Requires considerable assistance and frequent medical care 40 Disabled; requires special care and assistance 30 Severely disabled; hospital admission is indicated although death not imminent 20 Very sick; hospital admission necessary; active supportive treatment necessary 10 Moribund; fatal processes progressing rapidly 0 Dead
NS12 Neurosurgery
Tumour
Toronto Notes 2011
Treatment • low grade diffuse astrocytoma close follow-up, radiation, chemotherapy, surgery all valid options surgery: not curative, trend towards better outcomes radiotherapy alone or post-op prolongs survival (retrospective evidence) chemotherapy: usually reserved for tumour progression • high grade astrocytomas (anaplastic astrocytoma and GBM) surgery gross total resection: maximal safe resection + fractionated radiation with 2 cm margin + concomitant and adjuvant temozolomide – except: extensive dominant lobe GBM, significant bilateral involvement, end of life near, extensive brainstem involvement stereotactic biopsy if resection not possible, followed by fractioned radiation with 2 cm margin expectant (based on functional impairment – Karnofsky score 98% sensitive/specific), CT with contrast 2nd choice • audiogram, brainstem auditory evoked potentials, caloric tests Treatment • conservative: serial imaging • radiation: stereotactic radiosurgery is the treatment of choice • surgery if: 1. lesion >3 cm; 2. brainstem compression; 3. edema; 4. hydrocephalus curable if complete resection (almost always possible) operative complications: CN VII, VIII dysfunction (only significant disability if bilateral), CSF leak
Figure 13. Vestibular Schwannoma (tumour in cerebello-pontine angle)
Pituitary Adenoma • primarily from anterior pituitary, 3rd-4th decades, M=F • incidence in autopsy studies approximately 20% • classification microadenoma 2.5 cm in largest diameter. Results: A total of 105 patients had favourable outcome: 63 (44.4%) had full recovery and 42 (29.6%) had mild deficits. 37 patients had an unfavourable outcome, 24 died in hospital and 13 had moderate to severe disability. Multivariate logistic regression showed improved outcome associated with being male (OR 9.81, P=0.002), having a presenting GCS >12 (OR 6.20, P=0.019), being sepsis-free throughout hospitalization (OR 761.49, P50, associated with trauma
No lucid interval, hemiparesis, Pupillary changes
Crescentic mass
Craniotomy if bleed >1 cm
Poor
Chronic Subdural Hematoma
Ruptured subarachnoid bridging vessels
Age >50, EtOH abusers, anti-coagulated
Often asymptomatic Minor H/A, confusion, signs of increased ICP
Hypodense crescentic mass
Burr hole to drain; craniotomy if reoccurs
Good
Subarachnoid Hemorrhage
Trauma, spontaneous Age 55-60 (aneurysms, 20% cases under idiopathic, AVM) age 45
Sudden onset thunderclap headache, signs of increased ICP
High density blood (sensitivity decreases over time)
Conservative: Poor: 50% mortality NPO, IV NS, ECG, Foley, 30% of survivors have BP 120-150, vasospasm moderate to severe disability prophylaxis (nimodipine); open vs. endovascular surgery to repair if rebleed
Intracerebral Hemorrhage
HTN, vascular abnormality, tumours, infections, coagulopathy
High density blood
Medical: decrease BP, control ICP Surgical: Craniotomy
Age >55, male, TIA-like symptoms, drug use (cocaine, signs of increased ICP EtOH, amphetamine)
Poor: 44% mortality due to cerebral herniation
Extradural (“Epidural”) Hematoma Etiology • temporal-parietal skull fracture: 85% are due to ruptured middle meningeal artery. Remainder of cases are due to bleeding from middle meningeal vein, dural sinus, or bone/diploic veins Epidemiology • young adult, male > female = 4:1; rare before age of 2 or after age 60 Clinical Features • in 60%, there is lucid interval of several hours between concussion and coma • then, obtundation, hemiparesis, ipsilateral pupillary dilatation • signs and symptoms depend on severity but can include H/A, N/V, amnesia, altered LOC, HTN and respiratory distress • deterioration can take hours to days Investigations • CT without contrast: high density biconvex mass against skull, “lenticular-shaped,” usually with uniform density and sharp margins, usually limited by suture lines Treatment • admit, observe, head elevation • mannitol pre-op if elevated ICP/brain herniation • craniotomy to evacuate clot, follow up CT
1 2
1. Compression of ventricles (midline shift) 2. Blood
Figure 15. Extradural Hematoma on CT
NS16 Neurosurgery
Blood
Calcium antagonists for aneurysmal subarachnoid haemorrhage (Review) Cochrane Review 2008; Issue 3. Introduction: This study looked to review the evidence in regards as to whether calcium antagonists improve the outcome in patients with aneurysmal subarachnoid haemorrhage. Methods/Population: The review included 3361 patients presenting with aneurysmal subarachnoid haemorrhage from 16 randomised controlled trials comparing treatment with calcium antagonists vs. control from 1980 to March 2006. Results: The results were based mainly on one large trial of oral nimodipine, which showed a RR of 0.67 (95% CI 0.55 to 0.81) and the evidence for other calcium agonists was not statistically significant. Conclusion: The authors endorse the use of oral nimodipine in patients with aneurysmal subarachnoid haemorrhage.
Toronto Notes 2011
Prognosis • good with prompt management, as the brain is often not damaged • worse prognosis if bilateral Babinski or decerebration pre-op • death is usually due to respiratory arrest from uncal herniation (injury to the midbrain)
Subdural Hematoma ACUTE SUBDURAL HEMATOMA Etiology • rupture of vessels that bridge the subarachnoid space (e.g. cortical artery, large vein, venous sinus) or cerebral laceration Risk Factors • trauma, anticoagulants, alcohol, cerebral atrophy, infant head trauma (see Pediatrics) Clinical Features • no lucid period, signs and symptoms can include altered LOC, pupillary irregularity, hemiparesis
CT Density and MRI Appearance of Blood Time
CT
MRI -T1
MRI -T2
Acute (45 yrs, known HTN, and putamen/thalamic/ posterior fossa ICH (yield ~ 0%) • surgical craniotomy with evacuation of clot, treatment of source of ICH (i.e. AVM, tumour, cavernoma), ventriculostomy to treat hydrocephalus indications symptoms of raised ICP or mass effect rapid deterioration (especially if signs of brainstem compression) favourable location, e.g. cerebellar, non-dominant hemisphere young patient (10 poor prognosis: massive hemorrhage (especially dominant lobe), low GCS/coma, lost brainstem function medical reasons [e.g. very elderly, severe coagulopathy, difficult location (e.g. basal ganglia, thalamus)] Prognosis • 30-day mortality rate 44%, mostly due to cerebral herniation • rebleed rate 2-6%, higher if HTN poorly controlled
Intracranial Aneurysms Epidemiology • prevalence 1-4% (20% have multiple) • female > male; age 35-65 years Risk Factors • autosomal dominant polycystic kidney disease (15%) • fibromuscular dysplasia (7-21%) • AVMs • connective tissue diseases (Ehlers-Danlos, Marfan’s) • family history • bacterial endocarditis • Osler-Weber-Rendu syndrome (hereditary hemorrhagic telangiectasia) • atherosclerosis and HTN • trauma Types (Figure 4, NS3) • saccular (berry) most common type located at branch points of major cerebral arteries (Circle of Willis) 85-95% in carotid system, 5-15% in vertebrobasilar circulation • fusiform atherosclerotic more common in vertebrobasilar system, rarely rupture • mycotic secondary to any infection of vessel wall, 20% multiple 60% Streptococcus and Staphylococcus 3-15% of patients with SBE
Toronto Notes 2011
Cerebrovascular Disease
Neurosurgery NS21
Table 7. 5-year Cumulative Rupture Risk in Unruptured Aneurysms Based on Size and Location Cavernous Carotid
AC/MC/IC
Vertebrobasilar/PC/PComm
anterior circulation , deep/eloquent location, basilar artery bifurcation/apex, older age, presence of comorbidities, presence of vasospasm clipping: superficial > deep, broad aneurysmal base, branching arteries at the aneurysm base, tortuosity/atherosclerosis of afferent vessels, dissection, hematoma, acute brainstem compression • unruptured aneurysms average 1% annual risk of rupture: risk dependent on size and location of aneurysm no clear evidence on when to operate: need to weigh life expectancy risk of morbidity/mortality of SAH (20%/50%) vs. surgical risk (2%/5%) generally treat unruptured aneurysms >10 mm consider treating when aneurysm 7-9 mm in middle-aged, younger patients or patients with a family history of aneurysms follow smaller aneurysms with serial angiography
Carotid Stenosis Definition • narrowing of the internal carotid artery lumen due to atherosclerotic plaque formation, usually near common carotid bifurcation into internal and external carotids Risk Factors • for atherosclerosis: HTN, smoking, DM, CVD or CAD, dyslipidemia Clinical Features • may be asymptomatic • symptomatic stenosis may present as TIA, reversible ischemic neurologic deficit (RIND), or stroke • retinal insufficiency or infarct permanently or temporarily (amaurosis fugax), (see Neurology, N47 and Ophthalmology, OP37) • middle cerebral artery (MCA) occlusive symptoms
International Subarachnoid Aneurysm Trial (ISAT) of Neurosurgical Clipping versus Endovascular Coiling in 2143 Patients with Ruptured Intracranial Aneurysms: A Randomised Comparison of Effects on Survival, Dependency, Seizures, Rebleeding, Subgroups, and Aneurysm Occlusion Lancet 2005; 366:809-17 This randomized trial aimed to compare endovascular detachable coiled treatment against craniotomy and clipping for ruptured intracranial aneurysms in patients who were considered eligible for either modality of therapy. Conclusion: In patients with ruptured intracranial aneurysms suitable for both treatments, endovascular coiling is more likely to result in independent survival at 1 year than neurosurgical clipping; the survival benefit continues for at least 7 years. The risk of late rebleeding is low, but is more common after endovascular coiling than after neurosurgical clipping.
NS22 Neurosurgery
Cerebrovascular Disease/Vascular Malformations
Prevention of Disabling and Fatal Strokes by Successful Carotid Endarterectomy in Patients Without Recent Neurological Symptoms: Randomised Controlled Trial Lancet 2004; 363:1491-1502 Study: Asymptomatic Carotid Surgery Trial (ACST), a randomized, controlled trial with follow-up at 5 years. Patients: 3120 asymptomatic patients with significant carotid artery stenosis were randomized equally between immediate carotid endarterectomy (CEA) and indefinite deferral of CEA and were followed for up to 5 years (mean 3.4 years). Main Outcome: Any stroke (including fatal or disabling). Conclusions: In asymptomatic patients with significant carotid artery stenosis, immediate CEA reduced the net 5-year stroke risk from about 12% to about 6%. Half of this 5-year benefit involved disabling or fatal strokes.
Toronto Notes 2011
Investigations • CBC, PTT/INR (hypercoagulable states) • fundoscopy: cholesterol emboli in retinal vessels (Hollenhorst plaques) • auscultation over carotid bifurcation for bruits • carotid duplex Doppler ultrasound: determines size of lumen and blood flow velocity, safest but least accurate, unable to scan above mandible • angiogram: “gold standard” but invasive and 1/200 risk of stroke (not for screening) • MRA: safer than angiogram, may overestimate stenosis Treatment • control of HTN, lipids, diabetes • antiplatelet agents (ASA ± dipyridamole, clopidogrel) ~25% relative risk reduction • carotid endarterectomy (generally if symptomatic and >70% stenosis, see Tables 8 and 9) • endovascular angioplasty ± stenting Prognosis Table 8. Symptomatic Carotid Stenosis: North American Symptomatic Carotid Endarterectomy Trial (NASCET) % Stenosis on Angiogram
Risk of Major Stroke or Death Medical Rx
Medical + Surgical Rx
70-99 %
26% over 2 years
9% over 2 years
50-69%
22% over 5 years
16% over 5 years
6 cm
1 2 3
Location Noneloquent Eloquent
0 1
Deep venous drainage Not present Present
0 1
AVM grades calculated by adding the 3 individual Spetzler-Martin Scale scores from the above table. E.g. A 2 cm tumour in noneloquent location without deep venous drainage = Grade I.
Definition • tangle of abnormal vessels/arteriovenous shunts, with no intervening capillary beds or brain parenchyma • congenital Epidemiology • prevalence ~0.14%, male:female = 2:1, average age at diagnosis = 33 years • 15-20% of patients with hereditary hemorrhagic telengiectasia (Osler-Weber-Rendu syndrome) will have cerebral AVMs Clinical Features • hemorrhage (40-60%) – small AVMs are more likely to bleed due to direct high pressure AV connections • seizures (50%) – more common with larger AVMs • mass effect • focal neurological signs secondary to ischemia (high flow g “steal phenomena”) • localized headache, increased ICP • bruit (especially with dural AVMs) • may be asymptomatic (“silent”)
Toronto Notes 2011
Vascular Malformations/Cavernous Malformations
Neurosurgery NS23
Investigations • MRI (flow void), MRA • angiography (7% will also have one or more associated aneurysms) Treatment • decreases risk of future hemorrhage and seizure surgical excision is treatment of choice stereotactic radiosurgery (SRS) is preferred for small (50, previous hx of cancer, pain unrelieved by bed rest, constitutional symptoms. Infection. Increased ESR, IV drug use, immunosuppressed, fever. Compression Fracture Age >50, trauma, prolonged steroid use.
Differential Diagnosis • herniated disk • neoplasm (neurofibroma, schwannoma) • synovial cyst, abscess • hypertrophic bone/spur
Cervical Disc Syndrome Etiology • nucleus pulposus herniates through annulus fibrosis and impinges upon nerve root
RED FLAGS for Back Pain BACK PAIN Bowel/Bladder (retention or incontinence) Anesthesia (saddle) Constitutional symptoms Khronic disease Parasthesia Age >50 or C5-C6 (C6 root) Clinical Features • pain down arm in nerve root distribution, worse with neck extension, ipsilateral rotation and lateral flexion (all compress the ipsilateral neural foramen) • LMN signs and symptoms • central cervical disc protrusion causes myelopathy as well as nerve root deficits Investigations • if red flags: C-spine x-ray, CT, MRI (imaging of choice) • consider EMG, nerve conduction studies if diagnosis uncertain
© Glen Oomen 2002
Important Dermatomes and Myotomes C2 – Angle of jaw C4 – Collar of shirt “C3,4,5 keeps the diaphragm alive” T4 – Nipple line T6 – Xiphoid T10 – Umbilical T12 – Suprapubic “L3 above the knee” “S2,3,4 – Keeps your stool off the floor”
Toronto Notes 2011
Extradural Lesions
Treatment • conservative no bedrest unless severe radicular symptoms activity modification, patient education (reduce sitting, lifting) physiotherapy, exercise programs analgesics, collar, traction may help • surgical indications intractable pain despite adequate conservative treatment for >3 months progressive neurological deficit anterior cervical discectomy is usual surgical choice
Neurosurgery NS25
Disc herniations impinge the nerve root at the level below the interspace (i.e. C5-6 disc affects the C6 nerve root).
Prognosis • 95% improve spontaneously in 4-8 weeks Table 10. Lateral Cervical Disc Syndromes C4-5
C5-6
C6-7
C7-T1
Root Involved
C5
C6
C7
C8
Incidence
2%
19%
69%
10%
Sensory
Shoulder
Thumb
Middle finger
Ring finger, 5th finger
Motor
Deltoid, biceps, supraspinatus
Biceps
Triceps
Digital flexors, intrinsics
Reflex
No change
Biceps, Brachioradialis
Triceps
Finger jerk (Hoffmann’s sign)
Cervical Stenosis (Cervical Spondylosis) Definition • cervical spondylosis is chronic disc degeneration and associated facet arthropathy • resultant syndromes include mechanical neck pain, radiculopathy (root compression), myelopathy (spinal cord compression) and combinations
Sensory Fibres Fasciculus gracilis/cuneatus: joint position, fine touch, vibration Spinothalamic tract: Pain and temperature Motor Fibres Corticospinal tract: skilled movements
Dorsal funiculus Posterior spinal artery Dorsal horn (sensory)
Epidemiology • typically begins at age 40-50, men > women, most commonly at the C5-C6 > C6-C7 levels Pathogenesis • with neck extension, the cervical cord is pinched. With neck flexion, the canal dimensions increase slightly to relieve pressure on the cervical cord Clinical Features • insidious onset of mechanical neck pain exacerbated by excess vertebral motion (particularly rotation and lateral bending with a vertical compressive force – Spurling’s test). Pain is worse with neck extension, relieved with flexion • occipital headache is common • radiculopathy may involve 1 or more roots, and symptoms include neck, shoulder and arm pain, paresthesias and numbness • cervical myelopathy may be characterized by weakness (upper > lower extremity), decreased dexterity and sensory changes. UMN findings such as hyperreflexia, clonus and Babinski reflex may be present. The most worrisome complaint is lower extremity weakness (corticospinal tracts) • myelopathy may be associated with funicular pain, characterized by burning and stinging ± Lhermitte’s sign (lightning-like sensation down the back with neck flexion) Investigations • x-ray of cervical spine ± flexion/extension or oblique views (studied for changes in Luschka and facet joints, osteophytes and disc space narrowing), MRI, CT, EMG Treatment • NSAIDS, moist heat, strengthening and range of motion exercises, analgesics, cervical collar, cervical traction • surgical indications: myelopathy with motor impairment, progressive neurologic impairment, intractable pain
Lateral horn (motor) Ventral horn (motor) Anterior spinal artery Vascular territory
Fasciculus gracilis Fasciculus cuneatus Lateral corticospinal tract (efferent)
Lateral funiculus Ventral funiculus Spinothalamic tract Anterior (afferent) corticospinal tract (efferent) © Takami Iijima 2009
Figure 21A. Axial section of Cervical Spine with Vascular and Functional Territories Fasciculus gracilis Fasciculus cuneatus Lateral Posterior spinal artery corticospinal tract (efferent) Dorsal horn (sensory) Dorsal funiculus
Lateral funiculus Lateral horn (motor) Ventral horn Ventral funiculus (motor) Spinothalamic tract Anterior spinal artery (afferent) Anterior corticospinal tract Vascular territory (efferent) © Takami Iijima 2009
Figure 21B. Axial section of Thoracic Spine with Vascular and Functional Territories
NS26 Neurosurgery
Extradural Lesions
Dorsal funiculus Lateral Posterior spinal artery corticospinal tract (efferent) Dorsal horn (sensory)
Lateral Lateral horn funiculus (motor) Ventral horn Ventral funiculus (motor) Anterior spinal artery Spinothalamic tract (afferent) Anterior corticospinal tract (efferent) Vascular territory © Takami Iijima 2009
Figure 21C. Axial section of Lumbar Spine with Vascular and Functional Territories Dorsal funiculus Lateral Posterior spinal artery corticospinal tract (efferent) Dorsal horn (sensory)
Lateral funiculus Ventral horn (motor) Ventral funiculus Anterior spinal artery Spinothalamic tract Anterior (afferent) corticospinal tract (efferent) Vascular territory © Takami Iijima 2009
Figure 21D. Axial section of Sacral Spine with Vascular and Functional Territories
Toronto Notes 2011
Lumbar Disc Syndrome Etiology • laterally herniated lumbar disc compresses nerve root, central herniation causes cauda equina or lumbar stenosis (neurogenic claudication) Epidemiology • common (>95% of herniated lumbar disks) – L5 and S1 roots Clinical Features • leg pain > back pain • limited back movement (especially forward flexion) due to pain • motor weakness, dermatomal sensory changes, reflex changes • exacerbation with coughing, sneezing or straining. Relief with flexing the knee or thigh • nerve root tension signs straight leg raise (SLR: Lasegue’s test) or crossed SLR (pain should occur at less than 60 degrees) suggests L5, S1 root involvement femoral stretch test suggests L2, L3 or L4 root involvement Investigations • x-ray spine (only to rule out other lesions), CT, MRI • myelogram and post-myelogram CT (only if MRI is contraindicated) Treatment • conservative (same as cervical disc disease) • surgical indications same as cervical disc + cauda equina syndrome Prognosis • 95% improve spontaneously within 4 to 8 weeks Table 11. Lateral Lumbar Disc Syndromes L3-4
L4-5
L5-S1
Root Involved
L4
L5
S1
Incidence
5 minutes amnesia • unstable spine • use of alcohol • poor social support (i.e. no friend/relative to monitor for next 24 hours) • if there is any doubt, especially with children KEY POINTS • never do lumbar puncture in head injury unless increased ICP has been ruled out • all patients with head injury have C-spine injury until proven otherwise • alcohol may not be the cause of coma – there may also be a hematoma • low BP after head injury means injury elsewhere • must clear spine both radiologically AND clinically (will require re-assessment if/when patient improves clinically)
Toronto Notes 2011
Neurotrauma
Neurosurgery NS31
Head Injury Epidemiology • male to female: 2-3:1 Pathogenesis • acceleration/deceleration: contusions, subdural hematoma, axon and vessel shearing/ mesencephalic hematoma • impact: skull fracture, concussion, epidural hematoma • penetrating: worse with high velocity and/or high missile mass low velocity: highest damage to structures on entry/exit path high velocity: highest damage away from missile tract Scalp Injury • rich blood supply • considerable blood loss (vessels contract poorly when ruptured) • minimal risk of infection due to rich vascularity Skull Fractures • depressed fractures: double density on skull x-ray (outer table of depressed segment below inner table of skull), CT with bone windows is gold standard • simple fractures (closed injury): no need for antibiotics, no surgery • compound fractures (open injury): increased risk of infection, surgical debridement within 24 hours is necessary internal fractures into sinus may lead to meningitis, pneumocephalus risk of operative bleed may limit treatment to antibiotics • basal skull fractures: not readily seen on x-ray, rely on clinical signs retroauricular ecchymoses (Battle’s sign) periorbital ecchymoses (raccoon eyes) hemotympanum CSF rhinorrhea, otorrhea (suspect CSF if halo or target sign present); suspect with Lefort II/ III midface fracture
Head Injury can involve: Scalp, Skull, Meninges, Brain
Etiologies • MVA (30-55%) • Falls (15-35%) • Gun Shot Wound (5-20%)
Layers of Scalp SCALP Skin Connective tissue (dense) Aponeurosis (galea) Loose connective tissue Periosteum
Cranial Nerve Injury • most traumatic causes of cranial nerve injury do not warrant surgical intervention • surgical intervention CN II – local eye/orbit injury CN III, IV, VI – if herniation secondary to mass CN VIII – repair of ossicles • CN injuries that improve CN I – recovery may occur in a few months; most do not improve CN III, IV, VI – majority recover CN VII – recovery with delayed lesions CN VIII – vestibular symptoms improve over weeks, deafness usually permanent (except when resulting from hemotympanum) Arterial Injury • e.g. carotid-cavernous (C-C) fistula, carotid/vertebral artery dissection Intracranial Bleeding (see Blood, NS15 and Cerebrovascular Disease, NS17)
Brain Injury Primary Impact Injury • mechanism of injury determines pathology: penetrating injuries, direct impact low velocity: local damage high velocity: distant damage possible (due to wave of compression), concussion • concussion: a trauma-induced alteration in mental status American Academy of Neurology (AAN) Classification (see sidebar) no parenchymal abnormalities on CT • coup (damage at site of blow) • contre-coup (damage at opposite site of blow) acute decompression causes cavitation followed by a wave of acute compression
AAN Classification Grade 1: altered mental status 15 min Grade 3: any loss of consciousness
NS32 Neurosurgery
Neurotrauma
Management Associated with AAN Concussion Grades AAN Management Options Grade 1
• Examine 15 min for amnesia and other symptoms • Return to normal activity if symptoms clear within 15 mins
2
• Remove from activity for 1 day, then reexamine • CT or MRI if H/A or other symptoms worsen or last >1 week • Return to normal activity after 1 week without symptoms
3
• Emergent neuro exam + imaging; if initial exam is normal, may go home with close follow up • Admit if any signs of pathology or persistent abnormal mental status • CT or MRI if H/A or other symptoms • If brief concussion (1 min), return to normal activity only after 2 weeks without symptoms
SIADH hyponatremia DI hypernatremia
• contusion (hemorrhagic) high density areas on CT ± mass effect commonly occurs with brain impact on bony prominences (inferior frontal lobe, pole of temporal lobe) • diffuse axonal injury/shearing wide variety of damage results may tear blood vessels (hemorrhagic foci) often the cause of decreased LOC if no space occupying lesion on CT Secondary Pathologic Processes • same subsequent biochemical pathways for each traumatic etiology • delayed and progressive injury to the brain due to high glutamate release g NMDA g cytotoxic cascade cerebral edema intracranial hemorrhages ischemia/infarction raised ICP, intracranial HTN hydrocephalus Extracranial Conditions • hypoxemia due to trauma to the chest, upper airway, brainstem extremely damaging to vulnerable brain cells leads to ischemia, raised ICP • hypercarbia leads to raised ICP (secondary to vasodilation) • systemic hypotension caused by blood loss (e.g. ruptured spleen) loss of cerebral autoregulation leads to decreased CPP, ischemia • hyperpyrexia leads to increased brain metabolic demands g ischemia • fluid and electrolyte imbalance iatrogenic (most common) SIADH caused by head injury diabetes insipidus (DI) from head injury may lead to cerebral edema and raised ICP • coagulopathy Intracranial Conditions • raised ICP due to traumatic cerebral edema OR traumatic intracranial hemorrhage
Direction of Force
Coup
Contrecoup
Figure 25. CT Showing Coup-Contre-Coup Injury
Toronto Notes 2011
Brain Injury Outcomes • mildly traumatic (GCS 13-15): post-concussive symptoms: H/A, fatigue, dizziness nausea, blurred vision, diploplia, memory impairment, tinnitus, irritability, low concentration; 50% at 6 weeks, 14% at 1 year • moderately traumatic (GCS 9-12): proportional to age (>40) and CT findings; 60% good recovery, 26% moderately disabled, 7% severely disabled, 7% vegetative/dead • severe (GCS ≤8): difficult to predict, correlates with post-resuscitation GCS (especially motor) and age
Late Complications of Head/Brain Injury • seizures: 5% of head injury patients develop seizures incidence related to severity and location of injury (increased with local brain damage or intracranial hemorrhage) post-traumatic seizure may be immediate, early, or late presence of early (within first week) post-traumatic seizure raises incidence of late seizures • meningitis: associated with CSF leak from nose or ear • hydrocephalus: acute hydrocephalus or delayed normal pressure hydrocephalus (NPH)
Toronto Notes 2011
Neurotrauma
Neurosurgery NS33
Spinal Cord Injury (SCI) • see Orthopaedics, OR22 and Emergency Medicine, ER9 Neurogenic and Spinal Shock 1. neurogenic shock: hypotension that follows SCI (SBP usually ≤80 mmHg) caused by interruption of sympathetics (unopposed parasympathetics) below the level of injury loss of muscle tone due to skeletal muscle paralysis below level of injury venous pooling (relative hypovolemia) blood loss from associated wounds (true hypovolemia) 2. spinal shock: transient loss of all neurologic function below the level of the spinal cord injury, causing flaccid paralysis and areflexia for variable periods Whiplash-Associated Disorders • definition: traumatic injury to the soft tissue structures in the region of the cervical spine due to hyperflexion, hyperextension or rotational injury to the neck Initial Management of SCI • major causes of death in SCI are aspiration and shock • the following patients should be treated as having a SCI until proven otherwise: all victims of significant trauma minor trauma patients with decreased LOC or complaints referable to neck or back pain or SCI (weakness, abdominal breathing, numbness/tingling, priapism) Stabilization and Initial Evaluation in the Hospital 1. ABC’s 2. immobilization: maintain backboard/head-strap and use log-rolling during transfers 3. hypotension: maintain sBP ≥90 mmHg with pressors if necessary: dopamine is agent of choice (avoid phenylephrine – causes reflex bradycardia) hydration (cautiously to prevent pulmonary edema) atropine (for bradycardia associated with hypotension) 4. oxygenation 5. NG tube to suction: prevents N/V, decompresses abdomen to facilitate breathing 6. foley catheter to urometer (to monitor ins and outs) 7. DVT prophylaxis 8. temperature regulation 9. monitor CBC/electrolytes 10. focused history mechanism of injury (hyperflexion, extension, axial loading, etc.) weakness/numbness/tingling in extremities 11. palpate spine for point tenderness a “step-off ” deformity widened interspinous space 12. motor level assessment – grade with American Spinal Injury Association (ASIA) Standards skeletal muscle exam rectal exam for voluntary anal sphincter contraction 13. sensory level assessment (grade with ASIA Standards) sensation to pinprick (spinothalamic tract) sensation in face (spinal trigeminal tract can sometimes descend as low as C4) sensation to light touch (anterior spinothalamic tract in anterior cord) proprioception (posterior columns) 14. evaluation of reflexes 15. signs of autonomic dysfunction: altered levels of perspiration, bowel or bladder incontinence, priapism 16. radiographic evaluation 3 view C-spine x-rays (AP, lateral and odontoid) to adequately visualize C1 to C7-T1 junction oblique views to assess integrity of articular masses and lamina flexion-extension views to disclose occult instability CT scan (bony injuries) ± MRI (soft tissue injuries) Medical Management Specific to SCI • methylprednisolone (given within 8 hours of injury) • ± decompression in acute, nonpenetrating SCI (Fehlings and Tator, 1999)
Does early decompression improve neurological outcome of spinal cord injured patients? Appraisal of the literature using a metaanalytical approach Spinal Cord 2004; 42:503-512. Introduction: This study was aimed at determining whether neurological outcome was improved with early (24 hours) surgery or conservative management. Methods/Population: A Medline search found 37 articles with a total of 1683 eligible patients (traumatic spinal injury), 919 with complete paralysis and 764 with an ‘incomplete’ neurological deficit. Outcome was determined by calculating the percentage of patients with sensory or motor function improvement according to the Frankel’s scale. Results: Early decompression resulted in improved outcome compared to both late and conservative treatments, for both complete and incomplete injury (p0.05), however all other subgroups showed significant heterogeneity. Conclusion: Although statistically promising, with the lack of patient homogeneity in recent studies, the current recommendation is that early surgical decompression be considered as an optional practice following traumatic spinal cord injuries.
Methylprednisolone treatment in acute spinal cord injury: the myth challenged through a structured analysis of published literature The Spine Journal 2006; 6:335-343 Introduction: Beginning in the late 70’s and through to the early 90’s the National Acute Spinal Cord Injury Studies (NASCIS) were conducted in three large randomized controlled trials, and from those trials the recommendation to give patients methylprednisolone (MP) within 8 hours of spinal injury. Critical evaluation of each of these trials over the years led these authors to assess the literature (including the NASCIS trials) to re-evaluate the evidence behind this recommendation. Methods/Population: A critical appraisal of the data obtained through the NASCIS studies along with a literature review of other trials investigating the effect of MP on acute spinal injury. Results: The authors raise several important issues with each of NASCIS II and III, including the fact that statistical analysis was completed on only 30% of the population in NASCIS II and benefits were only observed in ‘incomplete’ spinal injuries. NASCIS III was evaluated for lack of explanation on optimum MP timing along with failure to report motor changes post-therapy on both the right and left side of the body, suggesting that changes in one side were not significant, arguing against the meaningfulness of the results. Other prior studies failed to reproduce the results of NASCIS, but there is the confounding factor that studies since NASCIS have all included MP since it was thought to be unethical to randomize individuals to a placebo group. Conclusion: The authors conclude that based on these and other major criticisms of the NASCIS II and III, many neurosurgical departments have stopped using MP as a standard routine in acute spinal cord injury, and also note that its use is not currently recommended by the Food and Drug Administration in the United States. They suggest further investigation is warranted to answer this question conclusively.
NS34 Neurosurgery
Neurotrauma
Toronto Notes 2011
Fractures of the Spine Fractures and Fracture-Dislocations of the Thoracic, Thoracolumbar and Lumbar Spine • assess ligamentous instability using flexion/extension x-ray views of C-spine ± MRI • thoracolumbar spine unstable if 4/6 segments disrupted (3 columns divided into left and right) anterior column: anterior half of vertebral body, disc and anterior longitudinal ligament middle column: posterior half of vertebral body, disc and posterior longitudinal ligament posterior column: posterior arch, facet joints, pedicle, lamina and supraspinous, interpinous and ligamentum ligaments Type 1
Type 3
Figure 26. Odontoid Fracture Classification
© Hidenori Miyagawa 2006
Type 2
Types of Injury (Dennis Classification) • compression fracture (58%) produced by flexion posterior ligament complex (supraspinous and interspinous ligaments, ligamentum flavum and intervertebral joint capsules) remain intact fractures are stable but lead to kyphotic deformity • burst fracture (17%) stable: anterior and middle columns parted with bone retropulsed nearby hallmark is pedicle widening on AP X-ray spinal cord (seen on x-ray and CT); posterior column is uninjured unstable: same as the stable but with posterior column disruption (usually ligamentous) • flexion distraction injury (6%) hyperflexion and distraction of posterior elements middle and posterior columns fail in distraction classic: Chance = horizontal fracture through posterior arch, pedicles, posterior vertebral body can be purely ligamentous, i.e. through PLL and disc • fracture-dislocation (6%) anterior and cranial dislocation of superior vertebral body g 3 column failure three types: flexion-rotation flexion-distraction shear/hyperextension (rare) Fractures of the Cervical Spine Types of Injury • C1 vertebral fracture (Jefferson fracture) vertical compression forces the occipital condyles of the skull down on the C1 vertebra (atlas), pushing the lateral masses of the atlas outward and disrupting the ring of the atlas also can cause an occipital condylar fracture • odontoid process fracture causes C1 and odontoid of C2 to move independently of C2 body this occurs because normally C1 vertebra and odontoid of C2 are a single functional unit alar and transverse ligaments on posterior aspect of odontoid most commonly remain intact following injury patients often report a feeling of instability and present holding their head with their hands • C2 vertebral fracture (hangman fracture, traumatic spondylolisthesis of axis): bilateral fracture through the pars interarticularis of C2 with subluxation of C2 on C3 usually neurologically intact • clay shoveler’s fracture avulsion of spinous process, usually C7 Imaging • AP spine x-ray (open-mouth and lateral view), CT Treatment • immobilization in cervical collar or halo vest until healing occurs, (usually 2-3 months) • Type II and III odontoid fractures consider surgical fixation for comminution, displacement or inability to maintain alignment with external immobilization onfirm stability after recovery with flexion-extension x-rays
Toronto Notes 2011
Neurotrauma
Neurosurgery NS35
Neurologically Determined Death Definition • irreversible and diffuse brain injury resulting in absence of clinical brain function • cardiovascular activity may persist for up to two weeks Criteria of Diagnosis • prerequisites: no CNS depressant drugs/neuromuscular blocking agents, no drug intoxication/ poisoning, temperature >32ºC, no electrolyte/acid-base/endocrine disturbance • absent brainstem reflexes: absent pupillary light reflex absent corneal reflexes absent oculocephalic response absent caloric responses (e.g. no deviation of eyes to irrigation of each ear with 50 cc of ice water – allow 1 min after injection, 5 min between sides) absent pharyngeal and tracheal reflexes absent cough with tracheal suctioning absent respiratory drive at PaCO2 >60 mmHg or >20 mmHg above baseline (apnea test) • 2 evaluations separated by time, usually performed by two specialists (e.g. anesthetist, neurologist, neurosurgeon) • confirmatory testing: flat EEG, absent perfusion assessed with cerebral angiogram
Altered Level of Consciousness Evaluation of Patient History • previous/recent head injury (hematoma) • sudden collapse (ICH, SAH) • cardiovascular surgery, prolonged cardiac arrest (hypoxia) • limb twitching, incontinence, tongue biting (seizure, post-ictal state) • recent infection (meningitis) • other medical problems (diabetes mellitus, renal failure, hepatic encephalopathy) • psychiatric illness (drug overdose) • telephone witnesses, read ambulance report, check for medic-alert bracelet • neurologic symptoms (headache, visual changes, focal weakness) Physical Examination • Glasgow Coma Scale (see sidebar, Neurotrauma, NS30) • pupils: reactivity and symmetry, papilledema (increased ICP) • reflexes: corneal reflex: normal = bilateral blinking response gag reflex: normal = gag oculocephalic reflex (doll’s eye): normal = eyes move in opposite direction of head, as if trying to maintain fixation of a point vestibulocochlear response (cold caloric): normal = nystagmus fast phase away from stimulated ear deep tendon reflexes plantar reflexes: normal = flexor plantar response • tone • spontaneous involuntary movements • assess for meningeal irritation, increased temperature • asses for head injury, Battle’s sign, raccoon eyes, skin rashes, and joint abnormalities that may suggest vasculitis
Coma Definition • an unrousable state in which patients show no meaningful response to environmental stimuli Pathophysiology • lesions affecting the cerebral cortex bilaterally, the reticular activating system (RAS) or their connecting fibres • focal supratentorial lesions do no alter consciousness except by herniation (compression on the brainstem or on the contralateral hemisphere) or by precipitating seizures
Caloric Reflexes COWS Cold Opposite Warm Same
NS36 Neurosurgery
Neurotrauma/Pediatric Neurosurgery
Toronto Notes 2011
Classification • structural lesions (tumour, pus, blood, infarction, CSF): 1/3 of comas supratentorial mass lesion – leads to herniation infratentorial lesion – compression of or direct damage to the RAS or its projections • metabolic disorders/diffuse hemispheric damage: 2/3 of comas deficiency of essential substrates (e.g. oxygen, glucose, vitamin B12) exogenous toxins (e.g. drugs, heavy metals, solvents) endogenous toxins/systemic metabolic diseases (e.g. uremia, hepatic encephalopathy, electrolyte imabalances, thyroid storm) infections (meningitis, encephalitis) trauma (concussion, diffuse shear axonal damage) Investigations and Management • ABCs • labs: electrolytes, TSH, LFTs, Cr, BUN, Ca, Mg, PO4, toxin screen, glucose • CT/MRI, LP, EEG
Persistent Vegetative State Definition • a condition of complete unawareness of the self and the environment accompanied by sleepwake cycles with either complete or partial preservation of hypothalamic and brainstem autonomic function • “awake but not aware” • follows comatose state Etiology/Prognosis • most commonly caused by cardiac arrest or head injury • due to irreversible loss of cerebral cortical function BUT intact brainstem function • average life expectancy is 2-5 years
Pediatric Neurosurgery Spinal Dysraphism SPINA BIFIDA OCCULTA Definition • congenital absence of a spinous process and a variable amount of lamina • no visible exposure of meninges or neural tissue Epidemiology • 15-20% of the general population; most common at L5 or S1
© Jen Polk 2002
Hair tuft
Figure 27. Spina Bifida Occulta
Etiology • failure of fusion of the posterior neural arch Clinical Features • no obvious clinical signs • presence of lumbosacral cutaneous abnormalities (dimple, sinus, port-wine stain, or hair tuft) should increase suspicion of an underlying anomaly (lipoma, dermoid, diastomatomyelia) Investigations • plain film – absence of the spinous process along with minor amounts of the neural arch • U/S or MRI to exclude spinal anomalies Treatment • requires no treatment MENINGOCELE (SPINA BIFIDA APERTA) Definition • herniation of meningeal tissue and CSF through a defect in the spine, without associated herniation of neural tissue
Toronto Notes 2011
Pediatric Neurosurgery
Neurosurgery NS37
Etiology • primary failure of neural tube closure Meninges
© Jen Polk 2002
Clinical Features • most common in lumbosacral area • usually no disability, low incidence of associated anomalies and hydrocephalus Investigations • plain films, CT, MRI, U/S, echo, genitourinary (GU) investigations Treatment • surgical excision and tissue repair (excellent results)
Figure 28. Meningocele
MYELOMENINGOCELE Definition • herniation of meningeal and CNS tissue through a defect in the spine
Clinical Features • sensory and motor changes distal to anatomic level producing varying degrees of weakness • urinary and fecal incontinence • 65-85% of patients with myelomeningocele have hydrocephalus • most have Type II Chiari malformation, see NS38 Investigations • plain films, CT, MRI, U/S, echo, GU investigations Treatment • surgical closure to preserve neurologic status and prevent CNS infections Prognosis • operative mortality close to 0%, 95% 2-year survival • 80% have IQ >80 (but most are 80-95), 40-85% ambulatory, 3-10% have normal urinary continence • early mortality usually due to Chiari malformation complications (respiratory arrest and aspiration), whereas late mortality is due to shunt malfunction
Intraventricular Hemorrhage (IVH) • see Pediatrics, P74
Hydrocephalus in Pediatrics Etiology • congenital aqueductal anomalies, primary aqueductal stenosis in infancy secondary gliosis due to intrauterine viral infections (mumps, varicella, TORCH) Dandy-Walker malformation (2-4%) Chiari malformation, especially Type II myelomeningocele • acquired post meningitis post hemorrhage (SAH, IVH) masses (vascular malformation, neoplastic) Clinical Features • symptoms and signs of hydrocephalus are age related in pediatrics • increased head circumference (HC), bulging anterior fontanelle, widened cranial sutures • irritability, lethargy, poor feeding and vomiting • “cracked pot” sound on cranial percussion • scalp vein dilation (increased collateral venous drainage) • sunset sign – forced downward deviation of eyes • episodic bradycardia and apnea
Meninges
Spinal cord Roots © Jen Polk 2002
Etiology • same as meningocele
Figure 29. Myelomeningocele
NS38 Neurosurgery
Pediatric Neurosurgery
Toronto Notes 2011
Investigations • skull x-ray, U/S, CT, MRI, ICP monitoring Treatment • similar to adults (see Hydrocephalus, NS7)
Dandy-Walker Malformation Definition • atresia of foramina of Magendie and Luschka, resulting in complete or incomplete agenesis of the cerebellar vermis with widely separated, hypoplastic cerebellar hemispheres posterior fossa cyst, enlarged posterior fossa dilatation of 4th ventricle (also 3rd and lateral ventricles) • associated anomalies hydrocephalus (90%) agenesis of corpus callosum (17%) occipital encephalocele (7%) Epidemiology • 2-4% of pediatric hydrocephalus Clinical Features • 20% are asymptomatic, seizures occur in 15% • symptoms and signs of hydrocephalus combined with a prominent occiput in infancy • ataxia, spasticity, poor fine motor control common in childhood Investigations • ultrasound, CT, MRI Treatment • asymptomatic patients require no treatment • associated hydrocephalus requires surgical treatment supratentorial lateral ventricular or cystoperitoneal shunt Progosis • 75-100% survival, 50% have normal IQ
Chiari Malformations Definition • malformations at the medullary-spinal junction Etiology • unclear, likely maldevelopment/dysgenesis during fetal life
Chiari II
Figure 30. Chiari Malformations
© Camillia Matuk
Chiari I
Categories • Type I (cerebellar ectopia) definition: cerebellar tonsils lie below the level of the foramen magnum epidemiology: average age at presentation 15 years clinical features: many are asymptomatic scoliosis brain compression central cord syndrome (65%) syringomyelia (50%) foramen magnum compression syndrome (22%) cerebellar syndrome (11%) hydrocephalus (10%) • Type II definition: part of cerebellar vermis, medulla and 4th ventricle extend through the foramen magnum often to midcervical region epidemiology: present in infancy clinical features: findings due to brainstem and lower cranial nerve dysfunction syringomyelia, hydrocephalus in >80%
Toronto Notes 2011
Pediatric Neurosurgery
Neurosurgery NS39
Investigations • MRI or CT myelography Treatment • indications for surgical decompression Type I: symptomatic patients (early surgery recommended; GPi) Psychiatric: depression, mania, anxiety, apathy (STN > GPi)
Dystonia
Contralateral primary (generalized) dystonias; cervical and tardive dystonias (GPi) Contralateral secondary dyskinesia (i.e. drug-induced: L-dopa, neuroleptics; STN)
Preferred target (primary dystonia): stereotactic ablation (pallidotomy/ stimulation of posteroventral GPi Secondary dystonia: stimulation of anterodorsal STN Stimulation of ventral posterior lateral thalamic nucleus (VPL)
Primary dystonia: 51% reduction in Burke-Fahn-Marsden Dystonia Scale (BFMDS) score Secondary dystonia: 62-89% improvement in dystonias Delayed effects: weeks months
Intracerebral hemorrhage, infection, seizure (1%–4%) Minor effects on cognitive functioning (esp. decreased lexical fluency; STN > GPi)
Tremor
Contralateral appendicular ET (first disorder to be treated by DBS; DBS is viable alternative to Rx) Intention (cerebellar) tremor (IT) resulting from demyelination of cerebellar outflow tracts (i.e. in multiple sclerosis) Brainstem tremor (Holmes tremor)
Preferred target: stereotactic ablation (thalamotomy)/stimulation of Vim nucleus of thalamus Other targets: stimulation of caudal zona incerta Parkinsonian tremor: stimulation of anterodorsal STN
Durable reductions in essential tremor rating scale (ETRS) scores Reduced dosage of medications Conflicting data on vocal/facial tremor
Intracerebral hemorrhage, infection, seizure (1%–4%) Paresthesias/pain Dysarthria Ataxia Minor effects on cognitive functioning (esp. decreased lexical fluency) Tolerance may develop over time
Neuropsychiatric Disorders • see Tourette’s Syndrome, Obsessive Compulsive Disorder and Depression sections in Neurology, N29 and Psychiatry, PS15, PS7 Table 15. Surgical Targets for Neuropsychiatric Disorders Disorder
Indications
Procedures
Obsessive Compulsive Disorder (OCD)
Severe symptoms refractory to medical management
Anterior capsulotomy/stimulation Currently under investigation of the anterior limb of the internal Reportedly 25-75% response rate capsule (IC)
Outcomes
Intracerebral hemorrhages (1%-2%) Mild effects on cognitive functioning Anxiety ± panic disorder (case report)
Tourette’s Syndrome
Severe symptoms refractory to medical management
Stimulation of midline intralaminar Currently under investigation nuclei of the thalamus Reportedly >70% reduction in vocal Stimulation of motor and limbic or motor tics + urge portions of GPi Stimulation of the anterior limb of the IC
Intracerebral hemorrhages (1%-2%) Mild sexual dysfunction
Major Depressive Disorder (MDD)
Severe depression refractory to medical management and ECT
Stimulation of the subgenual cingulate cortex
Intracerebral hemorrhages (1%-2%) Pain, headache Worsening mood, irritability
Currently under investigation Reportedly 60% response rate; 35% remission rate
Morbidity
Toronto Notes 2011
Functional Neurosurgery/Surgical Management of Epilepsy
Neurosurgery NS41
Chronic Pain Table 16. Surgical Targets for Chronic Pain Disorder
Indications
Procedures
Outcomes
Morbidity
Neuropathic Pain
Severe, intractable, organic neuropathic pain (i.e. post-stroke pain, phantom limb pain, trigeminal neuralgia, chronic low-back pain, complex regional pain syndrome)
Preferred target: stimulation of the contralateral ventral posterior lateral (VPL) and medial (VPM) thalamic nuclei ± periventricular/ periaqueductal grey matter (PVG/PAG) Other targets: stimulation of the contralateral IC Stimulation of the contralateral motor cortex
47% improvement in perception of pain intensity Less favourable results in central pain syndromes and poorly localized pain
Intracerebral hemorrhages (1%-2%) Paraesthesia Anxiety ± panic disorder
Nociceptive Pain
Severe, intractable, organic nociceptive pain
Bilateral (most common) stimulation of the PVG/PAG
Reportedly 63% improvement in perception of pain intensity
Intracerebral hemorrhages (1%-2%) Paraesthesia Anxiety ± panic disorder
Surgical Management of Epilepsy Neurosurgical Treatment of Epilepsy • see Neurology, N8 for the medical treatment of epilepsy Indications • medically refractory seizures, usually defined as seizures resistant to two first line anti-seizure medications used in succession • identification of a distinct epileptogenic region through clinical history, EEG, MRI, and neuropsychological testing. Other localizing investigations include magnetoencephalography, SPECT and PET • if a distinct epileptogenic region cannot be identified, the patient may be a candidate for a palliative procedure such as corpus callosotomy Procedure • most commonly adults: resection of the hippocampus and parahippocampal gyrus for mesial temporal lobe epilepsy arising from mesial temporal sclerosis children: resection of an epileptogenic space-occupying lesion • hemispherectomy and corpus callosotomy are less common Outcomes and Goals • freedom from seizures • 41-79% of adult patients are seizure free for 5 years after temporal lobe resection • 58-78% of children are seizure free after surgery • surgery is associated with improvements in preexisting psychiatric conditions such as depression and anxiety, a well as improvement in quality of life measures Morbidity • 0.4-4% of surgical patients will have partial hemianopsia, aphasia, motor deficit, sensory deficit, or cranial nerve palsy following anterior mesial temporal lobectomies • most patients will have some decline in verbal memory following dominant temporal lobectomy and in visuospatial memory in non-dominant temporal resection • the degree of memory decline stabilizes after 1-2 years Predictors • positive predictive factors for seizure freedom following anteromedial temporal lobe resection hippocampal sclerosis (unilateral) focal localization of interictal epileptiform discharges absence of preoperative generalized seizures tumoural cause complete resection of the lesion
A Randomized, Controlled Trial of Surgery for Temporal-lobe Epilepsy NEJM 2001; 345:311-8 This randomized control trial evaluates the efficacy and safety of neurosurgery for temporal lobe epilepsy. Methods: 80 patients with poorly controlled temporal-lobe epilepsy were randomized for surgery (n=40) or for continued treatment with antiepileptic drugs (n=40). The primary outcome was freedom from seizures that impair awareness of self and surroundings during the period of 1 year. Secondary outcomes included frequency and severity of seizures, quality of life, disability and death. Conclusions: In patients with poorly controlled temporal-lobe epilepsy, surgery is superior to prolonged medical therapy.
NS42 Neurosurgery
Surgical Management for Trigeminsl Neuralgia
Toronto Notes 2011
Surgical Management for Trigeminal Neuralgia Medical Therapy for Trigeminal Neuralgia • see Neurology, N18 for medical management
Surgical Therapy for Trigeminal Neuralgia • reserved for cases refractory to medical management Surgical Options • trigeminal nerve branch procedures local blocks (phenol, alcohol) neurectomy of the trigeminal branch • nerve branches V1 at the supraorbital, supratrochlear or infraorbital nerves V2 at the foramen rotundum V3 block at the foramen ovale • percutaneous trigeminal rhizotomy glycerol injection mechanotrauma via catheter balloon injection of sterile boiling water • radiofrequency thermocoagulation • microvascular decompression posterior fossa craniotomy with microsurgical exploration of the root entry zone, displacement of the vessel impinging on the nerve with placement of a non-absorbable Teflon felt
Toronto Notes 2011
Common Medications
Neurosurgery NS43
Common Medications The following are guidelines ONLY; follow clinical judgment and up-to-date prescription recommendations in practice; dosages refer to adults unless otherwise specified
Table 17. Common Medications Drug Name
Dosing Schedule
Indications
Side Effects
lorazepam (Ativan®)
4 mg IV over 2 minutes, q10-15 minutes (do not exceed 8 mg/12hr)
Status epilepticus
Drowsiness, sedation Other CNS depressants, digoxin (increases digoxin levels)
Common Interactions Contraindications
Comments
carbamazepine (Tegretol®)
Trigeminal neuralgia (tic douloureux): 100 mg PO bid, increase by 200 mg/day up to a maximum of 1,200 mg/day 200 mg tid Seizures: 200 mg PO bid, increase by 200 mg (inpatient: q3 days; outpatient: q7 days) until therapeutic level achieved (usual optimum dosage: 800-1,200 mg/day; range: 600-2,000 mg/day)
Trigeminal neuralgia Seizures
Worsening of seizures, heart failure, arrhythmias, AV block, aplastic anemia, agranulocytosis, thrombocytopenia, hepatitis, erythema multiforme, StevensJohnson syndrome
Lithium (increases lithium toxicity), MAOI Other meds may increase carbamazepine levels orhave decreased effects
Hypersensitivity to TCAs, previous bone marrow suppression, MAOI in past 14 days
Monitor CBC (potential hematological toxicity)
phenytoin (Dilantin®)
Seizures: Loading dose: Seizures 18 mg/kg slow IV or 300-600 mg Status epilepticus PO/day divided bid/tid Maintenance: 200-500 mg IV/day (max. rate: 37 weeks) P: number of premature infants delivered (20 to 37 weeks) A: number of abortions (loss of intrauterine pregnancy prior to viability of fetus L) due to progesterone-induced smooth muscle relaxation and uterine enlargement Increased CO and thus increased GFR leads to decreased creatinine (normal in pregnancy 35-44 mmol/L), uric acid, and BUN
Neurologic
Increased incidence of carpal tunnel syndrome and Bell’s palsy
Endocrine
Thyroid: moderate enlargement and increased basal metabolic rate Increased total thyroxine and thyroxine binding globulin (TBG) Free thyroxine index and TSH levels are normal Adrenal: maternal cortisol rises throughout pregnancy (total and free) Calcium: decreased total maternal Ca due to decreased albumin Free ionized Ca (i.e. active) proportion remains the same due to parathyroid hormone (PTH), results in: increased bone resorption and gut absorption, increased bone turnover (but no loss of bone density due to estrogen inhibition)
Cervix
Goodell’s sign, Chadwick’s sign, Hegar’s sign (see Physical Signs of Pregnancy, OB2)
Obstetrics OB3
Beta-hCG Rule of 10s 10 IU at time of missed menses 100,000 IU at 10 weeks (peak) 10,000 IU at term
OB4 Obstetrics
Prenatal Care
Toronto Notes 2011
Prenatal Care Family doctors and midwives to consider OB consultation if: • Insulin-dependent GDM • VBAC • HTN • Multiple gestation • Malpresentation • Active antepartum hemorrhage • PTL/PPROM • Failure to progress/descend • Induction/augmentation if high risk • Tears: 3rd or 4th degree • Retained placenta Note: Guidelines vary by institution.
Advise all women capable of becoming pregnant to supplement their diet with 0.4 mg/day of folic acid (CTFPHC Grade II-2-A Evidence).
• provided by obstetrician, family doctor, midwife, or multidisciplinary team (based on patient preference and risk factors) • Antenatal Records (province specific)
Preconception Counselling • 3-8 weeks gestational age (GA) is a critical period of organogenesis, so early preparation is vital • past medical history: optimize medical illnesses and necessary medications prior to pregnancy (see Medical Conditions in Pregnancy, OB11, and Drugs in Pregnancy, OB52) • supplementation folic acid: encourage diet rich in folic acid and supplement 8-12 wks preconception to prevent neural tube defects (NTDs) 0.4-1 mg daily in all women, 5 mg if previous NTD, anti-epileptic medications, diabetes mellitus or BMI >35 kg/m2) and continue for T1 of pregnancy iron supplementation, prenatal vitamins • risk modification lifestyle: balanced nutrition and physical fitness infection screening: rubella, HBsAg, VDRL, Pap smear, gonorrhea/chlamydia, HIV, toxoplasmosis, CMV, TB, varicella genetic testing as appropriate for high risk groups (see Prenatal Screening section, Table 4, OB7); consider genetics referral in known carriers, recurrent pregnancy loss/stillbirth, family members with developmental delay or birth anomalies social: alcohol, smoking, drug use, domestic violence use Antenatal Psychosocial Health Assessment (ALPHA) form to screen for antenatal risk factors associated with poor postpartum family outcomes (woman abuse, child abuse, postpartum depression, marital dysfunction and increased physical illness)
Initial Prenatal Visit • within 12 weeks of the first day of LMP or earlier if 35 years old • fill out Antenatal Records
Ask every woman about abuse – not just those whose situations raise suspicion of abuse AND ask as early as possible in pregnancy. Be careful not to congratulate women on pregnancy, as many are unplanned and may be unwanted.
History • gestational age by dates from the first day of the LMP • if LMP unreliable, get a dating ultrasound (see below) • estimated date of confinement (EDC) using Naegle’s Rule: 1st day of LMP + 7 days – 3 months e.g. LMP = 1 Apr 2011, EDC = 8 Jan 2012 (modify if cycle >28 days by adding number of days >28) • history of present pregnancy (e.g. bleeding, nausea, vomiting) • history of all previous pregnancies: GTPAL, year, sex, weight, gestational age, mode of delivery, length of labour, complications • past medical history, past gynecological history • prescription and non-prescription medications • family history: genetic disease, birth defects, multiple gestation • social history: smoking, alcohol, drug use, domestic violence (use ALPHA form) Physical Examination • complete exam to obtain baseline • BP and weight important for interpreting subsequent changes • pelvic exam
Tests for HIV, prenatal and genetic screening are voluntary and require proper counseling and informed consent before proceeding.
Investigations • bloodwork CBC, blood group and type, Rh antibodies, infection screening as per preconception counselling • urine R&M, C&S screen for bacteriuria and proteinuria • pelvic exam Pap smear (unless done within last 6-12 mo), cervical culture for N. gonorrhoeae (GC) and C. trachomatis, bacterial vaginosis (BV) vaginal swab
Toronto Notes 2011
Prenatal Care
Counselling • exercise under physician guidance absolute contraindications ruptured membranes, preterm labour, hypertensive disorders of pregnancy, incompetent cervix, IUGR, multiple gestation (>3), placenta previa after 28th week, persistent 2nd or 3rd trimester bleeding, uncontrolled type I diabetes, thyroid disease, or other serious cardiovascular, respiratory or systemic disorder relative contraindications previous spontaneous abortion, previous preterm birth, mild/moderate cardiovascular or respiratory disorder, anemia (Hb ≤100 g/L), malnutrition or eating disorder, twin pregnancy after 28th week, other significant medical conditions • nutrition Canada’s Food Guide to Healthy Eating suggests: 3-4 servings of milk products daily (greater if multiple gestation) a daily caloric increase of ~100 cal/d in the 1st trimester, ~300 cal/d in the second and third trimesters and ~450 cal/d during lactation daily multivitamin should be continued in the 2nd trimester for women who do not consume an adequate diet; otherwise routine vitamin supplementation is not necessary (avoid excess vitamin A) see Drug and Food Safety in Pregnancy, OB52 • nutrients important during pregnancy folate: 0.4-5 mg per day supports maternal increase in blood volume, growth of maternal and fetal tissue, decreases incidence of neural tube defects foods rich in folic acid include: spinach, lentils, chick peas, asparagus, broccoli, peas, Brussels sprouts, corn and oranges calcium: 1200-1500 mg per day maintains integrity of maternal bones, skeletal development of fetus, breast milk production vitamin D: 400 IU promotes calcium absorption iron: 0.8 mg/d in T1, 4-5 mg/d in T2 and >6 mg/d in T3 supports maternal increase in blood cell mass, supports fetal and placental tissue required amounts exceed normal body stores and typical intake, and therefore need supplemental iron iron is the only known nutrient for which requirements during pregnancy cannot be met by diet alone (see Iron Deficiency Anemia, OB11) essential fatty acids (EFA) – supports fetal neural and visual development contained in vegetable oils, margarines, peanuts, fatty fish • weight gain: optimal gain depends on pre-pregnancy weight (varies from 6.8-18.2 kg) • work: strenuous work, extended hours and shift work during pregnancy may be associated with greater risk of low birth weight, prematurity, and spontaneous abortion • travel: not harmful, but stress related to travel may be associated with preterm labour air travel is acceptable in second trimester but discouraged after 36 weeks • sexual intercourse: may continue except in patients at risk for abortion, preterm labour, or placenta previa; breast stimulation may induce uterine activity and is discouraged in high-risk patients near term • address social issues including physical or sexual abuse • smoking: assist/encourage to reduce or quit smoking • alcohol: encourage abstinence from alcohol during pregnancy • genetic screening must be offered to all women (see Prenatal Screening, OB7 and Chromosomal Screening, OB9)
Obstetrics OB5
Risk Factors for Neural Tube Defects GRIMM • Genetics: family history of NTD (risk of having second child with NTD is increased to 2-5%), consanguinity, chromosomal (characteristic of trisomy 13, 18, and 21) • Race: European Caucasians > than African Americans, 3-fold higher in Hispanics • Insufficient vitamins: zinc and folate • Maternal chronic disease (e.g. diabetes) • Maternal use of anti-epileptic drugs General population risk for NTD is 0.1%.
Expected Weight Gain BMI (kg/m2)
Weight (kg)
25
12.7-18.2 11.3-15.9 6.8-11.3
General Rule: 1-3.5 kg/week during T1, then 0.45 kg/week until delivery
Subsequent Prenatal Visits Timing • for uncomplicated pregnancies, q4-6 weeks until 28 weeks, q2 weeks from 28 to 36 weeks and weekly from 36 weeks until delivery Assess at Every Visit • record estimated GA • history of present pregnancy: fetal movements, uterine bleeding, leaking, cramping • physical exam: BP, weight gain, symphysis fundal height (SFH), Leopold’s maneuvers (T3) for lie, position and presentation of fetus • investigations: urinalysis for glucosuria, ketones, proteinuria; fetal heart tones starting at 12 weeks using Doppler U/S
Symphysis Fundal Height (SFH) 12 weeks: Uterine fundus at pubic symphysis 20 weeks: Fundus at umbilicus SFH should be within 2 cm of GA between 20 and 36 weeks 37 weeks: Fundus at sternum
OB6 Obstetrics
Small for dates: Date miscalculation IUGR Fetal Demise Oligohydramnios
Prenatal Care
Toronto Notes 2011
Leopold’s Maneuvers • performed after 30-32 weeks gestation • first maneuver: to determine which fetal part is lying furthest away from the pelvic inlet • second maneuver: to determine the location of the fetal back • third maneuver (Pawlick’s Grip): to determine which fetal part is lying above the pelvic inlet • fourth maneuver: to locate the fetal brow
A. First
B. Second
C. Third
D. Fourth
© Emilie McMahon 2004
Large for dates: Date miscalculation Multiple gestation Polyhydramnios
Figure 2. Leopold’s Maneuvers (T3)
Reprinted with permission from Essentials of Clinical Examination Handbook, 6th ed. Lincoln, McSheffrey, Tran, Wong.
Prenatal Fetal Monitoring DDx of Decreased Fetal Movements DASH Death of fetus Amniotic fluid decreased Sleep cycle of fetus Hunger/Thirst
Fetal Movements • generally first noticed (“quickening”) at 18-20 wks in primigravidas; can occur 1-2 wks earlier in multigravidas • if mother is concerned about decreased movement: mother chooses a time when fetus is normally active to count movements (usually recommended after 28 wks) if 15 bpm from baseline, lasting >15 s in 20 min
Definition • fetal heart rate (FHR) tracing ≥20 minutes using an external Doppler to assess FHR and its relationship to fetal movement (see Fetal Monitoring in Labour section, OB33) Indication • any suggestion of uteroplacental insufficiency or suspected fetal distress Table 2. Classification of Antepartum Non-Stress Test Parameter
Normal NST (Previously “Reactive”)
Atypical NST (Previously “Non-Reactive”)
Abnormal NST (Previously “Non-Reactive”)
Baseline
110-160 bpm
100-110 bpm or >160 bpm for 30 min Erratic baseline
Variability
6-25 bpm (moderate) 5 (absent or minimal) for 40-80 min ≤5 for 80 min ≤5 (absent or minimal) for 10 min Sinusoidal
Decelerations
None or occasional variable 60 sec Late deceleration(s)
Accelerations in Term Fetus
2 accelerations with acme of ≥15 bpm, lasting 15 sec over 10 bpm, lasting 10 sec in 80 min
Action
FURTHER ASSESSMENT OPTIONAL, based on total clinical picture
FURTHER ASSESSMENT REQUIRED
URGENT ACTION REQUIRED An overall assessment of the situation and further investigation with U/S or BPP is required. Some situations will require delivery
Adapted from SOGC, Fetal Health Surveillance: Antepartum and Intrapartum Consensus Guideline, September 2007.
Toronto Notes 2011
Prenatal Care
Obstetrics OB7
Operating Characteristics • false positive rate depends on duration; false negative rate = 0.2-0.3% Interpretation • normal (reassuring NST): at least 2 accelerations of FHR >15 bpm from the baseline lasting >15 seconds, in 20 minutes • abnormal (non-reassuring NST): 1 week discrepancy from EDC based on LMP • nuchal translucency ultrasound (NTUS) at 11-14 weeks GA measures the amount of fluid behind the neck of the fetus early screen for serious congenital anomalies (Down syndrome) NT measurement is necessary for the FTS and IPS Part 1 • fetal growth and anatomy ultrasound routinely done at 18-20 weeks GA (margin of error ± 7 days) (see Pediatrics, P43 for congenital anomalies) • earlier or subsequent ultrasounds performed when medically indicated Table 6. Comparison of FTS, MSS and IPS First Trimester Screen (FTS)
Maternal Serum Screen (MSS)
Integrated Prenatal Screen (IPS)
11-14 wks
15-18 wks
Nuchal translucency on 12 wk U/S FTS at 11-14 wks MSS + inhibin A at 15-18 wks
Measures 1. Nuchal translucency on U/S 2. Beta-hCG 3. Pregnancy-associated plasma protein A (PAPP-A)
Measures 1. Maternal serum alpha-fetoprotein (MSAFP) 2. Beta-hCG 3. Unconjugated estrogen (estriol or uE3)
Risk estimate for 1. Down syndrome (Trisomy 21): increased NT, increased beta-hCG, decreased PAPP-A
Risk estimate for 1. Open neural tube defect (oNTD): increased MSAFP (sensitivity 80-90%) 2. Trisomy 21: decreased MSAFP, increased beta-hCG, decreased uE3 Note: does not measure risk of oNTD and (sensitivity 65%) should be combined with MSAFP at 3. Trisomy 18: decreased MSAFP, 16 weeks decreased beta-hCG, decreased uE3 Useful where patient wants results within (sensitivity 80%) the first trimester Only offered alone if patient missed the More accurate estimate of Down time window for IPS or FTS syndrome risk than MSS, sensitivity 8% baseline false positive rate for t21, ~85% (when combined with age) lower for oNTD and t18 5% false positive rate Patients with positive screen should be Patients with positive screen should be offered U/S or amniocentesis offered CVS or amniocentesis
Risk estimate for oNTD, Trisomy 21, Trisomy 18 Sensitivity ~85-90% 2% false positive rate Patients with positive screen should be offered U/S and/or amniocentesis
Note: In twins, FTS, MSS and IPS are not applicable; screen with NT for chromosomal abnormalities and MSAFP for oNTDs.
Toronto Notes 2011
Prenatal Care
Obstetrics OB9
CHROMOSOMAL SCREENING Indications • maternal age >35 (increased risk of chromosomal anomalies) • risk factors in current pregnancy: teratogen exposure abnormal U/S abnormal prenatal screen (FTS, MSS or IPS) • past history/family history of: previous pregnancy with chromosomal anomaly or genetic disease either parent a known carrier of a genetic disorder or balanced translocation family history of chromosomal anomaly, genetic disorder, birth defect, or undiagnosed mental retardation consanguinity three or more spontaneous abortions AMNIOCENTESIS • U/S-guided transabdominal extraction of amniotic fluid Indications • identification of genetic anomalies (15-16 weeks gestation) as per indications above • assessment of fetal lung maturity (T3) via the L/S ratio (lecithin:sphingomyelin) if >2:1, respiratory distress syndrome (RDS) is less likely to occur • assessment of amniotic fluid bilirubin concentration in Rh-isoimmunized pregnancies Advantages • also screens for oNTD (acetylcholinesterase and amniotic AFP) – 96% accurate • in women >35 years, the risk of chromosomal anomaly (1/180) is greater than the risk of miscarriage from the procedure • more accurate genetic testing than CVS
DDx of Increased MSAFP • Incorrect GA • >1 fetus (e.g. twins) • Fetal demise • oNTD • Abdominal wall defects (e.g. omphalocele)
DDx of Decreased MSAFP • Incorrect GA • Gestational trophoblastic neoplasia • Missed abortion • Chromosomal anomalies • Maternal diabetes
L/S Ratio (Lecithin:Sphingomyelin Ratio) Lecithin levels increase rapidly after 35 weeks gestation, whereas sphingomyelin levels remain relatively constant. The L/S ratio is a measure of fetal lung maturity – less than 2:1 indicates pulmonary immaturity. Presence of blood or meconium in the amniotic fluid can affect the ratio.
Disadvantages • 0.5% risk of spontaneous abortion and risk of fetal limb injury • results take 14-28 days CHORIONIC VILLUS SAMPLING (CVS) • biopsy of fetal-derived chorion using a trans-abdominal needle or trans-cervical catheter at 10-12 weeks Advantages • enables pregnancy to be terminated earlier than with amniocentesis • rapid karyotyping and biochemical assay within 48 hours, including FISH analysis • high sensitivity and specificity Disadvantages • 1-2% risk of spontaneous abortion and risk of fetal limb injury • does not screen for open neural tube defects • 1-2% incidence of genetic mosaicism g false negative results ISOIMMUNIZATION SCREENING Definition • isoimmunization: antibodies (Ab) produced against a specific RBC antigen (Ag) as a result of antigenic stimulation with RBC of another individual Etiology • maternal-fetal circulation normally separated by placental barrier, but sensitization can occur and can affect the current pregnancy, or more commonly, future pregnancies • in pregnancy, anti-Rh Ab produced by a sensitized Rh-negative mother can lead to fetal hemolytic anemia • overall risk of isoimmunization of an Rh-negative mother with an Rh-positive ABO-compatible infant is 16% (2% antepartum, 7% within 6 months of delivery, and 7% in the second pregnancy) • sensitization routes incompatible blood transfusions previous fetal-maternal transplacental hemorrhage (e.g. ectopic pregnancy) invasive procedures in pregnancy (e.g. prenatal diagnosis, cerclage, D&C) any type of abortion labour and delivery
Compared to CVS, amniocentesis has a higher accuracy of prenatal cytogenetic diagnosis (99.8% vs. 97.5%) and lower risk of spontaneous abortion (0.5% vs. 1-2%).
OB10 Obstetrics
Rh Antibody Titre A positive titre (≥1:16) indicates an increased risk of fetal hemolytic anemia.
Prenatal Care
Toronto Notes 2011
Investigations • routine screening at first visit for blood group, Rh status, and antibodies are measured by the indirect Coombs test • if Rh positive with antibodies present, the severity of fetal anemia is determined primarily by antibody concentration Ab titres 4,000 g (8.8 lbs) elective C/S for predicted birthweight >4,500 g (9.9 lbs) (controversial) • monitoring during labour monitor blood glucose q1h with patient on insulin and dextrose drip aim for blood glucose between 3.5 to 6.5 mmol/L to reduce the risk of neonatal hypoglycemia Postpartum • insulin requirements dramatically drop with expulsion of placenta (source of insulin antagonists) • no insulin is required for 48-72 hours postpartum in most Type 1 DM • monitor glucose q6h, restart insulin at two-thirds of pre-pregnancy dosage when glucose >8 mmol/L
Toronto Notes 2011
Medical Conditions in Pregnancy
Obstetrics OB13
B. GESTATIONAL DIABETES MELLITUS Screening + Diagnosis • at 24-28 weeks GA • pregnant females age >25 or age 1 risk factor (see sidebar) • 1-hour, 50 g Oral Glucose Challenge Test (OGCT): not fasting PG 25 Obesity Ethnicity (Aboriginal, Hispanic, Asian, African) FHx of DM Previous history of GDM Previous child with birthweight >4.0 kg
Management • treat both GDM and IGT • tight glycemic control optimal as in Type 1 and Type 2 DM • monitoring and timing of delivery as for Type 1 and Type 2 DM • stop insulin and diabetic diet postpartum • follow-up with 2-hour, 75 g OGTT 6 weeks-6 months postpartum Prognosis • most maternal and fetal complications are related to hyperglycemia and its effects Long Term Maternal Complications • Type 1 and Type 2 DM: risk of progressive retinopathy and nephropathy • GDM: 50% risk of developing Type 2 DM in next 20 years Table 8. Complications of DM in Pregnancy Maternal
Fetal
Obstetric • Hypertension/preeclampsia (especially if pre-existing nephropathy/proteinuria): insulin resistance is implicated in etiology of hypertension • Polyhydramnios: maternal hyperglycemia leads to fetal hyperglycemia, which leads to fetal polyuria (a major source of amniotic fluid)
Growth Abnormalities • Macrosomia: maternal hyperglycemia leads to fetal hyperinsulinism resulting in accelerated anabolism • Intrauterine growth restriction (IUGR): due to placental vascular insufficiency
Diabetic Emergencies • Hypoglycemia • Ketoacidosis • Diabetic coma
Delayed Organ Maturity • Fetal lung immaturity: hyperglycemia interferes with surfactant synthesis (respiratory distress syndrome)
End-organ involvement or deterioration (occur in DM1 and DM2, not in GDM) • Retinopathy • Nephropathy
Congenital Anomalies (occur in DM1 and DM2, not in GDM) • 2-7x increased risk of cardiac (VSD), NTD, GU (cystic kidneys), GI (anal atresia), and MSK (sacral agenesis) anomalies due to hyperglycemia Note: Pregnancies complicated by GDM do not manifest an increased risk of congenital anomalies because GDM develops after the critical period of organogenesis (in T1)
Other • Pyelonephritis/UTI: glucosuria provides a culture medium for E. coli and other bacteria • Increased incidence of spontaneous abortion (in DM1 and DM2, not in GDM): related to pre-conception glycemic control
Labour and Delivery • Preterm labour/prematurity: most commonly in patients with hypertension/preeclampsia. Preterm labour is associated with poor glycemic control but the exact mechanism is unknown • Increased incidence of stillbirth • Birth trauma: due to macrosomia, can lead to difficult vaginal delivery and shoulder dystocia Neonatal • Hypoglycemia: due to pancreatic hyperplasia and excess insulin secretion in the neonate • Hyperbilirubinemia and jaundice: due to prematurity and polycythemia • Hypocalcemia: exact pathophysiology not understood, may be related to functional hypoparathyroidism • Polycythemia: hyperglycemia stimulates fetal erythropoietin production
OB14 Obstetrics
Medical Conditions in Pregnancy
Toronto Notes 2011
Hypertension in Pregnancy Hypertension in Pregnancy Adverse Maternal Conditions • DBP >100 mmHg • HELLP • Cerebral haemorrhage • Renal dysfunction – oliguria 5% of pre-pregnancy weight
Jaundice in Pregnancy Epidemiology • affects 1 in 1500 pregnancies
HELLP Syndrome Hemolysis Elevated Liver enzymes Low Platelets
Etiology • viral hepatitis (most common) • unique to pregnancy (see Table 9) cholestatic jaundice of pregnancy HELLP syndrome hepatic rupture, hematoma and infarct acute fatty liver of pregnancy (AFLP) hyperemesis gravidarum (rarely causes hepatic dysfunction) • pre-existing conditions, see Gastroenterology, Liver/Biliary Tract, G31, G44
Toronto Notes 2011
Medical Conditions in Pregnancy
Obstetrics OB17
Table 9. Conditions Causing Jaundice in Pregnancy
Definition
HELLP Syndrome
Cholestatic Jaundice of Pregnancy
Hepatic Infact, Hematoma, and Rupture
Acute Fatty Liver of Pregnancy (AFLP)
Hemolysis, Elevated Liver enzymes, Low Platelets
Clinical syndrome characterized by intense pruritus that precedes jaundice by 7-14 days Pathogenesis unknown, may be due to increased sensitivity to high levels of estrogen or abnormal progestational steroids
Rare consequence of preeclampsia, typically occuring in T3 Vasospasm-induced hepatic infarction can lead to hematoma formation; hematoma can lead to rupture
Form of hepatic failure with coagulopathy and encephalopathy characterized by microvesicular fatty infiltrates in liver parenchyma Pathogenesis unknown
Pathogenesis unknown
Epidemiology
Affects 20% of women with severe preeclampsia Presents >27 weeks GA (11% sooner); up to 30% of cases present AFTER delivery and with no prevoius signs of hypertension
17-29 weeks GA High incidence in Chile and Scandinavia; rare in Asian and African populations
1 in 7000 deliveries 3rd trimester (28-40 weeks GA) Maternal mortality as high as 75%; resolution of hepatic dysfunction with delivery or termination of pregnancy
Clinical Features
Epigastric, RUQ or chest pain, N/V, symptoms of preeclampsia (headache, blurred vision, thirst) ± jaundice Atypical presentations: asymptomatic reduction in platelet count, “flu-like” symptoms
Intense pruritis (usually, worst on palms and soles of feet) ± icterus (1-2 weeks later) Steatorrhea unusual
Hepatic rupture: RUQ abdominal pain, abdominal distention, nausea/vomiting, and hypertension, followed by shock
Acute nausea/vomiting, severe upper abdominal pain preceding jaundice Confusion Preeclampsia Pruritus Range in presentation: • Mild • Fulminant: GI bleeding, hepatic coma, renal failure and true hepatic failure (coagulopathy and encephalopathy)
Investigations
AST (70-663 U/L), total bilirubin slightly increased, low platelet count (7-99), elevated LDH ± elevated D-dimers, tissue polypeptide antigen (TPA) and fibronectin, fragmented RBCs on smear Liver biopsy (rarely done): periportal hemorrhage and fibrin deposition with periportal necrosis; macro- and microvesicular fatty deposits (NOT pericentral as in AFLP)
ALT ALT Hypoglycemia Preeclampsia and HELLP features Liver biopsy to establish diagnosis: • Microvesicular fatty infiltrates of the central zone hepatocytes • Oil Red O stain on frozen tissue • Electron microscopy on glutaraldehyde fixed tissue If liver biopsy not possible, CT most useful
Management
Supportive care (in ICU) and prompt delivery
Ursodeoxycholic acid Aggressive: rapid delivery and (20-25 mg/kg/day) trauma surgery to repair liver Pruritus: cholestyramine Prophylactic vitamin K before delivery Consider induction of labour (see Induction of Labour, OB35)
Early diagnosis with prompt delivery followed by maximal supportive care • ABCs, mechanical ventilation, transfusion of blood products • Hepatic encephalopathy treatment – lactulose, catharsis • Treat hypoglycemia
Notes
Complications: sepsis, multi-system organ failure, hepatic failure, DIC, death (rare)
Selenium may be protective against cholestasis Strong familial predisposition Correlates with oral contraceptive sensitivity
Recovery begins with delivery Persistent or increasing hyperbilirubinemia and complications: • Should not be interpreted as indications for liver transplantation • Aggressive supportive measures
Complications include death (mother and fetus) if untreated
OB18 Obstetrics
Medical Conditions in Pregnancy
Toronto Notes 2011
Urinary Tract Infection (UTI) Treat asymptomatic bacteriuria in pregnancy because of increased risk of progression to cystitis, pyelonephritis and probable increased risk of PRETERM LABOUR.
Etiology • increased urinary stasis from mechanical and hormonal (progesterone) factors • organisms include GBS as well as those that occur in non-pregnant women Epidemiology • most common medical complication of pregnancy • asymptomatic bacteriuria in 2-7% of pregnant women depending on parity and socioeconomic factors • note: asymptomatic bacteriuria should be treated in pregnancy Clinical Features • may be asymptomatic • dysuria, urgency, and frequency in cystitis • fever, flank pain, costovertebral angle tenderness in pyelonephritis Investigations • urinalysis, urine C&S • VCUG, cystoscopy, and renal function tests in recurrent infections Management • uncomplicated UTI first line: amoxicillin (250-500 mg PO q8h x 7 days) alternatives: TMP-SMX (Septra®) or nitrofurantoin (avoid sulpha drugs during last 6 weeks of pregnancy due to displacement of bilirubin from albumin and increased kernicterus in the newborn) follow with monthly urine cultures • pyelonephritis hospitalization and IV antibiotics Prognosis • complications if untreated: acute cystitis, pyelonephritis, and possible preterm premature rupture of membranes (PPROM) • recurrence is common
T1
T1-T3
To mom: respiratory, infected blood products To baby: transplacental
To mom: blood, saliva, semen, vaginal secretions To baby: transplacental, breast milk
To mom: intimate mucocutaneous contact, To baby: transplacental, during delivery
To mom: blood, semen, vaginal secretions To baby: in utero, during delivery, breast milk
To mom: raw meat, unpasteurized T3 (but most severe if infected goat’s milk, cat feces/urine in T1); only concern if primary To baby: transplacental infection during pregnancy
DNA virus
DNA virus
RNA retrovirus
ssRNA togavirus To mom: respiratory droplets (highly contagious) To baby: transplacental
To mom: sexual contact To baby: transplacental
Parvovirus B19
Spirochete (Treponema pallidum)
Protozoa (Toxoplasma gondii)
Erythema Infectiosum (Fifth Disease)
Hepatitis B
*Herpes Simplex Virus
HIV
*Rubella
Syphilis
*Toxoplasmosis
Spontaneous abortion (SA), stillbirth, hydrops in utero
1/3 in utero, 1/3 at delivery, 1/3 breastfeeding
Delivery (if genital lesions present); less commonly in utero
Fever, N/V, fatigue, jaundice, elevated liver enzymes
Flu-like, rash, arthritis; often asymptomatic
Clinical diagnosis
Serologic screening for all pregnancies
Serology, viral PCR, maternal AFP; if IgM present, follow fetus with U/S for hydrops
Congenital toxoplasmosis (chorioretinitis, hydrocephaly, intracranial calcification, MR, microcephaly) NB: 75% initially asymptomatic at birth
Risk of PTL, multisystem involvement, fetal death
Serologic testing; all pregnant women screened (immune if titre >1:16); infection if IgM present or >4x increase in IgG
Majority subclinical; may have IgM and IgG serology; flu-like symptoms PCR of amniotic fluid
See Infectious Diseases, ID26 VDRL screening for all pregnancies; if positive, requires confirmatory testing
SA or congenital rubella Rash (50%), fever, posterior auricular or occipital syndrome (hearing loss, cataracts, CV lesions, MR, IUGR, lymphadenopathy, arthralgia hepatitis, CNS defects, osseous changes)
IUGR, preterm labour, premature See Infectious Diseases, ID29 Serology, viral PCR rupture of membranes All pregnant women are offered screening
Disseminated herpes (20%); Painful vesicular lesions CNS sequelae (35%); self-limited infection
T3 Prematurity, low birth weight, neonatal death 10% vertical transmission if asymptomatic HBsAg +ve; 85-90% if HBsAg and HBeAg +ve
10-20 weeks GA
Self-limiting in mother; spiramycin decreases fetal morbidity but not rate of transmission
Pen G 2.4 M U IM 1 dose if early syphilis 3 doses if late syphilis monitor VDRL monthly If Pen G allergic: consider desensitization before treatment
No specific treatment; offer vaccine following pregnancy Do not administer during pregnancy (live attenuated)
Triple antiretroviral therapy decreases transmission to 500 RNA copies/ml, unknown prenatal care, patient request
Acyclovir for symptomatic women, suppressive therapy at 36 wks controversial C/S if active genital lesions, even if remote from vulva
Rx neonate with HBIG and vaccine (at birth, 1, 6 mos); 90% effective
If hydrops occurs, consider fetal transfusion
No specific treatment; Serologic screen; isolate virus from urine or secretion maintain good hygiene and avoid high risk situations culture
VZIG for mother if exposed, decreases congenital varicella syndrome Note: Do not administer vaccine during pregnancy (live attenuated)
Management
Asymptomatic or flu-like 5-10% develop CNS involvement (mental retardation, cerebral calcification, hydrocephalus, microcephaly, deafness, chorioretinitis)
Diagnosis Clinical, ± vesicle fluid culture, ± serology
Effects on Mother
Congenital varicella syndrome Fever, malaise, (limb aplasia, chorioretinitis, vesicular pruritic lesions cataracts, cutaneous scars, cortical atrophy, IUGR, hydrops), preterm labour (prematurity)
Effects on Fetus
Medical Conditions in Pregnancy
* Indicates TORCH infection
To mom: blood/organ transfusion, T1-T3 sexual contact To baby: transplacental, during delivery, breast milk
DNA virus (herpes family)
*CMV
13-30 weeks GA, and 5d pre- to 2d post-delivery
To mom: direct, respiratory, To baby: transplacental
Varicella zoster virus (herpes family)
Chicken Pox
Greatest Transmission Risk to Fetus
Source of Transmission
Agent
Infection
Table 10. Infections During Pregnancy
Toronto Notes 2011 Obstetrics OB19
Infections During Pregnancy
OB20 Obstetrics
Medical Conditions in Pregnancy/Bleeding in Pregnancy
Toronto Notes 2011
Venous Thromboembolism (VTE) Epidemiology • incidence 0.5-3/1,000 pregnancies occurring with approximately equal frequency in all three trimesters and postpartum Risk Factors • previous VTE, age >35, obesity, infection, bedrest/immobility, shock/dehydration, thrombophilias (congenital and acquired, see Hematology) Table 11. Risk Factors for VTE Specific to Pregnancy
Virchow’s Triad for VTE 1. Hypercoagulable state 2. Stasis 3. Endothelial damage
Hypercoagulability
Stasis
Endothelial
Increased factors: II, V, VII, VIII, IX, X, XII, fibrinogen Increased platelet aggregation Decreased protein S, tPA, factors XI, XIII Increased resistance to activated protein C Antithrombin can be normal or reduced
Increased venous distensibility Decreased venous tone 50% decrease in venous flow in lower extremity by T3 Uterus is mechanical impediment to venous return
Vascular damage at delivery (C/S or SVD) Uterine instrumentation Peripartum pelvic surgery
Clinical Features • most DVTs occur in the iliofemoral or calf veins with a predilection for the left leg • signs of a pulmonary embolism are non-specific (as in non-pregnant women) • unexplained spontaneous fetal loss Investigations • duplex venous Doppler sonography for DVT • CXR and V/Q scan for PE • due to hypercoagulability, the normal scale for D-dimer levels must be adjusted (controversial)
Bleeding in Pregnancy Definitions • First Trimester Bleeding: vaginal bleeding within the first 12 weeks • Second Trimester Bleeding: 6000 mIU/mL or 6 weeks gestational age (transabdominal) specific finding on transvaginal U/S is a tubal ring • culdocentesis (rarely done) • laparoscopy (for definitive diagnosis) Treatment • goals of treatment: conservative (preserve tube if possible) • surgical (laparoscopy) linear salpingostomy if tube salvageable salpingectomy if tube damaged or ectopic is ipsilateral recurrence 15% risk of persistent trophoblast; must monitor beta-hCG titres weekly until they reach non-detectable levels if patient is Rh negative give anti-D gamma globulin (Rhogam®) may require laparotomy • medical = methotrexate use 50 mg/m2 body surface area; given in a single IM dose this is 1/5 to 1/6 chemotherapy dose, therefore minimal side effects (reversible hepatic dysfunction, diarrhea, gastritis, dermatitis) follow beta-hCG levels weekly until beta-hCG is non-detectable plateau or rising levels suggest persisting trophoblastic tissue (requires further treatment) 67% success rate; up to 25% will require a 2nd dose tubal patency following methotrexate treatment approaches 80% Prognosis • 9% of maternal deaths during pregnancy • 40-60% of patients will become pregnant again after surgery • 10-20% will have subsequent ectopic gestation Suspected Ectopic Pregnancy
Interventions for Tubal Ectopic Pregnancy Cochrane Database of Systematic Reviews 2007, Issue 1 Study: Cochrane Review of randomized controlled trials comparing treatments in women with tubal ectopic pregnancy. Patients: Women with a diagnosis of tubal ectopic pregnancy. Intervention: Surgery-salpingectomy/ salpingostomy by open surgery or by laparoscopy, medical treatment, and expectant management. Main outcome: Primary treatment success, defined as an uneventful decline in serum beta-hCG to undetectable levels by the initial treatment. Results: Intramuscular MTX therapy and salpingostomy yielded similar treatment success rates (82-95% for MTX therapy vs. 80-92% for salpingostomy).
1. Positive urine beta-hCG 2. Abdominal pain 3. Vaginal bleeding
Intrauterine pregnancy
Vital signs stable
Vital signs unstable
Transvaginal U/S Serum beta-hCG
Surgery
5000 OR hepatic/renal/hematological disease OR poor compliance OR unable to follow-up
Methotrexate
Consider surgical management if using methotrexate, follow-up is more frequent
Figure 3. Algorithm for Suspected Ectopic Pregnancy
Toronto Notes 2011
Antepartum Hemorrhage
Obstetrics OB23
Antepartum Hemorrhage Definition • vaginal bleeding from 20 weeks to term Differential Diagnosis • bloody show (shedding of cervical mucous plug) – most common etiology in T3 • placenta previa • abruptio placentae – most common pathological etiology in T3 • vasa previa • marginal sinus bleeding • cervical lesion (cervicitis, polyp, ectropion, cervical cancer) • uterine rupture • other: bleeding from bowel or bladder, placenta accreta, abnormal coagulation
Placenta Previa Definition • abnormal location of the placenta near, partially or completely over the cervical os Etiology • idiopathic Epidemiology • incidence = 0.5-0.8% of all pregnancies Risk Factors • history of placenta previa (4-8% recurrence risk) • multiparity • increased maternal age • multiple gestation • uterine tumour (e.g. fibroids) or other uterine anomalies • uterine scar due to previous abortion, C/S, D&C, myomectomy Clinical Features • classification total: placenta completely covers the internal os partial: placenta partially covers the internal os marginal: within 2 cm of os but does not cover any part of os – causes potential risk of hemorrhage during cervical effacement and dilatation low lying (NOT a previa): placenta in lower segment but clear of os (can also bleed, but usually in labour) • history PAINLESS bright red vaginal bleeding (recurrent), may be minimized and cease spontaneously, but can become catastrophic mean onset of bleeding is 30 wks GA, but onset depends on degree of previa (total placenta previa bleed earlier, marginal bleed at onset of labour) • physical exam uterus soft and non-tender presenting part high or displaced • complications fetal perinatal mortality low but still higher than with a normal pregnancy prematurity (bleeding often dictates early C/S) intrauterine hypoxia (acute or IUGR) fetal malpresentation PPROM risk of fetal blood loss from placenta, especially if incised during C/S maternal 1 pack/day), excessive alcohol consumption, cocaine • multiparity and/or maternal age >35 (felt to reflect parity) • PPROM • rapid decompression of a distended uterus (polyhydramnios, multiple gestation) • uterine anomaly, fibroids • trauma (e.g. motor vehicle collision, maternal battery) Clinical Features • classification total (fetal death inevitable) vs. partial external/revealed/apparent: blood dissects downward toward cervix internal/concealed (20%): blood dissects upward toward fetus most are mixed • presentation PAINFUL vaginal bleeding, uterine tenderness, uterine contractions pain: sudden onset, constant, localized to lower back and uterus ± fetal distress, fetal demise (15% present with demise), bloody amniotic fluid
Abruptio placentae is the most common cause of DIC in pregnancy.
Complications • fetal complications: perinatal mortality 25-60%, prematurity, intrauterine hypoxia • maternal complications: 4000 g Etiology/Risk Factors • maternal obesity, gestational diabetes mellitus, past history of macrosomic infant, prolonged gestation, multiparity Clinical Features • increased risk of perinatal mortality • cephalopelvic disproportion (CPD) and birth injuries (shoulder dystocia, fetal bone fracture) more common • complications of DM in labour (see Medical Conditions in Pregnancy, OB11) Investigations • serial SFH • further investigations if mother at high risk or SFH >2 cm ahead of GA • U/S predictors polyhydramnios third trimester abdominal circumference (AC) >1.5 cm/week head circumference (HC)/AC ratio 4500 g in diabetic women no evidence that prophylactic C/S improves outcomes • early induction of labour is not recommended for non-diabetic mothers • risks and benefits of early induction (risk of C/S vs. risk of dystocia) must be weighed in diabetic mothers, as current research is unclear
Toronto Notes 2011
Growth Discrepancies
Polyhydramnios/Oligohydramnios Table 16. Polyhydramnios and Oligohydramnios Polyhydramnios
Oligohydramnios
Definition
Amniotic fluid volume (AFV) >2,000 cc at any stage in pregnancy U/S criteria: >8 x 8 cm (3.1 x 3.1 in) pocket of amniotic fluid
Amniotic fluid index of 5 cm (2 in) or less • Important sign of chronic placental insufficiency
Etiology
Idiopathic most common (40%) Maternal: • Type 1 DM: abnormalities of transchorionic flow Maternal-fetal: • Chorioangiomas • Multiple gestation • Fetal hydrops (increased erythroblastosis) Fetal: • Chromosomal anomaly (up to 2/3 of fetuses have severe polyhydramnios) • Respiratory: cystic adenomatoid malformed lung • CNS: anencephaly, hydrocephalus, meningocele • GI: tracheoesophageal fistula, duodenal atresia, facial clefts (interfere with swallowing)
Early onset oligohydramnios: • Decreased production: renal agenesis or dysplasia, urinary obstruction, posterior urethral valves (male), chronic hypoxemia and IUGR resulting in shunting away from the kidneys to ensure perfusion of the brain • Increased loss: prolonged amniotic fluid leak (although most often labour ensues) Late onset oligohydramnios: • Amniotic fluid normally decreases after 35 weeks • Common in post-term pregnancies • U/S Doppler studies (umbilical cord and uterine artery)
Epidemiology
Occur in 0.2 to 1.6% of all pregnancies
Occur in ~4.5% of all pregnancies Severe form in 41 weeks (~12%)
Clinical Features and Complications
Pressure symptoms from overdistended uterus (dyspnea, edema, hydronephrosis) Uterus large for dates, difficulty palpating fetal parts and hearing fetal heart tones Cord prolapse, placental abruption, malpresentation, preterm labour, uterine dysfunction and PPH
Cord compression Increased risk of adverse fetal outcomes Early onset: • 15-25% have fetal anomalies • Amniotic fluid bands (T1) can lead to Potter’s facies, limb deformities, abdominal wall defects Late onset: • Pulmonary hypoplasia • Marker for infants who may not tolerate labour well
Management
Determine underlying cause: • Screen for maternal disease/infection • Complete fetal U/S evaluation Depends on severity: • Mild to moderate cases require no treatment • If severe, hospitalize and consider therapeutic amniocentesis
Always warrants admission and investigation: • Rule out rupture of membranes (ROM) • Fetal monitoring (NST, CTG, BPP) • U/S Doppler studies (umbilical cord and uterine artery) Maternal hydration with oral or IV fluids to help increase amniotic fluid Vesicoamniotic shunt: if etiology is related to fetal obstuctive uropathy; however, pulmonary function may not be restored with restoration of amniotic fluid Injection of fluid via amniocentesis will improve condition for ~1 wk – may be most helpful for visualizing any associated fetal anomalies Consider delivery if term Amnio-infusion may be considered during labour via intra-uterine catheter
Prognosis
Two to five-fold increase in risk of perinatal mortality
Poorer with early onset High mortality related to congenital malformations and pulmonary hypoplasia when diagnosed during T2
Obstetrics OB29
OB30 Obstetrics
Normal Labour and Delivery
Toronto Notes 2011
Normal Labour and Delivery Frontal Fontanelle or Anterior Fontanelle
Occipital Fontanelle or Posterior Fontanelle
Right Occiput Transverse
Occiput Anterior
Occiput Posterior
Left Occiput Anterior
Right Occiput Posterior
© Simon Ip 2009
Sagittal Fontanelle
Biparietal Diameter 9.5 cm
Figure 6. Fetal Positions
The Fetus • fetal lie orientation of the long axis of the fetus with respect to the long axis of the uterus (longitudinal, transverse, oblique) Presenting Parts include: Occiput for vertex Sacrum for breech Mentum for face
Fetal lie: long axis of fetus compared to long axis of uterus Fetal presentation: fetal part at pelvic outlet Fetal position: position of presenting part relative to pelvis
• fetal presentation fetal part presenting at pelvic outlet breech (complete, frank, footling) – see Figure 8, OB40 cephalic (vertex, face, asynclitic) transverse (shoulder) compound (fetal extremity prolapses along with presenting part) all except vertex are considered malpresentations (see High Risk Labour and Delivery, OB37) • fetal position position of presenting part of the fetus relative to the maternal pelvis occiput anterior (OA): most common presentation (“normal”) – left OA most common occiput posterior (OP): most rotate spontaneously to OA; may cause prolonged second stage of labour occiput transverse (OT): leads to arrest of dilatation normally, fetal head enters maternal pelvis and engages in OT position subsequently rotates to OA position (or OP in a small percentage of cases) • attitude flexion/extension of fetal head relative to shoulders brow presentation: head partially extended (requires C/S) face presentation: head fully extended – mentum posterior always requires C/S, mentum anterior will deliver vaginally • station position of presenting part relative to ischial spines – determined by vaginal exam at ischial spines = station 0 = engaged cm above (–5 g –1) or cm below (+1 g +5)
Toronto Notes 2011
Normal Labour and Delivery
Obstetrics OB31
The Cervix • dilatation: latent phase: 0-3 cm; active phase: 4-10 cm • effacement: thinning of the cervix by percentage or length of cervix (cm) • consistency: firm vs. soft • position: posterior vs. anterior • application: contact between the cervix and presenting part (i.e. well or poorly applied) • see Bishop score (Table 20, OB36)
Definition of Labour • true labour: regular, painful contractions of increasing intensity associated with progressive dilatation and effacement of cervix and descent of presenting part, or progression of station preterm (>20 but 42 weeks GA) • false labour: Braxton-Hicks contractions irregular contractions, with unchanged intensity and long intervals, occur throughout pregnancy and not associated with any dilatation, effacement or descent often relieved by rest or sedation
Analgesic and Anaesthetic Techniques in Labour and Birth
• pain or anxiety leads to high endogenous catecholamines, which produce a direct inhibitory effect on uterine contractility Non-pharmacologic Pain Relief Techniques • reduction of painful stimuli maternal movement, position change, counter-pressure, abdominal compression • activation of peripheral sensory receptors superficial heat and cold immersion in water during labour touch and massage, acupuncture and acupressure transcutaneous electrical nerve stimulation (TENS) intradermal injection of sterile water aromatherapy • enhancement of descending inhibitory pathways attention focusing and distraction hypnosis and self-hypnosis music and audio analgesia biofeedback Pharmacologic Methods • nitrous oxide (e.g. self-administered Entonox®) • narcotics (usually combined with anti-emetic) • pudental nerve block • perineal infiltration with local anesthetic • regional anaesthesia (epidural block)
Four Stages of Labour First Stage of Labour • latent phase uterine contractions typically infrequent and irregular slow cervical dilatation (usually to 3-4 cm) and effacement • active phase rapid cervical dilatation to full dilatation (nulliparous ~1.2 cm/h, multiparous ~1.5 cm/h) phase of maximum slope on cervical dilatation curve (see Figure 9, OB43) painful, regular contractions q2-3 min, lasting 45-60 seconds contractions strongest at fundus, weakest at lower segment Second Stage of Labour • from full dilatation to delivery of the baby • mother feels a desire to bear down and push with each contraction • women may choose a comfortable position that enhances pushing efforts and delivery upright (semi-sitting, squatting) and LLDP are supported in the literature • progress measured by descent
Course of Normal Labour Stage
Nulliparous
Multiparous
First
6-18 hours
2-10 hours
Second 30 min-3 hours 5-30 minutes Third
5-30 minutes
5-30 minutes
Signs of Placental Separation 1. Gush of blood 2. Lengthening of cord 3. Uterus becomes globular 4. Fundus rises
Continuous Support for Women During Childbirth Cochrane Database of Systematic Reviews 2007, Issue 3 Study: Systematic review of 16 RCTs from 11 countries, 13,391 women in labour. Intervention: Continuous support during labour vs. usual care. Outcome: Effects on mothers and their babies. Results: Continuous intrapartum support increased likehood of shorter labour, spontaneous vaginal birth, decrease in analgesia use, and a decrease in dissatisfaction with childbirth experience. Greatest benefit when provider is not a health care professional.
Normal Labour and Delivery
Toronto Notes 2011
Third Stage of Labour • separation and expulsion of the placenta • can last up to 30 minutes before intervention indicated • start oxytocin IV drip or give 10 U IM after delivery of anterior shoulder in anticipation of placental delivery, otherwise give after delivery of placenta • routine oxytocin administration in third stage of labour can reduce the risk of PPH by >40% Fourth Stage of Labour • first postpartum hour • monitor vital signs and bleeding • repair lacerations • ensure uterus is contracted (palpate uterus and monitor uterine bleeding) • inspect placenta for completeness and umbilical cord for presence of 2 arteries and 1 vein • 3rd and 4th stages of labour most dangerous to the mother (i.e. hemorrhage)
The Cardinal Movements of the Fetus During Delivery
1. Head floating, before engagement
2. Engagement, descent, flexion
3. Further descent, internal rotation
4. Complete rotation, beginning extension
5. Complete extension
6. Restitution (external rotation)
7. Delivery of anterior shoulder
8. Delivery of posterior shoulder
Figure 7. Cardinal Movements of Fetus During Delivery Adapted from illustration in Williams Obstetrics, 19th Ed.
© Danielle Bader
OB32 Obstetrics
Toronto Notes 2011
Normal Labour and Delivery
Obstetrics OB33
Fetal Monitoring in Labour • see online Fetal Heart Rate Tutorial Vaginal Exam • membrane status • cervical effacement (thinning), dilatation, consistency, position, application • fetal presenting part, position, station • bony pelvis size and shape • monitor progress of labour at regular intervals and document in a partogram Intrapartum Fetal Monitoring • intermittent fetal auscultation with Doppler device q15-30 minutes for one minute in first stage active phase following a contraction, q5 minutes during second stage when pushing has begun • continuous electronic FHR monitoring reserved for non-reassuring auscultation, prolonged labour, and labour which is induced or augmented routine use of continuous electronic monitoring shown to lead to higher intervention rates and no improvement in outcome for the neonate techniques for continuous monitoring include external (Doppler) vs. internal (fetal scalp electrode) monitoring • fetal scalp sampling should be used in conjunction with electronic FHR monitoring and contraction monitoring (cardiotocometry – CTG) to resolve the interpretation of nonreassuring patterns Electronic Fetal Heart Rate (FHR) Monitoring • FHR measured by Doppler; contractions measured by tocometer • described in terms of baseline FHR, variability (short term, long term) and periodicity (accelerations, decelerations) • Baseline FHR normal range is 110-160 bpm parameter of fetal well-being vs. distress • Variability physiologic variability is a normal characteristic of FHR effect of vagus nerve on fetal heart normal variability indicates fetal acid-base status is acceptable can only be assessed by electronic fetal monitoring (CTG) variability decreases intermittently even in healthy fetus • Periodicity accelerations: increase of ≥15 bpm lasting ≥15 seconds, in response to fetal movement or uterine contraction (or ≥10 bpm lasting ≥10 sec if 160) Maternal Factors Fever Hyperthyroidism Anemia
Fetal Bradycardia (FHR 60 sec in duration with slow return to baseline
Approach to the Management of Abnormal FHR Ensure fetal tracing Call for help Change position to LLDP 100% O2 by mask Stop oxytocin Correct maternal hypotension Fetal scalp pH/fetal scalp electrode Vaginal exam to rule out cord prolapse Rule out fever, dehydration, drug effects, prematurity Amnioinfusion or tocolytics in selected cases C/S when necessary
Variable Decelerations • Variable in shape, onset, and duration • Most common type of periodicity seen during labour • Often with abrupt drop in FHR; usually no effect on baseline FHR or variability • Due to cord compression or, in second stage, forceful pushing with contractions Complicated Variable Decelerations • To 60 sec • Loss of variability or decrease in baseline after deceleration • Biphasic deceleration • Slow return to baseline • Baseline tachycardia or bradychardia Late Decelerations • Uniform shape with onset late in contraction, nadir after peak of contraction, and slow return to baseline • May cause decreased variability and change in baseline FHR • Due to fetal hypoxia and acidemia, maternal hypotension or uterine hypertonus • Usually a sign of uteroplacental insufficiency (an ominous sign)
Uterine contraction (baseline)
End of contraction
Variable Deceleration
BPM 160
FHR
140
Variable in duration, intensity, and timing
120 100
Uterine contraction
BPM
Late Deceleration
Onset of deceleration
160
Nadir of deceleration
140
FHR
120 100
Recovery time
30 seconds of lag time Acme of contraction Onset of contraction
Uterine contraction End of contraction
Table 19. Classification of Intrapartum EFM Tracings Normal Tracing (Category 1)
Atypical Tracing* (Category 2)
Abnormal Tracing* (Category 3)
Baseline
110-160 bpm
Bradycardia 100-100 bpm Tachycardia >160 for 30-80 min Rising baseline
Bradycardia 160 bpm for >80 min Erratic baseline
Variability
6-25 bpm ≤5 bpm for 10 min
Decelerations
None Early decelerations Occasional uncomplicated variable decelerations
Repetitive (≥3) uncomplicated variable decelerations Occasional late decelerations Any prolonged deceleration 2-3 min
Repetitive (≥3) complicated variable decelerations Repetitive late decelerations Any prolonged deceleration >3 min
Accelerations
Accelerations spontaneous or during scalp stimulation
Absent with scalp stimulation
Nearly absent
Action
EFM may be interrupted for ≤30 min if mother/fetus stable
Further assessment required
Action required: review clinical situation, obtain scalp pH, prepare for possible delivery
Adapted from SOGC guidelines, September 2008 *Previous classification was “reassuring” vs. “non-reassuring”, but distinction is now made between tracings that have some concerning changes but do not require immediate action (atypical) versus those with major concerns requiring immediate intervention (abnormal).
Toronto Notes 2011
Normal Labour and Delivery/Induction of Labour
Obstetrics OB35
Fetal Oxygenation • uterine contractions during labour decrease uteroplacental blood flow, which results in reduced oxygen delivery to the fetus • most fetuses tolerate this reduction in flow and have no adverse effects • distribution of oxygen to the fetus depends on maternal, uteroplacental and fetal factors • maternal factors decreased maternal oxygen carrying capacity significant anemia (iron deficiency, hemoglobinopathies) carboxyhemoglobin (smokers) decreased uterine blood flow hypotension (blood loss, sepsis) regional anesthesia maternal positioning chronic maternal conditions vasculopathies (lupus, Type 1 DM, chronic HTN) antiphospholipid syndrome cyanotic heart disease COPD • uteroplacental factors uterine hypertonus hyperstimulation secondary to oxytocin, prostaglandins or normal labour placental abruption uteroplacental dysfunction placental abruption placental infarction (dysfunction marked by IUGR, oligohydramnios, abnormal Doppler studies) chorioamnionitis placental edema (diabetes, hydrops) placental senescence (post dates) • fetal factors cord compression oligohydramnios cord prolapse or entanglement decreased fetal oxygen carrying capability significant anemia (isoimmunization, feto-maternal bleed) carboxyhemoglobin (exposure to smokers) • fetal response to hypoxia/asphyxia decreased movement, tone, and breathing activities redistribution of fetal blood flow increased flow to brain, heart, and adrenals decreased flow to kidneys, lungs, gut, liver and peripheral tissues increase in blood pressure transient fetal bradycardia followed by fetal tachycardia anaerobic metabolism (decreased pH)
Induction of Labour Definition • artificial initiation of labour before its spontaneous onset for the purpose of delivery of the fetus and placenta Prerequisites for Labour Induction • capability for C/S if necessary • maternal short, thin, soft, anterior cervix with open os (“inducible” or “ripe”) if cervix is not ripe, use prostaglandin vaginal insert (Cervidil®), prostaglandin gel (Prepidil®), or Foley catheter • fetal reassuring fetal heart tracing cephalic presentation adequate fetal monitoring available • likelihood of success determined by Bishop score (see Table 20) cervix considered unfavourable if 41 weeks) • maternal factors significant antepartum hemorrhage gestational HTN other maternal medical problems, e.g. diabetes, renal or lung disease • maternal-fetal factors isoimmunization, PROM, chorioamnionitis, post-term pregnancy • fetal factors suspected fetal jeopardy as evidenced by biochemical or biophysical indications fetal demise, severe IUGR Risks • failure to achieve labour and/or vaginal birth • uterine hyperstimulation and fetal compromise • uterine rupture • uterine atony and PPH • maternal side effects to medications
Evidence for Cervical Ripening Methods (SOGC Guidelines) • Meta-analysis of five trials has concluded that the use of oxytocin to ripen the cervix is not effective. • Since the best dose and route of misoprostol for labour induction with a live fetus are not known and there are concerns regarding hyperstimulation, the use of misoprostol for induction of labour should be within clinical trials only (Level Ib evidence) or in cases of intrauterine fetal death to initiate labour.
Use of Prostaglandins in Cervical Ripening and Induction Intravenous Prostaglandin for Induction of Labour • Prostaglandin E2 and F2 alpha can be used for cervical ripening and induction of labour. A meta-analysis comparing intravenous prostaglandin with oxytocin concluded that intravenous prostaglandin was no more likely to result in vaginal delivery (RR 0.85). Prostaglandins were associated with significantly more maternal side effects including gastrointestinal problems, thrombophlebitis and pyrexia. Currently, there is not enough evidence to draw any conclusions about the relative effects of prostaglandins vs. oxytocin and the choice is between the patient and the physician. • Intravaginal prostaglandins are associated with higher rate of uterine hypertonus, uterine hyperstimulation, and fetal heart rate abnormalities. • Prostaglandins are associated with reduced rate of C/S, instrumental vaginal delivery, and failed induction. Cochrane Review. The Cochrane Library, 2000. Issue 2
Contraindications • maternal prior classical or inverted-T incision or uterine surgery (e.g. myomectomy) unstable maternal condition gross CPD (although diagnosis cannot be made until active labour) active maternal genital herpes invasive cervical carcinoma pelvic structure deformities • maternal-fetal placenta previa or vasa previa cord presentation • fetal fetal distress, malpresentation, preterm fetus without lung maturity
Induction Methods CERVICAL RIPENING Definition • use of medications or other means to soften, efface and dilate cervix to increase likelihood of induction success • ripening of an unfavourable cervix (Bishop score 30 mm has high negative predictive value for PTL before 34 weeks) • identification of bacterial vaginosis (Rx – metronidazole) and ureaplasma urealyticum (Rx – erythromycin) infections – routine screening not supported by current data but it is reasonable to screen high risk women • fetal fibronectin – a glycoprotein in amniotic fluid and placental tissue functioning to maintain integrity of chorionic-decidual interface in asymptomatic women positive if >50 ng/mL in symptomatic women (i.e. preterm contractions), fetal fibronectin is most effectively combined with U/S detecting cervical length if cervical length is not short and fetal fibronectin is negative, preterm labour is highly unlikely
PTL Recurrence Definition: 3 or more consecutive lost pregnancies prior to 20th week of gestation • 15% sporadic loss of 1 pregnancy • 2% experience 2 consecutive pregnancy losses • 0.4-1% experience 3 consecutive pregnancy losses
Positive fetal fibronectin in cervicovaginal fluid (>50 ng/mL) at 24 weeks gestation predicted spontaneous PTL at 2 cm dilated or 80% effaced or documented change in cervix Management A. Initial • transfer to appropriate facility if stable • hydration (NS at 150 mL/hour) • bed rest in LLDP • sedation (morphine) • avoid repeated pelvic exams (increased infection risk) • U/S examination of fetus (GA, BPP, position, placenta location, estimated fetal weight) • prophylactic antibiotics; controversial but may help delay delivery, important to consider if PPROM B. Suppression of Labour – Tocolysis • does not inhibit preterm labour completely, but may buy time to allow for betamethasone valerate (Celestone®) and/or transfer to appropriate centre • requirements (all must be satisfied) preterm labour live, immature fetus, intact membranes, cervical dilatation of 41 weeks and expectant management elected – serial fetal surveillance: fetal movement count by the mother AFV ± NST (modified BPP) Prognosis • if >41 weeks, perinatal mortality 2-3x higher (due to progressive uteroplacental insufficiency) • morbidity increased with hypertension in pregnancy, DM, abruption, IUGR and multiple gestation
Intrauterine Fetal Death Definition • fetal death in utero after 20 weeks GA Epidemiology • 1% of pregnancies
Vaginal Delivery After C-Section (VBAC) • Rate of VBAC ranges from 60-82% • No significant difference in maternal deaths or hysterectomies between VBAC or C-section • Uterine rupture more common in VBAC group • Evidence regarding fetal outcome is lacking Safety of vaginal birth after caesarean section: a systematic review. Obstet Gynecol 2004; 103(3):420-9
OB42 Obstetrics
High Risk Labour and Delivery/Complications of Labour and Delivery
DIC: Generalized coagulation and fibrinolysis leading to depletion of coagulation factors Obstetrical Causes • Abruption • PIH • Fetal demise • PPH DIC-specific Bloodwork • Platelets • aPTT and PT • FDP (fibrin degradation products) • Fibrinogen Treatment • Treat underlying cause • Supportive • Fluids • Blood products • FFP, platelets, cryoprecipitate • Consider anti-coagulation as VTE prophylaxis
Toronto Notes 2011
Etiology • 50% idiopathic • 50% secondary to HTN, DM, erythroblastosis fetalis, congenital anomalies, umbilical cord or placental complications, intrauterine infection, APLA syndrome Clinical Features • decreased perception of fetal movement by mother • SFH and maternal weight not increasing • absent fetal heart tones (not diagnostic) • high maternal serum alpha-fetoprotein (MSAFP) Management • diagnosis: absent cardiac activity and fetal movement on U/S required for diagnosis • determine secondary cause maternal: HbA1C, Kleihauer-Betke, VDRL, ANA, antibody screens, INR/PTT, serum/urine toxicology screens, cervical and vaginal cultures, TORCH screen fetal: chromosomes, cord blood, skin biopsy, genetics evaluation, autopsy placenta: pathology, bacterial cultures Treatment • induction of labour • monitor for maternal coagulopathy [10% risk of disseminated intravascular coagulation (DIC)] • parental psychological care: referral to grieving support programs encourage partner to stay for support, ask couple to hold the newborn (inform that bruising, facial marks pay be present) make neonatal footprint, take a photo of the newborn, encourage to name the child early follow-up within 2 weeks to assess mental well-being (depression, anxiety) comprehensive discussion within 3 months about final investigation and post-mortem results, help make plans for future pregnancies
Complications of Labour and Delivery Meconium in Amniotic Fluid
Maternal Mortality Causes • Thrombo-embolism • Cardiac event • Suicide • Sepsis • Ectopic pregnancy • Hypertension • Amniotic-fluid embolism • Hemorrhage * In Canada (2005), lifetime risk of maternal death is 1 in 11,000
Dark green or black meconium is associated with lower APGARs and increased risk of meconium aspiration.
Epidemiology • usually not present early in labour • in general, meconium may be present in up to 25% of all labours; usually NOT associated with poor outcome, but extra care is required at time of delivery to avoid aspiration Etiology • likely cord compression ± uterine hypertonus • may indicate undiagnosed breech • increasing meconium during labour may be a sign of fetal distress Clinical Features • timing: early (prior to ROM) or late (after ROM with clear fluid) • consistency thin meconium: light green or yellow, not usually associated with poor outcome thick meconium: dark green or black, pea-soup consistency, associated with lower APGARs and increased risk of meconium aspiration Treatment • call respiratory therapy, neonatology or pediatrics to delivery room • oropharynx suctioning upon head expulsion or immediately after delivery if baby not breathing spontaneously (do NOT stimulate infant before) • consider amnioinfusion of ~800 mL of IV NS over 50-80 min during active stage of labour and a maintenance dose of ~3 mL/min until delivery • closely monitor FHR for signs of fetal distress
Abnormal Progression of Labour (Dystocia) Definition • expected patterns of descent of the presenting part and cervical dilatation fail to occur in the appropriate time frame; can occur in all stages of labour (see Figure 9) • during active phase: >4 hrs of 1 hr with no descent during active pushing
Toronto Notes 2011
Complications of Labour and Delivery
Etiology • Power (leading cause): contractions (hypotonic, incoordinate), inadequate maternal explusive efforts • Passenger: fetal position, attitude, size, anomalies (hydrocephalus) • Passage: pelvic structure (cephalopelvic disproportion), maternal soft tissue factors (tumours, full bladder or rectum, vaginal septum) • Psyche: hormones released in response to stress can bring about dystocia psychological and physiological stress should be evaluated as part of the management once dystocia has been diagnosed
Cervical Dilatation (cm)
10
A
E D
B
8 6
C
The 4 Ps of Dystocia Power Passenger Passage Psyche
A. Average Multipara B. Average Primigravida
4
C. Arrrest of active phase
2
D. Protracted active phase 0 2 4 6 8 10 12 14 16 18 20 22 24 26 28 30 32
Obstetrics OB43
E. Prolonged latent phase
The 4 Types of Pelvis GAAP Gynecoid (50%) – commonest, obstetrically ideal Anthropoid (25%) Android (20%) Platypelloid (5%)
Labour (hrs)
Figure 9. Normal and Abnormal Courses of the First Stage of Labour
Arrest Disorder (Curve C) • arrest of dilatation dilatation progress does not occur for ≥2 hrs in a patient who has entered the active phase arrest usually occurs at a cervical dilatation of 5-8 cm • arrest of descent no progress in station for >1 hr during second stage should search for factors causing CPD (nearly 50% require C/S) CPD diagnosed if adequate contractions measured by intrauterine pressure catheter (IUPC) with no descent/dilatation for >2 hours if CPD ruled out, IV oxytocin and amniotomy can be attempted Protraction Disorders (Curve D) • protraction of dilatation: slope of cervical dilatation 1000 mL with C/S • early – within first 24h postpartum • late – after 24h but within first 6 weeks Epidemiology • incidence 5-15%
Uterine atony is the most common cause of PPH.
DDx of Early PPH – 4 T’s 1. Tone (atony) 2. Tissue (retained placenta, clots) 3. Trauma (laceration, inversion) 4. Thrombin (coagulopathy) DDx of Late PPH 1. Retained products 2. ± endometritis 3. Sub-involution of uterus
Etiology (4 T’s) 1. Tone uterine atony most common cause of PPH avoid by giving oxytocin with delivery of the anterior shoulder or placenta occurs within first 24 hours due to: labour (prolonged, precipitous, induced, augmented) uterus (infection, over-distention) placenta (abruption, previa) maternal factors (grand multiparity, gestational HTN) halothane anesthesia 2. Tissue retained placental products retained blood clots in an atonic uterus gestational trophoblastic neoplasia 3. Trauma laceration (vagina, cervix, uterus), episiotomy, hematoma (vaginal, vulvar, retroperitoneal), uterine rupture, uterine inversion 4. Thrombin coagulopathy most identified prior to delivery (low platelets increases risk) includes hemophilia, DIC, aspirin use, ITP, TTP, vWD (most common) therapeutic anti-coagulation Investigations • assess degree of blood loss and shock by clinical exam • explore uterus and lower genital tract for evidence of tone, tissue or trauma • may be helpful to observe red-topped tube of blood – no clot in 7-10 minutes indicates coagulation problem Management • ABCs • 2 large bore IVs and crystalloids • CBC, coagulation profile, cross and type 4 units pRBCs • treat underlying cause Medical Therapy • oxytocin 20 U/L NS or RL IV continuous infusion in addition can give 10 U intramyometrial (IMM) after delivery of the placenta • methylergonavine maleate (ergotamine) 0.25 mg IM/IMM q5min up to 1.25 mg; can be given as IV bolus of 0.125 mg (may exacerbate HTN) • carboprost (Hemabate®) (synthetic PGF-2 alpha analog) 0.25 mg IM/IMM q15 min to max 2 mg (major prostaglandin side effects and contraindicated in cardiovascular, pulmonary, renal and hepatic dysfunction) Local Control • bimanual compression: elevate the uterus and massage through patient’s abdomen • uterine packing (mesh with antibiotic treatment) • intrauterine Senstaken-Blakemore catheter for balloon tamponade – may slow hemorrhage enough to allow time for correction of coagulopathy or for preparation of an OR
Toronto Notes 2011
Operative Obstetrics/Puerperal Complications
Surgical Therapy (Intractable PPH) • D&C (beware of vigorous scraping which may cause Asherman’s syndrome) • laparotomy with bilateral ligation of uterine artery (may be effective), internal iliac artery (not proven), ovarian artery, or hypogastric artery • hysterectomy (last option) with angiographic embolization if post-hysterectomy bleeding RETAINED PLACENTA Definition • placenta undelivered after 30 minutes postpartum Etiology • placenta separated but not delivered • abnormal placental implantation (placenta accreta, placenta increta, placenta percreta) Risk Factors • placenta previa, prior C/S, post-pregnancy curettage, prior manual placental removal, uterine infection Clinical Features • incomplete placenta removed • risk of postpartum hemorrhage and infection Investigations • explore uterus • assess degree of blood loss Management • 2 large bore IVs, type and screen • Brant maneuver (firm traction on umbilical cord with one hand applying suprapubic pressure to avoid uterine inversion by holding uterus in place) • oxytocin 10 IU in 20 mL NS into umbilical vein • manual removal if above fails • D&C if required UTERINE INVERSION Definition • turning inside out (inversion) of the uterus through cervix ± vaginal introitus Etiology/Epidemiology • often iatrogenic (excess cord traction with fundal placenta) • excessive use of uterine tocolytics • more common in grand multiparous (lax uterine ligaments) • 1/1500-1/2000 deliveries Clinical Features • can cause profound vasovagal response with vasodilation and hypovolemic shock • shock may be disproportionate to maternal blood loss Management • urgent management essential, call anesthesia • ABCs – initiate IV crystalloids • can use tocolytic drug (e.g. terbutaline) or nitroglycerin IV to relax uterus and aid replacement • replace uterus without removing placenta • remove placenta manually and withdraw slowly • IV oxytocin infusion (only after uterus replaced) • re-explore uterus • may require GA ± laparotomy
Obstetrics OB49
OB50 Obstetrics
Puerperal Complications
Toronto Notes 2011
Postpartum Pyrexia Etiology of Post-Partum Pyrexia B-5W Breast: engorgement, mastitis Wind: atelectasis, pneumonia Water: UTI Wound: episotomy, C/S site infection Walking: DVT, thrombophlebitis Womb: endometritis
Definition • fever >38°C on any 2 of the first 10 days postpartum, except the first day Etiology • Wind (atelectasis, pneumonia), Water (UTI), Wound (C/S incision or episiotomy site), Walking (pelvic thrombophlebitis, DVT), Breast (engorgement, mastitis – S. aureus), Womb (endometritis) Investigations • detailed history and physical exam, relevant cultures • for endometritis: blood and genital cultures Treatment • depends on etiology infection: empiric antibiotics, adjust when sensitivities available endometritis/wound infection: clindamycin + gentamycin IV mastitis: penicillin or cephalosporin DVT: anticoagulants • prophylaxis against post-C/S endometritis: begin antibiotic immediately after cord clamping and administer only 1-3 doses – cefazolin is most common choice
Risk Factors for Endometritis C-section, intrapartum chorioamnionitis, prolonged labour, prolonged ROM, multiple vaginal examinations
ENDOMETRITIS • definition: infection of uterine myometrium and parametrium • clinical features: fever, chills, abdominal pain, uterine tenderness, foul-smelling discharge or lochia • treatment: depends on infection severity; oral antibiotics if well, IV with hospitalization in moderate to severe cases VENOUS THROMBOEMBOLISM • see OB20
Mastitis • definition: inflammation of mammary glands • must rule out inflammatory carcinoma, as indicated • differentiate from mammary duct ectasia – mammary duct(s) beneath nipple clogged and dilated ± ductal inflammation ± nipple discharge (thick, grey to green), often postmenopausal women Table 21. Lactational versus Non-Lactational Mastitis Lactational
Non-Lactational
Epidemiology
More common than non-lactational Often 2-3 wks postpartum
Periductal mastitis most common Mean age 32 y
Etiology
S. aureus
May be sterile May be infected with S. aureus or other anaerobes Smoking is risk factor May be associated with mammary duct ectasia
Symptoms
Unilateral localized pain Tenderness Erythema
Subareolar pain May have subareolar mass Discharge (variable colour) Nipple inversion
Treatment
Heat or ice packs Continued nursing/pumping Antibiotics (dicloxacillin/cephalexin) (Erythromycin if pen-allergic)
Broad-spectrum antibiotics and I&D Total duct excision (definitive)
Abscess
Fluctuant mass Purulent nipple discharge Fever, leukocytosis D/c nursing, IV antibiotics (nafcillin/oxacillin), I&D usually required
If mass does not resolve, FNA to exclude cancer and U/S to assess presence of abscess Treatment includes antibiotics, aspiration or I&D (tends to recur) May develop mammary duct fistula A minority of non-lactational abscesses may occur peripherally in breast with no associated periductal mastitis (usually S. aureus)
Toronto Notes 2011
Puerperal Complications/Postpartum Care
Postpartum Mood Alterations POSTPARTUM BLUES • 85% of new mothers, onset day 3-10; extension of the “normal” hormonal changes and adjustment to a new baby • self-limited, should resolve by 2 weeks • manifested by mood lability, depressed affect, increased sensitivity to criticism, tearfulness, fatigue, irritability, poor concentration/despondency POSTPARTUM DEPRESSION (PPD) • definition: major depression occurring in a woman within 6 months of childbirth (see Psychiatry, PS10) • epidemiology: 10-20%, risk of recurrence 50% • risk factors personal or family history of depression (including PPD) prenatal depression or anxiety stressful life situation poor support system unwanted pregnancy colicky or sick infant • clinical features: suspect if the “blues” last beyond 2 weeks, or if the symptoms in the first two weeks are severe (e.g. extreme disinterest in the baby, suicidal or homicidal/infanticide ideation) • assessment: Edinburgh Postnatal Depression Scale or other • treatment: antidepressants, psychotherapy, supportive care, ECT if refractory • prognosis: interferes with bonding and attachment between mother and baby so it can have long term effects POSTPARTUM PSYCHOSIS • definition: onset of psychotic symptoms over 24-72 hours within first month postpartum, can present in the context of depression • epidemiology: rare (0.2%)
Postpartum Care Postpartum Office Visit at 6 Weeks Care of Baby • assess weight, feeding, immunization • encourage breastfeeding if no contraindications Care of Mother (The 10 Bs) • Be careful: do not use douches or tampons for 4-6 weeks post-delivery • Be fit: encourage gradual increases in walking, Kegel exercises • Birth control: assess for use of contraceptives; breastfeeding is NOT an effective method of birth control • Bladder: assess for urinary incontinence, maintain high fluid intake • Bleeding: (see Lacerations, OB47), 300 µg of RhIG should be given if Rh+ fetus and Rh– mother or extensive bleeding at delivery • Blood pressure: especially if gestational HTN • Blood tests: glucose, CBC (for anemia as sign of hematomas, retained placenta) • Blues: see Postpartum Mood Alterations, OB51 • Bowel: fluids and high-fibre foods, bulk laxatives; for hemorrhoids/perineal tenderness: pain meds, doughnut cushion, Sitz baths, ice compresses • Breast and pelvic exam: watch for Staphyloccoccal or Streptococcal mastitis/abscess, ± Pap smear at 6 weeks Physiological Changes Postpartum • uterus weight rapidly diminishes through catabolism, cervix loses its elasticity and regains firmness should involute ~1 cm below umbilicus per day in first 4-5 days, reaches non-pregnant state in 4-6 weeks postpartum • ovulation resumes in ~45 days for non-lactating women and within 3-6 months for lactating women • lochia: normal vaginal discharge postpartum decreases and changes in colour from red (lochia rubra; presence of erythrocytes) g yellow (lochia serosa) g white (lochia alba; residual leukorrhea) over 3-6 weeks foul smelling lochia suggests endometritis
Obstetrics OB51
OB52 Obstetrics
Postpartum Care/Drug and Food Safety During Pregnancy and Breastfeeding
The acronym “BUBBLES” for what to ask about when rounding on postpartum care. Modify this for C/S or vaginal delivery Baby care and breastfeeding (latch, amount) Uterus – firm or boggy? Bladder function – Voiding well? Dysuria? Bowel function – Passing gas or stool? Constipated? Lochia or discharge – Any blood? Episiotomy/laceration/incision – Pain controlled? Symptoms of VTE – Dyspnea? Calf pain?
Toronto Notes 2011
Breastfeeding Problems • inadequate milk: consider domperidone • breast engorgement: cool compress, manual expression/pumping • nipple pain: clean milk off nipple after feeds, moisture cream, topical steroid if needed • mastitis: treat promptly (see OB50) • inverted nipples: makes feeding difficult • maternal medications: may require pediatric consultation (see OB54) Bladder Dysfunction • pelvic floor prolapse can occur after vaginal delivery • stress or urge urinary incontinence common • increased risk with instrumental delivery or prolonged second stage • conservative management: pelvic floor retraining with Kegel exercises, vaginal cones or pessaries, lifestyle modifications (e.g. limit fluid, caffeine intake) • surgical management: minimally invasive procedures (tension-free vaginal tape, transobturator tape, midurethral sling) (see Gynecology, GY34) Puerperal Pain • “after pains” common in first 3 days due to uterine contractions; encourage simple analgesia • ice packs can be used on perineum if painful • encourage regular analgesia and stool softener
Drug and Food Safety During Pregnancy and Breastfeeding • most drugs cross the placenta to some extent • very few drugs are teratogenic, but very few drugs have proven safety in pregnancy • use any drug with caution and only if necessary
Antibiotics Drug Resources during Pregnancy and Breastfeeding • Motherisk at the Hospital for Sick Children in Toronto: www.motherisk.org • Hale, T. Medications and Mothers’ Milk, 11th Edition. Pharmasoft Publishing, 2004.
• safest: ampicillin, cephalosporins • erythromycin: maternal liver damage (acute fatty liver) used only if contraindication to penicillin use • tetracyclines: stain infant’s teeth, may affect long bone development • sulpha drugs: anti-folate properties, therefore theoretical risk in T1; risk of kernicterus in T3 • metronidazole: anti-metabolite, therefore theoretical risk in T1 • chloramphenicol: grey baby syndrome (fetal circulatory collapse 2° to toxic accumulation) • fluoroquinolones: risk of cartilage damage (in dog and rat studies)
Other Medications and Substances • analgesics: acetaminophen preferable to ASA or ibuprofen Documented adverse effects, contraindicated: • ACE inhibitors: fetal renal defects, IUGR, oligohydramnios • tetracycline: see above • retinoids (e.g. Accutane®): CNS, craniofacial, cardiac, and thymic anomalies • DES (and other estrogenic or androgenic compounds): vaginal adenosis, adenocarcinoma, uterine malformation in females exposed to DES in utero • misoprostol: Mobius syndrome (congenital facial paralysis with or without limb defects) Documented adverse effects, weigh benefits vs. risks and consider medication change: • anticonvulsants phenytoin associated with fetal hydantoin syndrome in 5-10% (IUGR, mental retardation, facial dysmorphogenesis, congenital anomalies) valproate associated with oNTD in 1% carbamazepine associated with oNTD in 1-2% generally recommended to remain on the lowest dose anticonvulsant appropriate for their condition • lithium: Ebstein’s cardiac anomaly, goitre, hyponatremia • warfarin: increased incidence of spontaneous abortion, stillbirth, prematurity, IUGR fetal warfarin syndrome (nasal hypoplasia, epiphyseal stippling, optic atrophy, mental retardation, intracranial hemorrhage)
Toronto Notes 2011
Drug and Food Safety During Pregnancy and Breastfeeding
Obstetrics OB53
Substances • alcohol: increased incidence of abortion and stillbirth, congenital anomalies fetal alcohol syndrome (growth retardation, CNS involvement and facial anomalies) • cigarette smoke: decreased birth weight, placenta previa/abruption, spontaneous abortion, preterm labour, stillbirth • cocaine: microcephaly, growth retardation, prematurity, MR
Immunizations Intrapartum • administration is dependent on the risk of infection vs. risk of immunization complications • safe: tetanus toxoid, diphtheria, influenza, hepatitis B • avoid live vaccines (risk of placental and fetal infection): polio, measles/mumps/rubella, varicella • contraindicated: rubella, oral typhoid Postpartum • rubella vaccine for all non-immune mothers • hepatitis B vaccine should be given to infant within 12h of birth if maternal status unknown or positive – follow-up doses at 1 and 6 months
Food Caffeine • diuretic and stimulant properties • readily crosses placenta • possible risk for miscarriage and fetal growth retardation at high doses (>200-300 mg/day); note some of this presumed risk may be due to confounders, such as cigarette smoking • based on a meta-analysis, consumption should be limited to no more than 150 mg per day from all sources during pregnancy and lactation Herbal Teas and Preparations • not enough scientific information about the safety of various herbs and herbal products to recommend their general use during pregnancy and lactation • some herbal teas can have toxic or pharmacological effects on the mother or fetus • chamomiles have been reported to exhibit adverse effects on the uterus Food Borne Illnesses • microbiological contamination of food may occur through cross-contamination and/or improper food handling listeriosis (Listeria monocytogenes) and toxoplasmosis (Toxoplasma gondii) are of concern during pregnancy avoid consumption of raw meats, fish, poultry, raw eggs, and unpasteurized dairy products wash all raw fruit and vegetables thoroughly avoid soft cheeses and pates as they may be sources of Listeria • chemical contamination of food current guideline for mercury of 0.5 ppm in fish is considered protective for the general population, including pregnant women Health Canada advises pregnant women to limit consumption of top predator fish such as shark, swordfish and fresh/frozen tuna (not canned tuna) to one meal per month
Sources of Caffeine 5 oz cup coffee: 40-180 mg 5 oz brewed tea: 20-90 mg 12 oz cola: 46 mg Red Bull®: 67 mg Dark chocolate bar: 10 mg 8 oz hot chocolate: 5 mg
Herbal Teas Considered Safe in Moderation (2-3 cups/day) Citrus peel Ginger Lemon balm Linden flower – not with prior cardiac condition Orange peel Rose hip
OB54 Obstetrics
Drug and Food Safety During Pregnancy and Breastfeeding/and Breastfeeding
Toronto Notes 2011
Radiation Radiation in Pregnancy Necessary amount to cause miscarriage: >5 rads Necessary amount to cause malformations: >20-30 rads
• ionizing radiation exposure is considered teratogenic at high doses if indicated for maternal health, should be done • imaging not involving direct abdominal/pelvic high dosage is not associated with adverse effects higher dosage to fetus: plain x-ray of lumbar spine/abdomen/pelvis, barium enema, CT abdomen, pelvis, lumbar spine • most investigations involve minimal radiation exposure (see Table 22) • radioactive isotopes of iodine are contraindicated • no known adverse effects from U/S or MRI Table 22. Approximate Fetal Doses from Common Diagnostic Procedures Examination Plain Film Abdomen Pelvis Lumbar spine Thoracic spine Chest (2 views) CT Abdomen (10 slices) Pelvis (1 slice with scout film) Lumbar spine (5 slices) Chest
Estimated Fetal Dose (rad)
Number of Exams Safe in Pregnancy
0.245 0.040 0.359 0.009 0.00007
20 125 13 555 71 429
2.600 0.250 3.500 0.2-0.6
1 20 1 20
Adapted from Valentin, 2000.
Breastfeeding and Drugs Breastfeeding: Contraindicated Drugs BREAST Bromocriptine/Benzodiazepines Radioactive isotopes/Rizatriptan Ergotamine/Ethosuximide Amiodarone/Amphetamines Stimulant laxatives/Sex hormones Tetracycline/Tretinoin
• safe penicillins, aminoglycosides, cephalosporins oral contraceptive use (low dose) – OCP will decrease quantity but not affect composition of breast milk medroxyprogesterone acetate • avoid chloramphenicol (bone marrow suppression) metronidazole (mutagenic in vitro) sulphonamides (hemolysis with G6PD deficiency) nitrofurantoin (hemolysis with G6PD deficiency) tetracycline lithium anti-neoplastics and immunosuppressants psychotropic drugs (relative)
Toronto Notes 2011
Common Medications
Obstetrics OB55
Common Medications Table 23. Common Medications Drug Name (Brand Name)
Dosing Schedule
Indications/Comments
betamethasone valerate (Celestone®)
12 mg IM q24h x 2 doses
Enhancement of fetal pulmonary maturity for PTL
carboprost (Hemabate®)
0.25 mg IM/IMM q15min; max 2 mg
Treatment of uterine atony
dexamethasone
6 mg IM q12h x 4 doses
Enhancement of fetal pulmonary maturity for PTL
dinoprostone (Cervidil®: PGE2 impregnated thread)
10 mg PV (remove after 12h) max of 3 doses
Induction of labour Advantage: can remove if uterine hyperstimulation
doxylamine succinate (Diclectin®)
2 tabs qhs + 1 tab qAM + 1 tab qPM max of 8 tabs/day
Each tablet contains 10 mg doxylamine succinate with vitamin B6 Used for hyperemesis gravidarum
folic acid
0.4-1 mg PO OD x 1-3 mo preconception and T1 5 mg PO OD with past Hx of NTD
Prevention of oNTD
methotrexate
50 mg/m2 IM or 50 mg po x 1 dose
For ectopic pregnancy or medical abortion
methylergonavine maleate (Ergotamine®)
0.25 mg IM/IMM q5min up to 1.25 mg or IV bolus 0.125 mg
Treatment of uterine atony
misoprostol (Cytotec®)
800-1000 µg PR x 1 dose 400 µg PO x 1 dose or 800 µg PV x 1 dose, 3 to 7 days after methotrexate
For treatment of PPH For medical abortion Also used for NSAID-induced ulcers (warn patients of contraindications)
oxytocin (Pitocin®)
0.5-2.0 mU/min IV, or 10 U/L N/S incr. by 1-2 mU/min q20-60min max of 36-48 mU/min 10 U IM at delivery of anterior shoulder and of placenta 20 U/L NS or RL IV continuous infusion
Augmentation of labour (also induction of labour)
PGE2 gel (Prostin® gel)
0.5 mg PV q6-12h; max of 3 doses
Induction of labour
Rh IgG (Rhogam®)
300 µg IM x 1 dose
Given to Rh negative women • Routinely at 28 wks GA • Within 72 hrs of birth of Rh +ve fetus positive • Positive Kleihauer-Betke test • With any invasive procedure in pregnancy • Ectopic pregnancy • Antepartum hemorrhage • Miscarriage or TA (dose: 50 µg IM only)
Prevention of uterine atony Treatment of uterine atony
Misoprostol (Cytotec®) is also indicated to protect against NSAIDinduced gastric ulcers in non-pregnant individuals. The use of misoprostol for cytoprotection is contraindicated in pregnancy. Warn female patients of this contraindication.
Common Discharge Medications Oxycodone IR 5-10 mg po q4-6h PRN Docusate sodium 100 mg PO BID
OB56 Obstetrics
References
Toronto Notes 2011
References American College of Obstetricians and Gynecologists www.acog.org The Society of Obstetricians and Gynaecologists of Canada www.sogc.org ABC of labour care: Care of the newborn in the delivery room. BMJ 1999; 318: 1403-1406. ABC of labour care: Obstetric emergencies. BMJ 1999; 318: 1342-1345. ABC of labour care: Operative delivery. BMJ 1999; 318: 1260-1264. ABC of labour care: Induction. BMJ 1999; 318: 995-998. ABC of labour care: Labour in special circumstances. BMJ 1999; 318: 1124-1127. ABC of labour care: Physiology and management of normal labour. BMJ 1999; 318: 793-796. ABC of labour care: Place of birth. BMJ 1999; 318: 721-723. ABC of labour care: Preterm labour and premature rupture of membranes. BMJ 1999; 318: 1059-1062. ABC of labour care: Relief of pain. BMJ 1999; 318: 927-930. ABC of labour care: Unusual presentations and positions and multiple pregnancy. BMJ 1999; 318: 1192-1194. Alfirevic Z. (2002). Oral misoprostol for induction of labour (Cochrane Review). The Cochrane Library, Issue 2. Antenatal Corticosteroid Therapy for Fetal Maturation. SOGC Clinical Practice Guidelines Policy Statement, December 1995: 53. Baskett, T. Essential Management of Obstetric Emergencies. 3rd ed. Clinical Press, Bristol, 1999. Bastian LA, Piscitelli JT. Is this patient pregnant? Can you reliably rule in or rule out early pregnancy by clinical examination? JAMA. 1997 Aug 20; 278(7): 586-91. Review Berghella V, et al. (2004). Cerclage for prevention of preterm birth in women with a short cervix found on transvaginal examination: A randomized trial. Amer J Obs Gyn. 191:1311-7. Boucher, M. Mode of Delivery for Pregnant Women infected by the Human Immunodeficiency Virus. SOGC Clinical Practice Guidelines. April 2001: 101. Boucher, M. and Gruslin, A. The Reproductive Care of Women living with Hepatitis C Infection. SOGC Clinical Practice Guidelines. October 2000: 96. Bricker L, Luckas M. (2002). Amniotomy alone for induction of labour (Cochrane Review). The Cochrane Library, Issue 2. Carol, Blenning. An Approach to the Postpartum Office Visit. American Family Physician. Dec 2005, 72(12); 2491 – 2496. Chodirker et al. Canadian Guidelines for Prenatal Diagnosis Part 1 & Part 2. SOGC Clinical Practice Guidelines. July 2001 and June 2000. No 105. Chyu JK, Strassner HT. Prostaglandin E2 for cervical ripening: a randomized comparison of cervidil vs. prepidil. AMJ Obstet Gynecol 1997. 177: 606-11. Crane, J. (2001). Induction of labour at term. SOGC Clinical Practice Guideline, 107:1-12. Fetal health Surveillance in Labour – Parts 1 and 2. SOGC Clinical Practical Guideline. January 1996, 45. Ford HB, Schust DJ. (2009) Recurrent Pregnancy Loss: Etiology, Diagnosis, and Therapy. Rev Obstet Gynecol. 2009 Spring; 2(2): 76–83. Gagnon A, and Wilson R. Obstetrical complications associated with abnormal maternal serum markers analytes. SOGC Clinical Practice Guidelines. Oct 2008, No 216; 918-932. Guidelines for exercise in pregnancy. SOGC Clinical Practical Guidelines. Committee Opinion. June 2003. Guidelines for the Management of Nausea and Vomiting in Pregnancy. SOGC Clinical Practice Guidelines Committee Opinion. November 1995: 12. Goldenberg et al. Epidemiology and causes of preterm birth. The Lancet. Jan 2008, Vol 371; 75 – 84. Guise JM, Berlin M, McDonagh M, Osterweil P, Chan B, Helfand M. Safety of vaginal birth after caesarean: a systematic review. Obstet Gynec. 2004 Mar; 103(3):420-9. Hannah, M. Post-term Pregnancy. SOGC Clinical Practice Guidelines Committee Opinion. March 1997: 15. Healthy Beginnings: Guidelines for Care during Pregnancy and Childbirth. SOGC Clinical Practice Guidelines Policy Statement. December 1998: 71. Hennessey MH, Rayburn WF, Stewart JD, Likes EC. Preeclampsia and induction of labour: a randomized comparision of prostaglandin E2 as an intracervical gel, with oxytocin immediately, or as a sustained – release vaginal insert. AMJ Obstet Gynecol. 1998. 179(5): 1204-9. Hod M, Bar J, Peled Y, Fried S, Katz I, Itzhak M, Ashkenazi S, Schindel B, Ben-Rafael Z. Antepartum management protocol. Timing and mode of delivery in gestational diabetes. Obstet Gynecol. 2009 Jan;113(1):206-17. Howarth GR, Botha DJ. (2002). Amniotomy plus intravenous oxytocin for induction of labour (Cochrane Review). The Cochrane Library, Issue 2. Kelly AJ, Tan B. (2002). Intravenous oxytocin alone for cervical ripening and induction of labour (Cochrane Review). The Cochrane Library, Issue 2. Kent, N. Prevention and Treatment of Venous Thromboembolism (VTE) in Obstetrics. SOGC Clinical Practice Guidelines. September 2000: 95. Koren, G. Motherisk Update: Caffeine Diring Pregnancy? Canadian Family Physician. April 2000. 46 : 801-3. Kotaska et al. Vaginal Delivery of Breech Presentation. SOGC Clinical Practice Guidelines. June 2009. No. 226; 557 – 566. Langlois S, Ford J, and Chitayat D. Carrier Screening for Thalassemia and hemoglobinopathies in Canada. SOGC Technical Update. Oct 2008, No 217; 950-959. Langlois S, Wilson R. Carrier Screening for Genetic Disorders in Individuals of Ashkenazi Jewish Descent. SOGC Clinical Practice Guidelines. Apr 2006, No 177; 324-332. Ling, F. and Duff, P. Obstetrics and Gynecology, Principles for practice. USA: McGraw-Hill Companies, 2002. Luckas M, Bricker L. (2000). Intravenous prostaglandin for induction of labour (Cochrane Review). The Cochrane Library, Issue 2. Meltwer, S., Leiter, L., Daneman, D., Gerstein, HC., Lau, D., Ludwig S., Yale, JF., Zinman, B., Lillie, D. 1998 Clinical Practice Guidelines for the Management of Diabetes in Canada. CMAJ. 1998, 159 (B suppl): S1-29. Menezes EV, Yakoob MY, Soomro T, Haws RA, Darmstadt GL, Bhutta ZA. (2009) Reducing stillbirths: prevention and management of medical disorders and infections during pregnancy. BMC Pregnancy Childbirth. 2009; 9(Suppl 1): S4. Midmer D, Biringer A, Carroll JC, Reid AJ, Wilson L, Stewart D, Tate M, Chalmers B. A Reference Guide for Providers: The ALPHA Form – Antenatal Psychosocial Health Assessment Form, 2nd Edition. Toronto: University of Toronto, Faculty of Medicine, Department of Family and Community Medicine, 1996. Ministry of Health and Long Term Care and Canadian Medical Association. Antenatal Record 1. Ontario. Ministry of Health and Long Term Care and Canadian Medical Association. Antenatal Record 2. Ontario. Money, D and Dobson, S. The prevention of early-onset group B streptococcal disease. SOGC Clinical Practice Guidelines. September 2004. Lowder JL, Burrows LJ, Krohn MA, Weber AM. Risk factors for primary and subsequent anal sphincter lacerations: a comparison of cohorts by parity and prior mode of delivery. Am J Obstet Gynecol. 2007 Apr;196(4):344.e1-5. Mount Sinai Hospital. First Trimester Combined Screening Program. 2001. North York General Hospital Genetics Program. Integrated Prenatal Screening. 1999. Ottinyer WS, Menara MK, Brost BC. A randomized control trial of prostaglandin E2 intracervical gel and a slow release vaginal for preinduction cervical ripening. AMJ Obstet Gynecol. 1998. 179(2): 349-53. Schuurmans, N., MacKinnon, C., Lane C., Etches, D. Prevention and Management of Postpartum Hemorrhage. SOGC Clinical Practice Guidelines. April 2000: 88. SOGC. Fetal Health Surveillance: Antepartum and Intrapartum Consensus Guideline, Sept. 2007. SOGC. Conservative Management of Urinary Incontinence, No. 186. Dec 2006. SOGC. Midurethral Minimally Invasive Sling Procedures for Stress Urinary Incontinence. No 213. Aug 2008. Statistics Canada. 2005. Induced Abortion Statistics, 82-223-XWE, page 16 of 32. Stewart, D. A broader context for maternal mortality. CMAJ, Jan 31 2006, 174 (3); 302 – 303. Stewart JD, Rayburn WF, Farmer KC, Ciles EM, Schipaul AH Jr., Stanley JR. Effectiveness of prostaglandin E2 intracervical gel (prepidil) with immediate oxytocin vs. vaginal insert (cervidil) for induction of labour. AMJ Obstet Gynecol 1998. 179: 1175-80. Summers A, Langlois S, Wyatt P, Wilson D. Prenatal Screening for Fetal Aneuploidy. SOGC Clinical Practice Guidelines. Feb 2007, No 187; 146-161. Van den Hof, M. and Crane, J. Ultrasound Cervical Assessment in Predicting Preterm Birth. SOGC Clinical Practice Guidelines. May 2001: 102.
OP
Ophthalmology Faazil Kassam, Kay Lam and Jenna Tessolini, chapter editors Alaina Garbens and Modupe Oyewumi, associate editors Adam Gladwish, EBM editor Dr. Wai-Ching Lam, staff editor Basic Anatomy Review . . . . . . . . . . . . . . . . . . . . 2 Differential Diagnoses of Common Presentations . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 Ocular Emergencies . . . . . . . . . . . . . . . . . . . . . . 5 The Ocular Examination . . . . . . . . . . . . . . . . . . . 5 Optics. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 The Orbit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 Globe Displacement Preseptal Cellulitis Orbital Cellulitis Lacrimal Apparatus. . . . . . . . . . . . . . . . . . . . . . 11 Dry Eye Syndrome Dacryocystitis Epiphora (Tearing) Dacryoadenitis Lids and Lashes . . . . . . . . . . . . . . . . . . . . . . . . 13 Lid Swelling Hordeolum (Stye) Ptosis Chalazion Trichiasis Blepharitis Entropion Xanthelasma Ectropion Conjunctiva . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Pinguecula Pterygium Subconjunctival Hemorrhage Conjunctivitis Sclera . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 Episcleritis Scleritis Cornea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 Foreign Body Herpes Zoster Corneal Abrasion Keratoconus Recurrent Erosions Arcus Senilis Corneal Ulcer Kayser-Fleischer Ring Herpes Simplex
Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Central Retinal Artery Occlusion (CRAO) Branch Retinal Artery Occlusion (BRAO) Central/Branch Retinal Vein Occlusion (CRVO/BRVO) Retinal Detachment (RD) Retinitis Pigmentosa Age-Related Macular Degeneration (ARMD) Glaucoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Primary Open Angle Glaucoma (POAG) Normal Pressure Glaucoma Secondary Open Angle Glaucoma Primary Angle Closure Glaucoma (PACG) Secondary Angle Closure Glaucoma Pupils . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 Pupillary Light Reflex Pupil Abnormalities Dilated Pupil (Mydriasis) Constricted Pupil (Miosis) Relative Afferent Pupillary Defect (RAPD) Malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 Lid Carcinoma Malignant Melanoma Metastases Ocular Manifestations of Systemic Disease. . . 34 HIV/AIDS Other Systemic Infections Diabetes Mellitus (DM) Hypertension Multiple Sclerosis (MS) TIA/Amaurosis Fugax Graves’ Disease Connective Tissue Disorders Giant Cell/Temporal Arteritis (GCA) Sarcoidosis
Lens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Cataracts Dislocated Lens (Ectopia Lentis)
Pediatric Ophthalmology . . . . . . . . . . . . . . . . . 38 Strabismus Amblyopia Leukocoria Retinoblastoma Retinopathy of Prematurity (ROP) Nasolacrimal System Defects Ophthalmia Neonatorum Congenital Glaucoma
Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 Posterior Vitreous Detachment (PVD) Vitreous Hemorrhage Endophthalmitis and Vitritis
Ocular Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Blunt Trauma Blow-Out Fracture Penetrating Trauma Chemical Burns Hyphema
The Uveal Tract . . . . . . . . . . . . . . . . . . . . . . . . . 20 Uveitis Anterior Uveitis (Iritis) Posterior Uveitis (Choroiditis)
Surgical Ophthalmology. . . . . . . . . . . . . . . . . . 43 Ocular Drug Toxicity . . . . . . . . . . . . . . . . . . . . . 44 Common Medications. . . . . . . . . . . . . . . . . . . . 44 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46
Toronto Notes 2011
Ophthalmology OP1
OP2 Ophthalmology
Basic Anatomy Review
Toronto Notes 2011
Basic Anatomy Review Lateral View
Tendon of superior rectus muscle Retina Choroid Ciliary muscle and body
Optic nerve
Bulbar conjunctiva
Tendon of medial rectus muscle
Retina
Lens
Palpebral conjunctiva
Lens
Anterior chamber Iris Bulbar conjunctiva
Tendon of lateral rectus muscle
Sclera
Eyelash Cornea
Cornea Ciliary muscle and body
Choroid Sclera
Retinal blood vessels
Fovea
Optic nerve Retinal blood vessels
Tendon of inferior rectus muscle
Conjunctival fornix
© Jenn Tse 2006
Meibomian gland
Superior View
Figure 1. Anatomy of the Eye RETINAL LAYERS (10) 1. Inner limiting membrane 2. Nerve fibre layer 3. Ganglion cell layer
LIGHT RAYS
CELL TYPES Vitreous humour Optic nerve fibres Ganglion cells
4. Inner plexiform layer Amacrine cells 5. Inner nuclear layer
Bipolar cells Horizontal cells
6. Outer plexiform layer
7. Outer nuclear layer 8. External limiting layer 9. Photoreceptor layer 10. Retinal pigment epithelium
Rod nuclei Cone nuclei Rod cells Cone cells
Pigment cells Bruch’s membrane Choroid © Sarah A. Kim 2005
Figure 2. Layers of the Retina
Toronto Notes 2011
Basic Anatomy Review/Differential Diagnoses of Common Presentations
Ophthalmology OP3
Lacrimal gland
Lacrimal canaliculus
Meibomian gland
Fundus of lacrimal sac Nasolacrimal duct
© Jenn Tse 2006
Inferior concha
Figure 3. Tear Drainage from the Eye (Lacrimal Apparatus)
Differential Diagnoses of Common Presentations Loss of Vision Loss of Vision Transient (seconds to hours) • Transient ischemic attack (TIA) • Migraine with aura
Acute (seconds to days)
Cornea/Anterior Segment • Corneal edema • Hyphema • Acute angleclosure glaucoma • Trauma/foreign body
Vitreous/Retina/ Cortical/Other Optic Nerve • Occipital • Vitreous infarction/ hemorrhage hemorrhage • Retinal • Cortical detachment blindness • Retinal artery/ • Functional vein occlusion (non-organic, • Acute macular diagnosis of lesion exclusion) • Optic neuritis • Temporal arteritis • Anterior ischemic optic neuropathy (AION)
Figure 4. Loss of Vision
Red Eye • lids/orbit/lacrimal system hordeolum/chalazion blepharitis foreign body/laceration dacryocystitis/dacryoadenitis • conjunctiva/sclera subconjunctival hemorrhage conjunctivitis dry eyes pterygium episcleritis/scleritis preseptal/orbital cellulitis • cornea foreign body keratitis abrasion, laceration ulcer
Chronic (weeks to months)
Cornea/Anterior Segment • Corneal dystrophy/ scarring/edema • Refractive error • Cataract • Glaucoma
Vitreous/Retina/ Cortical/Other Optic Nerve • Pituitary • Age-related adenoma macular • Medicationdegeneration induced (ARMD) (sildenafil, • Diabetic amiodarone) retinopathy • Nutritional • Retinal vascular deficiency insufficiency • Papilledema • Compressive optic neuropathy (intracranial mass, orbital mass) • Intraocular neoplasm • Retinitis pigmentosa (RP)
Top 3 in DDx of Acute Loss of Vision 1. Trauma/foreign body 2. Retinal artery/vein occlusion 3. Retinal detachment
Top 3 in DDx of Chronic Loss of Vision Reversible 1. Cataract 2. Refractive error 3. Corneal dystrophy Irreversible 1. Age-related macular degeneration 2. Glaucoma 3. Diabetic retinopathy
OP4 Ophthalmology
Differential Diagnoses of Common Presentations
Toronto Notes 2011
• anterior chamber uveitis (iritis, iridocyclitis) acute angle-closure glaucoma hyphema hypopyon • endophthalmitis Table 1. Common Differential Diagnosis of Red Eye
Not every red eye has conjunctivitis.
Conjunctivitis
Acute Iritis
Acute Angle Closure Glaucoma
Keratitis
Discharge
Bacteria: purulent Virus: serous Allergy: mucous
No
No
Profuse tearing
Pain
No
++ (tender globe)
+++ (nauseating)
++ (on blinking)
Photophobia
No
+++
+
++
Blurred Vision
No
++
+++
Varies
Pupil
Normal
Smaller
Fixed in mid-dilation
Same or smaller
Injection
Conjunctiva with limbal pallor
Ciliary flush
Diffuse
Diffuse
Cornea
Normal or opacified
Keratic precipitates
Steamy
Infiltrate, edema, epithelial defects
Intraocular pressure
Normal
Varies
Increased markedly
Normal or increased
Anterior chamber
Normal
Cells + flare
Shallow
Cells + flare or normal
Other
Large, tender pre-auricular Posterior synechiae (auricular) node if viral
Coloured halos Nausea and vomiting
Ocular Pain • differentiate from ocular ache: eye fatigue (asthenopia) • herpes zoster prodrome • trauma/foreign body • keratitis • corneal abrasion, corneal ulcer • acute angle-closure glaucoma • acute uveitis • scleritis, episcleritis • optic neuritis • ocular migraine Floaters • vitreous syneresis (shrinkage and collapse of vitreous gel) • posterior vitreous detachment (PVD) • vitreous hemorrhage • retinal tear/detachment • posterior uveitis Flashes of Light (Photopsia) • posterior vitreous detachment (PVD) • retinal tear/detachment • migraine with aura Photophobia (Severe Light Sensitivity) • corneal abrasion, corneal ulcer • keratitis • acute angle-closure glaucoma • iritis • meningitis, encephalitis • migraine Diplopia (Double Vision) • binocular diplopia: strabismus, CN palsy (III, IV, VI) secondary to ischemia, diabetes, tumour, trauma, myasthenia gravis, muscle restriction/entrapment, thyroid ophthalmopathy, internuclear ophthalmologia (INO) 2o to multiple sclerosis, brainstem infarct • monocular diplopia: refractive error, strands of mucus in tear film, keratoconus, cataracts, dislocated lens, peripheral iridotomy
Toronto Notes 2011
Differential Diagnoses of Common Presentations/Ocular Emergencies/Ocular Examination
Ophthalmology OP5
Ocular Problems in the Elderly • blepharitis • ptosis • entropion, ectropion • dry eyes, epiphora (excessive tearing) • presbyopia • cataracts • glaucoma • age-related macular degeneration • retinal artery/vein occlusion • temporal arteritis (arteritic ischemic optic neuropathy) Ocular Problems in the Contact Lens Wearer • superficial punctate keratitis (SPK)/dry eyes • solution hypersensitivity • tight lens syndrome • corneal abrasion • giant papillary conjunctivitis • sterile corneal infiltrates (immunologic) • infected ulcers (Pseudomonas, Acanthamoeba)
Ocular Emergencies These require urgent consultation to an ophthalmologist for management Sight Threatening • lid/globe lacerations • corneal ulcer • gonococcal conjunctivitis • acute iritis • acute angle-closure glaucoma • central retinal artery occlusion (CRAO) • intraocular foreign body • retinal detachment (especially macula threatening) • endophthalmitis Life Threatening • proptosis (rule out cavernous sinus fistula or thrombosis) • CN III palsy with dilated pupil (intracranial aneurysm or neoplastic lesion) • papilledema (must rule out intracranial mass lesion) • orbital cellulitis • giant cell (temporal) arteritis • leukocoria – white pupil (must rule out retinoblastoma)
The Ocular Examination Note: Sometimes vision may be blurry secondary to eye drops/ointment/mucus or applying too much pressure when patching. Encourage the patient to blink before starting the exam and wait until the patient’s vision is clear before continuing. VISION ASSESSMENT • always test visual acuity first • test best corrected visual acuity (BCVA) whenever possible (i.e. corrective lenses) • test each eye individually, starting with the right eye, and covering the untested eye • assess distance and near vision • improvement with a pinhole indicates an uncorrected refractive error Visual Acuity – Distance • Snellen Fraction (Figure 5) = testing distance (usually 20 feet or 6 metres) smallest line patient can read on the chart • e.g. 20/40 = what the patient can see at 20 feet (numerator), a “normal” person can see at 40 feet (denominator)
Example 1 SC 20/40 -1 20/80 +2 20/25 PH
V
Example 2 CC CF 3' HM
V
Note: RIGHT EYE visual acuity always listed on top. V Vision SC Without correction CC With correction 20/40 -1 All except one letter of 20/40 20/80+2 All of 20/80 plus two letters of 20/70 PH Visual acuity with pinhole correction CF Counting fingers HM Hand motion
Figure 5. Ophthalmology Nomenclature for Visual Acuity
OP6 Ophthalmology
• OD = oculus dexter = right eye • OS = oculus sinister = left eye • OU = oculus uterque = both eyes
A Snellen visual acuity of 20/20 equates to “normal” vision.
Infant and Child Visual Acuity • 6-12 months – 20/120 • 1-2 years – 20/80 • 2-4 years – 20/20
CF CF CF CF
CF CF
RIGHT EYE fields drawn on right side; LEFT EYE fields drawn on left side (as if seen through patient’s eyes). CF Able to count fingers in specified quadrant with peripheral vision Gross visual field deficit in specified quadrant using peripheral vision
Figure 6. Ophthalmology Nomenclature for Visual Fields by Confrontation
For patients with dark irises, test the pupils using an ophthalmoscope focused on the red reflex. This will provide a better view than using a penlight.
The Ocular Examination
Toronto Notes 2011
• testing hierarchy for low vision: Snellen acuity (20/x) counting fingers at x distance (CF) hand motion (HM) light perception with projection (LP with projection) light perception (LP) no light perception (NLP) • legal blindness is BCVA that is ≤20/200 in the better eye, or a limit to the binocular central field of vision 2/3 of nasal side of iris in shadow (Figure 9) EXTRAOCULAR MUSCLES
Changing fixation from distance to near results in the “near reflex”: 1. Eye convergence 2. Pupil constriction 3. Lens accommodation
Alignment • Hirschberg corneal reflex test examine in primary position of gaze (e.g. straight ahead) with patient focusing on distant object shine light into patient’s eyes from ~30 cm away corneal light reflex should be symmetric and at the same position on each cornea • strabismus testing as indicated (cover test, cover-uncover test, prism testing) (see Strabismus, OP38)
Toronto Notes 2011
The Ocular Examination
Ophthalmology OP7
Movement • examine movement of eyeball through six cardinal positions of gaze (Figure 8) • ask patient if diplopia is present in any position of gaze • observe for horizontal, vertical or rotatory nystagmus (rhythmic, oscillating movements of the eye) • resolving horizontal nystagmus at end gaze is usually normal • see side box for cranial nerve innervation of extraocular muscles EXTERNAL EXAMINATION • the four L’s lymph nodes (preauricular, submandibular) lids lashes lacrimal system
SR
IO
LR
IO
SR
MR MR IR
SO
LR SO
IR
© Sherry H. Lai 2006
Figure 8. Diagnostic Positions of Gaze to Isolate Primary Action of Each Muscle
• CN III – Superior, Medial and Inferior Rectus, Inferior Oblique • CN IV – Superior Oblique (SO) • CN VI – Lateral Rectus (LR)
SLIT-LAMP EXAMINATION Schematic drawing of the slit lamp
The ophthalmology note: Slit lamp exam ok injected 1+ edema 2+ cells ok 2+ NS
4 9
5
11 12
8 2 3
ok ok clear d+q ok ok
Eyelids/Eyelashes Conjunctiva/Sclera/Episclera Cornea/Iris/Anterior surface of lens
6 7
10
LLL SC K AC Iris Lens
Deep
Note: RIGHT EYE drawn on the left, LEFT EYE drawn on the right (as if looking at patient's face). LLL SC K AC d+q NS
Light source
Lids, lashes, lacrimal Sclera, conjunctiva Cornea Anterior chamber Deep (not shallow) and quiet (no cells in AC) Nuclear sclerosis (cataract)
1 Any abnormality or pathology is drawn on the sketch in the appropriate location, and is labelled (e.g. trichiasis, conjunctivitis/ episcleritis/scleritis, corneal abrasion/ulcer, foreign body, etc.)
Shallow Light source
© Doris Leung
Power switch (on/off) Slit lamp joystick control Locking knob Ocular Magnification adjustment knob Brightness adjustment lever Slit beam height adjustment knob Slit beam width adjustment knob Patient-positioning frame Forehead strap Patient chin rest Chin rest height adjustment knob
© Janice Wong and Danielle Bader
1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12.
Figure 7. Slit-Lamp
• systematically examine all structures of the anterior segment + anterior vitreous lids (including upper lid eversion if necessary), lashes, and lacrimal system conjunctiva and sclera cornea iris
Figure 9. Estimation of Anterior Chamber Depth
Central Corneal Thickness Average CCT = 550 µm A thick cornea overestimates IOP by GAT A thin cornea underestimates IOP by GAT
OP8 Ophthalmology
T
16 14
16
Ocular Examination/Optics
14
Note: RIGHT EYE lOP always listed on top. Note method used to measure lOP (Goldmann, Tonopen, airpuff).
Figure 10. Tonometry
Desired Myers Pattern on GAT
Note: Thick Myers overestimate the IOP and are a result of excess fluorescein
Quick Tips on Direct Ophthalmoscopy 1. Examine in a dark room 2. Ask patient to focus on a distant object 3. Match ophthalmoscope light aperture to size of pupil (i.e. smaller aperture for undilated eye) 4. Use moderate light intensity 5. Use your left/right eye and hand to examine patient’s left/right eye respectively 6. Get in close! Proximity to patient’s eye is key with hand resting on patient’s cheek
N D/M/V (normal disc, macula, vessels)
x
x
C:D 0.3
C:D 0.4
Note: RIGHT EYE drawn on the left, LEFT EYE drawn on the right (as if looking at patient's face). C:D Cup:Disc ratio x Fovea Any abnormality or pathology of the fundus is drawn on the sketch in the appropriate location, and is labelled (e.g. hemorrhages, neovascularization, cotton-wool spots, drusen, retinal tear/detachment, etc.).
Figure 11. Fundus
Structures Responsible for Refractive Power 1. Cornea (2/3) 2. Lens (1/3)
Diopter (D) = measurement of refractive power of a lens, equal to the reciprocal of the focal length in meters “Negative” lens = concave, corrects for myopia “Positive” lens = convex, corrects for hyperopia
Toronto Notes 2011
anterior chamber (for depth, cells and flare) to observe cells and flare 1. Dark room 2. High power beam 3. 1 mm beam height 4. Thin beam 5. Highest magnification 6. Approach at angle and focus on anterior chamber (space between cornea and lens) lens anterior vitreous • when necessary, use: fluorescein dye – stains Bowman’s membrane in de-epithelialized cornea; dye appears green with cobalt blue filterered light Rose Bengal dye – stains devitalized corneal epithelium • special lenses (78 or 90 diopter) used with the slit-lamp allow a binocular, stereoscopic view of the fundus and vitreous TONOMETRY • measurement of intraocular pressure (IOP) (Figure 10) • normal range is 10-21.5 mmHg (average 15 mmHg) • commonly measured by: Goldmann applanation tonometry (GAT) – gold standard, performed using the slit-lamp with special tip (prism) Tonopen – benefit is portability and use of disposable probe tips. Use when cornea is scarred/assymetric (GAT inaccurate) air puff (non-contact and least reliable) • use topical anesthetic for Goldmann and Tonopen OPHTHALMOSCOPY/FUNDOSCOPY • performed with: direct ophthalmoscope (monocular with small field of view, only posterior pole visualized) slit-lamp with 78 or 90 diopter lens (binocular view, visualization to mid-periphery of retina) indirect ophthalmoscopy with headlamp and 20 or 28 diopter lens (binocular view, visualization of entire retina to ora serrata/edge of retina) • best peformed with pupils dilated (see Table 8 for list of mydriatics and cycloplegics) 1. assess red reflex light reflected off the retina produces a “red reflex” when viewed from ~1 foot away anything that interferes with the passage of light will diminish the red reflex (e.g. large vitreous hemorrhage, cataract) 2. examine the posterior segment of the eye (Figure 11) vitreous optic disc (colour, cup:disc ratio, sharpness of disc margin) macula (~2 disc diameters temporal to disc), fovea (foveal light reflex) retinal vessels retinal background • contraindications to pupillary dilatation shallow anterior chamber – can precipitate acute angle-closure glaucoma iris-supported anterior chamber lens implant potential neurologic abnormality requiring pupil evaluation use caution with cardiovascular disease – mydriatics may cause tachycardia
Optics REFRACTION • two techniques used Flash/Streak Retinoscopy – refractive error determined objectively by the examiner by use of lenses and retinoscope Manifest – subjective trial using phoropter (device the patient looks through that is equipped with lenses) • a typical lens prescription would contain: sphere power in diopters (D), negative lens for myopes, positive lens for hyperopes cylinder power (in D) to correct astigmatism (always positive value), axis of cylinder in degrees “add” (bifocal/progressive reading lens) for presbyopes e.g. -1.50 + 1.00 x 120 degrees, add +2.00
Toronto Notes 2011
Optics
Ophthalmology OP9
REFRACTIVE EYE SURGERY • permanently alters corneal refractive properties by ablating tissue to change curvature of the cornea • used for correction of myopia, hyperopia, and astigmatism • common types include photorefractive keratectomy (PRK) and LASIK (see Surgical Ophthalmology, OP43) • potential risks/side-effects: infection, undercorrection/overcorrection, decreased night vision, corneal haze, dry eyes, regression, complete sever of corneal flap (LASIK only) Table 2. Optics Pathophysiology
Clinical Features
Treatment
Complications
Emmetropia
• Image of distant objects focus • No refractive error exactly on the retina (Figure 12)
Myopia
• Globe too long relative to • “Nearsightedness” • Correct with • Retinal tear/ refractive mechanisms, or • Usually presents in 1st or negative diopter/ detachment, refractive mechanisms too 2nd decade, stabilizes in concave/”negative” macular hole, strong 2nd and 3rd decade; rarely lenses to diverge light open angle • Light rays from distant object begins after age 25 except in rays (Figure 13) glaucoma focus in front of retina g blurring patients with diabetes • Refractive eye surgery • Complications of (distance) vision (Figure 12) or cataracts not prevented • Blurring of distance with refractive correction vision; near vision usually unaffected • Prevalence of 30-40% in U.S. population
Hyperopia
Astigmatism
Presbyopia
F
• Globe too short relative to • “Farsightedness” • When symptomatic, • Angle-closure refractive mechanisms, or • Youth: usually do not require correct with positive glaucoma, refractive mechanisms too glasses (still have sufficient Diopter/convex/”plus” particularly later weak accommodative ability to lenses to converge light in life as lens • Light rays from distant object focus image on retina), rays (Figure 13) enlarges focus behind retina g blurring of but may develop • Refractive eye surgery near ± distant vision (Figure 12) accommodative esotropia • May be developmental or due (see Strabismus, OP38) to any etiology that shortens • 30s-40s: blurring of near globe vision due to decreased accommodation, may need reading glasses • >50s: blurring of distance vision due to severely decreased accommodation • Light rays not refracted uniformly • Affects approximately 30% • Correct with cylindrical in all meridians due to of population, with lens (if regular), non-spherical surface of corneas prevalence increasing try contact lens (if or non-spherical lens (e.g. with age irregular) football-shaped) • Mild astigmatism • Refractive eye surgery • Two types: unnoticeable Regular – curvature • Higher amounts of uniformly different in astigmatism may cause meridians at right angles to blurry vision, squinting, each other asthenopia, or headaches Irregular – distorted cornea caused by injury, keratoconus (cone-shaped cornea), corneal scar, or severe dry eye
F
Myopia F
Hyperopia
Figure 12. Emmetropia and Refractive Errors
F Hyperopia corrected with positive converging lens
F Myopia corrected with negative diverging lens
Figure 13. Correction of Refractive Errors
• Normal aging process • If initially emmetropic, • Correct with positive (>40 years) person begins to hold diopter/convex/”plus” • Hardening/reduced deformability reading material farther lenses for reading of the lens results in decreased away, but distance vision accommodative ability remains unaffected • Accommodative power is 14D at • If initially myopic, person age 10, diminishes to 3.5D by 40 removes distance glasses to • Near images cannot be focused read onto the retina (focus is behind • If initially hyperopic, the retina as in hyperopia) symptoms of presbyopia occur earlier
Anisometropia • Difference in refractive errors between eyes
Emmetropia
• Second most common cause of amblyopia in children
OP10 Ophthalmology
The Orbit
Toronto Notes 2011
The Orbit Globe Displacement Table 3. Exophthalmos (proptosis) and Enophthalmos Exophthalmos (proptosis)
Enophthalmos
Definition
• Anterior displacement (protrusion) of the globe Exophthalmos generally refers to an endocrine etiology or protrusion of >18 mm (as measured by a Hertel exophthalmometer) Proptosis generally refers to other etiologies (e.g. cellulitis) or protrusion of 0.6, C:D ratio difference between eyes >0.2, or cup approaches disc margin • loss of peripheral vision most commonly precedes central loss • sequence of events: gradual pressure rise increased C:D ratio visual field loss • screening tests should include medical and family history visual acuity testing slit lamp exam to assess anterior chamber depth ophthalmoscopy to assess the disc features tonometry by applanation or indentation to measure the IOP visual field testing
OP28 Ophthalmology
Glaucoma Optic nerve head damage
Toronto Notes 2011 Visual field changes
10
20
Pallor and cupping of optic disc (C:D ratio 0.2-0.3) Small paracentral scotoma
Concentric enlargement (C:D ratio 0.5)
10
20
Arcuate defect
Superior expansion 20
Temporal central island
Advanced/total cupping
© Diana Dai 2005
10
Figure 19. Glaucomatous Damage Open- and Closed-Angle Glaucoma POAG PACG • Common (95%) • Rare (5%) • Chronic course • Acute onset • Painless eye • Painful red eye without redness • Extremely Ó IOP • Moderately Ó IOP • Hazy cornea • Normal cornea • Mid-dilated and pupil pupil unreactive • No N/V to light • No halos around • ± N/V, light abdominal pain • Halos around light
Risk Factors for POAG A FIAT Age Family History IOP African descent Thin Cornea
Contraindications to Dilating • Neurological abnormality requiring pupil assessment • Shallow anterior chamber • Iris-supported anterior chamber IOL
Primary Open Angle Glaucoma (POAG) • most common form, >95% of all glaucoma cases • due to obstruction of aqueous drainage within the trabecular meshwork and its drainage into the Canal of Schlemm • insidious and asymptomatic, so screening is critical for early detection Major Risk Factors • elevated intraocular pressure (>21 mmHg) • age: prevalence in 40 y.o. is 1-2% and in 80 y.o. 10% • ethnicity: African descent • familial (2-3x increased risk); polygenic Minor Risk Factors • myopia • hypertension • diabetes • hyperthyroidism (Graves’ disease) • chronic topical ophthalmic steroid use in steroid responders – yearly eye exams recommended if >4 weeks of steroid use • previous ocular trauma • anemia/hemodynamic crisis (ask about blood transfusions in past) Clinical Features • asymptomatic initially • insidious, painless, gradual rise in IOP due to restriction of aqueous outflow • bilateral, but usually asymmetric • earliest signs are optic disc changes increased cup:disc ratio (vertical C:D >0.6) significant cup:disc asymmetry between eyes (>0.2 difference)
Toronto Notes 2011
Glaucoma
thinning, notching of the neuroretinal rim flame shaped disc hemorrhage 360º of peripapillary atrophy nerve fibre layer defect large vessels become nasally displaced • visual field loss slow, progressive, irreversible loss of peripheral vision paracentral defects, arcuate scotoma and nasal step are characteristic late loss of central vision if untreated Treatment • medical treatment: decrease IOP by increasing the drainage and/or decreasing the production of aqueous (see Glaucoma Medications, Table 10, OP45) increase aqueous outflow topical cholinergics topical prostaglandin analogues topical alpha-adrenergics decrease aqueous production topical beta-blockers topical and oral carbonic anhydrase inhibitor topical alpha-adrenergics • laser trabeculoplasty, cyclophotocoagulation = selective destruction of ciliary body (for refractory cases) • trabeculectomy (see Surgical Ophthalmology, OP43) • optic nerve head examination, IOP measurement and visual field testing to monitor course of disease • pachymetry to measure corneal thickness
Normal Pressure Glaucoma • POAG with IOP in normal range • often found in women >60 but may occur earlier • damage to optic nerve may be due to vascular insufficiency Treatment • treat any causative underlying medical condition and lower the IOP further
Secondary Open Angle Glaucoma • increased IOP secondary to ocular/systemic disorders that clog the trabecular meshwork steroid-induced glaucoma traumatic glaucoma pigmentary dispersion syndrome pseudoexfoliation syndrome
Primary Angle Closure Glaucoma • 5% of all glaucoma cases • peripheral iris bows forward in an already susceptible eye with a shallow anterior chamber obstructing aqueous access to the trabecular meshwork • sudden shifting forward of the lens-iris diaphragm = pupillary block, results in inability of the aqueous to flow from the posterior chamber to the anterior chamber and a sudden rise in IOP (Figure 20) Risk Factors • hyperopia: small eye, big lens – large lens crowds the angle • age >70 • female • family history • more common in people of Asian and Inuit descent • mature cataracts • shallow anterior chamber • pupil dilation (topical and systemic anticholinergics, stress, darkness)
Ophthalmology OP29
Reduction of Intraocular Pressure and Glaucoma Progression Arch Ophthalmol 2002; 120:1268-1279 Study: Randomized controlled clinical trial. Patients: 255 participants, mainly selected through a population screening protocol, aged 50-80 with newly detected open-angle glaucoma, visual field defects, and a median intraocular pressure (IOP) of 20 mmHg. Intervention: Participants were randomized to either topical beta-blocker (betaxolol) plus argon laser trabeculoplasty or no initial treatment, with close observation for both groups. Median follow-up was 6 years. Main Outcome: Glaucoma progression as defined by visual field and optic disc abnormalities. Results: IOP was reduced by 25% (mean 5.1 mmHg) in the treatment group. Glaucoma progression was evident in 62% of individuals in the control group vs. only 45% in the treatment group (p=0.007). The progression was significantly later in the treatment group vs. the controls.
Rule of Fours 1/4 of general population after using 4 weeks of topical steroid 4x/day will develop an increase in IOP.
Medical Interventions for Primary Open Angle Glaucoma and Ocular Hypertension Cochrane Database of Systematic Reviews 2007, Issue 4. Study: Cochrane systematic review of 26 trials and meta-analysis of 10 trials investigating the effectiveness of topical pharmacological therapies for primary open angle glaucoma (POAG) or ocular hypertension (OHT). Patients: 4979 participants randomized in 26 trials. Patients had OHT with intraocular pressure (IOP) >21 mmHg or open angle glaucoma. Intervention: topical eye medications, including beta-blockers, dorzolamide, brimonidine, pilocarpine and epinephrine versus each other and placebo. Main outcome: Reduction of progression or prevention of onset of visual field defects. Results: Meta-analysis on all trials that tested drugs against placebo or untreated controls demonstrated that lowering IOP reduces incidence of glaucomatous visual field defects, with an odds ratio of 0.62 (95% CI 0.47-0.81). However, this result is of limited practical use since different therapies were pooled. No single drug demonstrated significant visual field protection. However, as a class, beta-blockers showed borderline significance in reducing onset of glaucoma in patients with OHT when compared to placebo, with an OR of 0.67 (95% CI 0.45-1.00). Conclusions: Lowering IOP can reduce progression of visual field defects in patients with OHT.
OP30 Ophthalmology A. Open angle with normal aqueous flow
3
Glaucoma/Pupils
B. Closed angle with abnormal aqueous flow
5 4
1 1. Aqueous flow 2. Ciliary body 3. Cornea 4. Lens 5. Blocked trabecular meshwork
© Simon Ip 2009
2
Figure 20. Normal Open Angle versus Angle Closure Glaucoma
Angle Closure Glaucoma BACH Tx with miotics and Beta-Blockers, Adrenergics, Cholinergics Hyperosmotic agents
Toronto Notes 2011
Clinical Features • unilateral, but other eye predisposed • red, painful eye = RED FLAG • decreased visual acuity, vision acutely blurred from corneal edema • halos around lights • nausea and vomiting, abdominal pain • fixed, mid-dilated pupil • corneal edema with conjunctival injection • marked increase in IOP; may be noticeable even to palpation (>40 mmHg) • shallow anterior chamber ± cells in anterior chamber Complications • irreversible loss of vision within hours to days if untreated • permanent peripheral anterior synechiae Treatment • refer to ophthalmologist laser iridotomy aqueous suppressants and hyperosmotic agents • immediate treatment important to: preserve vision prevent adhesions of peripheral iris to trabecular meshwork (peripheral anterior synechiae) resulting in permanent closure of angle • medical treatment (see Glaucoma Medications, Table 10, OP45) miotic drops (pilocarpine) to reverse pupillary block decrease IOP topical beta-blockers topical adrenergics topical cholinergics – pilocarpine 1-4% q15min, up to q5min systemic carbonic anhydrase inhibitors – IV acetazolamide 250-500 mg systemic hyperosmotic agents – oral glycerine 1 g/kg – IV mannitol 1 g/kg
Secondary Angle Closure Glaucoma Uveitis • inflamed iris adheres to lens (posterior synechiae) Neovascular Glaucoma • abnormal blood vessels develop on surface of iris (rubeosis iridis), in the angle, and within the trabecular meshwork • due to retinal ischemia associated with proliferative diabetic retinopathy and CRVO • treatment with laser therapy to retina reduces neovascular stimulus to iris vessels
Pupils
5 Targets of Retinal Signals • Pre-tectal nucleus (pupillary reflex/eye movements) • Lateral geniculate body of thalamus • Superior colliculus (eye movements) • Suprachiasmatic nucleus (optokinetic) • Accessory optic system (circadian rhythm)
• pupil size is determined by the balance between the sphincter muscle and the dilator muscle • sphincter muscle is innervated by the parasympathetic nervous system (PNS) carried by CN III: pre- and post-ganglionic fibres synapse in ciliary ganglion, and use acetylcholine as the neurotransmitter • dilator muscle is innervated by the sympathetic nervous system (SNS) first order neuron = hypothalamus brainstem spinal cord second order/preganglionic neuron = spinal cord sympathetic trunk via internal carotid artery superior cervical ganglion in neck third order/postganglionic fibres originate in the superior cervical ganglion, neurotransmitter is noradrenaline as a diagnostic test, 4% cocaine prevents the re-uptake of noradrenaline, and will cause dilation of normal pupil, but not one with loss of sympathetic innervation (Horner’s Syndrome)
Toronto Notes 2011
Pupils
Ophthalmology OP31
Pupillary Light Reflex • light shone directly into eye travels along optic nerve optic tracts both sides of midbrain • impulses enter both sides of midbrain via pretectal area and Edinger-Westphal nuclei • nerve impulses then travel down CN III bilaterally to reach the ciliary ganglia, and finally to the iris sphincter muscle, which results in direct and consensual light reflex
Pupil Abnormalities Denervation Hypersensitivity • when post-ganglionic fibres are damaged, the understimulated end-organ develops an excess of receptor and becomes hypersensitive • postganglionic parasympathetic lesions (i.e. Adie’s pupil) pupil will constrict with 0.125% pilocarpine (cholinergic agonist), normal pupil will not • postganglionic sympathetic lesions (this test is used to differentiate between pre- and postganglionic lesions in Horner’s syndrome) pupil will dilate with 0.125% adrenaline, normal pupil will not Local Disorders of Iris • posterior synechiae (adhesions between iris and lens) due to iritis can present as an abnormally shaped pupil • ischemic damage [e.g. post-acute angle-closure glaucoma (ACG)] ischemic damage usually at 3 and 9 o’clock positions result in a vertically oval pupil that reacts poorly to light • trauma (e.g. post intraocular surgery) Anisocoria • unequal pupil size • idiopathic/physiologic anisocoria 20% of population round, regular, males • pupil is tonic or reacts poorly to light (both direct and consensual) but constricts with accommodation • if decreased deep tendon reflexes = Adie’s syndrome • caused by benign lesion in ciliary ganglion; results in denervation hypersensitivity of parasympathetically innervated constrictor muscle dilute (0.125%) solution of pilocarpine will constrict tonic pupil but have no effect on normal pupil • pupil eventually gets smaller than pupil of unaffected eye
Toronto Notes 2011
Pupils
Ophthalmology OP33
Trauma • damage to iris sphincter from blunt or penetrating trauma • iris transillumination defects may be apparent using ophthalmoscope or slit lamp • pupil may be dilated (traumatic mydriasis) or irregularly shaped from tiny sphincter ruptures
Constricted Pupil (Miosis) Senile Miosis • decreased sympathetic stimulation with age Parasympathetic Stimulation • local or systemic medications such as: cholinergic agents: pilocarpine, carbachol cholinesterase inhibitor: phospholine iodide opiates, barbiturates Horner’s Syndrome • see Neurology, N24 • lesion in sympathetic pathway • difference in pupil size greater in dim light, due to decreased innervation of adrenergics to iris dilator muscle • associated with ptosis, anhydrosis of ipsilateral face/neck • application of cocaine 4% (blocks reuptake of noradrenaline) to eye does not result in pupil dilation (vs. physiologic anisocoria) • hydroxyamphetamine 1% (stimulates noradrenaline release) will dilate pupil if central or preganglionic lesion, not postganglionic lesion • postganglionic lesions result in denervation hypersensitivity, which will cause pupil to dilate with 0.125% adrenaline, whereas normal pupil will not • causes: carotid or subclavian aneurysm, brainstem infarct, demyelinating disease, cervical or mediastinal tumour, Pancoast tumour, goiter, cervical lymphadenopathy, surgical sympathectomy, Lyme disease, cervical ribs, tabes dorsalis, cervical vertebral fractures
Horner’s MAP Miosis Anhydrosis Ptosis
Iritis • miotic pupil initially • later, may be irregularly shaped pupil due to posterior synechiae • later stages non-reactive to light Argyll Robertson Pupil • both pupils irregular and 80% after 15 years Type 2 DM 20% at time of diagnosis 60% after 20 years
OP36 Ophthalmology
Clinically significant macular edema is defined as thickening of the retina at or within 500 µm of the centre of the macula.
Ocular Manifestations of Systemic Disease
Toronto Notes 2011
• proliferative 5% of patients with diabetes will reach this stage neovascularization of iris, disc, retina to vitreous neovascularization of iris (rubeosis iridis) can lead to neovascular glaucoma vitreous hemorrhage from bleeding, fragile new vessels, fibrous tissue can contract causing tractional retinal detachment high risk of severe visual loss 2o to vitreous hemorrhage, retinal detachment Screening Guidelines for Diabetic Retinopathy • Type 1 DM screen for retinopathy beginning annually 5 years after disease onset screening not indicated before the onset of puberty • Type 2 DM initial examination at time of diagnosis, then annually • pregnancy ocular exam in 1st trimester, close follow-up throughout as pregnancy can exacerbate DR gestational diabetics not at risk for retinopathy
Early Treatment Diabetic Retinopathy Study Early Treatment Diabetic Retinopathy Study Investigators: Aspirin effects on mortality and morbidity in patients with diabetes mellitus. ETDRS Report 14. JAMA 1992; 268:1292-1300. And other publications by the same group ETDRS Demonstrates • No benefit of aspirin in reduction in risk of progression of diabetic retinopathy, however no increased risk of hemorrhage either • Early treatment using panretinal photocoagulation reduces the risk of visual loss • Clinically significant macula edema should be treated by focal laser
Treatment • Diabetic Control and Complications Trial (DCCT) tight control of blood sugar decreases frequency and severity of microvascular complications • blood pressure control • focal laser for clinically significant macular edema • panretinal laser photocoagulation for proliferative diabetic retinopathy: reduces neovascularization, hence reducing the angiogenic stimulus from ischemic retina by decreasing retinal metabolic demand reduces risk of blindness • vitrectomy for non-clearing vitreous hemorrhage and retinal detachment in proliferative diabetic retinopathy • vitrectomy before vitreous hemorrhage does not improve the visual prognosis Lens Changes • earlier onset of senile nuclear sclerosis and cortical cataract • may get hyperglycemic cataract, due to sorbitol accumulation (rare) • changes in blood glucose levels (poor control) can suddenly cause refractive changes by 3-4 diopters Extra Ocular Muscle (EOM) Palsy • usually CN III infarct • pupil usually spared in diabetic CN III palsy, but get ptosis • may involve CN IV and VI • usually recover within few months Optic Neuropathy • visual acuity loss due to infarction of optic disc/nerve Inner limiting membrane Nerve fibre layer
Flame shaped hemorrhage
Ganglion cell layer Inner plexiform layer
Cotton wool spots
Hypertension retinopathy
Inner nuclear layer Optic disc
Outer plexiform layer Outer nuclear layer External limiting membrane Rod and cone outer segments
Pigmented epithelium
Figure 22. DM vs. HTN Retinopathy
Dot/Blot hemorrhage
Hard Exudate
Diabetes mellitus retinopathy
© Sherry H. Lai 2006
Diabetic Control and Complication Trial NEJM 1993; 329(14) DCCT trial shows intensive glycemic control will reduce the risk of diabetic retinopathy by 76%, and reduce the risk of worsening diabetic retinopathy by 54%.
Toronto Notes 2011
Ocular Manifestations of Systemic Disease
Ophthalmology OP37
Hypertension • retinopathy is the most common ocular manifestation • key features of chronic HTN retinopathy: AV nicking, blot retinal hemorrhages, microaneurysms, cotton wool spots • key features of acute HTN retinopathy: retinal arteriolar spasm, superficial retinal hemorrhage, cotton wool spots, optic disc edema Table 7. Keith-Wagener-Barker Classification Group 1
Mild arterial narrowing
Group 2
Obvious arterial narrowing with focal irregularities
Group 3
Group 2 plus Cotton-wool spots Hemorrhage and/or exudate
Group 4
Group 3 plus papilledema
Multiple Sclerosis (MS) • see Neurology, N49 Clinical Features • blurred vision and decreased colour vision: 2o to optic neuritis • central scotoma: due to damage to papillomacular bundle of retinal nerve fibres • diplopia: 2o to internuclear ophthalmoplegia (INO) • RAPD, ptosis, nystagmus, uveitis, optic atrophy, optic neuritis • white matter demyelinating lesions of optic nerve on MRI Treatment • IV steroids with taper to oral form for optic neuritis NOT oral steroids in isolation as this increases likelihood of developing MS later
Optic Neuritis Treatment Trial (ONTT) Optic Neuritis Study Group: The Optic Neuritis Treatment Trial. Three-year follow-up results. Arch Ophthalmol 1995; 113:136-137 ONTT recruited patients with acute new onset optic neuritis and studied outcome of three treatment regimes: oral steroid x 14d, IV steroid x 3d + oral steroid x 11d, and placebo x 14d. They found that oral steroid actually increases risk of recurrence, IV + oral steroid expedite recovery, and “no treatment” a viable therapeutic option. IV + oral steroid does not decrease risk of recurrence. Furthermore, brain MRI is most valuable in prediction of onset of MS.
TIA/Amaurosis Fugax • sudden, transient blindness from intermittent vascular compromise; ipsilateral carotid most frequent embolic source • typically monocular, lasting age/2 Females > (age + 10) /2
Does this Patient have GCA? JAMA 2002; 287:92-101 Rule in: jaw claudication and diplopia on history, temporal artery beading, prominence of the artery and tenderness over the artery on exam. Rule out: no temporal artery abnormalities on exam, normal ESR.
Clinical • more common in women >60 y.o. • abrupt monocular loss of vision, pain over the temporal artery, jaw claudication, scalp tenderness, polymyalgia rheumatica, and constitutional symptoms • ischemic optic atrophy 50% lose vision in other eye if untreated Diagnosis • temporal arterial biopsy + increased ESR (ESR can be normal, but likely 80-100 in first hour), CRP • if biopsy of one side is negative, biopsy the other side Treatment • high dose corticosteroid to relieve pain and prevent further ischemic episodes • if diagnosis of GCA is suspected clinically: start treatment + perform temporal artery biopsy to confirm diagnosis within 2 weeks of intial presentation (DO NOT WAIT TO TREAT)
Sarcoidosis • granulomatous uveitis with large “mutton fat” keratitic precipitates and posterior synechiae • neurosarcoidosis: optic neuropathy, oculomotor abnormalities, visual field loss Treatment • steroids and mydriatics
Pediatric Ophthalmology Strabismus • ocular misalignment, found in 3% of children • object not visualized simultaneously by fovea of each eye • often presents with parental concern about a wandering eye, crossing eye, or poor vision • types: heterotropia (paralytic or non-paralytic), heterophoria • distinguish from pseudostrabismus (prominent epicanthal folds, hypertelorism) • complications: amblyopia, cosmesis HETEROTROPIA • manifest deviation • deviation not corrected by the fusion mechanism (i.e. deviation is apparent when the patient is using both eyes) Types • exo- (lateral deviation), eso- (medial deviation) • hyper- (upward deviation), hypo- (downward deviation) • esotropia = “crossed-eyes”; exotropia = “wall-eyed” • pseudoesotropia: epicanthal folds give appearance of esotropia but Hirschberg test is normal, more common in Asians Tests • Hirschberg test (corneal light reflex): positive if the light reflex in the cornea of the two eyes is asymmetrical light reflex lateral to central cornea indicates esodeviation; light reflex medial to central cornea indicates exodeviation false positives occur if visual axis and anatomic pupillary axis of the eye are not aligned (angle kappa) • cover test (Figure 23) ask patient to fixate on target cover the fixating eye, the deviated eye will then move to fixate on the target if deviated eye moves inward = exotropia if deviated eye moves outward = esotropia • the deviation can be quantified using prisms
Toronto Notes 2011
Pediatric Ophthalmology
Ophthalmology OP39
HETEROPHORIA • latent deviation • deviation corrected in the binocular state by the fusion mechanism (i.e. deviation not seen when patient is using both eyes) • Hirschberg test will be normal (light reflexes symmetrical) • very common – majority are asymptomatic • may be exacerbated or become manifest with asthenopia (eye strain, fatigue) Tests • cover-uncover test (Figure 23) placing a cover over an eye with a phoria causes a breakdown of fixation of that eye, which allows it to move to a misaligned position uncovering the covered eye will allow it to return to a normal central position covered eye moves inward on removing cover = exophoria covered eye moves outward on removing cover = esophoria • alternate cover test alternating the cover between both eyes reveals the total deviation, both latent and manifest maintain cover over one eye for 2-3 seconds before rapidly shifting to other eye Cover tests
Esotropia
Eye moves
Exotropia
Eye moves
Esophoria
Eye moves
Exophoria
Eye moves
© Lori Waters 2005
Cover - Uncover tests
Figure 23. Cover and Cover-Uncover Tests for Detection of Tropias and Phorias
PARALYTIC STRABISMUS • incomitant strabismus (i.e. deviation varies in different positions of gaze) • reduction or restriction in range of eye movements Etiology • neural (CN III, IV, VI): ischemia (e.g. DM), MS, aneurysm, brain tumour, trauma • muscular: myasthenia gravis (neuromuscular junction pathology), Graves’ disease • structural: restriction/entrapment of extraocular muscles due to orbital inflammation, tumour, fracture of the orbital wall Clinical Features • mostly in adults, acquired • present mainly with diplopia • greatest deviation in field of action of the weakened muscle • visual acuity is usually unaffected in either eye, unless CN II is involved NON-PARALYTIC STRABISMUS • concomitant strabismus (i.e. deviation equal in all directions of gaze) • no restriction in range of eye movements • monocular, alternating, or intermittent Clinical Features • usually begins in infancy, up to age 8-10 • usually no diplopia (child suppresses the image from the misaligned eye) • deviated eye may become amblyopic if not treated when the child is young. Amblyopia treatment rarely successful after age 8-10 (see Amblyopia, OP39) • amblyopia usually does not develop if child has alternating strabismus or intermittency – allows neural pathways for both eyes to develop
All children with strabismus and/or possible reduced vision require prompt referral to an ophthalmologist.
OP40 Ophthalmology
Pediatric Ophthalmology
Toronto Notes 2011
Accommodative Esotropia • normal response to approaching object is the triad of the near reflex: convergence, accommodation and miosis • hyperopes must constantly accommodate – excessive accommodation can lead to esotropia in young children via over-activation of the near reflex • average age of onset is 2.5 years • usually reversible with correction of refractive error Non-accommodative Esotropia • accounts for 50% of childhood strabismus • most are idiopathic • may be due to monocular visual impairment (e.g. cataract, corneal scarring, anisometropia, retinoblastoma) or divergence insufficiency (ocular misalignment that is greater at distance fixation than at near fixation)
Amblyopia Definition • reduction of best corrected visual acuity due to cortical suppression of sensory input from an eye that is receiving blurred or conflicting visual information, leading to disruption of the normal development of visual pathways serving that eye Detection • “Holler Test”: young child upset if good eye is covered • quantitative visual acuity by age 3-4 years using picture charts and/or matching game (SheridanGardiner), testing each eye separately • amblyopia treatment less successful after age 8-10 years, but a trial should be given no matter what age • prognosis: 90% will have good vision restored and maintained if treated 2 mm • may delay surgery if the diplopia improves
Chemical Burns • alkali burns have a worse prognosis than acid burns because acids coagulate tissue and inhibit further corneal penetration • poor prognosis if cornea opaque, likely irreversible stromal damage • even with a clear cornea initially, alkali burns can progress for weeks (thus, very guarded prognosis) Treatment • irrigate at site of accident immediately with water or buffered solution IV drip for at least 20-30 minutes with eyelids retracted in emergency department swab upper and lower fornices to remove possible particulate matter • do not attempt to neutralize because the heat produced by the reaction will damage the cornea • cycloplegic drops to decrease iris spasm (pain) and prevent secondary glaucoma (due to posterior synechiae formation) • topical antibiotics and patching • topical steroids (by ophthalmologist) to decrease inflammation, use for less than two weeks (in the case of a persistent epithelial defect)
Surgical Ophthalmology • dacrocystorhinostomy (DCR) – excision of bone covering the nasolacrimal sac to restore tear drainage • LASIK (laser-assisted in-situ keratomileusis) – a microkeratome is used to create a corneal flap followed by laser remodeling of the stroma to correct refractive error • trabeculectomy – creation of a new outflow tract from anterior chamber to under conjunctiva; fibrosis prevented with mitomycin C or 5-FU injection during surgery • phacoemulsification (cataract extraction) – the use of ultrasonic waves to break up and aspirate a cataract followed by replacement with an artificial lens implant • vitrectomy – the use of small trochars to enter the posterior segment and remove vitreous; commonly used to treat vitreous hemorrhage and retinal detachment • pneumatic retinopexy – intraocular injection of air or an expandable gas in order to tamponade a retinal break • scleral buckle – a band is secured on the outside of the globe that indents the eye wall, thereby relieving tension on the retina around any tears/holes and allowing the tears/holes to remain sealed
Fluorescein lights up alkali so you can detect it and assess whether it has been removed.
OP44 Ophthalmology
Ocular Drug Toxicity/Common Medications
Toronto Notes 2011
Ocular Drug Toxicity Table 8. Drugs with Ocular Toxicity Amiodarone
Corneal microdeposits and superficial keratopathy (vortex keratopathy) Rare: ischemic optic neuropathy
Atropine, benztropine
Pupillary dilation (risk of angle closure glaucoma)
Bisphosphonates (Fosamax®, Actonel®)
Inflammatory eye disease (iritis, scleritis, episcleritis)
Chloroquine, hydroxychloroquine
Bull’s eye maculopathy Vortex keratopathy
Chlorpromazine
Anterior subcapsular cataract
Contraceptive pills
Decreased tolerance to contact lenses Migraine Optic neuritis Central vein occlusion
Digitalis
Yellow vision Blurred vision
Ethambutol
Optic neuropathy
Haloperidol (Haldol®)
Oculogyric crises Blurred vision
Indomethacin
Superficial keratopathy
Interferon
Retinal hemorrhages and cotton wool spots
Isoniazid
Optic neuropathy
Nalidixic acid
Papilledema
Steroids
Posterior subcapsular cataracts Glaucoma Papilledema (systemic steroids) Increased severity of HSV infections (geographic ulcers) Predisposition to fungal infections
Sulphonamides, NSAIDS
Stevens-Johnson syndrome
Tamsulosin (Flomax®)
Intraoperative Floppy Iris Syndrome (IFIS), which can complicate cataract surgery
Tetracycline
Papilledema (associated with pseudotumour cerebri)
Thioridazine
Pigmentary degeneration of retina
Vigabatrin
Retinal deposition with macular sparing, peripheral visual field loss
Vitamin A intoxication
Papilledema
Vitamin D intoxication
Band keratopathy
Common Medications TOPICAL OCULAR DIAGNOSTIC DRUGS Fluorescein Dye • water soluble orange-yellow dye • green under cobalt blue light (ophthalmoscope or slit lamp) • absorbed in areas of epithelial loss (ulcer or abrasion) • also stains mucus and contact lenses Rose Bengal Stain • stains devitalized epithelial cells and mucus Anesthetics • e.g. proparacaine HCl 0.5%, tetracaine 0.5% • indications: removal of foreign body and sutures, tonometry, examination of painful cornea • toxic to corneal epithelium (inhibit mitosis and migration) and can lead to corneal ulceration and scarring with prolonged use, therefore NEVER prescribe
Toronto Notes 2011
Common Medications
Ophthalmology OP45
Mydriatics • dilate pupils • two classes cholinergic blocking (e.g. tropicamide [Mydriacyl®]) dilation plus cycloplegia (lose accommodation) by paralysis of iris sphincter and the ciliary body indications: refraction, ophthalmoscopy, therapy for iritis adrenergic stimulating (e.g. phenylephrine HCl 2.5%) stimulate pupillary dilator muscles, no effect on accommodation usually used with tropicamide for additive effects side effects: hypertension, tachycardia, arrhythmias Table 9. Mydriatic Cycloplegic Drugs and Duration of Action Drugs
Duration of Action
Tropicamide (Mydriacyl®) 0.5%, 1%
4-5 hours
Cyclopentolate HCL 0.5%, 1%
3-6 hours
Homatropine HBr 1%, 2%
3-7 days
Atropine sulfate 0.5%, 1%
1-2 weeks
Scopolamine HBr 0.25%, 5%
1-2 weeks
GLAUCOMA MEDICATIONS Table 10. Glaucoma Medications Drug Category
Dose
Effect
Comment/Side Effects
Alpha-Agonist Non-selective • epinephrine HCl 1% (Epifrin®) • dipivalyl epinephrine 0.1% (Propine®) Alpha2-selective • brimonidine 0.2% (Alphagan®) • apraclonidine 0.5% (Lopidine®)
1 gtt OS/OD bid/tid
1. Non-selective – aqueous production + TM outflow 2. Selective – aqueous production + uveoscleral outflow
1. Non-selective – mydriasis, macular edema, tachycardia 2. Selective – contact allergy, hypotension in children
Beta-Blocker Non-selective • timolol (Timoptic®) • levobunolol (Betagan®) Beta1-selective • betaxolol (Betoptic®)
1 gtt OS/OD qd/bid
aqueous production
Bronchospasm (careful in asthma/COPD) CHF Bradycardia Hypotension Depression Heart block Impotence
Carbonic Anhydrase Inhibitor • dorzolamide (Trusopt®) • brinzolamide (Azopt®) • oral: acetazolamide (Diamox®)
1 gtt OS/OD tid Diamox®: 500 mg PO bid
aqueous production
**Must ask about sulfa allergy! Generally local side effects with topical preparations Oral: diuresis, fatigue, paresthesias, GI upset, etc.
Parasympathomimetic (cholinergic stimulating) • pilocarpine (Pilopine®) • carbachol (Isopto Carbachol®)
1-2 gtts OS/OD tid/qid
TM outflow
Miosis night vision GI motility Brow ache Headache heart rate
Prostaglandin Analogues • latanoprost (Xalatan®) • travaprost (Travatan®) • bimatoprost (Lumigan®)
1 gtt OS/OD qhs
uveoscleral outflow (uveoscleral responsible for 20% of drainage)
Iris colour change Periorbital skin pigmentation Lash growth Conjunctival hyperemia
Cosopt® = timolol + dorzolamide; Xalacom® = timolol + lantanoprost; Combigan® = timolol + brimonidine; DuoTrav® = tinolol + travaprost gtt = drop, gtts = drops
WET AGE-RELATED MACULAR DEGENERATION MEDICATIONS Vascular Endothelial Growth Factors (VEGF) Inhibitors • block vascular endothelial growth factor which prevents ocular angiogenesis and further development of choroidal neovascularization • administered via intravitreal injections • pegaptanib (Macugen®) is a selective anti-VEGF targeting VEGF isoform 165 • ranibizumab (Lucentis®) is a non-selective anti-VEGF agent • bevacizumab (Avastin®) is another non-selective anti-VEGF agent but is only FDA approved for metastatic breast cancer, colorectal cancer and non-small cell lung cancer. Therefore, its use in ophthalmologic is off-label
OP46 Ophthalmology
Common Medications/References/References
Toronto Notes 2011
TOPICAL OCULAR THERAPEUTIC DRUGS NSAIDs • used for less serious chronic inflammatory conditions • e.g. ketorolac (Acular®), diclofenac (Voltaren®), nepafenac (Nevanac®) drops Anti-Histamines • used to relieve red and itchy eye, often in combination with decongestants • sodium cromoglycate – stabilizes membranes Decongestants • weak adrenergic stimulating drugs (vasoconstrictor) • e.g. naphazoline, phenylephrine (Isopto Frin®) • rebound vasodilation with overuse; rarely can precipitate angle closure glaucoma Antibiotics • indications: bacterial conjunctivitis, keratitis, or blepharitis • commonly as topical drops or ointments, may give systemically • e.g. sulfonamide (sodium sulfacetamide, sulfisoxazole), gentamicin (Garamycin®), erythromycin, tetracycline, bacitracin, polymyxin B, fluoroquinolones (Ciloxan®, Ocuflox®, Vigamox®, Zymar®) Corticosteroids • e.g. fluorometholone (FML®), betamethasone, dexamethasone (Maxidex®), prednisolone (Predsol® 0.5%, Pred Forte® 1%), rimexolone (Vexol®) • primary care physicians should avoid prescribing topical corticosteroids due to risk of glaucoma, cataracts, and reactivation of HSV keratitis • complications potentiates herpes simplex keratitis and fungal keratitis as well as masks symptoms increased IOP, more rapidly in steroid responders (within weeks) posterior subcapsular cataract (within months)
References Complications: Your eyes & diabetic retinopathy. Canadian Diabetes Association Nov 2003. Diabetic Retinopathy. Diabetes Care 1998; 21(1):143-156. Diabetes in Ontario: an ICES Practice Atlas. ICES June 2003. Books Bradford C. Basic Ophthalmology for Medical Students and Primary Care Residents. 7th ed. San Francisco: American Academy of Ophthalmology, 1999. Wilson FM. Practical Ophthalmology: A Manual for Beginning Residents. 4th ed. American Academy of Ophthalmology, 2005. Friedman N, Pineda R, Kaiser P. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. Toronto: W.B. Saunders Company, 1998. Kanski JJ. Clinical Ophthalmology: A Systematic Approach. 6th ed. Oxford: Butterworth-Heinemann, 2007. Stein R, Stein H. Management of Ocular Emergencies. 4th ed. Montreal: Mediconcept, 2006. Guidelines Hux J, et al. Diabetes in Ontario: an ICES Practice Atlas. Toronto: Institute for Clinical Evaluative Sciences, 2003. The committee for the classification of retinopathy of prematurity. An international classification of retinopathy of prematurity. Arch Ophthalmology. 1984; 102: 1130-34. Images Red Atlas. www.redatlas.org Atlas of Ophthalmology www.atlasophthalmology.com/atlas/frontpage.jsf Lectures/Cases University of Michigan Kellogg Eye Centre www.kellogg.umich.edu/theeyeshaveit/index.html
OR
Orthopaedics Frederick Cheng, Chris Farlinger and Caroline Scott, chapter editors Alaina Garbens and Modupe Oyewumi, associate editors Adam Gladwish, EBM editor Dr. Jeremy Hall, Dr. Markku Nousiainen and Dr. Herbert von Schroeder, staff editors Basic Anatomy Review . . . . . . . . . . . . . . . . . . . . 2 Differential Diagnosis of Joint Pain. . . . . . . . . . 4 Fractures – General Principles . . . . . . . . . . . . . . 5 Fracture Description Management of Fractures Fracture Healing General Fracture Complications Orthopaedic Emergencies . . . . . . . . . . . . . . . . . 7 Trauma Patient Work-Up Open Fractures Septic Joint Osteomyelitis Compartment Syndrome Cauda Equina Syndrome Hip Dislocation Pelvis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 Pelvic Fracture Shoulder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 Shoulder Dislocation Rotator Cuff Disease Acromioclavicular (AC) Joint Pathology Clavicular Fracture Frozen Shoulder Humerus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Proximal Humeral Fracture Humeral Shaft Fracture Elbow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 General Principles Supracondylar Fracture Radial Head Fracture Olecranon Fracture Elbow Dislocation Epicondylitis Forearm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18 Radius and Ulna Fracture Monteggia Fracture Nightstick Fracture Galeazzi Fracture Wrist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 Colles’ Fracture Smith’s Fracture Complications of Wrist Fractures Scaphoid Fracture Hand . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Evaluation of Hand Complaints Spine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 Fractures of the Spine Cervical Spine Thoracolumbar Spine
Hip . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26 Hip Fracture Arthritis of the Hip Hip Dislocation after THA Femur . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 Femoral Diaphysis Fracture Distal Femoral Fracture Knee . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29 Evaluation of Knee Complaints Cruciate Ligament Tears Collateral Ligament Tears Meniscal Tears Quadriceps/Patellar Tendon Rupture Dislocated Knee Patella . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Patellar Fracture Patellar Dislocation Patellofemoral Syndrome Tibia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 Tibial Plateau Fracture Tibial Shaft Fracture Ankle. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 Evaluation of Ankle and Foot Complaints Ankle Fracture Ligamentous Injuries Foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35 Talar Fracture Calcaneal Fracture Achilles Tendonitis Achilles Tendon Rupture Plantar Fasciitis Bunions (Hallux Valgus) Metatarsal Fracture Pediatric Orthopaedics . . . . . . . . . . . . . . . . . . . 38 Fractures in Children Stress Fractures Evaluation of the Limping Child Epiphyseal Injury Slipped Capital Femoral Epiphysis (SCFE) Developmental Dysplasia of the Hip (DDH) Legg-Calve-Perthes Disease (Coxa Plana) Osgood-Schlatter Disease Congenital Talipes Equinovarus (Club Foot) Scoliosis Bone Tumours . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Benign Active Bone Tumours Benign Aggressive Bone Tumours Malignant Bone Tumours Articular Cartilage Defects . . . . . . . . . . . . . . . . 44 Properties of Articular Cartilage Common Medications. . . . . . . . . . . . . . . . . . . . 45 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46
Toronto Notes 2011
Orthopaedics OR1
OR2 Orthopaedics
Basic Anatomy Review
Toronto Notes 2011
Basic Anatomy Review C5 C6 C7 C8 T1
C7 C8 T1
Coracobrachialis
Musculocutaneous nerve Biceps brachii
Ulnar nerve
Median nerve
Medial cutaneous nerve of the arm (sensory)
Brachialis
Lateral cutaneous nerve of forearm (sensory)
Pronator teres Pronator teres Flexor carpi radialis Palmaris longus
Pronator quadratus
Medial cutaneous nerve of the forearm (sensory)
Flexor digitorum superficialis
Dorsal cutaneous branch (sensory)
Flexor digitorum profundus (lateral 2,3, digits) Adductor pollicis
Thenar muscles
Palmar interossei Palmaris brevis Hypothenar muscles
2, 3 lumbricals Palmar sensory branches of median nerve
Palmar cutaneous branch (sensory)
Dorsal interossei
4,5 lumbricals
Superficial terminal branches (sensory)
ANTERIOR VIEW Figure 1. Median, Musculocutaneous and Ulnar Nerves: Innervation of Upper Limb Muscles
© Lori Waters 2005
Flexor pollicis longus
Flexor carpi ulnaris
Flexor digitorum profundus (medial 4,5 digits)
Basic Anatomy Review
Axillary
Orthopaedics OR3 C5 C6 C7 C8
Subclavian
Subscapularis
Deltoids
Posterior Circumflex humeral
Teres major
Thoracoacromial Anterior
Axillary nerve
Lateral thoracic
Latissimus dorsi
Subscapular Brachial artery
Profunda brachii
Upper cutaneous nerve of the arm (sensory)
Superior ulnar collateral
Anterior and posterior ulnar recurrent
Radial
Supinator
Posterior interosseous nerve
Ulnar
Abductor pollicis longus
Anterior interosseous
Abductor pollicis brevis Deep palmar arch Superficial palmar arch
Arteries of the upper limb
Brachioradialis Extensor carpi radialis longus Extensor carpi radialis brevis
Inferior ulnar collateral Radial recurrent
Radial nerve
Extensor carpi ulnaris Extensor digiti minimi Extensor digitorum Extensor indicis
Superficial radial nerve (sensory)
ANTERIOR VIEW
POSTERIOR VIEW
Figure 2. (Left) Blood Supply to the Upper Limb (Right) Axillary and Radial Nerves: Innervation of the Upper Limb Table 1. Sensory and Motor Innervation of the Nerves in the Upper and Lower Extremities Nerve
Motor
Sensory
Axillary
Deltoid/Teres Minor
Lateral Upper Arm (Sergeant’s Patch)
Musculocutaneous
Biceps/Brachialis
Lateral Forearm
Radial
Triceps Wrist/Thumb/Finger Extensors
Lateral Dorsum of the Hand Medial Upper Forearm
Median
Wrist Flexors and Abductors Flexion of the 1st - 3rd Digits
Volar Thumb to Radial ½ of Ring Finger
Ulnar
Wrist Flexors and Adductors Flexion of the 4th - 5th Digits
Medial Forearm Medial Dorsum and Volar of Hand (Ulnar ½ of Ring and 5th Digit)
Tibial
Ankle Plantar Flexion Knee Flexion Great Toe Flexion
Sole of Foot
Superficial Peroneal
Ankle Eversion
Dorsum of Foot
Deep Peroneal
Ankle Dorsiflexion and Inversion Great Toe Extension
1st Web Space
Sural
Lateral Foot
Saphenous
Anteromedial Ankle
Quick Nerve Exam “Thumbs Up”: PIN (Radial Nerve) “OK Sign”: AIN (Median Nerve) “Spread Fingers”: Ulnar Nerve
© Lori Waters 2005
Toronto Notes 2011
Basic Anatomy Review/Differential Diagnosis of Joint Pain
ANTERIOR VIEW Common iliac artery Internal iliac artery External iliac artery Femoral nerve Lateral cutaneous nerve of the thigh Medial cutaneous nerve of the thigh Obturator nerve
Profunda femoris artery Femoral artery
Toronto Notes 2011
POSTERIOR VIEW
Superior gluteal nerve
Inferior gluteal nerve Sciatic nerve
Posterior cutaneous nerve of the thigh
Profunda femoris artery Femoral artery
Intermediate cutaneous nerve of the thigh
Common fibular (peroneal) nerve Saphenous nerve
Tibial nerve Common fibular (peroneal) nerve
Popliteal artery
Deep fibular (peroneal) nerve Superficial fibular (peroneal) nerve
Lateral cutaneous nerve of the calf Anterior tibial artery
Dorsalis pedis artery
Posterior tibial artery Anterior tibial artery Sural nerve
Calcaneal branch Lateral plantar nerve Medial plantar nerve Medial plantar artery Lateral plantar artery Plantar artery
Figure 3. Nerves and Arteries of Lower Limbs
Differential Diagnosis of Joint Pain Extrinsic • neurologic (nerve root compression, herpes zoster, etc.) • generalized (fibromyalgia, polymyalgia rheumatica, sickle cell (ischemic), dermato/polymyositis) • referred pain • pain originating from surrounding organs Intrinsic • articular arthritis (degenerative, rheumatoid, crystal-induced, septic, avascular necrosis) neoplastic traumatic (fracture, soft tissue damage, neuropathic arthropathy) • non-articular bursa, tendons, ligaments, muscle (bursitis, tendonitis, myositis)
© Michelle Lui 2005
OR4 Orthopaedics
Toronto Notes 2011
Fractures – General Principles
Orthopaedics OR5
Fractures – General Principles
Spongy bone Proximal epiphysis
Fracture Description 1. Integrity of Skin/Soft Tissue • closed: skin/soft tissue over and near fracture is intact • open: skin/soft tissue over and near fracture is lacerated or abraded, fracture exposed to outside environment, continuous bleeding from puncture site or fat droplets in blood suggest communication with fracture
Articular cartilage Epiphyseal line Periosteum Compact bone Medullary cavity
Diaphysis
2. Location (Figure 4) • epiphyseal: end of bone, forming part of the adjacent joint • metaphyseal: the flared portion of the bone at the ends of the shaft • diaphyseal: the shaft of a long bone (proximal, middle, distal) • physis: growth plate Distal epiphysis
3. Orientation/Fracture Pattern (Figure 5) • transverse: perpendicular fracture line, direct force, high energy • oblique: angular fracture line, angular or rotational force • butterfly: slight comminution at the fracture site which looks like a butterfly • segmental: a separate segment of bone bordered by fracture lines, high energy • spiral: complex, multi-planar fracture line, rotational force, low energy • comminuted/multi-fragmenary: more than 2 fracture fragments • intra-articular: fracture line crosses articular cartilage and enters joint • compression/impacted: impaction of bone, e.g. vertebrae, proximal tibia • torus: a buckle fracture of one cortex, often in children (Figure 49) • green-stick: an incomplete fracture of one cortex, often in children (Figure 49) • pathologic: fracture through bone weakened by disease/tumour
Figure 4. Schematic Diagram of the Long Bone
X-Ray Rule of 2’s 2 sides = bilateral 2 views = AP + lateral 2 joints = joint above + below 2 times = before + after reduction
4. Displacement (Figure 5) • nondisplaced: fracture fragments are in anatomic alignment • displaced: fracture fragments are not in anatomic alignment • distracted: fracture fragments are separated by a gap • angulated: direction of fracture apex, e.g. varus/valgus • translated: percentage of overlapping bone at fracture site • rotated: fracture fragment rotated about long axis of bone
C A
G
E B
D
C. Butterfly B. Oblique
D. Segmental
I
J
K
H
F E. Spiral
Varus/Valgus Displacement Varus = Apex away from midline Valgus = Apex toward midline NOTE: displacement refers to direction of distal fragment.
G. Shifted
F. Comminuted
A. Transverse
I. Rotated H. Angulated
K. Impacted J. Avulsion © Carly Vanderlee 2011
Figure 5. Fracture Types
Management of Fractures • ABCs, primary survey and secondary survey (ATLS protocol) rule out other fractures/injuries rule out open fracture • AMPLE history – Allergies, Medications, Past medical history, Last meal, Events surrounding injury consider pathologic fracture with history of only minor trauma • additional history/physical: baseline functional status – handedness (upper extremity) vs. ambulatory ability (lower extremity – note distances, stairs, and use of assistive devices such as canes, walkers, wheelchairs, etc.)
Indications for Open Reduction NO CAST N – non-union O – open fracture C – neurovascular compromise A – intra-articular fracture S – Salter-Harris 3,4,5 T – polytrauma
OR6 Orthopaedics
Reasons For Splinting • Reduces pain • Reduces further damage to vessels, nerves and skin • Reduces risk of inadvertently converting closed to open fracture • Facilitates patient transport
Fractures – General Principles
Toronto Notes 2011
occupation and smoking status mechanism of injury past medical history (note any contraindications to surgery or general anesthetic) neurovascular status • analgesia • imaging • splint extremity 1. obtain the reduction (refer to Table 22 for appropriate IV sedation) closed reduction apply traction in the long axis of the limb reverse the mechanism that produced the fracture reduce with IV sedation and muscle relaxation (fluoroscopy can be used if available) indications for open reduction – NO CAST (see sidebar, OR5) other indications include failed closed reduction cannot cast or apply traction due to site (e.g. hip fracture) pathologic fractures potential for improved function with open reduction and internal fixation (ORIF) potential complications of open reductions infection mal-union non-union implant failure new fracture re-check neurovascular status after reduction and obtain post-reduction x-ray 2. maintain the reduction external stabilization – splints, casts, traction, external fixator internal stabilization – percutaneous pinning, extramedullary fixation (screws, plates, wires), intramedullary fixation (rods) follow-up – evaluate bone healing 3. rehabilitate to regain function and avoid joint stiffness
Fracture Healing Normal Healing
Figure 6. Heterotopic Ossification of Femoral Diaphysis after Femur Fracture and Intramedullary Nailing
Heterotopic Ossification The formation of bone in abnormal locations (e.g. in muscle), secondary to pathology.
Avascular Necrosis Ischemia to bone due to disrupted blood supply; commonly in bones covered by cartilage.
Weeks 0-3
Hematoma, macrophages surround fracture site
Weeks 3-6
Osteoclasts remove sharp edges, callus forms within hematoma
Weeks 6-12
Bone forms within the callus, bridging fragments
Months 6-12
Cortical gap is bridged by bone
Years 1-2
Normal architecture is achieved through remodelling
Figure 7. Stages of Bone Healing
Evaluation of Healing: Tests of Union • clinical: no longer tender to palpation or stressing on physical exam • x-ray: trabeculae cross fracture site, visible callus bridging site on at least 3 of 4 cortices
General Fracture Complications Table 2. General Fracture Complications Early
Late
Local
Compartment syndrome Neurological injury Vascular injury Infection Implant failure Fracture blisters
Mal/non-union Avascular necrosis (AVN) Osteomyelitis Heterotopic ossification (HO) Post-traumatic arthritis/Joint stiffness Chronic regional pain syndrome type 1 (CRPS)/Reflex sympathetic dystrophy (RSD)
Systemic
Sepsis Deep vein thrombosis (DVT) Pulmonary embolus (PE) Acute respiratory distress syndrome (ARDS) Hemorrhagic shock
Fracture Blister Formation of vesicles or bullae that occur on edematous skin overlying a fractured bone.
CRPS/Reflex Sympathetic Dystrophy An exaggerated response to an insult in the extremities; characterized by intense pain, temperature asymmetry, edema and motor/sensory changes.
Toronto Notes 2011
Orthopaedic Emergencies
Orthopaedics OR7
Orthopaedic Emergencies Trauma Patient Work-Up Etiology • high energy trauma e.g. motor vehicle accidents, fall from height • may be associated with spinal injuries or life-threatening visceral injuries Clinical Presentation • local swelling, tenderness, deformity of the limbs and instability of the pelvis or spine • decreased level of consciousness • consider involvement of alcohol or other substances Investigations • trauma survey (see Emergency Medicine, Initial Patient Assessment/Management, ER2) • x-rays: lat cervical spine, AP chest, abdo x-ray, AP pelvis, AP and lateral of all long bones suspected to be injured • other views of pelvis: AP, inlet and outlet; Judet view for acetabular fracture (see Table 15 for classification of pelvic fractures) Treatment • ABCDEs and initiate resuscitation to life threatening injuries • assess genitourinary injury (rectal exam/vaginal exam mandatory) • external or internal fixation of all fractures • DVT prophylaxis Complications • hemorrhage – life threatening (may produce signs and symptoms of hypovolemic shock) • acute respiratory distress syndrome (ARDS) • fat embolism syndrome • venous thrombosis – DVT and PE • bladder/bowel injury • neurological damage • possible obstetrical difficulties in future • persistent sacro-iliac joint pain • persistent pain/stiffness/limp/weakness in affected extremities • post-traumatic arthritis of joints with intra-articular fractures • sepsis if missed open fracture
Orthopaedic Emergencies VON CHOP Vascular compromise Open fracture Neurological compromise/Cauda equina syndrome Compartment syndrome Hip dislocation Osteomyelitis/Septic arthritis Unstable Pelvic fracture
Buck’s Traction A system of weights, pulleys and ropes that are attached to the end of a patient’s bed exerting a longitudinal force on the distal end of a fracture, improving its alignment.
Open Fractures Definition • fractured bone in communication with the external environment Emergency Measures • removal of obvious foreign material • irrigate with normal saline • cover wound with sterile dressings • IV antibiotics (see Table 3) • tetanus status ± booster • splint fracture • NPO and prepare for OR (bloodwork, consent, ECG, CXR) operative irrigation and debridement within 6-8 hours to decrease risk of infection traumatic wound often left open to drain but vac dressing may be used re-examine with repeat I&D in 48 hrs
33% of patients with open fractures have multiple injuries.
OR8 Orthopaedics
Orthopaedic Emergencies
Toronto Notes 2011
Table 3. Gustilo Classification of Open Fractures Gustilo Grade
Length of Open Wound
Description
Antibiotic Regimen
I
10 cm
IIIA: Extensive soft tissue injury with adequate ability of soft tissue to cover wound IIIB: Extensive soft tissue injury with periosteal stripping and bone exposure; inadequate soft tissue to cover wound IIIC: Vascular injury/compromise
First generation cephalosporin (cefazolin) plus Gram-negative coverage (gentamicin) for at least 3 days For soil contamination, penicillin is added for clostridial coverage
*Any high injury, comminuted fracture, shot gun, farmyard/soil/water contamination, exposure to oral flora, or fracture more than 8 hours old is immediately classified as Grade III
Septic Joint
Plain Film Findings in a Septic Joint • Early (0-3 days) – usually normal. May show soft-tissue swelling or joint space widening from localized edema • Late (4-6 days) – joint space narrowing and destruction of cartilage
Serial C-reactive protein (CRP) can be used to monitor response to therapy.
Etiology • most commonly caused by Staphylococcus aureus in adults • consider coagulase-negative staph in patients with prior joint replacement • consider Neisseria gonorrhoeae in sexually active adults • most common route of infection is hematogenous Clinical Presentation • inability/refusal to bear weight, localized joint pain, erythema, warmth, swelling with pain on active and passive ROM, ± fever Investigations • x-ray (to r/o fracture, tumour, metabolic bone disease), ESR, CRP, WBC, blood cultures • joint aspirate (WBC >80,000 with >90% neutrophils, protein level >4.4 mg/dL, joint 2.5 cm vertical instability of the posterior pelvis Specific Complications (see General Fracture Complications, OR6) • hemorrhage (life-threatening) – 1500-3000 ml blood loss • injury to rectum or urogenital structures • obstetrical difficulties • persistent sacroiliac (SI) joint pain • post-traumatic arthritis of the hip with acetabular fractures • high risk of DVT/PE
Type B Open Book
© Seline McNamee
Type
Type A Unstable Vertical Fracture
Figure 11. Illustration of the Tile Classification of Pelvic Fractures
Shoulder Shoulder Dislocation • the glenohumeral joint is the most commonly dislocated joint in the body since stability is sacrificed for motion Prognosis • recurrence rate depends on age of 1st dislocation: 40 yrs = 2-4% Specific Complications • tuberosity fracture, glenoid rim fracture (Bankart lesion), humeral head impaction (Hill-Sachs lesion) • rotator cuff or capsular tear, shoulder stiffness • injury to axillary nerve/artery, brachial plexus • recurrent/unreduced dislocation (most common complication)
There are 4 Joints in the Shoulder: glenohumeral, acromioclavicular (AC), sternoclavicular (SC), scapulothoracic.
Factors Causing Shoulder Instability • Shallow glenoid • Loose capsule • Ligamentous laxity
4
5
3
2 1
6
ANTERIOR SHOULDER DISLOCATION (>90%) Mechanism • abducted and externally rotated arm or blow to posterior shoulder 7
8
9
1. Manubrium 2. Sternoclavicular joint 3. Clavicle 4. Coracoid process 5. Acromioclavicular joint 6. Acromion 7. Humerus 8. Glenohumeral joint 9. Scapula
Figure 12. Shoulder Joints
© Jason Raine
Clinical Features • pain • arm held in slight abduction, external rotation; internal rotation is blocked • “squared off ” shoulder • +ve apprehension test: apprehension with shoulder abduction and external rotation to 90o since humeral head is pushed anteriorly and recreates feeling of anterior dislocation • +ve relocation test: a posteriorly directed force applied during the apprehension test relieves apprehension since anterior subluxation is prevented
OR12 Orthopaedics
Shoulder
Toronto Notes 2011
© Lori Waters 2005
• +ve sulcus sign: presence of subacromial indentation with distal traction on humerus indicates inferior shoulder instability • neurovascular exam including: axillary nerve (sensory patch over deltoid and deltoid contraction) musculocutaneous nerve (sensory patch on lateral forearm and biceps contraction)
Anterior apprehension sign
Table 5. An EBM Perspective on Tests of Anterior Shoulder Instability Apprehension
Relocation
Surprise
Sensitivity
52.78%
45.83%
63.89%
Specificity
98.91%
54.35%
98.91%
PPV
97.73%
43.86%
98.22%
NPV
72.82%
56.26%
77.86%
An Evaluation of the Apprehension, Relocation, and Surprise Tests for Anterior Shoulder Instability. The American Journal of Sports Medicine 32:301-307 (2004).
Investigations • x-rays: AP, trans-scapular, axillary X-Ray Findings • dislocation axillary view: humeral head is anterior trans-scapular view: humeral head is anterior to the centre of the “Mercedes-Benz sign” • ± Hill-Sachs lesion: divot in posterior humeral head due to forceful impaction of an anteriorly dislocated humeral head against the glenoid rim (Figure 15) • ± bony Bankart lesion: avulsion of the anterior glenoid labrum (with attached bone fragments) from the glenoid rim
© Lori Waters 2005
© Lori Waters 2005
Sulcus sign
Posterior apprehension sign
Figure 13. Apprehension Tests
Treatment • closed reduction with IV sedation and muscle relaxation • 2 methods traction-countertraction: assistant stabilizes torso with a folded sheet wrapped across the chest while the MD applies gentle steady traction (see Figure 14) Stimson: while patient lies prone with arm hanging over table edge, hang a 5 lb weight on wrist for 15-20 min • obtain post-reduction x-rays • check post-reduction neurovascular status (NVS) • sling x 3 weeks, followed by shoulder rehabilitation
© Tabby Lulham 2010
POSTERIOR SHOULDER DISLOCATION (5%) • up to 60-80% are missed on initial presentation due to poor physical exam and radiographs
Figure 14. Traction-Countertraction
© Mary Sims 2003
Bankart
Mechanism • adducted, internally rotated, flexed arm • fall on an outstretched hand (FOOSH) • 3 E’s (epileptic seizure, EtOH, electrocution) • blow to anterior shoulder Clinical Features • arm is held in adduction and internal rotation; external rotation is blocked • anterior shoulder flattening, prominent coracoid, palpable mass posterior to shoulder • posterior apprehension (“jerk”) test: with patient supine, flex elbow 90º and adduct, internally rotate the arm while applying a posterior force to the shoulder; patient will “jerk” back with the sensation of subluxation Investigation • x-rays: AP, trans-scapular, axillary
Hill-Sachs
Figure 15. Anterior Dislocation Causing Hill-Sachs and Bankart Lesions
X-Ray Findings • dislocation AP view: partial vacancy of glenoid fossa (vacant glenoid sign) and >6 mm space between anterior glenoid rim and humeral head (positive rim sign), humeral head may resemble a lightbulb due to internal rotation (lightbulb sign) axillary view: humeral head is posterior trans-scapular view: humeral head is posterior to centre of “Mercedes-Benz sign” • reverse Hill-Sachs lesion (75% of cases): divot in anterior humeral head • reverse bony Bankart lesion: avulsion of the posterior glenoid labrum from the bony glenoid rim
Toronto Notes 2011
Shoulder
Orthopaedics OR13
Treatment • closed reduction: inferior traction on a flexed elbow with pressure on the back of the humeral head • obtain post-reduction x-rays • check post-reduction neurovascular status • sling x 3 weeks, followed by shoulder rehabilitation
Rotator Cuff Disease
Rotator Cuff Muscles
Table 6. Rotator Cuff Muscles Muscle
Muscle Attachments
Nerve Supply
Muscle Function
Supraspinatus
Scapula greater tuberosity of humerus
Suprascapular nerve
Abduction
Infraspinatus
Scapula greater tuberosity of humerus
Suprascapular nerve
External rotation
Teres Minor
Scapula greater tuberosity of humerus
Axillary nerve
External rotation
Subscapularis
Scapula lesser tuberosity of humerus
Subscapular nerve
Internal rotation and adduction
SPECTRUM OF DISEASE: IMPINGEMENT, TENDONITIS, MICRO OR MACRO TEARS Etiology • compression of rotator cuff tendons (primarily supraspinatus) and subacromial bursa between the head of the humerus and the acromion; leads to bursitis, tendonitis and, if left untreated, can lead to rotator cuff thinning and tear • anything that leads to a narrow subacromial space 1. glenohumeral muscle weakness leading to abnormal motion of humeral head 2. scapular muscle weakness leading to abnormal motion of acromion 3. acromial abnormalities such as congenital narrow space or osteophyte formation Clinical Features • night pain and difficulty sleeping on affected side • pain worse with active motion • weakness and loss of range of motion (e.g. trouble with overhead activities) • tenderness to palpation over greater tuberosity
SITS Supraspinatus Infraspinatus Teres minor Subscapularis
Acromioclavicular ligament
Coracoclavicular ligament
© Kevin Millar
• rotator cuff consists of 4 muscles that act to stabilize humeral head within the glenoid fossa
Supraspinatus Subscapularis Infraspinatus Teres minor Joint capsule
Figure 16. Muscles of the Rotator Cuff
Jobe’s test
Table 7. Rotator Cuff Special Tests Examination
Supraspinatus – place the shoulder in 90 degrees of abduction and Weakness with active resistance suggests 30 degrees of forward flexion and internally rotate the arm so that a supraspinatus tear the thumb is pointing toward the floor (Figure 17)
Lift-off Test
Subscapularis – internally rotate arm so dorsal surface of hand rests Inability to actively lift hand away from back on lower back. Patient instructed to actively lift hand away from suggests a subscapularis tear back against examiner resistance (Figure 17)
Posterior-cuff Test
Infraspinatus and Teres minor – arm positioned at patient’s side in 90 degrees of flexion. Patient instructed to externally rotate arm against the resistance of the examiner (Figure 17)
Weakness with active resistance suggests posterior cuff tear
Neer’s Test
Rotator Cuff Impingement – passive shoulder flexion (Figure 18)
Pain elicited between 130-170 degrees suggests impingement
HawkinsKennedy Test
Rotator Cuff Impingement – shoulder flexion to 90 degrees and passive internal rotation (Figure 19)
Pain with internal rotation suggests impingement
Painful Arc Test Rotator Cuff Tendinopathy – patient instructed to actively abduct the shoulder
Positive Test
Lift-off test
Pain with abduction greater than 90 degrees suggests tendinopathy Posterior cuff test
Figure 17. Rotator Cuff Tests – Jobe’s, Lift-Off, Posterior Cuff
© Tabby Lulham 2010
Test Jobe’s Test
OR14 Orthopaedics
Shoulder
Toronto Notes 2011
130-170º
© Tabby Lulham 2010
Investigations • x-rays: AP view may show high riding humerus relative to glenoid, evidence of chronic tendonitis • MRI: coronal/sagittal oblique and axial orientations are useful for assessing full/partial tears and tendinopathy, ± arthrogram: geyser sign (injected dye leaks out of joint through rotator cuff tear) • arthrogram: see full thickness tear, difficult to assess partial thickness tears
Figure 18. Neer’s Test
Treatment and Prognosis • mild (“wear”) treatment is non-operative (physiotherapy, NSAIDs) • moderate (“tear”) non-operative treatment ± steroid injection • severe (“repair”) impingement that is refractory to 2-3 months physio and 1-2 injections may require surgical repair, i.e. acromioplasty, rotator cuff repair
Acromioclavicular (AC) Joint Pathology • 2 main ligaments attach clavicle to scapula: acromioclavicular (AC) and coracoclavicular (CC) ligaments Mechanism • fall onto shoulder with adducted arm (fall onto tip of shoulder) Clinical Features • palpate step deformity between distal clavicle and acromion (with dislocation) • pain with adduction of shoulder and/or palpation over AC joint • limited ROM
© Erin Duff 2009
Investigations • x-rays: AP, Zanca view (10-15° cephalic tilt), axillary ± stress views (10 lb weight in patient’s hand)
Figure 19. Hawkins-Kennedy Test
Treatment • non-operative (most-common): sling 1-3 weeks, ice, analgesia • operative indications: AC and CC ligaments are both torn and/or clavicle displaced posteriorly procedure: excision of lateral clavicle with AC/CC ligament reconstruction
Clavicular Fracture Pneumothorax or pulmonary contusion are potential complications of severe acromioclavicular joint dislocation
• incidence: proximal (5%), middle (80%), or distal (15%) third of clavicle • common in children (unites rapidly without complications) Mechanism • fall on shoulder (87%), direct trauma to clavicle (7%), FOOSH (6%)
Associated Injuries with Clavicular Fractures • Up to 9% of clavicular fractures are associated with other fractures (most commonly rib fractures) • Majority of brachial plexus injuries are associated with proximal third fractures
Clinical Features • pain and tenting of skin • arm is clasped to chest to splint shoulder and prevent movement Treatment • evaluate neurovascular status of entire upper limb • proximal and middle third clavicular fractures sling x 1-2 weeks early ROM and strengthening once pain subsides if ends overlap >2 cm, consider ORIF • distal third clavicular fractures undisplaced (with ligaments intact): sling x 1-2 weeks displaced (CC ligament injury): ORIF Specific Complications (see General Fracture Complications, OR6) • cosmetic bump usually only complication • shoulder stiffness, weakness with repetitive activity • pneumothorax, injuries to brachial plexus and subclavian vessel (all very rare)
Toronto Notes 2011
Shoulder/Humerus
Orthopaedics OR15
Frozen Shoulder (Adhesive Capsulitis) Definition • disorder characterized by progressive pain and stiffness of the shoulder usually resolving spontaneously after 18 months Mechanism • primary adhesive capsulitis idiopathic, usually associated with diabetes mellitus may resolve spontaneously in 9-18 months • secondary adhesive capsulitis due to prolonged immobilization shoulder-hand syndrome – type of chronic regional pain syndrome (reflex sympathetic dystrophy) characterized by arm and shoulder pain, decreased motion and diffuse swelling following myocardial infarction, stroke, shoulder trauma Clinical Features • gradual onset (weeks to months) of diffuse shoulder pain with: decreased active and passive ROM pain worse at night and often prevents sleeping on affected side increased stiffness as pain subsides: continues for 6-12 months after pain has disappeared
Conditions Associated with an Increased Incidence of Adhesive Capsulitis • Prolonged immobilization (most significant) • Female gender • Age >49 years • Diabetes mellitus (5x) • Cervical disc disease • Hyperthyroidism • Stroke • Myocardial infarction • Trauma and surgery
Investigations • x-rays may be normal, or may show demineralization from disease Treatment • active and passive ROM (physiotherapy) • NSAIDs and steroid injections if limited by pain • MUA (manipulation under anesthesia) and early physiotherapy • arthroscopy for debridement/decompression
Humerus Proximal Humeral Fracture
Anatomic neck fractures disrupt blood supply to the humeral head and avascular necrosis (AVN) of the humeral head may ensue.
Greater tuberosity Lesser tuberosity
Mechanism • young: high energy trauma (MVA) • older: FOOSH from standing height in osteoporotic individuals
Anatomical neck
Clinical Features • pain, swelling, tenderness, painful ROM
Surgical neck
Investigations • test axillary nerve function (deltoid function and skin over deltoid) • x-rays: AP, trans-scapular, axillary are essential • CT scan: to evaluate for articular involvement and fracture displacement Classification • Neer classification is based on 4 fracture fragments: head, greater tuberosity, lesser tuberosity, shaft nondisplaced: displacement 45º dislocated/subluxed: humeral head dislocated/subluxed from glenoid Treatment • non-operative sling immobilization (nondisplaced): begin ROM in 7-10 days to prevent stiffness closed reduction (minimally displaced) • operative ORIF (anatomic neck fractures, displaced, dislocated) hemiarthroplasty may be necessary, especially in elderly Specific Complications (see General Fracture Complications, OR6) • AVN, axillary nerve palsy, malunion, post-traumatic arthritis
Figure 20. Fractures of the Proximal Humerus
Neer Classification Based on 4 parts of humerus 1. Greater Tuberosity 2. Lesser Tuberosity 3. Humeral Head 4. Shaft Two-part fracture: any of the 4 parts with 1 displaced Three-part fracture: displaced fracture of surgical neck + displaced greater tuberosity or lesser tuberosity Four-part fracture: displaced fracture of surgical neck + both tuberosities
OR16 Orthopaedics
Humerus/Elbow
Toronto Notes 2011
Humeral Shaft Fracture Mechanism • direct blows/MVA (most common), FOOSH, twisting injuries, metastases (in elderly) Clinical Features • pain, swelling, ± shortening, motion/crepitus at fracture site • must test radial nerve function before and after treatment Investigations • x-rays: AP and lateral radiographs of the humerus including the shoulder and elbow joints
Acceptable Humeral Shaft Deformities for Non-operative Treatment • 10º angulation
Galeazzi Fracture
© Chesley Sheppard
Mechanism • direct blow to forearm (holding arm up to protect face)
Figure 25. Nightstick Fracture Fracture of distal radius
Mechanism • usual cause is fall on the hand (mechanical axial loading of pronated forearm)
DRUJ
Investigations • x-rays shortening of distal radius >5 mm relative to the distal ulna widening of the DRUJ space on AP dislocation of radius with respect to ulna on true lateral
Dislocation of ulna
Treatment • ORIF of radius • if DRUJ is stable, splint with early ROM • if DRUJ is unstable, DRUJ pinning and long arm cast in supination x 6 weeks
© Desmond Ballance 2006
Definition • fracture of the distal radial shaft with disruption of the distal radioulnar joint (DRUJ) • most commonly in the distal 1/3 of radius near junction of metaphysis/diaphysis
Figure 26. Galeazzi Fracture
For all isolated radius fractures assess DRUJ to rule out a Galeazzi fracture.
Wrist Colles’ Fracture Definition • transverse distal radius fracture (about 2 cm proximal to the radiocarpal joint) with dorsal displacement ± ulnar styloid fracture
Mechanism • FOOSH Clinical Features • “dinner fork” deformity • swelling, ecchymosis, tenderness Investigations • findings on x-ray (Figure 27) Treatment • goal is to restore radial height, radial inclination (22º), volar tilt (11º) and articular congruity • closed reduction (think opposite of the deformity): hematoma block (sterile prep and drape, local anesthetic injection directly into fracture site) or conscious sedation closed reduction – traction with extension (exaggerate injury), then traction with ulnar deviation, pronation, flexion of distal fragment – not at wrist) dorsal slab/below elbow cast for 5-6 weeks x-ray q1 week to ensure reduction is maintained • obtain post-reduction films immediately; repeat reduction if necessary, consider external fixation or ORIF
2
Lateral view 3 4
5
6
AP view 1. Dorsal tilt 2. Dorsal displacement 3. Ulnar styloid fracture 4. Radial displacement 5. Radial tilt 6. Shortening
Figure 27. Colles’ Fracture and Associated Bony Deformity
© Willa Bradshaw 2005
Epidemiology • most common fracture in those >40 years, especially in women and those with osteoporotic bone
1
OR20 Orthopaedics
Wrist
Toronto Notes 2011
Smith’s Fracture Definition • volar displacement of the distal radius (i.e. reverse Colles’ fracture) Mechanism • fall onto the back of the flexed hand Treatment • usually unstable and needs ORIF • if patient is poor operative candidate, may attempt non-operative treatment • closed reduction with hematoma block (reduction opposite of Colles’) • long-arm cast in supination x 6 weeks
Complications of Wrist Fractures • most common complications are poor grip strength, stiffness, and radial shortening • distal radius fractures in individuals 3.5 mm is abnormal) angulation: between adjacent vertebral bodies (>11º is abnormal) disc or facet joint widening anterior soft tissue space (at C3 should be ≤3 mm; at C4 should be ≤8-10 mm) • oblique: evaluate pedicles and intervertebral foramen • ± swimmer’s view: lateral view with arm abducted 180º to evaluate C7-T1 junction if lateral view is inadequate (must see C7-T1 in all trauma situations) • ± lateral flexion/extension view: evaluate subluxation of cervical vertebrae Differential Diagnosis of C-Spine Pain • trapezial sprain, whiplash, cervical spondylosis, cervical stenosis, rheumatoid arthritis (spondylitis), traumatic injury C-SPINE INJURY • see Neurosurgery, NS34
Thoracolumbar Spine General Principles • spinal cord terminates at conus medullaris (L1) • individual nerve roots exit below pedicle of vertebra (i.e. L4 nerve root exits below L4 pedicle) Special Tests • Straight leg raise (SLR): passive lifting of leg (30-70o) reproduces radicular symptoms of pain radiating down post/lat leg to knee, ± into foot • Lasegue maneuver: dorsiflexion of foot during SLR makes symptoms worse or, if leg is less elevated, dorsiflexion will bring on symptoms • Femoral stretch test: with patient prone, flexing the knee of the affected side and passively extending the hip results in radicular pain Table 11. Lumbar Radiculopathy/Neuropathy Root
L4
L5
S1
Motor
Quadriceps (knee extension + hip adduction) Tibialis anterior (ankle inversion + dorsiflexion)
EHL (extensor hallucis longus) Gluteus medius (hip abduction)
Peroneus longus + brevis (ankle eversion) Gastrocnemius + soleus (plantar flexion)
Sensory
Medial malleolus
1st dorsal webspace and lateral Lateral foot leg
Reflex
Knee (Patellar)
Medial hamstring*
Ankle (Achilles)
Test
Femoral stretch
Straight leg raise
Straight leg raise
*Unreliable
Red Flags for BACK PAIN Bowel or bladder dysfunction Anesthesia (saddle) Constitutional symptoms/malignancy Khronic disease Paresthesias Age >50 IV drug use Neuromotor deficits
Canadian C-spine Rule (CCR) Used to guide imaging for alert (GCS = 15) and stable patients with suspected C-spine injury Obtain radiography if: • Age >65 • Paresthesia in the extremities • Inability to rotate neck >45° • Dangerous mechanism of injury (e.g. high speed MVA, fall from elevation > 5 ft, etc.) Reference: CJEM 2002;4(2):84-90
Immediate immobilization of C-spine at scene of accident with spine board, C-collar and sandbags.
OR24 Orthopaedics
Spine
Toronto Notes 2011
Differential Diagnosis of Back Pain 1. mechanical or nerve compression (>90%) • degenerative (disc, facet, ligament) • peripheral nerve compression (disc herniation) • spinal stenosis (congenital, osteophyte, central disc) • cauda equina syndrome
Cauda equina syndrome and ruptured aortic aneurysms are causes of low back pain that are considered surgical emergencies.
2. others • neoplastic (primary, metastatic, multiple myeloma) • infectious (osteomyelitis, TB) • metabolic (osteoporosis) • traumatic fracture (compression, distraction, translation, rotation) • spondyloarthropathies (ankylosing spondylitis) • referred (aorta, renal, ureter, pancreas)
Disc Prolapse Annulus fibrosus
Nerve root
Vertebra
Figure 32. Disc Herniation
© Kataryna Nemethy 2007
Nucleus pulposus
DEGENERATIVE DISC DISEASE • loss of vertebral disc height with age results in: bulging and tears of annulus fibrosus change in alignment of facet joints osteophyte formation • can cause back-dominant pain • management non-operative staying active with modified activity back strengthening NSAIDs do not treat with opioids; no proven efficacy of spinal traction or manipulation operative – rarely indicated decompression ± fusion no difference in outcome between non-operative and surgical management at 2 years Table 12. Types of Low Back Pain Mechanical Back Pain
Direct Nerve Root Compression
Disc Origin
Facet Origin
Spinal Stenosis
Root Compression
Pain Dominance
Back
Back
Leg
Leg
Aggravation
Flexion
Extension Standing, walking
Exercise, extension, walking, standing
Flexion
Onset
Gradual
More sudden
Congenital or acquired
Acute leg ± back pain
Duration
Long (weeks, months)
Shorter (days, weeks)
Acute or chronic history (weeks to months)
Short episodes Attacks (minutes)
Treatment
Relief of strain, exercise Relief of strain, exercise
Relief of strain, exercise
Relief of strain, exercise + surgical decompression if progressive or severe deficit
SPINAL STENOSIS • definition: narrowing of spinal canal 6 months
Toronto Notes 2011
Spine
Orthopaedics OR25
Table 13. Differentiating Claudication Neurogenic
Vascular
Aggravation
With standing or exercise Walking distance variable
Walking set distance
Alleviation
Change in position (usually flexion, sitting, lying down)
Stop walking
Time
Relief in ~10 min
Relief in ~2 min
Character
Neurogenic ± neurological deficit
Muscular cramping
Back Pain Back Dominant Constant Inflammatory Mechanical
Leg Dominant Intermittent
Disc Herniation (central)
Constant Disc Herniation (lateral)
Intermittent Spinal Stenosis
Facet Joint
Figure 33. Approach to Back Pain
MECHANICAL BACK PAIN • definition: back pain NOT due to prolapsed disc or any other clearly defined pathology • clinical features dull backache aggravated by activity morning stiffness no neurological signs • treatment: symptomatic (analgesics, PT) • prognosis: symptoms may resolve in 4-6 weeks, others become chronic LUMBAR DISC HERNIATION • definition: tear in annulus fibrosus allows protrusion of nucleus pulposus causing either a central, posterolateral or lateral disc herniation, most commonly at L5-S1 > L4-5 > L3-4 • etiology: usually a history of flexion-type injury which tears the annulus fibrosus allowing for protrusion of the nucleus pulposus • clinical features back dominant pain (central herniation) or leg dominant pain (lateral herniation) tenderness between spines at affected level muscle spasm ± loss of normal lumbar lordosis neurological disturbance is segmental and varies with level of central herniation motor weakness (L4, L5, S1) diminished reflexes (L4, S1) diminished sensation (L4, L5, S1) +ve straight leg raise +ve Lasegue test bowel or bladder symptoms, decreased rectal tone suggests cauda equina syndrome due to central disc hernation – surgical emergency • investigations: MRI • treatment symptomatic extension protocol (PT) NSAIDs 90% resolve in 3 months surgical discectomy reserved for progressive neurological deficit, failure of symptoms to resolve within 3 months or cauda equina syndrome due to central disc herniation
MRI abnormalities are quite common in both asymptomatic and symptomatic individuals and are not necessarily an indication for intervention without clinical correlation.
OR26 Orthopaedics
Spine/Hip
Spondylolysis
© Ryo Sakai 2007
Spondylolisthesis (anterior displacement)
Figure 34. Spondylolysis, Spondylolisthesis
Toronto Notes 2011
SPONDYLOLYSIS • definition: defect in the pars interarticularis with no movement of the vertebral bodies • etiology trauma: gymnasts, weightlifters, backpackers, loggers, labourers • clinical features: activity-related back pain • investigations oblique x-ray: “collar” break in the “Scottie dog’s” neck bone scan CT scan • treatment: activity restriction, brace, stretching exercise SPONDYLOLISTHESIS • definition: defect in pars interarticularis causing a forward slip of one vertebrae on another usually at L5-S1, less commonly at L4-5 • etiology: congenital (children), degenerative (adults), traumatic, pathological, teratogenic • clinical features: lower back pain radiating to buttocks Table 14. Classification and Treatment of Spondylolisthesis Class
Percentage of Slip
Treatment
1
0-25%
Symptomatic operative fusion only for intractable pain
2
25-50
3
50-75
4
75-100
5
>100
Decompression for spondylolisthesis and spinal fusion
Specific Complications • may present as cauda equina syndrome due to roots being stretched over the edge of L5 or sacrum
Hip Hip Fracture
DVT Prophylaxis in Hip Fractures LMWH (i.e. enoxaparin 40 mg SC bid) on admission, do not give 90 degrees or crossing legs for approximately 6 weeks after surgery) Epidemiology • occurs in 1-4% of primary THA and 10-16% of revision THAs • risk factors: neurological impairment, post-traumatic arthritis, revision surgery, substance abuse Treatment • external abduction splint to prevent hip adduction • constrained acetabular component for recurrent dislocation if no issue with position of acetabular/femoral implants Complications • sciatic nerve palsy in 25% (10% permanent) • heterotopic ossification (HO)
Femur Femoral Diaphysis Fracture Mechanism • high energy trauma (MVA, fall from height, gunshot wound) • in children, can result from low energy trauma (spiral fracture) Clinical Features • shortened, externally rotated leg (if fracture displaced) • inability to weight-bear • often open injury, always a Gustilo III Investigations • AP pelvis, AP/lateral hip, femur, knee Specific Complications • hemorrhage requiring transfusion • fat embolism leading to ARDS • extensive soft tissue damage • ipsilateral hip dislocation/fracture • nerve injury
Supracondylar
Treatment • stabilize patient • immobilize leg • ORIF with intramedullary nail, external fixator, or plate and screws within 24 hours • early mobilization and strengthening
Condylar
Intercondylar
Figure 37. Distal Femoral Fractures
© Paul Belletrutti 2003
Distal Femoral Fracture Mechanism • direct high energy force or axial loading • three types (Figure 37)
Toronto Notes 2011
Femur/Knee
Orthopaedics OR29
Clinical Features • direct high energy force or axial loading • extreme pain • knee effusion (hemarthrosis) • shortened, externally rotated leg if displaced Treatment • ORIF • early mobilization and strengthening Complications (see General Fracture Complications, OR6) • femoral artery tear • nerve injury • extensive soft tissue injury • angulation deformities
4
Physical Examination • general orthopaedic physical exam (do not forget to evaluate hip) Special Tests of the Knee • Anterior and Posterior drawer tests (see Figure 39) demonstrate torn ACL and PCL, respectively knee flexed at 90º, foot immobilized, hamstrings released if able to sublux tibia anteriorly, then ACL may be torn if able to sublux tibia posteriorly, then PCL may be torn • Lachmann test demonstrates torn ACL hold knee in 10-20º flexion, stabilizing the femur try to sublux tibia anteriorly on femur similar to anterior drawer test, more reliable due to less muscular stabilization • Posterior sag sign demonstrates torn PCL may give a false positive anterior draw sign flex knees and hips to 90º, hold ankles and knees view from the lateral aspect if one tibia sags posteriorly compared to the other, its PCL is torn • Pivot shift sign demonstrates torn ACL start with the knee in extension internally rotate foot, slowly flex knee while palpating and applying a valgus force normal knee will flex smoothly if incompetent ACL, tibia will sublux anteriorly on femur at start of maneuver. During flexion, the tibia will reduce and externally rotate about the femur (the “pivot”) reverse pivot shift (start in flexion, externally rotate, apply valgus and extend knee) suggests torn PCL • Collateral ligament stress test palpate ligament for “opening” of joint space while testing with knee in full extension, apply valgus force to test MCL, apply varus force to test LCL repeat tests with knee in 20º flexion to relax joint capsule opening only in 20º flexion due to MCL damage only opening in 20º of flexion and full extension is due to MCL, cruciate, and joint capsule damage
Posterior horn of lateral meniscus Anterior horn of lateral meniscus Posterior cruciate ligament Anterior cruciate ligament Posterior horn of medial meniscus Anterior horn of medial meniscus
© Jenn Platt 2004
1. 2. 3. 4. 5. 6.
6
Figure 38. Diagram of the Right Tibial Plateau
6 Degrees of Freedom of the Knee 1. Flex. and ext. 2. Ext. and int. rotation 3. Varus and valgus angulation 4. Ant. and post. glide 5. Med. and lat. shift 6. Compression and distraction
Knee History CLIPS Clicking Locking Instability Pain (location) Swelling
Physical examination difficult in acute knee injuries. Immobilize leg and reexamine in one week.
Anterior Drawer Test
Posterior Drawer Test
© Tabby Lulham 2010
History • general orthopaedic history • also inquire about common knee symptoms locking: mechanical block to extension torn meniscus/loose body in joint pseudo-locking: limited ROM without mechanical block effusion, muscle spasm after injury, arthritis painful clicking (audible) torn meniscus giving way: instability cruciate ligament or meniscal tear, patellar dislocation
5
2
Knee Evaluation of Knee Complaints
3
1
Figure 39. Anterior and Posterior Drawer Test
OR30 Orthopaedics
Knee
• Tests for meniscal tear Crouch compression test joint line pain when squatting (anterior pain suggests patellofemoral pathology) McMurray’s test useful collaborative information (see Figure 40) with knee in flexion, palpate joint line for painful “pop/click” internally rotate foot, varus stress, and extend knee to test lateral meniscus externally rotate foot, valgus stress, and extend knee to test medial meniscus
© Tabby Lulham 2010
Examination for Medial Meniscal Tear
Examination for Lateral Meniscal Tear
Toronto Notes 2011
Figure 40. McMurray Test
X-Rays • AP standing, lateral • skyline – tangential view with knees flexed at 45º to see patellofemoral joint • 3-foot standing view – useful in evaluating leg length and varus/valgus alignment • see Ottawa Knee Rules (Emergency Medicine, ER17)
Cruciate Ligament Tears • ACL tear much more common than PCL tear Table 17. Comparison of ACL and PCL Injuries Anterior Cruciate Ligament
Figure 41. T1 MRI of Torn ACL
Posterior Cruciate Ligament
Mechanism
Sudden deceleration Sudden posterior displacement of tibia when knee is Hyperextension and internal rotation of tibia on femur flexed or hyperextended (dashboard MVA injury)
History
Audible “pop” Immediate swelling Knee “giving way” Inability to continue activity
Audible “pop” Immediate swelling Pain with push off Cannot descend stairs
Physical
Effusion (hemarthrosis) Posterolateral joint line tenderness Positive anterior drawer Positive Lachmann Pivot shift Test for MCL, meniscal injuries
Effusion (hemarthrosis) Anteromedial joint line tenderness Positive posterior drawer Reverse pivot shift Other ligamentous, bony injuries
Treatment
Stable knee with minimal functional impairment: immobilization 2-4 weeks with early ROM and strengthening
Unstable knee or young person/high-demand lifestyle: ligament reconstruction Posterior sag
Collateral Ligament Tears • MCL tear more common than LCL tear Mechanism • valgus force to knee = medial collateral ligament • varus force to knee = lateral collateral ligament
Partial ligamentous tears are much more painful than complete ligamentous tears.
Clinical Features • swelling/effusion • tenderness above and below joint line medially (MCL) or laterally (LCL) • joint laxity with varus or valgus force to knee laxity with endpoint suggests partial tear laxity with no endpoint suggests a complete tear • test for other injuries (e.g. O’Donahue’s triad), common peroneal nerve injury Treatment • partial tear: immobilization x 2-4 weeks with early ROM and strengthening • complete tear or multiple ligamentous injuries: surgical repair of ligaments – not for MCL or LCL on their own
Meniscal Tears • medial tear much more common than lateral tear Tissue Sources for ACL Reconstruction 1. Hamstring 2. Middle 1/3 patellar tendon (bone-patellar-bone) 3. Allograft (e.g. cadaver)
Mechanism • twisting force on knee when it is partially flexed (e.g. stepping down and turning) • requires moderate trauma in young person but only mild trauma in elderly due to degeneration
Toronto Notes 2011
Knee
Clinical Features • immediate pain, difficulty weight-bearing, instability and clicking • increased pain with squatting and/or twisting • effusion (hemarthrosis) with insidious onset (24-48 hrs after injury) • joint line tenderness medially or laterally • locking of knee (if portion of meniscus mechanically obstructing extension) Investigations • MRI, arthroscopy Treatment • if not locked: ROM and strengthening • if locked or failed above: arthroscopic repair/partial meniscectomy
Quadriceps/Patellar Tendon Rupture Mechanism • sudden forceful contraction of quadriceps during an attempt to stop • more common in obese patients and those with pre-existing degenerative changes in tendon DM, SLE, RA, steroid use, renal failure on dialysis Clinical Features • inability to extend knee or weight-bear • possible audible “pop” • patella in lower or higher position with palpable gap above or below patella respectively • may have an effusion Investigations • ask patient to straight leg raise • knee x-ray to rule out patellar fracture • lateral view: patella alta with patella tendon rupture, patella baja with quadriceps tendon rupture Treatment • nonoperative treatment for incomplete tears with preserved extension of knee • surgical repair of tendon indicated for complete ruptures
Dislocated Knee Mechanism • high energy trauma • by definition, caused by tears of multiple ligaments Clinical Features • classified by relation of tibia with respect to femur anterior, posterior, lateral, medial, rotary • knee instability • effusion • pain • ischemic limb Investigations • x-rays: AP, lateral, skyline associated radiographic findings include tibial plateau fracture dislocations, proximal fibular fractures and avulsion of fibular head • ankle brachial index (abnormal if less than 0.9) • arteriogram if abnormal vascular exam Treatment • urgent closed reduction complicated by interposed soft tissue • assessment of peroneal nerve, tibial artery, and ligamentous injuries • repair of associated injuries; also may need decompressive fasciotomy especially if vascular repair undertaken fasciotomy • knee immobilization x 6-8 weeks
Orthopaedics OR31
OR32 Orthopaedics
Knee/Patella
Toronto Notes 2011
Specific Complications • high incidence of associated injuries popliteal artery tear peroneal nerve injury capsular tear • chronic: instability, stiffness, post-traumatic arthritis
Patella Patellar Fracture Mechanism • direct blow to the patella • indirect trauma by sudden flexion of knee against contracted quadriceps Undisplaced
Vertical
Lower/Upper Pole Comminuted Displaced
Osteochondral
Transverse
© Julie Saunders 2003
Clinical Features • marked tenderness • inability to extend knee or straight leg raise • proximal displacement of patella • patellar deformity • ± effusion Investigations • x-rays: AP, lateral, skyline • consider bipartite patella: congenitally unfused ossification centres with smooth margins on x-ray Treatment • non-displaced (2 mm) • comminuted: ORIF; may require partial/complete patellectomy
Figure 42. Types of Patellar Fractures
Patellar Dislocation Mechanism • lateral displacement of patella after contraction of quadriceps against a flexed knee Risk Factors • young, female • obesity • high-riding patella (patella alta) • knock-knees (genu valgum) • Q-angle (quadriceps angle) increased • shallow intercondylar groove • weak vastus medialis • tight lateral retinaculum
ASIS
Q-angle
Central patella
Tibial tuberosity
Figure 43. Q-Angle
© Michael Corrin 2005
Clinical Features • knee catches or gives way with walking • severe pain, tenderness anteromedially from rupture of capsule • weak knee extension or inability to extend leg unless patella reduced • +ve patellar apprehension test patient apprehensive when examiner laterally displaces patella • often recurrent, self-reducing Investigations • x-rays: AP, lateral, skyline view of patella check for fracture of medial patella and lateral femoral condyle Treatment • non-operative first knee immobilization x 4-6 weeks progressive weight bearing and isometric quadriceps strengthening • if recurrent surgical tightening of medial capsule and release of lateral retinaculum, possible tibial tuberosity transfer, or proximal tibial osteotomy
Toronto Notes 2011
Patellofemoral Syndrome
Patella/Tibia
Orthopaedics OR33
(Chondromalacia Patellae)
Mechanism • softening, erosion and fragmentation of articular cartilage, predominantly medial aspect of patella • commonly seen in active young females • predisposing factors malalignment causing patellar maltracking (patellofemoral syndrome) post-trauma deformity of patella or femoral groove recurrent patellar dislocation, ligamentous laxity excessive knee strain (athletes) Clinical Features • deep, aching anterior knee pain exacerbated by prolonged sitting (theatre sign), strenuous athletic activities, stair climbing, squatting • sensation of instability, pseudolocking • tenderness to palpation of underside of medially displaced patella • pain with extension against resistance through terminal 30-40º • swelling rare, minimal if present
Pain with firm compression of patella into medial femoral groove is pathognomonic of chondromalacia patellae.
Investigations • x-rays: AP, lateral, skyline Treatment • non-operative continue non-impact activities NSAIDs PT: quadriceps strengthening • surgical with refractory patients tibial tubercle elevation arthroscopic shaving/debridement lateral release of retinaculum
Tibia Tibial Plateau Fracture Mechanism • axial loading (e.g. fall from height) • femoral condyles driven into proximal tibia • can result from minor trauma in osteoporotics Clinical Features • lateral fractures more common than medial
Schatzker Classification
Classification • Schatzker classification (see sidebar)
Type
Description
I
Involvement of lateral plateau split fracture
Investigations • x-rays: AP, lateral, skyline
II
Involvement of lateral plateau: split depression fracture
III
Involvement of lateral plateau: pure depression fracture
IV
Medial plateau fracture
V
Bicondylar plateau fracture
VI
Bicondylar with metaphyseal/ diaphyseal involvement
Treatment • if depression on x-ray is 3 mm ORIF often requiring bone grafting to elevate depressed fragment Specific Complications (see General Fracture Complications, OR6) • ligamentous injuries • meniscal lesions • AVN • infection
OR34 Orthopaedics
Tibia/Ankle
Toronto Notes 2011
Tibial Shaft Fracture Tibial shaft fractures have high incidence of compartment syndrome and are often associated with soft tissue injuries.
Mechanism • numerous, including MVA, falls, sporting injuries Clinical Features • open vs. closed • amount of displacement • neurovascular status • most commonly fractured long bone • most common open fracture Investigations • x-rays: AP, lateral, skyline Treatment • closed minimally displaced: straight leg cast x 4-6 weeks with early weight bearing displaced: ORIF with reamed IM nail, plate and screws, or external fixator • open external fixation or IM nail vascularized coverage of soft tissue defects (often heal poorly)
Figure 44. Tibial Shaft Fracture Treated with Intra-medullary Nail and Screws
Specific Complications (see General Fracture Complications, OR6) • high incidence of neurovascular injury and compartment syndrome • poor soft tissue coverage
Ankle Evaluation of Ankle and Foot Complaints Special Tests • anterior drawer: examiner attempts to displace the foot anteriorly against a fixed tibia • talar tilt: foot is stressed in inversion and angle of talar rotation is evaluated by x-ray
Ottawa Ankle Rules (see Emergency Medicine, ER17) X-rays are only required if: Pain in the malleolar zone AND bony tenderness over the posterior aspect of the medial or lateral malleolus OR inability to weight bear both immediately after injury and in the E.R.
X-Ray • AP, lateral • mortise view: ankle at 15º of internal rotation gives true view of ankle joint joint space should be symmetric with no talar tilt • Ottawa Ankle Rules should guide use of x-ray (see sidebar) • ± CT to better characterize fractures
Ankle Fracture Mechanism • pattern of fracture depends on the position of the ankle when trauma occurs • generally involves ipsilateral ligamentous tears or transverse bony avulsion contralateral shear fractures (oblique or spiral) • classification systems Danis-Weber (see below) Lauge-Hansen: based on foot’s position and motion relative to leg Danis-Weber Classification (Figure 45) • based on level of fibular fracture relative to syndesmosis • Type A (infra-syndesmotic) pure inversion injury avulsion of lateral malleolus below plafond or torn calcaneofibular ligament ± shear fracture of medial malleolus • Type B (trans-syndesmotic) external rotation and eversion (most common) ± avulsion of medial malleolus or rupture of deltoid ligament spiral fracture of lateral malleolus starting at plafond
Toronto Notes 2011
Ankle/Foot
• Type C (supra-syndesmotic) pure external rotation avulsion of medial malleolus or torn deltoid ligament ± posterior malleolus may be avulsed with posterior tibio-fibular ligament fibular fracture is above plafond (called Maisonneuve fracture if at proximal fibula) frequently tears syndesmosis Treatment • undisplaced: non-weight bearing below knee cast • indications for ORIF all fracture-dislocations most of type B, and all of type C trimalleolar (medial, posterior, lateral) fractures talar tilt >10º medial clear space on XR greater than superior clear space open fracture/open joint injury • high incidence of post-traumatic arthritis
Ligamentous Injuries
Orthopaedics OR35
1 2
4 5
3
6
Normal Ankle
Type A
Type B
Type C
Legend 1. Posterior malleolus 2. Medial malleolus 3. Deltoid ligament 4. Syndesmosis 5. Lateral malleolus 6. Calcanofibular ligament
Medial Ligament Complex (deltoid ligament) • eversion injury • usually avulses medial or posterior malleolus and strains syndesmosis Lateral Ligament Complex (ATF, CF, PTF) • inversion injury • ATF most severely injured if ankle is plantar flexed • swelling and tenderness anterior to lateral malleolus • ++ ecchymoses • +ve ankle anterior drawer • may have significant medial talar tilt on inversion stress x-ray Treatment • microscopic tear (Grade I) rest, ice, compression, elevation (RICE) • macroscopic tear (Grade II) strap ankle in dorsiflexion and eversion x 4-6 weeks PT: strengthening and proprioceptive retraining • complete tear (Grade III) below knee walking cast 4-6 weeks PT: strengthening and proprioceptive retraining surgical intervention may be required if chronic symptomatic instability develops
Foot
Figure 45. Ring Principle of the Ankle and Danis-Weber Classification PTF CF ATF
PTT TC ATT
Talar Fracture Mechanism • axial loading or hyperdorsiflexion (MVA, fall from a height) • 60% of talus covered by articular cartilage • tenuous blood supply runs distal to proximal along talar neck high risk of AVN with displaced fractures Investigations • x-rays: AP, lateral • CT to better characterize fracture • MRI can clearly define extent of AVN Treatment • undisplaced: non-weight bearing below knee cast x 20-24 weeks • displaced: ORIF (high rate of nonunion, AVN)
TN Legend PTF: Posterior talofibular CF: Calcaneofibular ATF: Anterior talofibular PTT: Posterior tibiotalar TC: Tibiocalcaneal ATT: Anterior tibiotalar TN: Tibionavicular
Figure 46. Ankle Ligament Complexes
With a history of trauma from axial loading of lower limb always consider spinal injuries, femoral neck, tibial plateau, talar/calcaneal fractures.
OR36 Orthopaedics
Foot
Toronto Notes 2011
Calcaneal Fracture Mechanism • axial loading: fall from a height onto heels • 10% of fractures associated with compression fractures of thoracic or lumbar spine • 5% are bilateral Calcaneal Fracture Treatment Principles 1. Avoid wound complications 2. Restore articular congruity 3. Restore normal calcaneal width and height 4. Maximum functional recovery may take longer than 12 months
Physical Examination • swelling, bruising on heel/sole • wider, shortened, flatter heel when viewed from behind Investigations • x-rays: AP, lateral, oblique (Broden’s view) • loss of Bohler’s angle • CT – assess intraarticular extension Treatment • closed vs. open reduction is controversial • non-weight bearing cast approximately 3 months with early ROM and strengthening
Achilles Tendonitis Mechanism • chronic inflammation from activity or poor-fitting footwear • may also develop heel bumps (retrocalcaneobursitis) Physical Examination • pain, stiffness and crepitus with ROM • thickened tendon, palpable bump Treatment • rest, NSAIDs • gentle stretching, deep tissue calf massage • orthotics, open back shoes • DO NOT inject steroids (risk of tendon rupture)
Achilles Tendon Rupture Mechanism • loading activity, stop-and-go sports (e.g. squash, tennis, basketball) • secondary to chronic tendonitis, steroid injection The most common site of Achilles tendon rupture is 2-6 cm from its insertion where the blood supply is the poorest.
Clinical Features • audible pop, sudden pain with push off movement • sensation of being kicked in heel when trying to plantar flex • palpable gap • apprehensive toe off when walking • weak plantar flexion, +ve Thompson test: with patient prone, squeezing the calf muscles should passively plantar flex the foot to demonstrate intact Achilles tendon +ve test = no passive plantar flexion = ruptured tendon Treatment • low demand or elderly: cast foot in plantar flexion (to relax tendon) x 8-12 weeks • high demand: surgical repair, then cast as above x 6-8 weeks
Plantar Fasciitis (Heel Spur Syndrome) Mechanism • repetitive strain injury causing microtears and inflammation of plantar fascia • female:male = 2:1 • common in athletes (especially runners) • also associated with obesity, DM, seronegative and seropositive arthritis
Toronto Notes 2011
Foot
Orthopaedics OR37
Clinical Features • morning pain and stiffness • intense pain when walking from rest that subsides as patient continues to walk • swelling, tenderness over sole • greatest at medial calcaneal tubercle and 1-2 cm distal along plantar fascia • pain with toe dorsiflexion (stretches fascia) Investigations • plain radiographs to rule out fractures • often see exostoses (heel spurs) at insertion of fascia into medial calcaneal tubercle (see Figure 47) • spur is reactive to inflammation, not the cause of pain
Cuneiform
Talus
Navicular Bone spur
Calcaneus
Figure 47. X-Ray of Bony Heel Spur
Treatment • rest, ice, NSAIDs, steroid injection • PT: stretching, ultrasound • orthotics with heel cup to counteract pronation and disperse heel strike forces • endoscopic surgical release of fascia in refractory cases spur removal is not required
Bunions (Hallux Valgus) Mechanism • valgus alignment on 1st MTP (hallux valgus) causes eccentric pull of extensor and intrinsic muscles • reactive exostosis forms with thickening of the skin creating a bunion • most often associated with poor-fitting footwear but can be hereditary • 10x more frequent in women
Normal angle 15º
© Michelle Lui 2004
Clinical Features • painful bursa over medial eminence of 1st metatarsal head • pronation (rotation inward) of great toe • numbness over medial aspect of great toe Treatment • cosmetic and to relieve pain • non-operative first properly fitted shoes (low heel) and toe spacer • surgical osteotomy with realignment of 1st MTP joint
Figure 48. Hallux Valgus
Metatarsal Fracture • as with the hand, 1st, 4th, 5th metatarsals (MT) are relatively mobile, while the 2nd and 3rd are fixed (Table 18) • use Ottawa Foot Rules to determine need for x-ray (see sidebar) Table 18. Types of Metatarsal Fractures Fracture Type
Mechanism
Clinical
Treatment
Avulsion of base of 5th MT
Sudden inversion followed by contraction of peroneus brevis
Tender base of 5th MT
Requires ORIF if displaced
Midshaft 5th MT (Jones fracture)
Stress injury
Painful shaft of 5th MT
*NWB BK cast x 6 wks ORIF if athlete
Shaft 2nd, 3rd MT (March fracture)
Stress injury
Painful shaft of 2nd or 3rd MT Symptomatic
1st MT
Trauma
Painful 1st MT
ORIF if displaced otherwise NWB BK cast x 3 wks then walking cast x 2 wks
Shortened forefoot prominent base
ORIF (Lisfranc fracture)
Tarso-MT fracture – dislocation Fall onto plantar flexed foot or direct crush injury * NWB BK = Non weight bearing, below knee cast
Ottawa Foot Rules X-rays only required if: Pain in the midfoot zone AND bony tenderness over the navicular or base of the fifth metatarsal OR inability to weight bear both immediately after injury and in the ER.
OR38 Orthopaedics
Pediatric Orthopaedics
Toronto Notes 2011
Pediatric Orthopaedics Fractures in Children
© Sarah A. Kim 2005
Greenstick fractures are easy to reduce but can redisplace while in cast due to intact periosteum.
Proximal Radius
Proximal Radius
Figure 49. Greenstick (left) and Torus (right) Fractures
• type of fracture usually greenstick or buckle because periosteum is thicker and stronger adults fracture through both cortices • epiphyseal growth plate plate often mistaken for fracture and vice versa x-ray opposite limb for comparison mechanism which causes ligamentous injury in adults causes growth plate injury in children intra-articular fractures have worse consequences in children because they usually involve the growth plate • anatomic reduction gold standard with adults may cause limb length discrepancy in children (overgrowth) accept greater angular deformity in children (remodeling minimizes deformity) • time to heal shorter in children • always be aware of the possibility of child abuse make sure mechanism compatible with injury high index of suspicion, look for other signs, including x-ray evidence of healing fractures at other sites
Stress Fractures Mechanism • insufficiency fracture stress applied to a weak or structurally deficient bone • fatigue fracture repetitive, excessive force applied to normal bone • most common in adolescent athletes • tibia is most common site
Type I
Diagnosis and Treatment • localized pain and tenderness over the involved bone • plain films may not show fracture for 2 weeks • bone scan +ve in 12-15 days • treatment is rest from strenuous activities to allow remodeling (can take several months)
Type II
Evaluation of the Limping Child Type III
• see Pediatrics, P95
Epiphyseal Injury Table 19. Salter-Harris Classification of Epiphyseal Injury
Type IV
© Kimberly Chin
Type V
Figure 50. Salter-Harris Classification
SALT(E)R–Harris Type
Description
Treatment
I (Straight through; Stable)
Transverse through growth plate
Closed reduction and cast immobilization heals well, 95% do not affect growth
II (Above)
Through metaphysis and along growth plate
III (Low)
Through epiphysis to plate and along growth Anatomic reduction by ORIF to prevent growth arrest plate
IV (Through and through) Through epiphysis and metaphysis V (Ram)
Crush injury of growth plate
High incidence of growth arrest; no specific treatment
Toronto Notes 2011
Pediatric Orthopaedics
Orthopaedics OR39
Slipped Capital Femoral Epiphysis (SCFE) • type I Salter-Harris epiphyseal injury • most common adolescent hip disorder, peak at pubertal growth spurt • risk factors: male, obese, hypothyroid Etiology • multifactorial genetic: autosomal dominant, blacks > caucasians cartilaginous physis thickens rapidly under growth hormone (GH) effects sex hormone secretion, which stabilizes physis, has not yet begun overweight: mechanical stress trauma: causes acute slip Clinical Features • acute: sudden, severe pain with limp • chronic: limp with medial knee or anterior thigh pain • tender over joint capsule • restricted internal rotation, abduction, flexion Whitman’s sign: with flexion there is an obligate external rotation of the hip • pain at extremes of ROM Investigations • x-rays: AP, frog-leg, lateral radiographs posterior and medial slip if mild slip, AP view may be normal or show slightly widened growth plate compared with opposite side Treatment and Complications • mild/moderate slip: stabilize physis with pins in current position • severe slip: ORIF or pin physis without reduction and osteotomy after epiphyseal fusion • complications: AVN (most common), chondrolysis, pin penetration, premature OA, loss of ROM
In slipped capital femoral epiphysis, bilateral involvement occurs in about 25%.
Developmental Dysplasia of the Hip (DDH) • formerly called congenital dysplasia of the hip (CDH) • due to ligamentous laxity, muscular underdevelopment, and abnormal shallow slope of acetabular roof • spectrum of conditions that lead to hip subluxation and dislocation dislocated femoral head completely out of acetabulum dislocatable head in socket head subluxates out of joint when provoked dysplastic acetabulum, more shallow and more vertical than normal • painless (if painful suspect septic dislocation) Physical Examination • diagnosis is clinical limited abduction of the flexed hip (18 months: open reduction; pelvic and/or femoral osteotomy • complications redislocation, inadequate reduction, stiffness AVN of femoral head
Legg-Calve-Perthes Disease (Coxa Plana) • self-limited AVN of femoral head, presents at 4-10 years of age • etiology unknown, 20% bilateral, males > females, 1/10,000 • associations family history low birth weight abnormal pregnancy/delivery history of trauma to affected hip • key features AVN of proximal femoral epiphysis, abnormal growth of the physis, and eventual remodelling of regenerated bone Clinical Features • child with hip pain and limp • tender over anterior thigh • flexion contracture: decreased internal rotation, abduction of hip Investigations • x-rays may be negative early eventually, characteristic collapse of femoral head (diagnostic) • subchondral fracture • metaphyseal cyst Treatment • goal is to preserve ROM and preserve femoral head in acetabulum • PT: ROM exercises • brace in flexion and abduction x 2-3 years • femoral or pelvic osteotomy • prognosis better in males F, severity F>M
Figure 51. The Club Foot – Depicting the Gross and Bony Deformity
Scoliosis Definition • lateral curvature of spine with vertebral rotation Epidemiology • age: 10-14 years • more frequent and more severe in females
Clinical Features • ± back pain • 1º curve where several vertebrae affected • 2º curves above and below fixed 1º curve to try and maintain normal position of head and pelvis • asymmetric shoulder height when bent forward • Adam’s test: rib hump when bent forward • prominent scapulae, creased flank, asymmetric pelvis • associated posterior midline skin lesions in non-idiopathic scolioses café-au-lait spots, dimples, neurofibromas axillary freckling, hemangiomas, hair patches • associated pes cavus or leg atrophy • apparent leg length discrepancy X-Rays • 3-foot standing measure curvature – Cobb Angle (Figure 52) may have associated kyphosis Treatment • based on degree of curvature 20º or progressive: bracing (many types) that halt/slow curve progression but do NOT reverse deformity >40º, cosmetically unacceptable or respiratory problems: surgical correction (spinal fusion)
Cobb angle
© Crista Mason 2005
Etiology • idiopathic: most common (90%) • congenital: vertebrae fail to form or segment • neuromuscular: UMN or LMN lesion, myopathy • other: osteochondrodystrophies, neoplastic, traumatic • postural: leg length discrepancy, muscle spasm
Figure 52. Cobb Angle – used to monitor the progression of the scoliotic curve
In structural or fixed scoliosis, bending forwards makes the curve more obvious.
Postural scoliosis can be corrected by correcting the underlying problem.
OR42 Orthopaedics
Bone Tumours
Toronto Notes 2011
Bone Tumours • primary bone tumours are rare after 3rd decade • metastases to bone are relatively common after 3rd decade
Red Flags • Persistent skeletal pain • Localized tenderness • Spontaneous fracture • Enlarging mass/soft tissue swelling
Neoplasm
Codman’s triangle
Figure 53. Codman’s Triangle – a radiographic finding in malignancy, where the partially ossified periosteum is lifted off the cortex by neoplastic tissue
© j.a.platt 2005
Periosteum
Diagnosis • pain, swelling, tenderness, rarely regional adenopathy • routine x-ray location (which bone, diaphysis, metaphysis, epiphysis) size lytic/lucent vs. sclerotic involvement (cortex, medulla, soft tissue) matrix (radiolucent, radiodense or calcified) periosteal reaction margin (geographic vs. permeative) any pathological fracture soft tissue swelling • malignancy is suggested by rapid growth, warmth, tenderness, lack of sharp definition • staging should include bloodwork including liver enzymes CT chest bone scan bone biopsy should be referred to specialized centre prior to biopsy classified into benign, benign aggressive, and malignant MRI of affected bone
Benign Active Bone Tumours 1. Osteoid Osteoma • peak incidence in 2nd and 3rd decades, M:F = 3:1 • small, round radiolucent nidus (40 years old • present with anemia, anorexia, renal failure, nephritis, increased ESR, bone pain (cardinal early symptom), compression fractures, hypercalcemia • high incidence of infections (e.g. pyelonephritis/pneumonia)
Figure 57. X-Ray of Femoral Chondrosarcoma
OR44 Orthopaedics
Bone Tumours/Articular Cartilage Defects
• diagnosis CT-guided biopsy of lytic lesions at multiple bony sites serum/urine protein electrophoresis • treatment: chemotherapy, radiation, surgery for symptomatic lesions or impending fractures • see Hematology, H47
Most Common Tumours Metastatic to Bone Thyroid Breast Lung
Breast Lung
Melanoma Kidney Prostate
Toronto Notes 2011
Kidney
5. Bone Metastases • 2/3 from breast or prostate; also consider thyroid, lung, kidney • usually osteolytic; prostate occasionally osteoblastic • bone scan for MSK involvement, MRI for spinal involvement may be helpful • stabilization of impending fractures internal fixation, IM rods bone cement
BLT with a Kosher Pickle Breast Lung Thyroid Kidney Prostate
Articular Cartilage Defects Properties of Articular Cartilage • lacks blood supply and does not have innervation or lymphatic drainage • varies in thickness from 2 mm to 4 mm and is thickest at periphery of concave surfaces and central portions of convex surfaces • composed of type 2 collagen, water, proteoglycans, and chondrocytes • collagen provides resistance against tensile stresses and transmits vertical loads • water and proteoglycans provide turgor and elasticity and help to limit friction • chondrocytes synthesize the cartilage matrix and control matrix turnover rate Etiology • overt trauma or repeated minor trauma; most commonly from sports injuries • early stage osteoarthritis • genetic degenerative diseases such as osteochondritis dissecans Clinical Features • very similar to symptoms of osteoarthritis (joint line pain with possible effusion, etc.) • often have predisposing factors such as ligament injury, malalignment of the joint (varus/ valgus), obesity, bone deficiency (avascular necrosis, osteochondritis dissecans, ganglion bone cysts), inflammatory arthropathy, and familial osteoarthropathy • may have symptoms of locking or catching related to the torn/displaced cartilage Investigations • arthroscopy to visualize focal pathology and guide treatment strategy • MRI may also be used to visualize the defect Table 21. Outerbridge Classification of Chondral Defects Grade
Chondral Damage
I
Softening and swelling of cartilage
II
Fragmentation and fissuring 1/2 inch in diameter
IV
Erosion of cartilage down to bone
Treatment • arthroscopic lavage and debridement of the joint • marrow stimulation techniques (microfracture, drilling, abrasion arthroplasty) involves creating a site of bleeding where new growth/healing can take place • osteochondral grafts; also known as the OATS procedure or mosaicplasty involves transferring osteochondral fragments from non-weightbearing surface to area of defect • autologous chondrocyte implantation (ACI) currently only available in the U.S. and Europe involves harvesting patient’s cartilage, growing it in culture medium outside of the patient, then reinserting the newly cultured chondrocytes back to fill the chondral defect • osteochondral allograft; only used in limited circumstances when defect is very large
Toronto Notes 2011
Common Medications
Common Medications Table 22. Common Medications Drug Name
Dosing Schedule
Indications
Comments
cefazolin (Ancef®)
1-2 g IV q8h
Prophylactically before orthopaedic surgery
First generation cephalosporin; do not use with penicillin allergy
heparin
5000 IU SC q12h
To prevent venous thombosis and pulmonary emboli
Monitor platelets, follow PTT which should rise 1.5-2x
LMWH dalteparin (Fragmin®) enoxaparin (Lovenox®) fondaparinux (Arixtra®)
5000 IU SC OD 30-40 mg SC bid 2.5 mg SC OD
DVT prophylaxis esp. in hip and knee surgery
Fixed dose, no monitoring, improved bioavailability, increased bleeding rates
midazolam (Versed®)
0.02 mg/kg IV
Conscious sedation for short procedures
Medications used together during fracture reduction – monitor for respiratory depression
fentanyl (Sublimaze®)
0.5-3 µg/kg IV
Conscious sedation for short procedures
Short acting anesthetic used in conjunction with midazolam (Versed®)
triamcinolone (Aristocort®) – an injectable steroid
0.5-1 mL of 25 mg/mL
Suspension (injected into inflamed joint or bursa)
Potent anti-inflammatory effect Increased pain for 24 hours, rarely causes fat necrosis and skin depigmentation
naproxen (Naprosyn®)
250-500 mg bid
Pain due to inflammation, arthritis, soft tissue injury
NSAID, may cause gastric erosion and bleeding
misoprostol (Cytotec®)
200 µg qid
Prophylaxis of heterotopic ossification after THA
Use with indomethacin
indomethacin (Indocid®)
25 mg PO tid
Prophylaxis of heterotopic ossification after THA
Use with misoprostol
ibuprofen (Advil®, Motrin®)
200-400 mg tid
Pain (including post-op), inflammation (including arthritis)
NSAID, may cause gastric erosion and bleeding
propofol (Diprivan®)
1-2 mg/kg IV Maint 0.5 mg/kg
Conscious sedation for short procedures
Short acting anesthetic often used in conjunction with fentanyl (Sublimaze®)
Orthopaedics OR45
OR46 Orthopaedics
References
Toronto Notes 2011
References Textbooks Adams JC, Hamblen DL. Outline of fractures: including joint injuries. 11th ed. Toronto (ON): Churchill Livingstone, 1999. Blackbourne LH. Surgical recall. 3rd ed. LH Blackbourne. Philadelphia (PA): Lippincott Williams & Wilkins, 2002. Brinker MR. Review of orthopaedic trauma. Toronto (ON): W.B. Saunders Company, 2001. Brinker M, Miller M. Fundamentals of orthopaedics. Philadelphia (PA): W.B. Saunders, 1999. Dee R, Hurst LC, Gruber MA, Kottmeier SA, editors. Principles of orthopaedic practice. 2nd Toronto (ON): McGraw-Hill, 1997. Kao LD. Pre-test surgery. Toronto (ON): McGraw-Hill, 2002 Ochiai DH. The orthopaedic intern pocket survival guide. McLean (VA): International Medical Publishing, 2007. Rockwood CA Jr, Greene DP, Bucholz RU, Heckman JD, editors. Rockwood and Green’s fractures in adults. 4th ed. Philadelphia (PA): Lippincott Raven, 1996. Skinner HB. Current diagnosis and treatment in orthopaedics. 4th ed. New York (NY): McGraw-Hill, 2006. 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Rehabilitation of multiple ligament injured knee. Clin Sports Med. 2000;19(3): 545-571. Lawrence LL. The limping child. Emerg Med Clin North Am. 1998;169(4):911-929. Lo, I.K. NB, Woolfrey, M., Litchfield, R., & Kirkey, A. An evaluation of the apprehension, relocation, and surprise tests for anterior shoulder instability. American Journal of Sports Medicine, 2004;32, 301-307. Luime JJ, Verhagen AP, Miedema HS, Kuiper JI, Burdorf A, Verhaar JAN, Koes BW. An Evaluation of the Apprehension, Relocation, and Surprise Tests for Anterior Shoulder Instability. The American Journal of Sports Medicine. 2004;32:301-307. Mazzone MF. Common conditions of the Achilles tendon. Am Fam Physician. 2000;65(9):1805-1810. Steele PM, Bush-Joseph C, Bach Jr B. Management of acute fractures around the knee, ankle, and foot. Clin Fam Pract. 2000;2(3):661-705. Miller SL. Malignant and benign bone tumours. Radiol Clin North Am. 2000;39(4): 673-699. Oudjhane K. Imaging of osteomyelitis in children. Radiol Clin North Am. 2001;39(2): 251-266. Patel DR. Sports injuries in adolescents. Med Clin North Am. 2000;84(4):983-1007. Roberts DM, Stallard TC. Emergency department evaluation and treatment of knee and leg injuries. Emerg Med Clin North Am. 2000;18(1):67-84. Russell GV Jr. Complicated femoral shaft fractures. Orthop Clin North Am. 2002;33(1): 127-142. Solomon DH, Simel DL, Bates DW, Katz JN, Schaffer JL. The rational clinical examination. Does this patient have a torn meniscus or ligament of the knee? Value of the physical examination. JAMA. 2001;286(13):1610-20. St Pierre P. Posterior cruciate ligament injuries. Clin Sports Med. 1999;18(1): 199-221. Steele PM, Bush-Joseph C, Bach Jr B. Management of acute fractures around the knee, ankle, and foot. Clin Fam Pract. 2000;2(3):661-705. Stewart DG, Kay RM, Skaggs DL. Open fractures in children. Principles of evalution nd management. JBJS Am. 2005;87: 2784-2798. Swenson TM. The dislocated knee: physical diagnosis of the multiple-ligament-injured knee. Clin Sports Med. 2000;19(3):415-423. Zollinger PE, Tuinebreijer WE, Kreis RW, Breederveld RS. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomized trial. Lancet. 1999;354(9195): 2025-2028.
OT
Otolaryngology – Head and Neck Surgery Jayant Ramakrishna and Rebecca Zener, chapter editors Alaina Garbens and Modupe Oyewumi, associate editors Adam Gladwish, EBM editor Dr. Jonathan Irish and Dr. Blake Papsin, staff editors Basic Anatomy Review . . . . . . . . . . . . . . . . . . . . 2 Ear Nose Throat Head and Neck Anatomical Triangles of the Neck Differential Diagnoses of Common Presenting Problems . . . . . . . . . . . . . . . . . . . . . . 6 Dizziness Otalgia Hearing Loss Tinnitus Nasal Obstruction Hoarseness Neck Mass Hearing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 Normal Hearing Physiology Types of Hearing Loss Pure Tone Audiometry Speech Audiometry Impedance Audiometry Auditory Brainstem Response Otoacoustic Emissions Aural Rehabilitation Vertigo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 Evaluation of the Dizzy Patient Benign Paroxysmal Positional Vertigo Menière’s Disease (Endolymphatic Hydrops) Vestibular Neuronitis Labyrinthitis Vestibular Schwannoma (Acoustic Neuroma) Tinnitus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Diseases of the External Ear . . . . . . . . . . . . . . 16 Cerumen Impaction Exostoses Otitis Externa (OE) Malignant (Necrotizing) Otitis Externa (Skull Base Osteomyelitis) Diseases of the Middle Ear . . . . . . . . . . . . . . . 17 Acute Otitis Media and Otitis Media with Effusion Cholesteatoma Mastoiditis Otosclerosis Diseases of the Inner Ear . . . . . . . . . . . . . . . . . 19 Congenital Sensorineural Hearing Loss Presbycusis Sudden Sensorineural Hearing Loss Autoimmune Inner Ear Disease Drug Ototoxicity Noise-Induced Sensorineural Hearing Loss Inner Ear Diseases that cause Vertigo Temporal Bone Fractures
Facial Nerve Paralysis . . . . . . . . . . . . . . . . . . . . 22 Rhinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Allergic Rhinitis (Hay Fever) Vasomotor Rhinitis Sinusitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25 Acute Suppurative Sinusitis Chronic Sinusitis Epistaxis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Hoarseness . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 Acute Laryngitis Chronic Laryngitis Vocal Cord Polyps Vocal Cord Nodules Benign Laryngeal Papillomas Laryngeal Carcinoma Salivary Glands . . . . . . . . . . . . . . . . . . . . . . . . . 30 Sialadenitis Sialolithiasis Salivary Gland Neoplasms Parotid Gland Neoplasms Neck Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Approach to a Neck Mass Evaluation Congenital Neck Masses in Detail . . . . . . . . . . 33 Branchial Cleft Cysts/Fistulae Thyroglossal Duct Cysts Cystic Hygroma (Lymphangioma) Neoplasms of the Head and Neck . . . . . . . . . . 34 Thyroid Carcinoma Pediatric Otolaryngology . . . . . . . . . . . . . . . . . 38 Acute Otitis Media (AOM) Otitis Media with Effusion (OME) Adenoid Hypertrophy Sleep-Disordered Breathing in Children Acute Tonsillitis Peritonsillar Abscess (Quinsy) Tonsillectomy Airway Problems in Children Signs of Airway Obstruction Acute Laryngotracheobronchitis (Croup) Acute Epiglottitis Subglottic Stenosis Laryngomalacia Foreign Body Deep Neck Space Infection Common Medications . . . . . . . . . . . . . . . . . . . 47 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48
Toronto Notes 2011
Otolaryngology OT1
OT2 Otolaryngology
Basic Anatomy Review
Toronto Notes 2011
Basic Anatomy Review Ear External
Middle
Temporalis fascia and muscle Triangular fossa Antihelix Scapha
Auditory ossicles
Malleus
Helix
Inner
Incus
Semicircular canals
Stapes
Vestibular nerve
Helical crus
Cochlear nerve
Vestibulocochlear nerve (CN VIII)
Tragus Facial nerve (CN VII) Cochlea
Antiragus Lobule External acoustic Tympanic meatus membrane
© Aarti Inamdar
Eustachian tube © Susan Park 2009
Figure 1. Surface Anatomy of the External Ear; Anatomy of Ear Tympanic membrane viewed through speculum
View into tympanic cavity after removal of tympanic membrane Pars flaccida Neck of malleus Lateral process of malleus Incus long process Stapes Tendon of stapedius muscle Long process of Malleus Umbo (Flat portion) Fossa of round (cochlear) window Cone of light
Pars tensa
Tensor tympani tendon Tensor tympani muscle
Tympanic Plexus (branch of CN IX) Hypotympanum Annulus © Diana Dai 2006
Figure 2. Normal Appearance of Right Tympanic Membrane on Otoscopy
Nose Adenoid Sphenoid sinus Superior turbinate Drainage into Nasal Cavity Superior meatus: sphenoid (via sphenodethmoidal recess), posterior ethmoid sinuses Middle meatus: frontal, maxillary, anterior ethmoid sinuses Inferior meatus: nasolacrimal duct
Middle turbinate Middle meatus
Speculum View of Right Nostril
Inferior meatus Palatine process of maxilla Soft palate Opening for Eustachian tube
Figure 3. Nasal Anatomy
© Jason Raine 2003
Inferior turbinate
Toronto Notes 2011
Basic Anatomy Review
Otolaryngology OT3
Anterior ethmoid a. Frontal sinus
Posterior ethmoid a.
Kiesselbach’s plexus Septal branch of sphenopalatine a.
Branch from superior labial a.
Greater palatine a.
© Victoria Roswell 2002
Sphenoid sinus
Figure 4. Nasal Septum and its Arterial Supply (see Epistaxis section for detailed blood supply) Nasopharynx: skull base to soft palate Oropharynx: soft palate to hyoid bone Laryngopharynx: hyoid bone to inferior cricoid cartilage
Frontal sinus Orbit Ethmoid sinus Lamina papyracea Osteomeatal complex Maxillary sinus Nasal cavity Teeth
Figure 5. Anatomy of the Four Paranasal Sinuses: Maxillary, Ethmoid, Sphenoid, and Frontal
Reprinted from Dhillon R.S, and East CA. Ear, Nose and Throat and Head and Neck Surgery, 2nd ed. Copyright 1999, with permission from Elsevier.
Throat
Coronal Section
Superior View Valeculla Epiglottis
Anterior
Vestibule
Vestibular folds (false cords) Vocal folds (true cords) Trachea
Posterior View
Pyriform fossa Arytenoid cartilage
Figure 6. Anatomy of a Normal Larynx; Superior View of Larynx on Indirect Laryngoscopy
Posterior
© Glen Oomen 2002
Thyroid cartilage
OT4 Otolaryngology
Basic Anatomy Review
Toronto Notes 2011
Head and Neck Temporal branch Zygomatic branch
Superficial temporal a.
Buccal branch Styloid process Mastoid process Stylomastoid foramen
Maxillary a.
Angular a.
Occipital a.
Lateral nasal a.
Facial n. (VII) Posterior belly of digastric m. Parotid gland Mandibular branch Marginal mandibular branch Cervical branch
Posterior auricular a. Ascending pharyngeal a.
Figure 7. Extratemporal Segment of Facial Nerve Branches of Facial Nerve (in order from superior to inferior) Ten Zebras Broke My Car
Internal carotid a.
Inferior labial a.
External carotid a. Facial a. Lingual a.
Common carotid a.
Superior thyroid a.
© Sean Wang 2007
© M. Romanova 2010
Superior labial a.
Figure 8. Blood Supply to the Face
Branches of the External Carotid Artery (in order from inferior to superior) Some Angry Lady Figured Out PMS Hyoid bone
Post. belly digastric muscle Ant. belly digastric muscle Hyoid bone Sternohyoid muscle Omohyoid muscle
Common carotid artery bifurcation Sternocleidomastoid muscle Posterior Triangle Trapezius muscle
External carotid artery Carotid bifurcation Common carotid artery Internal jugular vein Anterior belly of omohyoid muscle Sternohyoid muscle Median cricothyroid ligament Cricoid cartilage
Thyrohyoid membrane Thyroid cartilage Sternocleidomastoid muscle
Anterior Triangle Posterior belly of omohyoid muscle Trachea
Thyroid gland Clavicle
Figure 9. Anatomy of the Neck
Anatomical Triangles of the Neck Paired Parasympathetic Ganglia of the Head and Neck Ciliary – pupillary constriction Pterygopalatine – lacrimal gland, nasal mucosa Submandibular – submandibular, sublingual glands Otic – parotid gland
Function of Facial Nerve “Ears, Tears, Face, Taste” Ears – stapedius muscle Tears – lacrimation (lacrimal gland) and salivation (parotid) Face – muscles of facial expression Taste – sensory anterior 2/3 of tongue (via chorda tympani)
• anterior triangle: bounded by anterior border of sternocleidomastoid (SCM), midline of neck, and lower border of mandible divided into: submental triangle: bounded by both anterior bellies of digastrics and hyoid bone digastric triangle: bounded by anterior and posterior bellies of digastric, and inferior border of mandible carotid triangle: bounded by sternocleidomastoid, anterior belly of omohyoid, and posterior belly of digastric – contains: tail of parotid, submandibular gland, hypoglossal nerve, carotid bifurcation, and lymph nodes
Toronto Notes 2011
Basic Anatomy Review
Otolaryngology OT5
• posterior triangle: bounded by posterior border of sternocleidomastoid, anterior border of trapezius, and middle third of clavicle divided into: occipital triangle: superior to posterior belly of the omohyoid subclavian triangle: inferior to posterior belly of omohyoid contains: spinal accessory nerve and lymph nodes Table 1. Lymphatic Drainage of Nodal Groups and Anatomical Triangles of Neck Nodal Group/Level
Location
Drainage
1. Suboccipital (S)
Base of skull, posterior
Posterior scalp
2. Retroauricular (R)
Superficial to mastoid process
Scalp, temporal region, ext. auditory meatus, post. pinna
3. Parotid-preauricular (P)
In front of ear
External auditory meatus, anterior pinna, soft tissues of frontal and temporal regions, root of nose, eyelids, palpebral conjunctiva
4. Submental (Level IA)
(Midline) Anterior bellies of digastric muscles, tip of mandible, and hyoid bone
Floor of mouth, anterior oral tongue, anterior mandibular alveolar ridge, lower lip
5. Submandibular (Level IB)
Anterior belly of digastric muscle, stylohyoid muscle, body of mandible
Oral cavity, anterior nasal cavity, soft tissues of the mid-face, submandibular gland
6. Upper jugular (Levels IIA and IIB)
Skull base to inferior border of hyoid bone along SCM muscle
Oral cavity, nasal cavity, naso/oro/hypopharynx, larynx, parotid glands
7. Middle jugular (Level III)
Inferior border of hyoid bone to inferior border of cricoid cartilage along SCM muscle
Oral cavity, naso/oro/hypopharynx, larynx
8. Lower jugular* (Level IV)
Inferior border of cricoid cartilage to clavicle along SCM muscle
Hypopharynx, thyroid, cervical esophagus, larynx
9. Posterior triangle** (Levels VA and VB)
Posterior border of SCM, anterior border of trapezius, from skull base to clavicle
Nasopharynx and oropharynx, cutaneous structures of the posterior scalp and neck
10. Anterior compartment*** (Level VI)
(Midline) Hyoid bone to suprasternal notch Thyroid gland, glottic and subglottic larynx, apex of between the common carotid arteries piriform sinus, cervical esophagus
*Virchow node: left lower level IV supraclavicular node **Includes some supraclavicular nodes ***Includes pretracheal, precricoid, paratracheal, and perithyroidal nodes
CCA
TC
IJV
CC
ITA
SPG TG
RRLN
IPG VN (CN X) LRLN
Figure 10. Anatomy of Thyroid Gland
STA CCA IJV ITA RRLN TC CC SPG TG IPG VN (CN X) LRLN
– – – – – – – – – – – –
Superior thyroid artery Common carotid artery Internal jugular vein Inferior thyroid artery Right recurrent laryngeal nerve Thyroid cartilage Cricoid cartilage Superior parathyroid gland Thyroid gland Inferior parathyroid gland Vagus nerve (CN X) Left recurrent laryngeal nerve
© Marisa Bonofiglio 2003
STA
• Left-sided enlargement of a supraclavicular node (Virchow’s node) may indicate an abdominal malignancy. • Right-sided enlargement may indicate malignancy of the mediastinum, lungs, or esophagus. • Occipital and/or posterior auricular node enlargement may indicate rubella.
OT6 Otolaryngology
Differential Diagnoses of Common Presenting Problems
Toronto Notes 2011
Differential Diagnoses of Common Presenting Problems Dizziness Dizziness
* Common causes
True Vertigo True nystagmus and vertigo caused by a peripheral lesion will never last longer than a couple of weeks because of compensation. Central lesions do not compensate, hence nystagmus and vertigo will pass.
5 D’s of Vertebrobasilar Insufficiency Drop attacks Diplopia Dysarthria Dizziness Dysphagia
Non-Vertiginous
Peripheral (Vestibular)
Central
Organic Diseases
Functional
*Benign paroxysmal positional vertigo (BPPV) *Labyrinthitis *Vestibular neuronitis *Menière’s disease Recurrent vestibulopathy Temporal bone fracture Superior semicircular canal dehiscence Ototoxic drug exposure Autoimmune inner ear disease Perilymph fistula Cholesteatoma
*Cerebrovascular disorders Vertebrobasiliar insufficiency Transient ischemic attacks Wallenberg’s syndrome Cerebellar infarction *Migrainous vertigo *Multiple sclerosis Tumorous CPA tumours Posterior fossa tumours Glomus tumours Inflammation Meningitis Cerebellar abscess Trauma: cerebellar contusion Toxic: alcohol, hypnotics, drugs
Cardiac Arrhythmias Aortic stenosis Vasovagal Orthostatic hypotension Anemia Peripheral neuropathy Visual impairment
Depression Anxiety Panic disorder (hyperventilation) Personality disorder Phobic dizziness
Figure 11. Differential Diagnosis of Dizziness
Otalgia 1. Local Causes Table 2. Differential Diagnosis of Otalgia – Local Causes Etiology
External Ear Pain
Middle and Inner Ear Pain*
Infection
a. Otitis externa b. Herpes simplex/zoster c. Auricular cellulitis d. External canal abscess
a. Acute otitis media b. Otitis media with effusion c. Mastoiditis, myringitis, skull base infections (malignant otitis in diabetics)
Trauma
Frostbite, burns, hematoma, lacerations
Traumatic perforation, barotrauma
Other
Neoplasm of external canal, foreign body, cerumen impaction
Neoplasm, Wegener’s, cholesteatoma
* primarily mediated by TM stretching
2. Referred Pain (from CN V, IX and X) – Ten T’s + 2 • Eustachian Tube • TMJ Syndrome (pain in front of the ears) • Trismus (spasm of masticator muscles; early symptom of tetanus) • Teeth • Tongue • Tonsil (tonsillitis, tonsillar cancer, post-tonsillectomy) • Tic (glossopharyngeal neuralgia) • Throat (cancer of larynx) • Trachea (foreign body; tracheitis) • Thyroiditis • Ramsay Hunt syndrome (Geniculate Herpes) • ± CN VII palsy (e.g. Bell’s palsy)
Toronto Notes 2011
Differential Diagnoses of Common Presenting Problems
Otolaryngology OT7
Hearing Loss Hearing Loss
* Common causes
Conductive
Sensorineural
External Ear
Middle Ear
Congenital
Acquired
*Impacted cerumen *Otitis externa *Foreign body Keratosis obturans Exostoses, osteomas Tumour of canal Congenital stenosis/microtia
*Acute otitis media *Otitis media with effusion *TM perforation *Otosclerosis Tympanosclerosis Eustachian tube dysfunction Cholesteatoma Ossicular malformations Ossicular discontinuity Hemotympanum Middle ear tumour
Genetic *Non-syndrome associated Syndrome associated Intrauterine infections (i.e. TORCH) Teratogens Perinatal hypoxia Prematurity/low birth weight Hyperbilirubinemia
*Presbycusis *Noise-induced Menière’s disease Labyrinthitis Sudden SNHL Autoimmune inner ear disease Ototoxic drug exposure Temporal bone trauma Infectious Postmeningitis Syphilis Viral: mumps, CMV, HSV Neoplastic *Acoustic neuroma CPA tumours Vascular occlusion/emboli Auditory neuropathy
The incidence of Menière’s disease has decreased since the introduction of H. influenzae and S. pneumoniae vaccines.
Figure 12. Differential Diagnosis of Hearing Loss
Tinnitus Tinnitus
* Common causes
Subjective Only heard by patient (common)
Objective Can be heard by others (rare)
Otologic *Presbycusis Noise-induced hearing loss Otitis media with effusion Menière’s disease Otosclerosis Cerumen Foreign body against TM Drugs *ASA NSAIDs Aminoglycosides Antihypertensives Heavy metals Metabolic Hyper/hypothyroidism Hyperlipidemia Vitamin A, B, Zinc deficiency Neurologic Head trauma Multiple sclerosis Acoustic neuroma CPA tumours Psychiatric Anxiety Depression
Vascular *Benign intracranial hypertension Arteriovenous malformation Glomus tympanicum Glomus jugulare Arterial bruits: High-riding carotid artery Vascular loop Persistent stapedial artery Carotid stenosis Venous hum: High jugular bulb Hypertension Hyper/hypothyroidism Mechanical Patulous eustachian tube Palatal myoclonus Stapedius muscle spasm
Figure 13. Differential Diagnosis of Tinnitus
Tinnitus is most commonly associated with SNHL.
Glomus Tympanicum/Jugulare Tumour Signs and Symptoms • Pulsatile tinnitus • Hearing loss • Blue mass behind TM • Brown’s sign (blanching of the TM with pneumatic otoscopy)
OT8 Otolaryngology
Differential Diagnoses of Common Presenting Problems
Toronto Notes 2011
Nasal Obstruction Table 3. Differential Diagnosis of Nasal Obstruction Acquired Nasal Cavity • Rhinitis • Acute/chronic • Vasomotor • Allergic • Polyps • Foreign bodies • Enlarged turbinates • Tumour • Benign – inverting papilloma • Malignant • Squamous cell carcinoma (SCC) • Esthesioneuroblastoma (olfactory neuroblastoma) • Adenocarcinoma
Congenital Nasal Cavity • Nasal dermoid cyst • Encephalocele • Glioma • Choanal atresia Nasal Septum • Septal deviation • Septal hematoma/abscess • Dislocated septum
Nasal Septum • Septal deviation • Septal hematoma/abscess • Dislocated septum Nasopharynx • Adenoid hypertrophy • Tumour • Benign – juvenile nasopharyngeal angiofibroma (JNA) • Malignant – nasopharyngeal carcinoma Functional • Tunnel nose syndrome: absence of feeling in nose prevents the sensation of aeration through nostrils
Hoarseness Table 4. Differential Diagnosis of Hoarseness
Lung malignancy is the most common cause of vocal cord paralysis.
Infectious
• Acute/chronic laryngitis • Laryngotracheobronchitis (croup)
Inflammatory
• Gastro-esophageal reflux (GERD) • Vocal cord polyps/nodules • Lifestyle: smoking, chronic ETOH use
Trauma
• External laryngeal trauma • Endoscopy and endotracheal tube (e.g. intubation granuloma)
Neoplasia
• Benign tumour • Papillomas (HPV infection)
Cysts
• Retention cysts
Systemic
• Endocrine • Hypothyroidism • Virilization
Neurologic (vocal cord paralysis due to superior ± recurrent laryngeal nerve injury)
• Central lesions • Cerebrovascular accident (CVA) • Head injury • Multiple sclerosis (MS) • Skull base tumours • Arnold-Chiari Malformation • Peripheral lesions • Unilateral • Lung malignancy • Iatrogenic injury – thyroid, parathyroid surgery, carotid endarterectomy, patent ductus arteriosus (PDA) ligation • Bilateral • Iatrogenic injury: bilateral thyroid surgery, forceps delivery • Neuromuscular • Myasthenia gravis
Functional
• Psychogenic aphonia (hysterical aphonia)
Congenital
• Laryngomalacia • Laryngeal Web • Laryngeal Atresia
• Malignant tumour • Squamous cell carcinoma (SCC) • Connective tissue disease • Rheumatoid arthritis (RA) • SLE
Toronto Notes 2011
Differential Diagnoses of Common Presenting Problems/Hearing
Otolaryngology OT9
Neck Mass Neck Mass
Inflammatory/Infections
Congenital
Midline Reactive lymphadenopathy TB or atypical mycobacteria Infectious mononucleosis Abscesses Cat scratch fever Sarcoidosis Kawasaki’s HIV
Thyroglossal duct cyst Thyroid tumour/goitre Pyramidal lobe of thyroid gland Ranula
Neoplastic
Lateral
Malignant
Branchial cleft cyst Cystic hygroma
Lymphoma Thyroid Sarcoma Salivary gland neoplasm Rhabdomyosarcoma Neuroblastoma
Benign Salivary gland neoplasm Lipoma Fibroma Vascular
Head and neck primary Infraclavicular primary Leukemia
Figure 14. Differential Diagnosis of a Neck Mass
Hearing Normal Hearing Physiology • Conductive pathway (External auditory canal to cochlea) – Air conduction of sound energy down the EAC Vibration of the tympanic membrane (area effect) Sequential vibration of the middle ear ossicles: malleus, incus, stapes (lever effect) Transmission of amplified vibrations from the stapes footplate in the middle ear to the oval window of the cochlea in the inner ear Pressure differential on Cochlear fluid creates movement along the basilar membrane within the Cochlea from base to apex • Neural pathway (Nerve to brain) – Basilar membrane vibration stimulates overlying hair cells in the Organ of Corti Stimulation of bipolar neurons in the spiral ganglion of the cochlear division of CN VIII Cochlear nucleus Superior Olivary Nucleus Lateral lemniscus Inferior colliculus Sylvian Fissure of Temporal Lobe
Order of the Neural Pathway (with corresponding waves on ABR) E COLI Eighth cranial nerve (I – II) Cochlear nucleus (III) Superior Olivary nucleus Lateral leminiscus (IV – V) Inferior colliculus
Types of Hearing Loss 1. Conductive Hearing Loss (CHL) • the conduction of sound to the cochlea is impaired • can be caused by external and middle ear disease 2. Sensorineural Hearing Loss (SNHL) • due to a defect in the conversion of sound into neural signals or in the transmission of those signals to the cortex • can be caused by disease of the cochlea, acoustic nerve (CN VIII), brainstem, or cortex 3. Mixed Hearing Loss • the conduction of sound to the cochlea is impaired, as well as transmission through the cochlea to the cortex Auditory Acuity • whispered-voice test: mask one ear and whisper into the other • tuning fork tests (see Table 5) (audiogram is of greater utility) • sensitivity depends on which tuning fork used (256 Hz, 512 Hz, 1024 Hz) Rinne test: 512 Hz tuning fork is struck and held firmly on mastoid process to test bone conduction (BC). The tuning fork is then placed beside the pinna to test air conduction (AC) If AC >BC g positive Rinne, which is normal Weber test: 512 Hz tuning fork is held on vertex of head and patient states whether it is heard centrally (Weber negative) or is lateralized to one side (Weber right, Weber left) can place vibrating fork on patient’s chin while they clench their teeth, or directly on teeth to elicit more reliable response will only lateralize if difference in hearing loss between ears is >6 dB
Weber Test Lateralization = Ipsilateral conductive hearing loss or contralateral sensorineural hearing loss. When conductive hearing loss is present, the Weber test is more sensitive in detecting the CHL than the Rinne test.
HL = Intensity x Duration
OT10 Otolaryngology
Hearing
Toronto Notes 2011
Table 5. The Interpretation of Tuning Fork Tests Frequency of Tuning Fork (Hz) 256 512 1024
Minimum hearing loss to have NEGATIVE Rinne (BC > AC) (dB) 15 30 45
Examples
Weber
Rinne
Normal or bilateral sensorineural hearing loss
Central
AC>BC (+) bilaterally
Right-sided conductive hearing loss, normal left ear
Lateralizes to Right
BC>AC (–) right
Right-sided sensorineural hearing loss, normal left ear
Lateralizes to Left
AC>BC (+) bilaterally
Right-sided severe sensorineural hearing loss or dead right ear, normal left ear
Lateralizes to Left
BC>AC (–) right*
* a vibrating tuning fork on the mastoid stimulates the cochlea bilaterally, therefore in this case, the left cochlea is stimulated by the Rinne test on the right, i.e. a false negative test. These tests are not valid if the ear canals are obstructed with cerumen (i.e. will create conductive loss)
Pure Tone Audiometry Range of Frequencies Audible to Human Ear: 20 to 20,000 Hz Most sensitive frequencies: 1,000 to 4,000 Hz Range of human speech: 500 to 2,000 Hz
• a threshold is the lowest intensity level at which a patient can hear the tone 50% of the time • thresholds are obtained for each ear for frequencies 250 to 8000 Hz • air conduction thresholds are obtained with headphones and measure outer, middle, inner ear, and auditory nerve function • bone conduction thresholds are obtained with bone conduction oscillators which bypass the outer and middle ear Degree of Hearing Loss • determined on basis of the pure tone average (PTA) at 500, 1000, and 2000 Hz 250
Air conduction thresholds can only be equal to or greater than bone conduction thresholds.
500
1000 2000 4000 8000 -10 0 10 20 30 40 50 60 70 80 90 100 110 120
250
A. Normal Audiogram 250
500
1000 2000 4000 8000 -10 0 10 20 30 40 50 60 70 80 90 100 110 120
C. Conductive Hearing Loss (Otosclerosis)
250
500
500
B. Conductive Hearing Loss (Otitis Media)
1000 2000 4000 8000 -10 0 10 20 30 40 50 60 70 80 90 100 110 120
250
D. Sensorineural Hearing Loss (Noise Induced)
Figure 15. Types of Hearing Loss and Associated Audiograms of a Left Ear
PURE TONE PATTERNS Degree of Hearing Loss Decibel Loss
Degree of Hearing Loss
0 to 20 dB
Normal
21 to 40 dB
Mild
41 to 55 dB
Moderate
56 to 70 dB
Moderate – Severe
71 to 90 dB
Severe
≥91 dB
Profound
1. Conductive Hearing Loss (CHL) (Figure 15B and 15C) • bone conduction (BC) in normal range • air conduction (AC) outside of normal range • gap between AC and BC thresholds >10 dB (an air-bone gap) 2. Sensorineural Hearing Loss (SNHL) (Figure 15D and 15E) • both air and bone conduction thresholds below normal • gap between AC and BC 10 dB (an air-bone gap)
1000 2000 4000 8000 -10 0 10 20 30 40 50 60 70 80 90 100 110 120
HEARING LEVEL (dB)
Audiogram Legend for a Left Ear x = AC Unmasked > = BC Unmasked = AC Masked ] = BC Masked
HEARING LEVEL (dB)
Hearing loss most often occurs at higher frequencies. Noise-induced (occupational) HL is seen at 4000 Hz. HL associated with otosclerosis is seen at 2000 Hz (Carhart’s notch).
500
1000 2000 4000 8000 -10 0 10 20 30 40 50 60 70 80 90 100 110 120
E. Sensorineural Hearing Loss (Presbycusis)
Toronto Notes 2011
Hearing
Otolaryngology OT11
Speech Audiometry Speech Reception Threshold (SRT) • lowest hearing level at which patient is able to repeat 50% of two syllable words which have equal emphasis on each syllable (spondee words) • SRT and best pure tone threshold in the 500 to 2000 Hz range (frequency range of human speech) usually agree within 5 dB. If not, suspect a retrocochlear lesion or functional hearing loss • used to assess the reliability of the pure tone audiometry
Speech Discrimination
Speech Discrimination Test • percentage of words the patient correctly repeats from a list of 50 monosyllabic words • tested at a level 35 to 50 dB > SRT, therefore degree of hearing loss is taken into account • patients with normal hearing or conductive hearing loss score >90% • score depends on extent of SNHL • rollover effect: a decrease in discrimination as sound intensity increases are typical of a retrocochlear lesion (e.g. acoustic neuroma) • investigate further if scores differ more than 20% between ears • used as best predictor of hearing aid response, as if patient has HL and prior word discrimination, hearing aids may not be helpful
Impedance Audiometry Tympanogram • the eustachian tube equalizes the pressure between external and middle ear • tympanograms graph the compliance of the middle ear system against pressure gradient ranging from to –400 to +200 mmH2O • tympanogram peak occurs at the point of maximum compliance where the pressure in the external canal is equivalent to the pressure in the middle ear • normal range: –100 to +50 mmH2O High
Type B
Type C
Compliance
Type A
Low
–
O Air Pressure
+
• Normal pressure peak at 0 • Note: with otosclerosis, peak is still at 0 mmH2O but has a lower amplitude • Note: with ossicular chain discontinuity, peak is still at 0 mmH2O but has a higher amplitude
–
O Air Pressure • No pressure peak • Poor TM mobility indicative of middle ear effusion (OME) or perforated TM
+
–
O Air Pressure
+
• Negative pressure peak • Indicative of eustachian tube dysfunction or early stage otitis media without effusion
Figure 16. Tympanograms
Static Compliance • volume measurement reflecting overall stiffness of the middle ear system • normal range: 0.3 to 1.6 cc • negative middle ear pressure and abnormal compliance indicate middle ear pathology • in a type B curve, ear canal volumes of greater than 2 cc in children and 2.5 cc in adults indicate TM perforation or presence of a patent ventilation tube Acoustic Stapedial Reflexes • stapedius muscle contracts 2º to loud sound • acoustic reflex thresholds = 70 to 100 dB greater than hearing threshold; if hearing threshold >85 dB, reflex likely absent • stimulating either ear causes bilateral and symmetrical reflexes • for reflex to be present, CN VII must be intact and no conductive hearing loss in monitored ear • if reflex is absent without conductive or severe sensorineural loss g suspect CN VIII lesion • acoustic reflex decay test = ability of stapedius muscle to sustain contraction for 10 s at 10 dB • normally, little reflex decay occurs at 500 and 1000 Hz • with cochlear hearing loss, acoustic reflex thresholds = 25 to 60 dB • with retrocochlear hearing loss (acoustic neuroma) g absent acoustic reflexes or marked reflex decay (>50%) within 5 seconds
% of words identified
Speech Discrimination
90 to 100%
Excellent
80 to 90%
Good
60 to 80%
Fair
40 to 60%
Poor
90% of cases causes: head injury, viral infection (URTI), degenerative disease, idiopathic results in slightly different signals being received by the brain from the two balance organs resulting in sensation of movement Diagnosis • history and positive Dix-Hallpike maneuver Dix-Hallpike Positional Testing (see website for video and illustrations) • the patient is rapidly moved from a sitting position to a supine position with the head hanging over the end of the table, turned to one side at 45º holding the position for 20 seconds • onset of vertigo is noted and the eyes are observed for nystagmus • see sidebar for the 5 signs of BPPV Treatment • reassure patient that process resolves spontaneously • particle repositioning maneuvers Epley maneuver (performed by MD) Brandt-Daroff exercises (performed by patient) • surgery for refractory cases • anti-emetics for nausea/vomiting • drugs to suppress the vestibular system delay eventual recovery and are therefore not used
BPPV is the most common cause of episodic vertigo. Patients often are symptomatic when rolling over in bed or moving their head to a position of extreme posterior extension.
5 Signs of BPPV Seen with Dix-Hallpike Maneuver • Geotropic rotatory nystagmus (nystagmus MUST be present for a positive test) • Fatigues with repeated maneuver • Reversal of nystagmus upon sitting up • Latency of ~20 seconds • Crescendo/decrescendo vertigo lasting 20 seconds
Patients can wear Frenzel’s magnifying eyeglasses during the Dix-Hallpike Maneuver, which inhibit visual fixation and allow for better visualization of the eyes.
Supine
Sitting
1
6 5
Turn on side
UT
4
Supine
PSC 3
2 2
6
Supine
1
Supine
5
4 A. Epley Maneuver
Figure 17. Epley and Dix-Hallpike Maneuvers
UT – Utricle PSC – Posterior Semicircular Canal
B. Dix-Hallpike Maneuver
© Ardis Cheng 2006
3
OT14 Otolaryngology
Vertigo
Toronto Notes 2011
Menière’s Disease (Endolymphatic Hydrops) Definition • episodic attacks of tinnitus, hearing loss, aural fullness, and vertigo lasting minutes to hours Features of Meniere’s Disease Vertigo, Tinnitus, Aural Fullness and Hearing Loss
Proposed Etiology • inadequate absorption of endolymph leads to endolymphatic hydrops (over accumulation) that distorts the membranous labyrinth Epidemiology • peak incidence 40 to 60 years • bilateral in 35% of cases
Drop Attacks (Tumarkin’s Otolithic Crisis) are sudden falls occurring without warning and without LOC.
Before proceeding with gentamicin treatment, perform a CT Head to rule out CPA tumour as the cause of symptoms.
Clinical Features • syndrome characterized by vertigo, fluctuating low frequency sensorineural hearing loss (SNHL), tinnitus, and aural fullness • ± drop attacks (Tumarkin crisis), ± nausea and vomiting • vertigo disappears with time (minutes to hours), but hearing loss remains • early in the disease: fluctuating sensorineural hearing loss (SNHL) • later stages: persistent tinnitus and low-frequency hearing loss • attacks come in clusters and can be debilitating to the patient • may be triggered by stress Treatment • acute management may consist of bed rest, antiemetics, antivertiginous drugs [e.g. betahistine (Serc®)], and low molecular weight dextrans (not commonly used) • long term management may include: medical: low salt diet, diuretics (e.g. hydrochlorothiazide, triamterene, amiloride) local application of gentamicin to destroy vestibular end-organ, results in complete SNHL Serc® prophylactically to decrease intensity of attacks surgical: selective vestibular neurectomy or transtympanic labyrinthectomy • must monitor opposite ear as bilaterality occurs in 35% of cases
Vestibular Neuronitis Definition • acute onset of disabling vertigo often accompanied by nausea, vomiting and imbalance without hearing loss that resolves over days leaving a residual imbalance that lasts days to weeks Etiology • thought to be due to a viral infection (e.g. measles, mumps, herpes zoster) • ~30% of cases have associated URTI symptoms • other: microvascular events, diabetes, autoimmune process • considered to be the vestibular equivalent of Bell’s palsy, sudden hearing loss, and acute vocal cord palsy Clinical Features • acute phase: severe vertigo with nausea, vomiting, and imbalance lasting 1 to 5 days irritative nystagmus (fast phase towards the offending ear) patient tends to veer towards affected side • convalescent phase: imbalance and motion sickness lasting days to weeks spontaneous nystagmus away from affected side gradual vestibular adaptation requires weeks to months • incomplete recovery likely with the following risk factors: elderly, visual impairment, poor ambulation • repeated attacks can occur Treatment • acute phase: bed rest, vestibular sedatives (Gravol®), diazepam • convalescent phase: progressive ambulation especially in the elderly vestibular exercises: involve eye and head movements, sitting, standing, and walking
Toronto Notes 2011
Vertigo/Tinnitus
Otolaryngology OT15
Labyrinthitis Definition • acute infection of the inner ear resulting in vertigo and hearing loss Etiology • may be serous (viral), or purulent (bacterial) • occurs as a complication of acute and chronic otitis media, bacterial meningitis, cholesteatoma, and temporal bone fractures • bacterial: S. pneumoniae, H, influenzae, M. catarrhalis, P. aeruginosa, P. mirabilis • viral: rubella, CMV, measles, mumps, varicella zoster Clinical Features • sudden onset of vertigo, nausea, vomiting, tinnitus, and unilateral hearing loss, with no associated fever or pain • meningitis is a serious complication Investigations • CT head • if meningitis is suspected: lumbar puncture, blood cultures Treatment • treat with IV antibiotics, drainage of middle ear ± mastoidectomy
Vestibular Schwannoma (Acoustic Neuroma) Definition • schwannoma of the vestibular portion of CN VIII Pathogenesis • starts in the internal auditory canal and expands into cerebellar pontine angle (CPA), compressing cerebellum and brainstem • when associated with type 2 neurofibromatosis (NF2): bilateral acoustic neuromas, café-au-lait skin lesions and multiple intracranial lesions Clinical Features • usually presents with unilateral SNHL or tinnitus • dizziness and unsteadiness may be present, but true vertigo is rare as tumour growth occurs slowly • facial nerve palsy and trigeminal (V1) sensory deficit (corneal reflex) are late complications Diagnosis • MRI with gadolinium contrast is the gold standard • audiogram – SNHL • poor speech discrimination relative to the hearing loss • stapedial reflex absent or significant reflex decay • acoustic brainstem reflexes (ABR) – increase in latency of the 5th wave • vestibular tests: normal or asymmetric caloric weakness (an early sign) Treatment • expectant management if tumour is very small, or in elderly • definitive management is surgical excision • other options: gamma knife, radiation
Tinnitus Definition • an auditory perception in the absence of an acoustic stimuli, likely related to loss of input to neurons in central auditory pathways and resulting in abnormal firing History • subjective vs. objective (see Figure 13) • continuous vs. pulsatile (vascular in origin) • unilateral vs. bilateral • associated symptoms: hearing loss, vertigo, aural fullness, otalgia, otorrhea
Acoustic neuroma is the most common intracranial tumour causing SNHL and the most common cerebellopontine angle tumour.
In the elderly, unilateral tinnitus or SNHL is acoustic neuroma until proven otherwise.
Hitzelberger sign: hypoesthesia of external auditory canal due to CN VII compression by an acoustic neuroma.
OT16 Otolaryngology
Tinnitus/Diseases of the External Ear
Toronto Notes 2011
Investigations • audiology • if unilateral: ABR, MRI/CT to exclude a retrocochlear lesion CT to diagnose glomus tympanicum (rare) MRI or angiogram to diagnose AVM • if suspect metabolic abnormality: lipid profile, TSH Treatment • if a cause is found, treat the cause (e.g. drainage of middle ear effusion, embolization or excision of AVM) • with no treatable cause: 50% will improve, 25% worsen, 25% remain the same • avoid loud noise, ototoxic meds, caffeine, smoking • tinnitus workshops • identify situations where tinnitus is most bothersome (e.g. quiet times), mask tinnitus with soft music or “white noise” • hearing aid if coexistent hearing loss • tinnitus instrument: combines hearing aid with white noise masker • trial of tocainamide
Diseases of the External Ear Cerumen Impaction Cerumen impaction is the most common cause of conductive hearing loss for those aged 15-50 years.
Syringing Indications: • Totally occlusive cerumen with pain, decreased hearing, or tinnitus Contraindications: • Non-occlusive cerumen • Previous ear surgery • Only hearing ear • TM perforation Complications: • Failure • Otitis externa • TM perforation • Trauma • Pain • Vertigo • Tinnitus • Otitis media Method: • Establish that TM is intact • Gently pull the pinna superiorly and posteriorly • Using warm water, aim the syringe nozzle upwards and posteriorly to irrigate the ear canal
Etiology • ear wax is a mixture of secretions from ceruminous and pilosebaceous glands, squames of epithelium, dust, and debris Risk Factors • hairy or narrow ear canals, in-the-ear hearing aids, cotton swab usage, osteomata Clinical Features • hearing loss (conductive) • ± tinnitus, vertigo, otalgia, aural fullness Treatment • ceruminolytic drops (bicarbonate solution, olive oil, glycerine, Cerumenol®, Cerumenex®) • syringing • manual debridement (by MD)
Exostoses Definition • bony protuberances in the external auditory canal composed of lamellar bone Etiology • possible association with swimming in cold water Clinical Features • usually an incidental finding • if large, they can cause cerumen impaction or otitis externa Treatment • no treatment required unless symptomatic
Otitis Externa (OE) Etiology • bacteria (~90% of OE): Pseudomonas aeruginosa, Pseudomonas vulgaris, E. coli, S. aureus • fungus: Candida albicans, Aspergillus niger Risk Factors • associated with swimming (“swimmer’s ear”) • mechanical cleaning (Q-tips®), skin dermatitides, aggressive scratching • devices that occlude the ear canal: hearing aids, headphones, etc.
Toronto Notes 2011
Diseases of the External Ear/Diseases of the Middle Ear
Clinical Features • acute: pain aggravated by movement of auricle (traction of pinna or pressure over tragus) otorrhea (sticky yellow purulent discharge) conductive hearing loss ± aural fullness 2º to obstruction of external canal by swelling and purulent debris post-auricular lymphadenopathy complicated OE exists if the pinna and/or the periauricular soft tissues are erythematous and swollen • chronic: pruritus of external ear ± excoriation of ear canal atrophic and scaly epidermal lining, ± otorrhea, ± hearing loss wide meatus but no pain with movement of auricle tympanic membrane appears normal
Otolaryngology OT17
Pulling on the pinna is extremely painful in otitis externa, but is usually well tolerated in otitis media.
Treatment • clean ear under magnification with irrigation, suction, dry swabbing, and C&S • bacterial etiology antipseudomonal otic drops (e.g. gentamicin, ciprofloxacin) or a combination of antibiotic and steroid (e.g. Garasone® or Cipro HC®) do not use aminoglycoside if the tympanic membrane (TM) is perforated because of the risk of ototoxicity introduction of fine gauze wick (pope wick) if external canal edematous ± 3% acetic acid solution to acidify ear canal (low pH is bacteriostatic) systemic antibiotics if either cervical lymphadenopathy or cellulitis • fungal etiology repeated debridement and topical antifungals (gentian violet, Mycostatin® powder, boric acid, Locacorten®, Vioform® drops) • ± analgesics • chronic otitis externa (pruritus without obvious infection) g corticosteroid alone e.g. diprosalic acid
Malignant (Necrotizing) Otitis Externa (Skull Base Osteomyelitis) Definition • osteomyelitis of the temporal bone Epidemiology • occurs in elderly diabetics and immunocompromised patients Etiology • rare complication of otitis externa • Pseudomonas infection in 99% of cases Clinical Features • otalgia and purulent otorrhea that is refractory to medical therapy • granulation tissue on the floor of the auditory canal Complications • lower cranial nerve palsies • systemic infection, death Management • imaging: high resolution temporal bone CT scan, gadolinium scan, technetium scan • requires hospital admission, debridement, IV antibiotics, hyperbaric O2 • may require OR for debridement of necrotic tissue/bone
Diseases of the Middle Ear Acute Otitis Media (AOM) and Otitis Media with Effusion (OME) • see Pediatric Otolaryngology, OT38
Gallium and Technetium Scans Gallium scans are used to show sites of active infection. Gallium is taken up by PMNs and therefore only lights up when active infection is present. It will not show the extent of osteomyelitis. Technetium scans provide information about osteoblastic activity and as such are used to demonstrate sites of osteomyelitis. Technetium scans help with diagnosis whereas gallium scans are useful in follow-up.
OT18 Otolaryngology
Diseases of the Middle Ear
Toronto Notes 2011
Cholesteatoma Definition • a cyst composed of keratinizing squamous epithelium occurring in the middle ear, mastoid and temporal bone • two types: congenital and acquired (see below) Congenital • presents as a “small white pearl” behind an intact tympanic membrane (anterior and medial to the malleus) or as a conductive hearing loss • believed to be due to aberrant migration of external canal ectoderm during development • not associated with otitis media/eustachian tube dysfunction Mechanisms of Cholesteatoma Formation 1. Epithelial migration through TM perforation (2° acquired) 2. Invagination of TM (1° acquired) 3. Metaplasia of middle ear epithelium (congenital) 4. Basal cell hyperplasia (congenital)
Acquired (more common) • generally occurs as a consequence of otitis media and chronic eustachian tube dysfunction • frequently associated with retraction pockets in the pars flaccida (1° acquired) and marginal perforations (2° acquired) of the tympanic membrane • the associated chronic inflammatory process causes progressive destruction of surrounding bony structures Clinical Features • symptoms: history of otitis media (especially if unilateral), ventilation tubes, ear surgery progressive hearing loss (predominantly conductive although may get sensorineural hearing loss in late stage) otalgia, aural fullness, fever • signs: retraction pocket in TM, may contain keratin debris TM perforation granulation tissue, polyp visible on otoscopy malodorous, unilateral otorrhea Complications Table 8. Complications of Cholesteatoma Local
Intracranial
Ossicular erosion: conductive hearing loss
Meningitis
Inner ear erosion: SNHL, dizziness and/or labyrinthitis
Sigmoid sinus thrombosis
Temporal bone infection: mastoiditis, petrositis
Intracranial abscess (subdural, epidural, cerebellar)
Facial paralysis
Investigations • audiogram and CT scan Treatment • there is no conservative therapy for cholesteatoma • surgical: mastoidectomy ± tympanoplasty ± ossicular reconstruction
Mastoiditis Definition • complication of AOM • infection (usually subperiosteal) of mastoid air cells, most commonly seen approximately two weeks after onset of untreated or inadequately treated acute suppurative otitis media Mastoiditis is now rare due to rapid and effective treatment of acute otitis media with antibiotics.
Etiology • acute mastoiditis caused by the same organisms as AOM: S. pneumoniae, S. pyogenes, S. aureus, H. influenzae Clinical Features • classic triad otorrhea tenderness to pressure over the mastoid retroauricular swelling with protruding ear • fever, hearing loss, ± TM perforation (late) • CT radiologic findings: opacification of mastoid air cells by fluid and interruption of normal trabeculations of cells
Toronto Notes 2011
Diseases of the Middle Ear/Diseases of the Inner Ear
Otolaryngology OT19
Treatment • IV antibiotics with myringotomy and ventilation tubes – usually all that is required acutely • cortical mastoidectomy: debridement of infected tissue allowing aeration and drainage • indications for surgery: failure of medical treatment after 48 hours symptoms of intracranial complications aural discharge persisting for 4 weeks and resistant to antibiotics
Otosclerosis Definition • fusion of stapes footplate to oval window so that it cannot vibrate Etiology • autosomal dominant, variable penetrance approximately 40% • female > male, progresses during pregnancy (hormone responsive) Clinical Features • progressive conductive hearing loss first noticed in teens and 20’s (may progress to sensorineural hearing loss if cochlea involved) • ± pulsatile tinnitus • tympanic membrane normal ± pink blush (Schwartz’s sign) associated with the neovascularization of otosclerotic bone • characteristic dip at 2,000 Hz (Carhart’s notch) on audiogram (see Figure 15)
Otosclerosis is the 2nd most common cause of conductive hearing loss in 15 to 50 year olds (after cerumen impaction).
Treatment • monitor with serial audiograms if coping with loss • hearing aid (air conduction, bone conduction, BAHA) • stapedectomy or stapedotomy (with laser or drill) with prosthesis is definitive treatment
Diseases of the Inner Ear Congenital Sensorineural Hearing Loss Hereditary Defects • non-syndrome associated (70%): often idiopathic, autosomal recessive connexin 26 (GJB2) most common • syndrome associated (30%): Waardenburg’s – white forelock, heterochromia iridis, wide nasal bridge and increased distance between medial canthi Pendred’s – deafness associated with thyroid gland disorders, SLC26A4 gene, enlarged vestibular aqueducts Treacher-Collins – first and second branchial cleft anomalies Alport’s – hereditary nephritis Prenatal TORCH Infections • toxoplasmosis, rubella, cytomegalovirus (CMV), herpes simplex, others (e.g. HIV, syphilis) Perinatal • Rh incompatibility • anoxia • hyperbilirubinemia • birth trauma (hemorrhage into inner ear) Postnatal • meningitis • mumps • measles High Risk Registry (for Hearing Loss in Newborns) • risk factors: low birth weight/prematurity perinatal anoxia (low APGARs) kernicterus – bilirubin >25 mg/dL craniofacial abnormality family history of deafness in childhood
Congenital SNHL is decreasing in incidence due to the availability of vaccines and improved neonatal care.
OT20 Otolaryngology
Diseases of the Inner Ear
Toronto Notes 2011
1st trimester illness – TORCH infections neonatal sepsis ototoxic drugs perinatal infection, including post-natal meningitis consanguinity • 50-75% of newborns with sensorineural hearing loss have at least one of the above risk factors, and 90% of these have spent time in the NICU • presence of any risk factor: auditory brainstem response (ABR) study performed before leaving NICU and at 3 months adjusted age • early rehabilitation improves speech and school performance
Presbycusis Definition • sensorineural hearing loss associated with aging (staging in 5th and 6th decades) Etiology • hair cell degeneration • age related degeneration of basilar membrane • cochlear neuron damage • ischemia of inner ear
Presbycusis is the most common cause of SNHL.
Clinical Features • progressive, gradual bilateral hearing loss initially at high frequencies, then middle frequencies • loss of discrimination of speech especially with background noise present – patients describe people as mumbling • recruitment phenomenon: inability to tolerate loud sounds • tinnitus Treatment • hearing aid if patient has difficulty functioning, hearing loss >30-35 dB, and good speech discrimination • ± lip reading, auditory training, auditory aids (doorbell and phone lights)
Sudden Sensorineural Hearing Loss
Sudden sensorineural hearing loss may easily be confused with ischemic brain events. It is important to keep a high index of suspicion especially with elderly patients presenting with sudden sensorineural hearing loss as well as vertigo.
Clinical Features • presents as a sudden onset of significant SNHL (usually unilateral) ± tinnitus, aural fullness • usually idiopathic, rule out other causes: autoimmune causes – ESR, rheumatoid factor, ANA MRI to rule out tumour and/or CT to rule out ischemic/hemorrhagic stroke if associated with any other focal neurological signs (e.g. vertigo, ataxia, abnormality of CN V or VII, weakness) Treatment • treat with oral corticosteroids within 3 days of onset: prednisone 1-2 mg/kg/day, tapering over 2 weeks Prognosis • depends on degree of hearing loss • 70% resolve spontaneously within 10 to 14 days • 20% experience partial resolution • 10% experience permanent hearing loss
Autoimmune Inner Ear Disease Etiology • idiopathic • may be associated with systemic autoimmune diseases (ie. rheumatoid arthritis, SLE), vasculitides (i.e. Wegener’s, polyarteritis nodosa) and allergies Epidemiology • most common between ages 20-50 Clinical Features • rapidly progressive or fluctuating bilateral SNHL • ± tinnitus, aural fullness, vestibular symptoms (i.e. ataxia, disequilibrium, vertigo)
Toronto Notes 2011
Diseases of the Inner Ear
Otolaryngology OT21
Investigations • autoimmune work-up: CBC, ESR, ANA, rheumatoid factor Treatment • high-dose corticosteroids: treat early for at least 30 days • consider cytotoxic medication for steroid non-responders
Drug Ototoxicity Aminoglycosides • toxic to hair cells by any route: oral, IV, and topical (if the TM is perforated) • destroys sensory hair cells – outer first, inner second (therefore OAEs are lost first) • high frequency hearing loss develops earliest • ototoxicity occurs days to weeks post-treatment • streptomycin and gentamicin (vestibulotoxic), kanamycin and tobramycin (cochleotoxic) • must monitor with peak and trough levels when prescribed, especially if patient has neutropenia and/or history of ear or renal problems • q24h dosing recommended, with amount determined by creatinine clearance, not serum creatinine • aminoglycoside toxicity displays saturable kinetics therefore once daily dosing presents less risk than divided daily doses • duration of treatment is the most important predictor of ototoxicity • treatment: immediately stop aminoglycosides Salicylates • hearing loss with tinnitus, reversible if discontinued Antimalarials (Quinine) • hearing loss with tinnitus • reversible if discontinued but can lead to permanent loss Others • many antineoplastics agent are ototoxic (weigh risks vs. benefits) • loop diuretics
Noise-Induced Sensorineural Hearing Loss Pathogenesis • 85 to 90 dB over months or years causes cochlear damage • early-stage hearing loss at 4000 Hz (because this is the resonance frequency of the temporal bone), extends to higher and lower frequencies with time (see Figure 15D) • speech reception not altered until hearing loss >30 dB at speech frequency, therefore considerable damage may occur before patient complains of hearing loss • difficulty with speech discrimination, especially in situations with competing noise Phases of Hearing Loss • dependent on: intensity of sound and duration of exposure • temporary threshold shift: when exposed to loud sound, decreased sensitivity or increased threshold for sound may have associated aural fullness and tinnitus with removal of noise, hearing returns to normal • permanent threshold shift: hearing does not return to previous state Treatment • hearing aid • prevention: ear protectors: muffs, plugs machinery which produces less noise limit exposure to noise with frequent rest periods regular audiologic follow-up
Inner Ear Diseases that cause Vertigo • see Vertigo, OT12 benign paroxysmal positional vertigo (BPPV) Menière’s disease (endolymphatic hydrops) vestibular neuronitis labyrinthitis acoustic neuroma (AN), (vestibular schwannoma)
Short exposures to louder sounds can cause significant SNHL.
Limits of Noise Causing Damage Continuous sound pressure >85 dB Single sound impulse >135 dB
OT22 Otolaryngology
Diseases of the Inner Ear/Facial Nerve (CN VII) Paralysis
Toronto Notes 2011
Temporal Bone Fractures • rarely are temporal bbone fractures purely transverse or longitudinal, often it is a mixed picture Table 9. Features of Temporal Bone Fractures (see Figure 18) 1
Transverse (1) 2
© Teddy Cameron 2002
Figure 18. Types of Temporal Bone Fractures
Signs of Basilar Skull Fracture Battle’s Sign: ecchymosis of the mastoid process of the temporal bone Racoon Eyes CSF Rhinorrhea/Otorrhea Cranial Nerve involvment (facial palsy CN VII, nystagmus CN VI, facial numbness CN V)
The halo sign is the double ringed appearance of CSF fluid on white filter paper as it separates out from blood.
Hemotympanum can be indicative of temporal bone trauma.
Longitudinal (2)
Extension
Into bony labyrinth and internal auditory meatus
Into middle ear
Incidence
10 to 20%
70 to 90%
Etiology
Frontal/occipital trauma
Lateral skull trauma
CN pathology
CN VII palsy (50%)
CN VII palsy (10 to 20%)
Hearing loss
Sensorineural loss due to direct cochlear injury
Conductive hearing loss secondary to ossicular injury
Vestibular symptoms
Sudden onset vestibular symptoms due to direct semicircular canal injury (vertigo, spontaneous nystagmus)
Rare
Other features
Intact external auditory meatus, tympanic membrane ± hemotympanum Spontaneous nystagmus CSF leak in eustachian tube to nasopharynx ± rhinorrhea (risk of meningitis)
Torn tympanic membrane or hemotympanum Bleeding from external auditory canal Step formation in external auditory canal CSF otorrhea Battle’s sign = mastoid ecchymoses Raccoon eyes = periorbital ecchymoses
* Classic types of fractures are uncommon with modern injury (MVA); often see a combination of pure types
Diagnosis • otoscopy • do not syringe or manipulate external auditory meatus due to risk of inducing meningitis via TM perforation • CT head • audiology, facial nerve tests (for transverse fractures), Schirmer’s test (of lacrimation), stapedial reflexes if CN VII palsy • if suspecting CSF leak: look for halo sign (see sidebar), send fluid for beta-2-transferrin Treatment • ABC’s • medical – expectant, prevent otogenic meningitis • surgical – explore temporal bone, indications: CN VII palsy (immediate and complete) gunshot wound depressed fracture of external auditory meatus early meningitis (mastoidectomy) bleeding intracranially from sinus CSF otorrhea (may resolve spontaneously) Complications • acute otitis media ± labyrinthitis ± mastoiditis • meningitis/epidural abscess/brain abscess • post-traumatic cholesteatoma
House-Brackmann Facial Nerve Grading System Grade I: Normal facial motor function Grade II: Mild dysfunction – Slight weakness – Normal symmetry and tone at rest – Complete eye closure Grade III: Moderate dysfunction – Obvious weakness Grade IV: Moderately severe dysfunction – Obvious weakness ± disfiguring asymmetry – Incomplete eye closure – No forehead motion – Mouth asymmetric motion Grade V: Severe dysfunction – Barely perceptible motion of mouth – Asymmetric at rest Grade VI: Total paralysis – No movement
Facial Nerve (CN VII) Paralysis Etiology • supranuclear and nuclear (MS, poliomyelitis, cerebral tumours) • infranuclear – see table below Treatment • treat according to etiology plus provide corneal protection with artificial tears, nocturnal lid taping, tarsorrhaphy, gold weighting of upper lid • facial paralysis that does not resolve with time or with medical treatment will often be referred for possible reanimation techniques to restore function common reanimation techniques include: direct facial nerve anastomosis interpositional grafts anastomosis to other motor nerves muscle transpositions
Toronto Notes 2011
Facial Nerve (CN VII) Paralysis/Rhinitis
Otolaryngology OT23
Table 10. Differential Diagnosis of Peripheral Facial Paralysis (PFP) Etiology
Incidence
Findings
Investigations
Treatment, Follow-up, and Prognosis (Px)
Bell’s Palsy
80 to 90% of PFP
Idiopathic, (HSV) infection of the facial nerve Diagnosis of exclusion
Risk Factors: Diabetes Pregnancy Viral prodrome (50%)
Hx: Acute onset Numbness of ear Schirmer’s test Recurrence (12%) + FHx (14%) Hyperacusis (30%)
Stapedial reflex absent Audiology normal (or baseline) Electromyogram (EMG) – best measure for prognosis Topognostic testing MRI with gadolinium – enhancement of CN VII and VIII High resolution CT
Rx: Protect the eye to prevent exposure keratitis with patching or tarsorraphy Systemic steroids may lessen degeneration and hasten recovery Consider antiviral (acyclovir)
P/E: Paralysis or paresis of all muscle groups on one side of the face Absence of signs of CNS disease Absence of signs of ear or CPA diseases
Ramsay-Hunt Syndrome (Herpes Zoster Oticus) Varicella zoster infection of CN VII/VIII
4.5 to 9% of PFP Risk Factors: >60 years Impaired immunity Cancer Radiotherapy Chemotherapy (see OT22)
Temporal Bone Fracture 20% have PFP Longitudinal (90%)
Hx: Hyperacusis SNHL Severe pain of pinna, mouth, or face P/E: Vesicles on pinna, ext. canal (errupt 3-7 days after onset of pain) Associated herpes zoster ophthalmicus (uveitis, keratoconjunctivitis, optic neuritis, or glaucoma) Hx: Blow to side of head
Stapedial reflex absent Audiology – SNHL Viral ELISA studies to confirm MRI with gadolinium (86% of facial nerves enhance)
40% have PFP
Iatrogenic
Hx: Blow to frontal or occipital area
Px: 90% recover spontaneously and completely overall; >90% recovery if paralysis was incomplete Poorer if hyperacusis, >60 yrs, diabetes, HTN, severe pain Rx: Pt. should avoid touching lesions to prevent spread of infection Systemic steroids can relieve pain, vertigo, avoid postherpetic neuralgia Acyclovir may lessen pain, aid healing of vesicles F/U: 2 to 4 weeks Px: Poorer prognosis than Bell’s palsy; 22% recover completely, 66% incomplete paralysis, 10% complete paralysis
Skull X-rays CT head
Px: Injury usually due to stretch or impingement; may recover with time
Skull X-rays CT head
Px: Nerve transection more likely
P/E: Trauma to side of head Neuro findings consistent with epidural/subdural bleed Transverse (10%)
F/U: Spontaneous remission should begin within 3 weeks of onset Delayed (3 to 6 months) recovery portends at least some functional loss
P/E: Trauma to front or back of head Variable (depending on level of injury) Wait for lidocaine to wear off EMG
Rx: Exploration if complete nerve paralysis No exploration if any movement present
Source: Paul Warrick. icarus.med.utoronto.ca/carr/manual/afnptable.html
Rhinitis Definition • inflammation of the lining (mucosa) of the nasal cavity Table 11. Classification of Rhinitis Inflammatory
Non-Inflammatory
• Perennial non-allergic • Asthma, ASA sensitivity • Allergic • Seasonal • Perennial • Atrophic • Primary: Klebsiella ozena (especially in elderly) • Acquired: post-surgery if too much mucosa or turbinate has been resected • Infectious • Viral: e.g. rhinovirus, influenza, parainfluenza, etc. • Bacterial: e.g. S. aureus • Fungal • Granulomatous: TB, syphilis, leprosy • Non-infectious • Sarcoidosis • Wegener’s granulomatosis • Irritant • Dust • Chemicals • Pollution
• Rhinitis medicamentosa • Topical decongestants • Hormonal • Pregnancy • Estrogens • Thyroid • Idiopathic vasomotor
Rhinitis medicamentosa is rebound congestion due to the overuse of intranasal vasoconstrictors. For prevention, use of these medications for only 5-7 days is recommended.
OT24 Otolaryngology
Rhinitis
Toronto Notes 2011
Table 12. Nasal Discharge: Character and Associated Conditions Character
Associated Conditions
Watery/mucoid
Allergic, viral, vasomotor, CSF leak (halo sign)
Mucopurulent
Bacterial, foreign body
Serosanguinous
Neoplasia
Bloody
Trauma, neoplasia, bleeding disorder, hypertension/vascular disease
Allergic Rhinitis (Hay Fever) Definition • rhinitis characterized by an IgE-mediated hypersensitivity to foreign allergens • acute-and-seasonal or chronic-and-perennial • perennial allergic rhinitis often confused with recurrent colds
Congestion reduces nasal airflow and allows the nose to repair itself. Treatment should focus on the initial insult rather than target this defense mechanism.
Etiology • when allergens contact the respiratory mucosa, specific IgE antibody is produced in susceptible hosts • concentration of allergen in the ambient air correlates directly with the rhinitis symptoms Epidemiology • age at onset usually 5 days) of nasal drops and sprays (Dristan®, Otrivin®) Clinical Features • chronic intermittent nasal obstruction, varies from side to side • rhinorrhea: thin, watery • nasal allergy must be ruled out • mucosa and turbinates: swollen, pale between exposure • symptoms are often more severe than clinical presentation suggests Treatment • elimination of irritant factors • parasympathetic blocker (Atrovent® nasal spray) • steroids (e.g. beclomethasone, fluticasone) • surgery (often of limited lasting benefit): electrocautery, cryosurgery, laser treatment or removal of inferior or middle turbinates • vidian neurectomy (rarely done) • symptomatic relief with exercise (increased sympathetic tone)
Sinusitis Development of Sinuses • sinus pneumatization begins in 3rd-4th month of fetal life • maxillary sinus first to develop • neonate – clinically significant ethmoid and maxillary buds present • age 9 – maxillary full grown; frontal and sphenoid cells starting • age 18 – frontal and sphenoid cells full grown Pathogenesis of Sinusitis • inflammation of the mucosal lining of the paranasal sinuses • anything that blocks mucus from exiting the sinuses predisposes them to inflammation • all sinuses drain to a common prechamber under the middle meatus called the osteomeatal complex Definition • inflammation of the mucosal lining of the sinuses Classification • acute: 3 months Table 13. Etiologies of Sinusitis Ostial Obstruction
Inflammation
• URTI • Allergy
Mechanical
• Septal deviation • Turbinate hypertrophy • Polyps • Tumours • Adenoid hypertrophy • Foreign body • Congenital abnormalities i.e. cleft palate
Immune
• Wegener’s granulomatosis • Lymphoma, leukemia • Immunosuppressed patients (e.g. neutropenics, diabetics, HIV) • Cystic fibrosis • Immotile cilia (Kartagener’s)
Systemic Direct Extension
Dental
• Infection
Trauma
• Facial fractures
Source: Dr. J. Chapnik. icarus.meds.utoronto.ca/otolaryngology/OTL300/sinusitis.pdf
FESS = Functional Endoscopic Sinus Surgery Opening of the entire osteomeatal complex in order to facilitate drainage while sparing the sinus mucosa.
OT26 Otolaryngology
Sinusitis
Toronto Notes 2011
Acute Suppurative Sinusitis
Acute Sinusitis Complications Consider hospitalization if any of the following are suspected 1. Orbital (Chandler’s classification) a. Periorbital cellulitis b. Orbital cellulitis c. Subperiosteal abscess d. Orbital abscess e. Cavernous sinus thrombosis 2. Intracranial a. Meningitis b. Abscess 3. Bony a. Subperiosteal frontal bone abscess (“Pott’s Puffy Tumour”) b. Osteomyelitis 4. Neurologic a. Superior orbital fissure syndrome CN III/IV/VI palsy, immobile globe, dilated pupils, ptosis, V1 hypoesthesia) b. Orbital apex syndrome (as “4a” above, plus neuritis, papilledema, decreased acuity)
Definition • acute infection and inflammation of the paranasal sinuses • clinical diagnosis requiring at least 2 major symptoms or 1 major and 2 minor symptoms � major symptoms � minor symptoms � facial pain/pressure � headache facial fullness/congestion � halitosis nasal obstruction � fatigue purulent/discoloured nasal discharge � dental pain hyposmia/anosmia � cough fever � ear pressure/fullness Etiology • viral vs. bacterial (viral etiology is more common) • children are more prone to a bacterial etiology, but viral is still more common • maxillary sinus most commonly affected • must rule out fungal causes (mucormycosis) in immunocompromised hosts (especially if painless, bloodless mucosa on examination) • organisms viral: rhinovirus, influenza, parainfluenza bacterial: S. pneumoniae (35%), H. influenzae (35%), M. catarrhalis, anaerobes (dental) Clinical Features • sudden onset of nasal blockage/congestion and/or nasal discharge/posterior nasal drip • ± facial pain or pressure, hyposmia • signs more suggestive of a bacterial etiology are erythematous nasal mucosa, mucopurulent discharge, pus originating from the middle meatus and the presence of nasal polyps or a deviated septum • acute viral rhinosinusitis lasts 10 days, consider bacterial etiology Management • anterior rhinoscopy • x-ray/CT scan not recommended unless complications are suspected (i.e. sub-periorbital abscess or intracranial spread – Pott’s Puffy tumour) • symptoms improving within 5 days: symptomic relief and expectant management • moderate symptoms that worsen or persist beyond 5 days: institute an intranasal corticosteroid spray and continue for 14 days if symptomic relief is noted within 48 hrs • severe symptoms that worsen or persist beyond 5 days and are refractory to intranasal corticosteroid (INCS): clarithromycin or Clavulin® therapy ± INCS ± referral to a specialist • surgery if medical therapy fails: FESS
Chronic Sinusitis Definition • inflammation of the paranasal sinuses lasting >3 months
Allergic fungal rhinosinusitis is a chronic sinusitis affecting mostly young, immunocompetent, atopic individuals. Treatment options include FESS ± intranasal topical steroids, antifungals and immunotherapy.
Chronic Sinusitis Complications 1. Polyps 2. Mucocele
Etiology • can result from any of the following: inadequate treatment of acute sinusitis untreated nasal allergy allergic fungal rhinosinusitis anatomic abnormality e.g. deviated septum (predisposing factor) underlying dental disease ciliary disorder e.g. cystic fibrosis, Kartagener’s chronic inflammatory disorder e.g. Wegener’s • organisms: bacterial: S. pneumoniae, H. influenzae, M. catarrhalis, S. pyogenes, S. aureus, anaerobes fungal: Aspergillus Clinical Features (similar to acute, but less severe) • chronic nasal obstruction • purulent nasal discharge • pain over sinus or headache
Toronto Notes 2011
Sinusitis/Epistaxis
Otolaryngology OT27
• halitosis • yellow-brown post-nasal discharge • chronic cough • maxillary dental pain Treatment • antibiotics for 3 to 6 weeks for infectious etiology augmented penicillin (Clavulin®), macrolide (clarithromycin), fluoroquinolone (levofloxacin), clindamycin, Flagyl® • topical nasal steroid, saline spray • surgery if medical therapy fails or fungal sinusitis Surgical Treatment • functional endoscopic sinus surgery g open osteomeatal complex • balloon sinoplasty
Epistaxis Blood Supply to the Nasal Septum (Figure 4) 1. Superior posterior septum: internal carotid g ophthalmic g anterior/posterior ethmoidal 2. Posterior septum: external carotid g internal maxillary g sphenopalatine artery g nasopalatine 3. Lower anterior septum: external carotid g facial artery g superior labial artery g nasal branch external carotid g internal maxillary g descending palatine g greater palatine • these arteries all anastomose to form Kiesselbach’s plexus, located at Little’s area (anterior portion of the cartilaginous septum) • bleeding from above middle turbinate is internal carotid, and from below is external carotid
90% of nose bleeds occu in Little’s area.
Table 14. Etiology of Epistaxis Type
Causes
Local
Trauma (most common) • Fractures: facial, nasal • Self-induced: digital, foreign body
Tumours • Benign: polyps, inverting papilloma, angiofibroma • Malignant: squamous cell carcinoma, esthesioneuroblastoma
Iatrogenic: nasal, sinus, orbit surgery
Inflammation • Rhinitis: allergic, non-allergic • Infections: bacterial, viral, fungal
Barometric changes Nasal dryness: dry air, ± septal deformities Septal perforation
Idiopathic
Chemical: cocaine, nasal sprays, ammonia, etc. Systemic
Coagulopathies • Meds: anticoagulants, NSAIDs • Hemophilias, von Willebrand’s • Hematological malignancies • Liver failure, uremia Vascular: hypertension, atherosclerosis, OslerWeber-Rendu (HHT) Others: Wegener’s, SLE
Investigations • CBC, PT/PTT (if indicated) • x-ray, CT as needed Treatment • aim is to localize bleeding and achieve hemostasis 1. First-aid • ABC’s • patient leans forward to minimize swallowing blood • constant firm pressure applied for 20 min on soft part of nose (not bony pyramid) 2. Assess Blood Loss (can be potentially fatal hemorrhage) • pulse, blood pressure, and other signs of shock • IV NS, cross match for 2 units packed RBCs if significant • IV NS if hypovolemic, or signs of shock
Special Cases • Adolescent male with unilateral recurrent epistaxis consider juvenile nasopharyngeal angiofibroma (JNA). This is the most common benign tumour of the nasopharynx • Thrombocytopenic patients – use resorbable packs to avoid risk of re-bleeding caused by pulling out the removable pack
OT28 Otolaryngology
Epistaxis/Hoarseness
Toronto Notes 2011
3. Determine Site of Bleeding • anterior/posterior hemorrhage defined by location in relationship to bony septum • insert cotton pledget of 4% topical lidocaine ± topical decongestant (i.e. Otrivin), visualize nasal cavity with speculum and aspirate excess blood and clots • if suspicion of bleeding disorder, coagulation workup 4. Control the Bleeding • first line topical vasoconstrictors (Otrivin®) • if first line fails and bleeding adequately visualized, cauterize with silver nitrate • do not cauterize both sides of the septum at one time due to risk of septal perforation from loss of septal blood supply A. Anterior hemorrhage treatment if fail to achieve hemostasis with cauterization: place anterior pack with half inch Vaseline®-soaked ribbon gauze strips or absorbable packing (i.e. Gelfoam®) layered from nasal floor toward nasal roof extending to posterior choanae for 2 to 3 days can also attempt packing with Merocel® or nasal tampons of different shapes can also apply Floseal® (hemostatic matrix consisting topical human thrombin and cross linked gelatin) if other methods fail B. Posterior hemorrhage treatment if unable to visualize bleeding source, then usually posterior source: place posterior pack using a Foley catheter, gauze pack or Epistat® balloon subsequently, layer anterior packing bilaterally antibiotics for any posterior pack or any pack in >48 hours admit to hospital with packs in for 3 to 5 days watch for complications: hypoxemia (naso-pulmonic reflex), toxic shock syndrome (Rx: remove packs immediately), pharyngeal fibrosis/stenosis, alar/septal necrosis, aspiration C. If anterior/posterior packs fail to control epistaxis arterial ligation of anterior/posterior ethmoid, branches of internal maxillary, external carotid artieriesselective embolization of branches of external carotid artery ± septoplasty 5. Prevention • prevent drying of nasal mucosa with humidifiers, saline spray, or topical ointments • avoidance of irritants • medical management of hypertension and coagulopathies
Hoarseness If hoarseness present for >2 weeks in a smoker, laryngoscopy must be done to rule out malignancy. Acute 2 weeks.
Vocal Cord Paralysis Unilateral: affected cord lies in the parmedian position, inadequate glottic closure during phonation weak, breathy voice. Bilateral: cords rest in midline therefore voice remains good but respiratory function is compromised and may present as stridor. Treatment options: voice therapy, injection laryngoplasty (collagen, fat), cord medialization.
Innervation of Larynx Internal branch of superior laryngeal nerve: sensory to larynx above cords. External branch of superior laryngeal nerve: motor to cricothyroid muscle. Recurrent laryngeal nerve: all motor to larynx except for cricothyroid muscle; sensory to larynx below cords.
Definitions • hoarseness: change in voice quality, ranging from voice harshness to voice weakness reflects abnormalities anywhere along the vocal tract from oral cavity to lungs • dysphonia: a general alteration in voice quality • aphonia: no sound emanates from vocal folds
Acute Laryngitis Etiology • viral: influenza, adenovirus • bacterial: Group A Streptococcus • acute voice strain g submucosal hemorrhage g vocal cord edema g hoarseness • environmental: toxic fume inhalation Clinical Features • URTI symptoms, hoarseness, aphonia, cough attacks, ± dyspnea • true vocal cords erythematous/edematous with vascular injection and normal mobility Treatment • usually self-limited, resolves within ~1 week • voice rest • humidification • hydration • avoid irritants (e.g. smoking) • treat with antibiotics if there is evidence of coexistent bacterial pharyngitis
Toronto Notes 2011
Hoarseness
Otolaryngology OT29
Chronic Laryngitis Definition • longstanding inflammatory changes in laryngeal mucosa Etiology • repeated attacks of acute laryngitis • chronic irritants (dust, smoke, chemical fumes) • chronic voice strain • chronic sinusitis with postnasal drip (PND) • chronic alcohol use • esophageal disorders: GERD, Zenker’s diverticulum, hiatus hernia • systemic: allergy, hypothyroidism, Addison’s Clinical Features • chronic dysphonia – rule out malignancy • cough, globus sensation, frequent throat clearing 2º to GERD • laryngoscopy: cords erythematous, thickened with ulceration/granuloma formation and normal mobility Treatment • remove offending irritants • treat related disorders e.g. antisecretory therapy for GERD • speech therapy with voice rest • ± antibiotics, ± steroids to decrease inflammation • laryngoscopy to rule out malignancy
Vocal Cord Polyps Definition • structural manifestation of vocal cord irritation • acutely, polyp forms 2º to capillary damage in the subepithelial space during extreme voice exertion Etiology • most common benign tumour of vocal cords • voice strain (muscle tension dysphonia) • laryngeal irritants (GERD, allergies, tobacco) Epidemiology • 30 to 50 years of age • M>F Clinical Features • hoarseness, aphonia, cough attacks ± dyspnea • pedicled or sessile polyp on free edge of vocal cord • typically polyp asymmetrical, soft and smooth • more common on the anterior 1/3 of the vocal cord • polyp are intermittent respiratory distress with large polyps Treatment • avoid irritants • endoscopic laryngeal microsurgical removal if persistent or if high risk of malignancy
Vocal Cord Nodules Definition • vocal cord callus • aka “screamer’s or singer’s nodules” Etiology • early nodules occur 2º to submucosal hemorrhage • mature nodules result from hyalinization which occurs with long term voice abuse • chronic voice strain • URTI, smoke, alcohol Epidemiology • frequently in singers, children, bartenders, and school teachers • F>M
Vocal Cords: Polyps vs. Nodules Polyps
Nodule
Unilateral, asymmetric
Bilateral
Acute onset Gradual onset May resolve Often follow a spontaneously chronic course Subepithelial capillary breakage
Acute: submucosal hemorrhage or edema Chronic: hyalinization within submucous lesion
Soft, smooth, fusiform, pedunculated mass
Acute: small, discrete nodules Chronic: hard, white, thickened fibrosed nodules
Surgical Surgical excision if excision if refractory persistent or in presence of risk factors for laryngeal cancer
OT30 Otolaryngology
Hoarseness/Salivary Glands
Toronto Notes 2011
Clinical Features • hoarseness worst at end of day • on laryngoscopy: red, soft nodules often bilateral at the junction of the anterior 1/3 and posterior 2/3 of the vocal cords – point of maximal cord vibration • chronic nodules may become fibrotic, hard, and white Treatment • voice rest • hydration • speech therapy • avoid irritants • surgery rarely indicated for refractory nodules
Benign Laryngeal Papillomas Etiology • human papilloma virus (HPV) types 6, 11 • possible hormonal influence, possibly acquired during delivery Epidemiology • biphasic distribution – 1) birth to puberty (most common laryngeal tumour) and 2) adulthood Clinical Features • hoarseness/”frog voice” and airway obstruction • can seed into tracheobronchial tree • highly resistant to complete removal • some juvenile papillomas resolve spontaneously at puberty • papillomas in adults may undergo malignant transformation • laryngoscopy shows wart-like lesions in supraglottic larynx and trachea Treatment • CO2 laser and microsurgery • adjuvants under investigation: interferon, cidofovir, acyclovir • Gardasil® HPV vaccine may prevent/decrease the incidence but more research is needed
Laryngeal Carcinoma • see Neoplasms of the Head and Neck, OT34
Salivary Glands Sialadenitis Definition • inflammation of salivary glands Etiology • viral most common (mumps) • bacterial causes: S. aureus, S. pneumoniae, H. influenzae • obstructive vs. non-obstructive • obstructive infection involves salivary stasis and bacterial retrograde flow Predisposing Factors • HIV • anorexia/bulimia • Sjogren’s syndrome • Cushing’s, hypothyroidism, DM • hepatic/renal failure • meds that increase stasis: diuretics, TCAs, beta-blockers, anticholinergics, antibiotics • sialolithiasis (can cause chronic sialadenitis)
Toronto Notes 2011
Salivary Glands
Clinical Features • acute onset of pain and edema of parotid or submandibular gland that may lead to marked swelling • ± fever • ± leukocytosis • ± suppurative drainage from punctum of the gland
Otolaryngology OT31
Mumps usually presents with bilateral parotid enlargement, ± sensorineural hearing loss, ± orchitis.
Investigations • U/S imaging to differentiate obstructive vs. non-obstructive sialadenitis Treatment • bacterial: treat with cloxacillin ± abscess drainage • viral: no treatment
Sialolithiasis Definition • ductal stone (mainly hydroxyapatite) leading to chronic sialadenitis • 80% in submandibular gland, F): 10% cysts, lymph nodes and adenomas: 10% oncocytoma: liver > bones
Pre-Malignant Disease • leukoplakia hyperkeratosis risk of malignant transformation 5 to 20% • erythroplakia red superficial patches adjacent to normal mucosa commonly associated with epithelial dysplasia associated with carcinoma in situ or invasive tumour in 40% of cases • dysplasia histopathologic presence of mitoses and prominent nucleoli involvement of entire mucosal thickness = carcinoma in situ associated progression to invasive cancer in 15 to 30% of cases Investigations • initial metastatic screen includes chest x-ray • scans of liver, brain, and bone only if clinically indicated • TNM (tumour, nodes, metastases) classification varies slightly depending on the specific type of head and neck tumour (see online tables) • TNM classification widely used for staging in order to: guide treatment indicate prognosis evaluate results of treatment facilitate accurate exchange of information • CT scan is superior to MRI for the detection of pathologic nodal disease and bone cortex invasion • MRI is superior to discriminate tumour from mucus and to detect bone marrow invasion • ± PET scans Treatment • treatment depends on: histologic grade of tumour stage physical and psychological health of patient facilities available expertise and experience of the medical and surgical oncology team
Toronto Notes 2011
Neoplasms of the Head and Neck
• in general: 1º surgery for malignant oral cavity tumours with radiotherapy reserved for salvage or poor prognostic indicators 1º radiotherapy for nasopharynx, oropharynx, hypopharynx, larynx malignancies with surgery reserved for salvage palliative chemotherapy for metastatic or incurable disease concomitant chemotherapy or alternating chemoradiotherapy may increase survival in resectable/unresectable disease chemotherapy has a role as induction therapy prior to surgery and radiation panendoscopy to detect primary disease when lymph node metastasis is identified anti-EGFR treatment (cetuximab, panitumumab) has a role as concurrent therapy with radiation (for advanced local and regional disease) Prognosis • synchronous tumours occur in 9 to 15% of patients • late development of 2nd primary most common cause of post-treatment failure after 36 months
Otolaryngology OT35 Access to PET scans across Canada is variable and there are evolving indications regarding its use in the field of head and neck cancer. PET improves diagnosis and treatment of patients with treated thyroid cancer who develop increased serum thyrogloblin without evidence of recurrence on conventional CT/MRI. Additionally, there is likely application in the following clinical circumstances: - Detection of an unknown primary - Determining the extent of local disease - Detection of residual disease after treatment Source: Cancer Care Ontario. “PET SCAN PRIMER: a guide to the implementation of positron emission tomography imaging in Ontario”. Members of the Ontario PET Steering Committee. Date: Aug 31, 2008
Table 16. Quick Look-Up Summary of Head and Neck Malignancies – Etiology and Epidemiology Etiology Oral Cavity 95% SCC others: sarcoma, melanoma, minor salivary gland tumour Nose and Paranasal Sinus 75 to 80% SCC then adenoCA and mucoepidermoid 99% in maxillary/ethmoid sinus 10% arise from minor salivary glands
Epidemiology
Risk Factors
50% on anterior 2/3 of tongue Mean age: 50 to 60 M>F Most common site of H&N cancers
Smoking/EtOH Poor oral hygiene Leukoplakia, erythroplakia UV light – lip Oral HPV infection
Mean age: 50 to 70 Rare tumours incidence in last 5 to 10 years
Wood/shoe/textile industry Hardwood dust (nasal/ethmoid sinus) Nickel, chromium (maxillary sinus) Air pollution Chronic sinusitis HPV infection – role unclear
Carcinoma of the Pharynx – Subtypes (Nasopharynx, Oropharynx, Hypopharynx and Larynx) Nasopharynx 90% SCC Incidence 0.8 per 100,000 Epstein-Barr virus (EBV) ~10% lymphoma 100x increased incidence in southern Salted fish Chinese Nickel exposure M:F= 2.4:1 Poor oral hygiene Mean age: 50 to 59 Southern Chinese Oropharynx 95% SCC – poorly differentiated
M:F = 4:1 Mean age: 50 to 70
Smoking/EtOH Oral HPV Infection
Hypopharynx 95% SCC 3 sites: 1. piriform sinus (60%) 2. post-cricoid (30%) 3. post pharyngeal wall (10%)
M>F Mean age: 50 to 70 8 to 10% of all H&N cancer
Smoking/EtOH
Larynx SCC most common 3 sites: 1. supraglottic (30 to 35%) 2. glottic (60 to 65%) 3. subglottic (1%)
45% of all H&N cancer M:F = 10:1 Mean age: 45 to 75
Smoking/EtOH Oral HPV Infection
Salivary Gland (see Salivary Gland) 40% mucoepidermoid 30% adenoid cystic 5% acinic cell 5% malignant mixed 5% lymphoma
Thyroid ( 90% benign – 10% malignant) 60 to 70% papillary 15 to 20% follicular 2 to 5% anaplastic 2 to 5% medullary 1 to 5% Hurthle cell 3% lymphoma 1 to 2% metastatic Parathyroid
Rate of malignancy: Parotid 15 to 25% Submandibular 37 to 43% Minor salivary >80% 3 to 6% of all H&N cancer Mean age: 55 to 65 M=F Children Adults 60 Nodules more common in females Malignancy more common in males
Rare tumour Mean age: 44 to 55 years
Radiation exposure Family history – papillary CA or multiple Endocrine neoplasia – MEN II Older age Male Papillary – Gardner’s, Cowden’s, familial adenomatous polyposis (FAP)
Treatment of locoregionally advanced head and neck cancer with concomitant highdose radiotherapy plus cetuximab improves locoregional control and reduces mortality without increasing the common toxic effects associated with radiotherapy to the head and neck. Bonner JA, et al. Radiotherapy plus Cetuximab for Squamous-Cell Carcinoma of the Head and Neck. NEJM 2006; 354(6):567-578.
Risk Factors for Head and Neck Cancer include: 1. Smoking 2. EtOH (this is synergistic with #1) 3. Radiation 4. Occupational/Environmental exposures 5. Oral HPV infection (independent of smoking and EtOH exposure)
HPV-16 accounts for over 90% of HPVpositive head and neck squamous cell carcinoma.
The smaller the salivary gland the greater the likelihood that a mass in the gland is malignant.
OT36 Otolaryngology
Neoplasms of the Head and Neck
Toronto Notes 2011
Table 17. Quick Look-Up Summary of Head and Neck Malignancies – Diagnosis and Treatment Clinical Features Oral Cavity Asymptomatic neck mass (30%) Non-healing ulcer ± bleeding Dysphagia, sialorrhea, dysphonia Oral fetor, otalgia leukoplakia or erythroplakia (pre-malignant changes or CIS)
Investigations
Treatment
Prognosis
Biopsy CT
1o surgery local resection ±neck dissection ±reconstruction 2o radiation
5 year: - T1/T2: 75% - T3/T4: 30 to 35% Poor prognostic indicators: Depth of invasion, close surgical margins location (tongue worse than floor of mouth) Cervical nodes, extra capsular spread
CT/MRI Biopsy
Surgery and radiation Chemoradiotherapy for unresectable disease
5 year: 30 to 60% Poor prognosis 2o to late presentation
Nasopharyngoscopy Biopsy CT/MRI
1o radiation 2o surgery
5 year survival: - I: 79% - II: 72% - III: 50 to 60% - IV: 36 to 42%
Biopsy CT
1o radiation 2o surgery local resection ±neck dissection ±reconstruction
Base of tongue – control rates T1: >90% T4: 13 to 52% Tonsils – cure rate T1/T2: 90 to 100% T4: 15 to 33% HPV-positive tumours have an approximately 20% improved overall survival rate
Pharyngoscopy Biopsy CXR r/o lung mets CT
1o radiation 2o surgery
T2/T3 cure rate: 60% T4/5 year survival: 25 to 40%
Laryngoscopy CT/MRI
1o radiation 2o surgery 1o surgery for bulky T4
5 year T4 >40% (surgery with radiation) Control rate early lesions >90% (radiation) 10 to 12% of small lesions fail radiotherapy
Fine needle aspirate CT
Surgery: Benign and malignant Lymph node sampling Post-op radiotherapy Chemo if unresectable
Parotid 10-year survival 85, 69, 43, and 14% for stages I to IV Submandibular 2 year: 82%, 5 year: 69% Minor salivary gland 10 year: 83, 52, 25, 23% for stages I to IV
FNA U/S
1o surgery I131 for metastatic deposits post-op TSH suppression
Recurrences occur within 5 years Need long-term f/u: clinical exam, thyroglobulin
Wide surgical excision Post-op monitoring of serum Ca
Recurrence rates: 1-year 27% 5-year 82% 10-year 91% Mean survival: 6 to 7 years
Nose and Paranasal Sinus Early symptoms: Unilateral nasal obstruction Epistaxis, rhinorrhea Late symptoms: 2o to invasion of nose, orbit, nerves, oral cavity, skin, skull base, cribriform plate Nasopharynx Cervical nodes (60 to 90%) Nasal obstruction, epistaxis Unilateral AOM ± hearing loss CN III to VI, IX to XII (25%) Proptosis, voice change, dysphagia Oropharynx Odynophagia, otalgia Ulcerated/enlarged tonsil Fixed tongue/trismus/dysarthria Oral fetor, bloody sputum Cervical lymphadenopathy (60%) Distal mets: lung/bone/liver (7%) Hypopharynx Dysphagia, odynophagia Otalgia, hoarseness Cervical lymphadenopathy Larynx Dysphagia, odynophagia, globus Otalgia, hoarseness, Dyspnea/stridor Cough/hemoptysis Cervical nodes (rare w/ glottic CA) Salivary Gland Painless mass CN VII – parotid mass Cervical lymphadenopathy Rapid growth Invasion of skin Constitutional signs/symptoms Thyroid Thyroid mass, cervical nodes Vocal cord paralysis Hyper/hypothyroidism Dysphagia Parathyroid Increased serum Ca Neck mass Bone disease, renal disease Pancreatitis
Toronto Notes 2011
Neoplasms of the Head and Neck
Otolaryngology OT37
Thyroid Carcinoma Table 18. Cytology results of FNA Samples Category
Characteristics
Non-diagnostic Benign
Macrofollicular or colloid adenomas, chronic autoimmune (Hashimoto’s) thyroiditis
Suspicious or indeterminate
Microfollicular or cellular neoplasm
Malignant
Types of Thyroid Cancer (from most common to least common) Papillary Follicular Medullary Anaplastic Lymphoma
Table 19. Thyroid Carcinoma Papillary 70 to 75% Incidence (% of all thyroid cancers)
Follicular
Medullary
Anaplastic
Lymphoma
10%
3 to 5% (10% familial 90% sporadic)
2 to 5%
10% in 6 mos
Non-surgical Combined radiation Chemotherapy (CHOP**)
** CHOP = cyclophosphamide, adriamycin, vincristine, prednisone
Approach to Thyroid Nodule • Recommendation A: all patients with thyroid nodules require evaluation of serum TSH and ultrasound any nodule >5 mm with suspicious sonographic features (hypoechoic, microcalcifications, increased nodular vascularity, infiltrative margins, height > width on transverse view) should undergo FNA when performing repeat FNA on initially non-diagnostic nodules, US-guided FNA should be employed • Recommendation B: nonpalpable, mostly cystic, or posteriorly located nodules should undergo US-guided FNA as the initial evaluative procedure
A minimum of hemithyroidectomy is required to confirm capsular invasion for diagnosing follicular or Hürthle cell CA.
Indications for post-op radioactive iodine ablation – I131 Adjuvant therapy – decrease mortality RAI therapy – treat persistent cancer
Table 20. Management of the Thyroid Nodule Treatment
Indications
Radioiodine therapy
Hyperthyroid with suspicious solid mass, that is HOT on thyroid scan
Chemotherapy and / or radiotherapy
Anaplastic CA or thyroid lymphoma
Surgical excision
Recurrent cyst that is “suspicious” on FNA or if patient is extremely anxious Malignancy other than anaplastic CA or thyroid lymphoma Solid “suspicious” mass that is “cold” on thyroid scan (excise to r/o capsular invasion) Hyperthyroid with suspicious solid mass, that is HOT on thyroid scan (hyperfunctioning)
*U/S findings: cystic: risk of malignancy 4 cm regardless of patient age (Evidence Grade B and above). Cooper DS, et al. Revised American Thyroid Association Management Guidelines for Patients with Thyroid Nodules and Differentiated Thyroid Cancer. Thyroid 2009; 19(11):1167-214.
OT38 Otolaryngology
Pediatric Otolaryngology
Toronto Notes 2011
Pediatric Otolaryngology Acute Otitis Media (AOM) Definition • acute inflammation of middle ear Epidemiology • 60 to 70% of children have at least 1 episode of AOM before 3 years of age • 18 months to 6 years most common age group • peak incidence January to April • one third of children have had ≥3 or more episodes by age 3 Etiology • S. pneumoniae – 35% of cases (incidence decreasing due to pneumococcus vaccine) • H. influenzae – 25% of cases • M. catarrhalis – 10% of cases • S. aureus and S. pyogenes (all beta-lactamase producing) • anaerobes (newborns) • Gram-negative enterics (infants) • viral Predisposing Factors • eustachian tube dysfunction/obstruction: swelling of tubal mucosa: upper respiratory tract infection (URTI) allergies/allergic rhinitis chronic sinusitis obstruction/infiltration of eustachian tube ostium: tumour – nasopharyngeal carcinoma (adults) adenoid hypertrophy (not due to obstruction but by maintaining a source of infection) barotrauma (sudden changes in air pressure) inadequate tensor palati function – cleft palate (even after repair) abnormal eustachian tube: Down syndrome (horizontal position of eustachian tube), Crouzon’s, and Apert’s syndrome • disruption of action of: cilia of eustachian tube – Kartagener’s syndrome mucus secreting cells capillary network that provides humoral factors, PMNs, phagocytic cells • immunosuppression/deficiency due to chemotherapy, steroids, diabetes mellitus, hypogammaglobulinemia, cystic fibrosis Risk Factors • bottle feeding, pacifier use • second-hand smoke • crowded living conditions (day care/group child care facilities) or sick contacts • male • family history Pathogenesis • obstruction of eustachian tube g air absorbed in middle ear g negative pressure (an irritant to middle ear mucosa) g edema of mucosa with exudate/effusion g infection of exudate from nasopharyngeal secretions Clinical Features • triad of otalgia, fever (especially in younger children), and conductive hearing loss • rarely tinnitus, vertigo, and/or facial nerve paralysis • otorrhea if tympanic membrane perforated • pain over mastoid process • infants/toddlers ear-tugging hearing loss, balance disturbances (mild) irritable, poor sleeping vomiting and diarrhea anorexia
Toronto Notes 2011
Pediatric Otolaryngology
• otoscopy of tympanic membrane (TM) hyperemia bulging, pus may be seen behind TM loss of landmarks: handle and long process of malleus not visible Treatment • antibiotic treatment hastens resolution – 10 day course 1st line: amoxicillin 80-90 mg/kg/day divided into two doses – safe, effective, and inexpensive if penicillin allergic: macrolide (clarithromycin, azithromycin), trimethoprimsulphamethoxazole (Bactrim®) 2nd line (for amoxicillin failures): double dose of amoxicillin (80 mg/kg/day), amoxicillin-clavulinic acid (Clavulin®) cephalosporins: cefuroxime axetil (Ceftin®), ceftriaxone IM(Rocephin®), cefaclor (Ceclor®), cefixime (Suprax®) AOM deemed unresponsive if clinical signs/symptoms and otoscopic findings persist beyond 48 hours of antibiotic treatment • symptomatic therapy: antipyretics/analgesics (e.g. acetaminophen) decongestants – may relieve nasal congestion but does not treat AOM • prevention: parent education about risk factors (see above) antibiotic prophylaxis – amoxicillin or macrolide shown effective at half therapeutic dose pneumococcal and influenza vaccine surgery: choice of surgical therapy for recurrent AOM depends on whether local factors (eustachian tube dysfunction) are responsible (use ventilation tubes), or regional disease factors (tonsillitis, adenoid hypertrophy, sinusitis) are responsible Indications for Myringotomy and Tympanostomy Tubes in Recurrent AOM and OME (tubes are more commonly inserted for OME, rarely for AOM) • persistent effusion >3 months (OME) • lack of response to >3 months of antibiotic therapy (OME) • persistent effusion for ≥3 months after episode of AOM (OME) • recurrent episodes of AOM (>7 episodes in 6 months) • bilateral conductive hearing loss of >20 dB (OME) • chronic retraction of the tympanic membrane or pars flaccida (OME) • bilateral OME lasting >4 to 6 mos • craniofacial anomalies predisposing to middle ear infections (e.g. cleft palate) (OME) • complications of AOM (see below) McIsaac WJ. Coyte PC. Croxford R. Asche CV. Friedberg J. Feldman W. Otolaryngologists’ perceptions of the indications for tympanostomy tube insertion in children. CMAJ. 162(9):1285-8, 2000 May 2. Myringotomy and tympanostomy tubes. In: 2000 clinical indicators compendium. Alexandria (VA): American Academy of Otolaryngology-Head and Neck Surgery; 1999.
Complications of AOM • otologic: TM perforation chronic suppurative OM ossicular necrosis cholesteatoma persistent effusion (often leading to hearing loss) • CNS: meningitis brain abscess facial nerve paralysis • other: mastoiditis labyrinthitis sigmoid sinus thrombophlebitis
Otitis Media with Effusion (OME) Definition • presence of fluid in the middle ear without signs or symptoms of ear infection Epidemiology • not exclusively a pediatric disease • follows AOM frequently in children • middle ear effusions have been shown to persist following an episode of AOM for 1 mo in 40% of children, 2 mo in 20% and 3+ mo in 10%
Otolaryngology OT39 Antibiotics for Acute Otitis Media in Children Cochrane Database of Systematic Reviews 2004;1 Study: Meta-analysis of Randomized Controlled Trials (RCTs) on children (>6 mo) with acute otitis media comparing any antibiotic regime to placebo. Data Sources: Cochrane Central Register of Controlled Trials (2003 issue 1), MEDLINE (January 2000 to March 2003), and EMBASE (January 1990 to March 2003) without language restrictions. Main Outcomes: 1) Pain at 24 hours, and 2-7 days. 2) Hearing measured by tympanometry at 1 and 3 months. Patients: Pain: 24 hours, 4 studies (n=717); 2-7 days 9 studies (n=2287). Hearing: 1 month, 3 studies (n=472); 3 months, 2 studies (n=370). Results: Treatment with antibiotics had no significant impact on pain at 24 hours. However, pain at 2-7 days was lower in the antibiotic groups with an NNT of 16 (p3 episodes/year over 3 consecutive years • any complication of tonsillitis: quinsy, parapharyngeal abscess, retropharyngeal abscess strep bacteremia: rheumatic heart disease, nephritis, arthritis Relative Contraindications • repaired cleft palate • hemophilia • epidermolysis bullosa • retrognathia
Airway Problems in Children DIFFERENTIAL DIAGNOSIS BY AGE GROUP Neonates (obligate nose breathers) • extralaryngeal: choanal atresia (e.g. CHARGE syndrome) nasopharyngeal dermoid, glioma, encephalocele glossoptosis – Pierre-Robin sequence, Down syndrome, lymphangioma, hemangioma • laryngeal: laryngomalacia – most common cause of stridor in children laryngocele vocal cord palsy (Arnold-Chiari malformations) glottic web subglottic stenosis laryngeal cleft • tracheal: tracheoesophageal fistula tracheomalacia vascular rings 2 to 3 Months • congenital: laryngomalacia vascular: subglottic hemangioma (more common), innominate artery compression, double aortic arch laryngeal papilloma • acquired: subglottic stenosis – post intubation tracheal granulation – post intubation tracheomalacia – post tracheotomy and tracheoesophageal fistula (TEF) repair
Otolaryngology OT43
OT44 Otolaryngology
Pediatric Otolaryngology
Toronto Notes 2011
Infants – Sudden Onset • foreign body aspiration • croup • bacterial tracheitis • caustic ingestion • epiglottitis Children and Adults • infection: Ludwig’s angina peritonsillar-parapharyngeal abscess retropharyngeal abscess • neoplastic: squamous cell carcinoma (SCC) (adults): larynx, hypopharynx retropharyngeal: lymphoma, neuroblastoma nasopharyngeal: carcinoma, rhabdomyosarcoma • allergic: angioneurotic edema polyps (suspect cystic fibrosis in children) • trauma: laryngeal fracture, facial fracture burns and lacerations post-intubation caustic ingestion • congenital: lingual thyroid/tonsil
Signs of Airway Obstruction
Symptoms and signs of airway obstruction require a full assessment to diagnose potentially serious causes.
Stridor • note quality, timing • body position important: lying prone: subglottic hemangioma, double aortic arch lying supine: laryngomalacia, glossoptosis • site of stenosis: vocal cords or above: inspiratory stridor subglottis and extrathoracic trachea: biphasic stridor distal tracheobronchial tree: expiratory stridor Respiratory Distress • nasal flaring • supraclavicular and intercostal indrawing • sternal retractions • use of accessory muscles of respiration • tachypnea • cyanosis • altered LOC Feeding Difficulty and Aspiration • supraglottic lesion • laryngomalacia • vocal cord paralysis • post laryngeal cleft g aspiration pneumonia • tracheoesophageal fistula
Acute Laryngotracheobronchitis (Croup) Signs of Croup – the 3 S’s Stridor Subglottic swelling Seal bark cough
• inflammation of tissues in subglottic space ± tracheobronchial tree • swelling of mucosal lining and associated with thick, viscous, mucopurulent exudate which compromises upper airway (subglottic space narrowest portion of upper airway) • normal function of ciliated mucous membrane impaired Etiology • viral: parainfluenzae I (most common), II, III, influenza A and B, RSV
Toronto Notes 2011
Pediatric Otolaryngology
Otolaryngology OT45
Clinical Features • age 4 months to 5 years • preceded by URTI symptoms • generally occurs at night • biphasic stridor and croupy cough (loud, sea-lion bark) • appear less toxic than epiglottitis • supraglottic area normal • rule out foreign body and subglottic stenosis • “steeple-sign” on AP x-ray of neck • if recurrent croup, think subglottic stenosis Treatment • racemic epinephrine via nebulizer q1 to 2h, prn • systemic corticosteroids (e.g. dexamethasone, prednisone) • adequate hydration • close observation for 3 to 4 hours • intubation if severe • hospitalize if poor response to steroids after 4 hours and persistent stridor at rest • consider alternate diagnosis if poor response to therapy (e.g. bacterial tracheitis)
Acute Epiglottitis • acute inflammation causing swelling of supraglottic structures of the larynx without involvement of vocal cords Etiology • H. influenzae type B • relatively uncommon condition due to HiB vaccine Clinical Features • any age, most commonly 1 to 4 years • rapid onset • toxic-looking, fever, anorexia, restlessness • cyanotic/pale, inspiratory stridor, slow breathing, lungs clear with decreased air entry • prefers sitting up, open mouth, drooling, tongue protruding, sore throat, dysphagia Investigations and Management • investigations and physical examination may lead to complete obstruction, thus preparations for intubation or tracheotomy must be made prior to any manipulation • stat ENT/anesthesia consult(s) • lateral neck radiograph – cherry-shaped epiglottic swelling (“thumb sign”) – only if stable • WBC (elevated), blood and pharyngeal cultures after intubation
When managing epiglottitis, it is important not to agitate the child, as this may precipitate complete obstruction.
Treatment • secure airway • IV access with hydration • antibiotics – IV cefuroxime, cefotaxime, or ceftriaxone • moist air • extubate when leak around tube occurs and afebrile • watch for meningitis
Subglottic Stenosis Congenital • diameter of subglottis 90% of cases • in the case of severe laryngomalacia, division of the aryepiglottic folds provides relief
Foreign Body Ingested • usually stuck at cricopharyngeus • coins, toys • presents with drooling, dysphagia, stridor if very large Foreign body inhalation is the most common cause of accidental death in children.
Aspirated • usually stuck at right mainstem bronchus • peanuts, carrot, apple core, popcorn, balloons • presentation stridor if lodged in trachea unilateral “asthma” if bronchial, therefore often misdiagnosed as asthma if impacts to totally occlude airway: cough, lobar pneumonia, atelectasis, mediastinal shift, pneumothorax
Batteries MUST be ruled out as a foreign body (vs. coins) as they are lethal and can erode into the aorta.
Diagnosis and Treatment • inspiration-expiration chest x-ray (if patient is stable) • bronchoscopy and esophagoscopy with removal • rapid onset, not necessarily febrile or elevated WBC
Deep Neck Space Infection Trismus means the pterygoids are involved and airway will become increasingly hard to access.
These investigations should be obtained carefully and the surgeon should consider accompanying the patient for the x-ray as the worst place to lose an airway is during imaging.
Ludwig’s angina is the prototypical infection of the submandibular and sublingual space.
In Ludwig’s angina, the floor of mouth feels hard on palpation.
• most commonly arise from an infection of the mandibular teeth, tonsils, parotid gland, deep cervical lymph nodes, middle ear, or the sinuses • often a rapid onset and may progress to fatal complications Etiology • usually mixed aerobic and anaerobes that represent the flora of the oral cavity, upper respiratory tract, and certain parts of the ears and eyes Clinical Features • sore throat or pain and trismus • dysphagia and odynophagia • stridor and dyspnea • late findings may include dysphonia and hoarseness • swelling of the face and neck, erythema • asymmetry of the oropharynx with purulent oral discharge • lymphadenopathy Diagnosis • lateral cervical view of the plain radiograph • CT • MRI Treatment • secure the airway • surgical drainage • maximum doses of IV systemic antimicrobials regimens according to the site of infection
Toronto Notes 2011
Common Medications
Common Medications Table 21. Antibiotics Generic Name (Brand Name) Dosing Schedule
Indications
Comments
amoxicillin (Amoxil®, Amoxi ®, Amox®)
Adult: 500 mg PO tid Streptococcus, Pneumococcus, Children: H. influenzae, Proteus coverage 80-90 mg/kg/day in 2 divided doses
In patients with infectious mononucleosis, may cause rash
piperacillin with tazobactam (Zosyn®)
3 g PO q6h
Gram-positive and negative aerobes and anaerobes plus Pseudomonas coverage
May cause pseudomembranous colitis
ciprofloxacin (Cipro®, Ciloxan®)
500 mg PO bid
Pseudomonas, Streptococci, MRSA, and most Gram-negative; no anaerobic coverage
Do not give quinolones to children
erythromycin (Erythrocin®, EryPed®, Staticin®, T-Stat®, Erybid®, Novorythro Encap®)
500 mg PO qid
Alternative to penicillin
Ototoxic
Table 22. Otic Drops Generic Name (Brand Name)
Dose
Indications / Notes
ciprofloxacin (Ciprodex®)
4 gtt in affected ear bid
For otitis externa and complications of otitis media Pseudomonas, Streptococci, MRSA, and most Gram-negative; no anaerobic coverage
neomycin, polymyxin B sulfate, and hydrocortisone (Cortisporin Otic®)
5 gtt in affected ear tid
For otitis externa Used for inflammatory conditions which are currently infected or at risk of bacterial infections May cause hearing loss if placed in inner ear
hydrocortisone and acetic acid (VoSol HC®)
5-10 gtt in affected ear tid
Bactericidal by lowering pH
tobramycin and dexamethasone (TobraDex®)
5-10 gtt in affected ear bid
For chronic suppurative otitis media Risk of vestibular or cochlear toxicity
Table 23. Nasal Sprays Generic Name (Brand Name)
Indications
Notes: General
Allergic rhinitis Chronic sinusitis
Requires up to four weeks of consistent use to have effect Long term use Dries nasal mucosa; get minor bleeding Patient should stop if epistaxis May sting Flonase® and Nasonex® not absorbed systemically
Allergic rhinitis
Immediate effect If no effect by 3 days then discontinue Use during allergy season
Acute sinusitis Rhinitis
Careful if patient has hypertension Short term use ( male • age: onset between 25-50 years of age • family history: depression, alcohol abuse, sociopathy • childhood experiences: loss of parent before age 11, negative home environment (abuse, neglect) • personality: insecure, dependent, obsessional • recent stressors: illness, financial, legal • postpartum 65 years old • high suicide risk due to social isolation, chronic medical illness • suicide peak: males aged 80-90; females aged 50-65 • often present with somatic complaints (e.g. changes in weight, sleep, energy) or anxiety symptoms • refer to Table 4 to compare with delirium and dementia Treatment • biological: antidepressants (see PS45), lithium, antipsychotics, anxiolytics, electroconvulsive therapy (ECT), light therapy • psychological individual therapy: psychodynamic, interpersonal, cognitive behavioural therapy family therapy group therapy • social: vocational rehabilitation, social skills training • experimental: deep brain stimulation, transcranial magnetic stimulation, vagal nerve stimulation Prognosis • one year after diagnosis of a MDE without treatment, 40% of individuals still have symptoms that are sufficiently severe to meet criteria for a full MDE, 20% continue to have some symptoms that no longer meet criteria for a MDE, 40% have no mood disorder
Antidepressants for Depression in Medical Illness Cochrane Database of Systematic Reviews 2010; Issue 3 This systematic review and meta-analysis of 51 RCTs (3603 patients) compared anti-depressants to placebo in patients with a physical disorder (eg. Cancer, MI) who have been diagnosed as depressed (including Major Depression, Adjustment Disorder, and Dysthymia). Conclusions: Antidepressants, including SSRIs and TCAs, cause a significant improvement in patients with a physical illness, as compared to placebo.
St. John’s Wort for Major Depression Cochrane Database of Sys Rev 2008; Issue 3 Study: Systematic review of trials that were (1) randomized, double-blinded (2) with patients with major depression (3) comparing St. John’s wort (hypericum extracts) with placebo or standard antidepressants and (4) included clinical outcomes. Patients: 5489 patients with major depression. Outcomes: 1. Effectiveness: treatment response measured by a depression scale 2. Safety: the proportion of patients who dropped out due to adverse effects. Intervention: St. John’s wort vs. placebo; St. John’s wort vs. standard antidepressants. Results: 29 trials, 5489 patients, with 18 comparisons with placebo and 17 with antidepressants. St John’s wort is more effective than placebo (response rate ratio=1.87, 95% CI), and similarly effective as antidepressants (RRR=1.02, 95%CI). Less adverse effects with hypericum extracts. However, the effect size is dependent on the country of origin.
Cognitive Therapy vs. Medications in the Treatment of Moderate to Severe Depression Arch Gen Psychiatry 2005; 62:409-416 Study: Randomized control trial. Patients: 240 outpatients with moderate to severe MDD, aged 18-70. Intervention: 16 weeks of paroxetine with or without augmentation with lithium carbonate or desipramine hydrochloride (n=120) versus cognitive behavioural therapy (n=60). Response up to 8 weeks was controlled by pill placebo (n=60) Main Outcomes: The Hamilton Depression Rating scale was used to determine response to treatment. Results: At 8 weeks, 50% (95%CI 41-59%) of patients on medication and 43% (95%CI 31-56%) of patients on CBT had responded in comparison to 25% (95%CI 16-38%) of patients on pill placebo. There was no significant difference between medication and CBT. At 16 weeks, 46% of patients on medication and 40% of patients on CBT achieved remission. Summary: There is no difference in efficacy between CBT vs. paroxetine in the treatment of moderate to severe depression.
PS10 Psychiatry
Mood Disorders
Toronto Notes 2011
DYSTHYMIA DSM-IV-TR Diagnostic Criteria for Dysthymic Disorder
Reprinted with permission from the Diagnostic and Statistical Manual of Mental Disorders, Text Revision, Fourth Edition, (Copyright 2000). American Psychiatric Association.
A. depressed mood for most of the day, for more days than not, as indicated either by subjective account or observation by others, for ≥2 years. Note: In children and adolescents, mood can be irritable and duration must be at least 1 year B. presence, while depressed, of ≥2 of the following poor appetite or overeating insomnia or hypersomnia low energy or fatigue low self-esteem poor concentration or difficulty making decisions feelings of hopelessness C. during the 2-year period (1 year for children or adolescents) of the disturbance, the person has never been without the symptoms in criteria A and B for more than 2 months at a time D. no MDE has been present during the first 2 years of the disturbance (1 year for children and adolescents); i.e. the disturbance is not better accounted for by chronic MDD, or MDD in partial remission E. there has never been a Manic Episode, a Mixed Episode, or a Hypomanic Episode, and criteria have never been met for Cyclothymic Disorder F. the disturbance does not occur exclusively during the course of a chronic Psychotic Disorder, such as Schizophrenia or Delusional Disorder G. the symptoms are not due to the direct physiological effects of a substance or a GMC H. the symptoms cause clinically significant distress or impairment in social, occupational, or other important areas of functioning Epidemiology • point prevalence: 3%; life prevalence: 6%; M:F = 1:2-3 Treatment • psychological principle treatment for dysthymia individual, group, and family therapy • biological antidepressant therapy (SSRIs/SNRIs) as an outpatient
Postpartum Mood Disorders Postpartum “Blues” • transient period of mild depression, mood instability, anxiety, decreased concentration, increased concern over own health and health of baby – considered to be normal emotional changes related to the puerperium • occurs in 50-80% of mothers; begins 2-4 days postpartum, usually lasts 48 hours, can last up to 10 days • does not require psychotropic medication • patient at increased risk of developing postpartum depression Health Canada Advises of Potential Adverse Effects of SSRls and Other Antidepressants on Newborns August 9, 2004 Health Canada was concerned that newborns exposed to SSRls and other antidepressants during the third trimester of pregnancy may be adversely affected, because of reports of complications at birth requiring longer hospitalization, breathing support and tube feeding. Advisory applied to: bupropion (used for depression or smoking cessation), citalopram, fluoxetine, fluvoxamine, mirtazapine, paroxetine, sertraline and venlafaxine. Conclusions: Physicians and patients should carefully consider risks, benefits and options for both the mother and unborn baby when treating depression in pregnant women. Consider tapering in the third trimester. Women should consult their doctors before stopping these medications.
Postpartum Depression (PPD) • diagnosis: MDE, onset within 4 weeks postpartum • clinical presentation typically lasts 2 to 6 months; residual symptoms can last up to 1 year may present with psychosis – rare (0.2%), usually associated with mania, but can be MDE severe symptoms include extreme disinterest in baby, suicidal and infanticidal ideation • epidemiology: occurs in 10% of mothers, risk of recurrence 50% • risk factors previous history of a mood disorder (postpartum or otherwise) psychosocial factors: stressful life events, unemployment, marital conflict, lack of social support, unwanted pregnancy, colicky or sick infant • treatment psychotherapy short-term safety of maternal SSRIs for breastfeeding infants established; long-term effects unknown supportive, non-directive counselling by trained home visitors if depression severe, consider ECT • prognosis: impact on child development – increased risk of cognitive delay, insecure attachment, behavioural disorders; treatment of mother improves outcome for child at 8 months through increased mother-child interaction
Toronto Notes 2011
Mood Disorders
Premenstrual Dysphoric Disorder (PMDD) DSM-IV-TR Diagnostic Criteria for Premenstrual Dysphoric Disorder
Reprinted with permission from the Diagnostic and Statistical Manual of Mental Disorders, Text Revision, Fourth Edition, (Copyright 2000). American Psychiatric Association.
A. in most menstrual cycles during the past year, five (or more) of the following symptoms were present for most of the time during the last week of the luteal phase, began to remit within a few days after the onset of the follicular phase, and were absent in the week post-menses, with at least one of the symptoms being one of the first four listed 1. markedly depressed mood, feelings of hopelessness, or self-deprecating thoughts 2. marked anxiety, tension, feeling of being “keyed up” or “on edge” 3. marked affective lability 4. persistent and marked anger, irritability, or increased interpersonal conflicts 5. decreased interest in usual activities 6. difficulty concentrating 7. lethargy, easily fatigued, lack of energy 8. change in appetite – overeating or specific food cravings 9. hypersomnia or insomnia 10. a sense of being overwhelmed or out of control 11. physical symptoms – breast tenderness or swelling, headaches, joint or muscle pain, sensation of bloating or weight gain B. the disturbance markedly interferes with work, school, social activities or relationships with others C. the disturbance is not merely an exacerbation of the symptoms of another disorder such as Major Depressive Disorder, Panic Disorder, Dysthymic Disorder or Personality Disorder D. Criteria A, B and C must be confirmed by prospective daily recordings and/or ratings during at least two consecutive symptomatic cycles Treatment • 1st line: SSRIs highly effective in treating PMDD fluoxetine and sertraline most studied can be used intermittently in luteal phase x 14 days • 2nd line clomipramine alpraxolam (Xanax®) for anxiety symptoms • 3rd line OCP containing progesterone drospirenone (e.g. Yasmin®) GnRH agonists (e.g. leuprolide) • if GnRH agonist completely relieves symptoms, may consider definitive surgery (i.e. total abdominal hysterectomy + bilateral salpingo-oophorectomy)
Bipolar Disorders BIPOLAR I / BIPOLAR II DISORDER Definition • Bipolar I Disorder disorder in which at least one manic or mixed episode has occurred commonly accompanied by at least 1 MDE but not required for diagnosis • Bipolar II Disorder disorder in which there is at least 1 MDE and at least 1 hypomanic episode no past manic or mixed episode Epidemiology • prevalence: 0.6-0.9%; M:F = 1:1 • age of onset: teens to 20’s Risk Factors • slight increase in upper socioeconomic groups • 60-65% of bipolar patients have family history of major mood disorders Classification • classification of bipolar disorder involves describing the current or most recent mood episode as either manic, hypomanic, mixed or depressed • the current or most recent episode can be further classified as without psychotic features, with psychotic features, with catatonic features, with postpartum onset, with seasonal pattern, with rapid cycling (at least 4 episodes of a mood disturbance in the previous 12 months that meet criteria for a Major Depressive, Manic, Mixed, or Hypomanic Episode)
Psychiatry PS11
PS12 Psychiatry A Randomized Controlled Trial of Cognitive Therapy for Bipolar Disorder: Focus on Longterm Change J Clin Psychiatry. 2006 Feb; 67(2):277-86 Study: Randomized, blinded clinical trial. Patients: 52 patients with DSM-IV bipolar 1 or 2 disorder. Intervention: Patients allocated to either a 6 month trial of cognitive therapy (CT) with emotive techniques or treatment as usual. Both groups received mood stabilizers. Main Outcomes: Relapse rates, dysfunctional attitudes, psychosocial functioning, hopelessness, self-control, medication adherence. Patients were assessed by independent raters blinded to treatment group. Results: At 6 months, CT patients experienced fewer depressive symptoms and fewer dysfunctional attitudes. There was a non-significant (p=.06) trend to greater time to depressive relapse. At 12 month follow up, CT patients had lower Young Mania Rating scores and improved behavioural self-control. At 18 months, CT patients reported less severity of illness. Conclusions: CT appears to provide benefits in the 12 months succeeding completion of therapy.
Mood Disorders/Anxiety Disorders
Toronto Notes 2011
Treatment • biological: mood stabilizers, anticonvulsants, antipsychotics, antidepressants, ECT (Note: Treatment of bipolar depression must be done extremely cautiously, as a switch from depression to mania can result. Monotherapy with antidepressants should be avoided) • psychological: supportive and psychodynamic psychotherapy, cognitive or behavioural therapy • social: vocational rehabilitation, leave of absence from school/work, drug and EtOH cessation, substitute decision maker for finances, sleep hygiene, social skills training, education for family members CYCLOTHYMIA Diagnosis • presence of numerous periods of hypomanic and depressive symptoms (not meeting criteria for MDE) for ≥2 years; never without symptoms for >2 months • no MDE, manic or mixed episodes; no evidence of psychosis • symptoms are not due to the direct physiological effects of a substance or GMC • symptoms cause clinically significant distress or impairment in social, occupational, or other important areas of functioning Treatment • similar to Bipolar I • anticonvulsants ± psychotherapy
Anxiety Disorders Definition • anxiety is a universal human characteristic involving tension, apprehension, or even terror, which serves as an adaptive mechanism to warn about an external threat by activating the sympathetic nervous system (fight or flight) • manifestations of anxiety can be described along a continuum of physiology, psychology, and behaviour physiology – main brain structure involved is the amygdala; neurotransmitters involved include serotonin, cholecystokinin, epinephrine, norepinephrine, dopamine psychology – one’s perception of a given situation is distorted which causes one to believe it is threatening in some way behaviour – once feeling threatened, one responds by escaping or facing the situation, thereby causing a disruption in daily functioning • anxiety becomes pathological when fear is greatly out of proportion to risk/severity of threat response continues beyond existence of threat or becomes generalized to other similar or dissimilar situations social or occupational functioning is impaired Differential Diagnosis Table 3. Differential Diagnosis of Anxiety Disorders Cardiovascular
Post-MI, arrhythmia, congestive heart failure, pulmonary embolus, mitral valve prolapse
Respiratory
Asthma, COPD, pneumonia, hyperventilation
Endocrine
Hyperthyroidism, pheochromocytoma, hypoglycemia, hyperadrenalism, hyperparathyroidism
Metabolic
Vitamin B12 deficiency, porphyria
Neurologic
Neoplasm, vestibular dysfunction, encephalitis
Substance-Induced
Intoxication (caffeine, amphetamines, cocaine, thyroid preparations, OTC for colds/decongestants), withdrawal (benzodiazepines, alcohol)
Other Psychiatric Disorders
Psychotic disorders, mood disorders, personality disorders (OCPD), somatoform disorders
Medical Workup of Anxiety Disorder • routine screening: physical examination, CBC, thyroid function test, electrolytes, urinalysis, urine drug screening • additional screening: neurological consultation, chest x-ray, electrocardiogram (ECG), CT scan
Toronto Notes 2011
Anxiety Disorders
Psychiatry PS13
Panic Disorder DSM-IV-TR Diagnostic Criteria for Panic Disorder without Agoraphobia
Reprinted with permission from the Diagnostic and Statistical Manual of Mental Disorders, Text Revision, Fourth Edition, (Copyright 2000). American Psychiatric Association.
A. both (1) and (2) (1) recurrent unexpected panic attacks: a discrete period of intense fear or discomfort, in which ≥4 of the following symptoms develop abruptly and reach a peak within 10 minutes palpitations, pounding heart, or accelerated heart rate sweating trembling or shaking sensations of shortness of breath or smothering feeling of choking chest pain or discomfort nausea or abdominal distress feeling dizzy, unsteady, lightheaded, or faint derealization (feelings of unreality) or depersonalization (being detached from oneself) fear of losing control or going crazy fear of dying paresthesias (numbness or tingling sensations), chills or hot flushes (2) at least one of the attacks has been followed by 1 month (or more) of ≥1 of the following persistent concern about having additional attacks worry about the implications of the attack or its consequences (e.g. losing control, having a heart attack, “going crazy”) a significant change in behavior related to the attacks B. absence of agoraphobia C. the panic attacks are not due to the direct physiological effects of a substance or GMC D. the panic attacks are not better accounted for by another mental disorder, such as Social Phobia, Specific Phobia, Obsessive-Compulsive Disorder, Post-Traumatic Stress Disorder, Separation Anxiety Disorder Epidemiology • prevalence: 1.5-5% (one of the top five most common reasons to see a family doctor); M:F = 1:2-3 • onset: average late 20’s, familial pattern Treatment • psychological supportive psychotherapy, relaxation techniques (visualization, box-breathing), cognitive behavioural therapy (correct distorted thinking, desensitization/exposure therapy) • biological SSRIs: fluoxetine, citalopram, paroxetine, fluvoxamine, sertraline SNRI: venlafaxine with SSRI/SNRIs start low, go slow, aim high to prevent non-compliance due to physical side effects, explain symptoms to expect prior to initiation other antidepressants (TCAs: clomipramine, imipramine, mirtazapine, MAOIs) consider avoiding bupropion due to stimulating effects benzodiazepines (short term, low dose, regular schedule, long half-life, no prn) Prognosis • 6-10 years post-treatment: 30% well, 40-50% improved, 20-30% no change or worse • clinical course: chronic, but episodic with psychosocial stressors Panic Disorder with Agoraphobia • agoraphobia anxiety about being in places or situations from which escape might be difficult (or embarrassing) or where help may not be available in the event of having an unexpected panic attack fears commonly involve situations: being out alone, being in a crowd, standing in a line, or travelling on a bus • situations are avoided, endured with anxiety or panic, or require companion • treatment: as per panic disorder
Generalized Anxiety Disorder (GAD) DSM-IV-TR Diagnostic Criteria for Generalized Anxiety Disorder
Reprinted with permission from the Diagnostic and Statistical Manual of Mental Disorders, Text Revision, Fourth Edition, (Copyright 2000). American Psychiatric Association.
A. excessive anxiety and worry (apprehensive expectation), occurring more days than not for at least 6 months, about a number of events or activities (such as work or school performance) B. the person finds it difficult to control the worry
Situational trigger
Panic attack
Increased anxiety and generalization to other situations
Mentally associated with situation
Figure 1. Panic Attack
Criteria for Panic Disorder (≥4) STUDENTS FEAR the 3 C’s Sweating Trembling Unsteadiness, dizziness Depersonalization, Derealization Excessive heart rate, palpitations Nausea Tingling Shortness of breath Fear of dying, losing control, going crazy 3 C’s: Chest pain, Chills, Choking
PS14 Psychiatry
Criteria for GAD (≥3) BE SKIM Blank mind Easily fatigued Sleep disturbance Keyed up Irritability Muscle tension
Anxiety Disorders
Toronto Notes 2011
C. the anxiety and worry are associated with ≥3 of the following 6 symptoms (with at least some symptoms present for more days than not for the past 6 months) Note: Only one item is required in children 1. restlessness or feeling keyed up or on edge 2. being easily fatigued 3. difficulty concentrating or mind going blank 4. irritability 5. muscle tension 6. sleep disturbance (difficulty falling or staying asleep, or restless unsatisfying sleep) D. the focus of the anxiety and worry is not confined to features of an Axis I disorder, such as panic disorder, social phobia, etc. E. the anxiety, worry, or physical symptoms cause clinically significant distress or impairment in social, occupational, or other important areas of functioning F. the disturbance is not due to the direct physiological effects of a substance or a GMC and does not occur exclusively during a Mood Disorder, a Psychotic Disorder, or a Pervasive Developmental Disorder Epidemiology • 1-year prevalence: 3-8%; M:F = 1:2 if considering only those receiving inpatient treatment, ratio is 1:1 • most commonly presents in early adulthood Treatment • lifestyle: caffeine and EtOH avoidance, sleep hygiene • psychological: psychotherapy, relaxation, mindfulness, and CBT • biological benzodiazepines (short term, low dose, regular schedule, long half-life, no prn) buspirone (tid dosing) others: SSRIs/SNRI, TCAs, beta-blockers avoid bupropion due to stimulating effects • combinations of above Prognosis • chronically anxious adults become less so with age • depends on pre-morbid personality functioning, stability of relationships, work, and severity of environmental stress • difficult to treat
Phobic Disorders Specific Phobia • definition: marked and persistent fear that is excessive or unreasonable, cued by presence or anticipation of a specific object or situation • lifetime prevalence 12-16%; M:F ratio variable • types: animal/insect, environment (heights, storms), blood/injection/injury, situational (airplane, closed spaces), other (loud noise, clowns) Social Phobia (Social Anxiety Disorder) • definition: marked and persistent fear of social or performance situations in which person is exposed to unfamiliar people or to possible scrutiny by others; person fears he/she will act in a way that may be humiliating or embarrassing (e.g. public speaking, initiating or maintaining conversation, dating, eating in public) • lifetime prevalence may be as high as 13-16%; M90 d
Acute: within 10 d Tardive: >90 d
Acute: within 30 d
Tardive: >90 d
Treatment
Acute: benztropine or diphenhydramine
Acute: lorazepam, propanolol or diphenhydramine; reduce or change neuroleptic to lower potency
Acute: benztropine (or benzodiazepine if side effects); reduce or change neuroleptic to lower potency
Tardive: no good treatment; may try clozapine; discontinue drug or reduce dose
Antiparkinsonian Agents (Anticholinergic Agents) • types benztropine (Cogentin®) 2 mg PO, IM or IV OD (~1-6 mg) amantadine (Symmetrel®) 100 mg PO bid (100-400 mg) diphenhydramine (Benadryl®) 25-50 mg PO/IM qid • do not always prescribe with neuroleptics give only if at high risk for acute EPS or if acute EPS develops • do not give these for tardive syndromes because they worsen the condition
Toronto Notes 2011
Pharmacotherapy
Antidepressants • onset of effect neurovegetative symptoms – 1-3 weeks emotional/cognitive symptoms – 2-6 weeks • may use mild stimulant (e.g. methylphenidate) for severe neurovegetative symptoms briefly and taper down as antidepressant effect increases • taper TCAs slowly (over weeks-months) because they can cause withdrawal reactions • tapering of any kind of antidepressant may be required based on the half-life of the medication and the patient’s individual sensitivity • it is important to be particularly vigilant over the first 2 weeks of therapy as neurovegetative symptoms may start to resolve while emotional and cognitive symptoms may not (patients may be particularly at risk for suicidal behaviour during this time) • treatment of bipolar depression: monotherapy with antidepressants is not advisable as a switch from depression to mania can occur. If the patient is medication-naïve, initiate therapy with a mood stabilizer plus an SSRI or buproprion. For patients taking mood stabilizers, consider adding or switching to lithium or lamotrigine, or adding an SSRI or buproprion Table 19. Common Antidepressants Class
Drug
Daily Starting Dose (mg)
Therapeutic Dose (mg)
SSRI
fluoxetine (Prozac®) fluvoxamine (Luvox®) paroxetine (Paxil®) sertraline (Zoloft®) citalopram (Celexa®) escitalopram (Cipralex®)
20 50-100 10 50 20 10
20-80 150-300 20-60 50-200 20-60 10-20
SNRI
venlafaxine (Effexor®) duloxetine (Cymbalta®)
37.5-75 40
75-225 40-60
NDRI
bupropion (Wellbutrin®)
100
300-450
TCA (3º Amines)
amitriptyline (Elavil®) imipramine (Tofranil®)
75-100 75-100
150-300 150-300
TCA (2º Amines)
nortriptyline (Aventyl®) desipramine (Norpramin®)
75-100 100-200
75-150 150-300
MAOI
phenelzine (Nardil®) tranylcypromine (Parnate®)
45 30
60-90 10-60
RIMA
moclobemide (Manerix®)
300
300-600
NASSA
mirtazapine (Remeron®)
15
15-45
(SSRI=selective serotonin reuptake inhibitors; SNRI=serotonin and norepinephrine reuptake inhibitors; NDRI=norepinephrine and dopamine reuptake inhibitors; TCA=tricyclic antidepressants; MAOI= monoamine oxidase inhibitors; RIMA=reversible inhibition of MAO-A; NASSA=noradrenergic and specific serotonin antagonists)
Treatment Strategies for Refractory Depression (see Figure 2) • optimization: ensuring adequate drug doses for the individual • augmentation: the addition of a medication that is not considered an antidepressant to an antidepressant regimen (e.g. thyroid hormone, lithium, atypical antipsychotics) • combination: the addition of another antidepressant to an existing treatment regimen (e.g. the addition of bupropion to an SSRI or SNRI) • substitute: change in the primary antidepressant (within or outside a class) Note: it is important to fully treat the symptoms of depression in order to decrease rates and severity of relapses Start SSRI or other first line agent Reassess every 1-2 weeks for 3-4 weeks Full response
No or partial response
Continue
Optimize dose Reassess regularly for 4-8 weeks
Full response
Partial response
Combine
Figure 2. Treatment of Depression
Augment
No response
Switch
Psychiatry PS45 Selective Serotonin Reuptake Inhibitors (SSRls) versus Other Antidepressants for Depression (Cochrane Review) Cochrane Database of Systematic Reviews 2004; Issue 3 This systematic review of 98 RCTs compared the efficacy of SSRls with other kinds of antidepressants in the treatment of patients with depressive disorders. Conclusions: There is no significant difference in the effectiveness of SSRls versus TCAs. Consider relative patient acceptability, toxicity and cost when choosing.
Tips On Choosing Antidepressants • All SSRIs have similar effectiveness, but consider side effect profiles and half-lives. • Bupropion causes less sexual dysfunction, weight gain, and sedation but is contraindicated for patients with history of seizure, stroke, brain tumour, brain surgery or closed head injury. Also used to treat eating disorders. Not recommended for anxiety because of stimulating effects. • Mirtazapine useful if insomnia or agitation are prominent, or to treat depression with cachexia. • Trazodone mainly used as adjunct for SSRI-induced sleep disturbances. • Sertraline, citalopram, and escitalopram have the least interactions with other drugs and are sleep-wake neutral. • Fluoxetine and paroxetine are the most activating drugs and should be taken in the morning. • Fluvoxamine is always sedating and should be taken in the evening.
How Long to Treat? 6-12 months: if first or second episode 2 years: if third episode, elderly, psychotic features, refractory depression, >2 episodes in 5 years
Psychopharmacology of SSRIs Post-Synaptic Effect/Side Effect Serotonin Receptor Stimulated 5HT1A centrally • Relief of depression • Anxiolytic effect 5HT2A in spinal • Sexual dysfunction: cord delayed ejaculation, anorgasmia, decreased interest/ libido • Rx with buproprion 5HT2C/5HT2A • Activation: anxiety, in brain insomnia • Worst with fluoxetine, paroxetine • Warn patients anxiety may worsen in first 1-2 weeks of treatment 5HT3A in gut • GI upset: nausea, vomiting, bloating • Take with food
PS46 Psychiatry
Pharmacotherapy
Toronto Notes 2011
Table 20. Commonly Used Antidepressants TCA
SSRI
Considerations
OCD (clomipramine) Melancholic depression
Anxiety states, OCD, eating disorders, For moderate/severe depression that Depression, anxiety disorders seasonal depression, typical and does not respond to SSRI atypical depression Atypical depression
Mode of Action
Block norepinephrine and serotonin reuptake
Block serotonin reuptake only
Irreversible inhibition of monoamine oxidase A and B Leads to norepinephrine and serotonin
Block norepinephrine and serotonin reuptake
Side Effects
Anticholinergic effects: (see Table 17) Noradrenergic effects: tremors, tachycardia, sweating, insomnia, erectile and ejaculation problems α-1 adrenergic effects: orthostatic hypotension Antihistamine effects: sedation, weight gain CNS: sedation, stimulation, seizure threshold CVS: HR, conduction delay
Fewer than TCA, therefore increased compliance CNS: restlessness, tremor, insomnia, headache, drowsiness GI: N/V, diarrhea, abdominal cramps, weight loss Sexual dysfunction: impotence, anorgasmia CVS: increased HR, conduction delay Serotonin syndrome, EPS, SIADH
Hypertensive crises with tyramine rich foods (e.g. wine, cheese) develop headache, flushes, palpitations, N/V, photophobia Dizziness, reflex tachycardia, postural hypotension, sedation, insomnia Weight gain Social dysfunction Energizing Minimal anticholinergic and antihistamine effects
Low dose side effects include insomnia (serotonergic) Higher dose side effects include: tremors, tachycardia, sweating, insomnia, dose-dependent increase in diastolic BP (noradrenergic)
Toxic in OD, but wider margin of safety than TCA
Tachycardia and N/V seen in acute overdose
Relatively safe in OD Risk in Overdose Toxic in OD 3 times therapeutic dose is lethal Presentation: anticholinergic effects, CNS stimulation, then depression and seizures ECG: QT (duration reflects severity) Treatment: activated charcoal, cathartics, supportive treatment, IV diazepam for seizure, physostigmine salicylate for coma Do not give ipecac, as can cause rapid neurologic deterioration and seizures Drug Interactions MAOI, SSRI EtOH
MAOI
SNRI
SSRIs inhibit P450 enzymes; therefore EtOH MAOI, SSRI will levels of drugs metabolized by Hypertensive crises with Does not seem to inhibit P450 system P450 system noradrenergic medications (e.g. TCA, decongestants, amphetamines) Serotonin syndrome with serotonergic drugs (e.g. SSRI, tryptophan, dextromethorphan)
NDRI
RIMA
NASSA
Considerations
Depression, seasonal depression
Depression unresponsive to other therapies
Useful in patients with insomnia, agitation or depression with cachexia
Mode of Action
Block norepinephrine and dopamine reuptake
Reversible inhibitor of monoamine oxidase A Leads to norepinephrine and serotonin
Enhance central noradrenergic and serotonergic activity by inhibiting presynaptic a-2 adrenergic receptors
Side Effects
CNS: dizziness, headache, tremor, insomnia CVS: dysrhythmia, hypertension GI: dry mouth, N/V, constipation, appetite Other: agitation, anxiety, anaphylactoid reaction
CNS: dizziness, headache, tremor, insomnia CVS: dysrhythmia, hypotension GI: dry mouth, N/V, diarrhea, abdominal pain, dyspepsia GU: delayed ejaculation Other: diaphoresis
CNS: somnolence, dizziness, seizure (rare) Endocrine: cholesterol, triglycerides GI: Constipation, ALT
Risk in Overdose Tremors and seizures seen in acute overdose
Risk of fatal overdose when combined Mild symptoms with overdose with citalopram or clomipramine
Drug Interactions MAOI Drugs that reduce seizure threshold: antipsychotics, systemic steroids, quinolone antibiotics, antimalarial drugs
MAOI, SSRI, TCA Narcotics
MAOI, SSRI, SNRI, RIMA
Toronto Notes 2011
Pharmacotherapy
Serotonin Syndrome • thought to be due to over-stimulation of the serotonergic system • can result from medication combinations such as SSRI+MAOI, SSRI+tryptophan, MAOI+meperidine, MAOI+tryptophan • rare but potentially life-threatening adverse reaction to SSRis, especially when switching from an SSRI to an MAOI • symptoms include nausea, diarrhea, palpitations, chills, restlessness, confusion, and lethargy but can progress to myoclonus, hyperthermia, rigor and hypertonicity • treatment: discontinue medication and administer emergency medical care as needed • important to distinguish from NMS (see side box, PS44) Discontinuation Syndrome • caused by the abrupt cessation of an antidepressant • observed most frequently with paroxetine, fluvoxamine, and venlafaxine • symptoms usually begin within l-3 days, and include: anxiety, insomnia, irritability, mood lability, N/V, dizziness, headache, dystonia, tremor, chills, fatigue, lethargy and myalgia • treatment: symptoms may last between l-3 weeks, but can be relieved within 24 hours by restarting antidepressant therapy at the same dose the patient was taking, and initiating a slow taper over several weeks • consider drug with longer half-life such as fluoxetine
Mood Stabilizers First-Line Lithium/Valproic Acid(± antipsychotic) • before initiating, get baseline: CBC, ECG (if patient >45 years old or cardiovascular risk), urinalysis, BUN, Cr, electrolytes, TSH • before initiating lithium: screen for pregnancy, thyroid disease, seizure disorder, neurological, renal, cardiovascular diseases • may need acute coverage with benzodiazepines or anti psychotics • use carbamazepine in non-responders and rapid cycling • can combine lithium and carbamazepine or valproic acid safely in lithium non-responders • olanzapine may be used as a mood stabilizer, in conjunction with other mood stabilizers • lithium and lamotrigine have established antidepressant efficacy Lithium Toxicity (see Table 21) • clinical diagnosis, as toxicity can occur at therapeutic levels • common causes • overdose • sodium or fluid loss • concurrent medical illness • clinical presentation • GI: severe nausea/vomiting and diarrhea • cerebellar: ataxia, slurred speech, lack of coordination • cerebral: drowsiness, myoclonus, choreiform or Parkinsonian movements, upper motor neuron signs, seizures, delirium, coma • management • discontinue lithium for several doses and begin again at a lower dose when lithium level has fallen to a nontoxic range • serum lithium levels, BUN, electrolytes • saline infusion • hemodialysis if lithium >2 mmol/L, coma, shock, severe dehydration, failure to respond to treatment after 24 hours, or deterioration Second-Line/ Adjuvant Mood Stabilizers Lamotrigine (Lamictal®) • indications: treatment of dysphoric mania, mixed episodes and rapid cycling BAD, bipolar type l depression, prevention of mania and depression • mechanism: may inhibit 5-HT3 receptors and potentiate DA activity • side effects • CNS: dizziness, headache, ataxia, nausea, somnolence, fever, anxiety • skin: rash, Stevens-Johnson syndrome (O.l %)
Psychiatry PS47
~·
Symptoms of Antidepressant Discontinuation FINISH Flu-like symptoms Insomnia Nausea Imbalance Sensory disturbances Hyperarousal (anxiety/agitation)
Sequenced Treatment A~ematives to Relieve Depression Journal of Psychosocial Nursing 1008; 46:11-14 Study: Prospectiverandomized anti-depressant treatment trial. Patients: 4000 patients with major depressive disorder. Objective: To compare the efficacy and tolerability of various antidepressant therapies through four sequential treatment levels. Intervention: l evel l-citalopram -> if relapse -> level1-citalopram + bupropion SR, sertraline, venlafaxine XR, or cognitivepsychotherapy. l evel 1A-switch to bupropion or venlafaxine XR. level 3-either mirtazapine or nortriptyline + lithium, T3. level4·tranylcypromine or venlafaxine XR + mirtazapine. Resu~s: Remission rates were18%for Levell , 17%for level1, 11-15%for level3, and 7-14%for level4. When more treatment steps are required, lower remission rates, greater degrees of tolerance, and higher rates of relapse.
....
,,
~}--------------------.
long term lithium use can lead to a nephropathy and diabetes insipidus in some patients.
~·
Lithium Side Effects LITHIVM Leukocytosis Insipidus (diabetes) Tremor, teratogenicity Hypothyroidism Increased weight "V" omiting, nausea Miscellaneous (e.g. EGG changes, acne)
PS48 Psychiatry
Pharmacotherapy
Toronto Notes 2011
Table 21. Commonly Used Mood Stabilizers Lithium
Lamotrigine (Lamictal®)
Divalproex (Epival®)
Carbamazepine (Tegretol®)
Indications
Maintenance therapy of bipolar disorder Treatment of acute mania Augmentation of antidepressants in MDE and OCD Schizoaffective disorder Chronic aggression and antisocial behaviour Recurrent depression
Treatment of bipolar disorder Rapid cycling bipolar disorder Mixed phase/Dysphoric mania Prevention of mania and depression
Maintenance therapy of bipolar disorder Treatment of acute mania Rapid cycling bipolar disorder Mixed phase/Dysphoric mania
Maintenance therapy of bipolar disorder Treatment of acute mania Rapid cycling bipolar disorder
Mode of Action
Unknown Therapeutic response within 7-14 days
May inhibit 5-HT3 receptors May potentiate DA activity
Depresses synaptic transmission Raises seizure threshold
Depresses synaptic transmission Raises seizure threshold
Dosage
Adult: 600-1500 mg/day Geriatric: 150-600 mg/day Usually once/day dosing
Starting: 12.5-15 mg/day Maximum: 500 mg/day Dose adjusted in patients taking other anti-convulsants
750-2500 mg/day Usually tid dosing
400-1600 mg/day Usually bid or tid dosing
17-50 mmol/L
350-700 µmol/L
Therapeutic Level Adult: 0.5-1.2 mmol/L (1.0-1.25 mmol/L Therapeutic plasma level not for acute mania) established Geriatric: 0.3-0.8 mmol/L Dosing based on therapeutic response Monitoring
Monitor serum levels until therapeutic Monitor for suicidality, particularly (always wait 12 hours after dose) when initiating treatment Then monitor biweekly or monthly until a steady state is reached, then q2 months Monitor thyroid function q6 months, creatinine q6 months, urinalysis q1 year
LFTs weekly x 1 month, then monthly, due to risk of liver dysfunction Watch for signs of liver dysfunction: nausea, edema, malaise
Weekly blood counts for first month, due to risk of agranulocytosis Watch for signs of blood dyscrasias: fever, rash, sore throat, easy bruising
Side Effects
GI: N/V, diarrhea, stomach pain GU: polyuria, polydipsia, GN, renal failure, nephrogenic DI CNS: fine tremor, lethargy, fatigue, headache Hematologic: reversible leukocytosis Other: teratogenic (Ebstein’s anomaly), weight gain, edema, psoriasis, hypothyroidism, hair thinning, muscle weakness, ECG changes
GI: liver dysfunction, N/V, diarrhea CNS: ataxia, drowsiness, tremor, sedation, cognitive blurring Other: hair loss, weight gain, transient thrombocytopenia, neural tube defects when used in pregnancy
GI: N/V, diarrhea, hepatic toxicity ( AST, ALT, LDH) CNS: ataxia, dizziness, slurred speech, drowsiness, confusion, nystagmus, diplopia Hematologic: transient leukopenia (10%), agranulocytosis, aplastic anemia Skin: rash (5% risk; should d/c drug because of risk of Stevens-Johnson syndrome) Other: neural tube defects when used in pregnancy
Interactions
NSAIDs decrease clearance
OCP
OCP
GI: N/V, diarrhea CNS: ataxia, dizziness, diplopia, headache, somnolence Skin: rash (should d/c drug because of risk of StevensJohnson syndrome), increased lamotrigine levels = increased risk of rash Other: anxiety
Toronto Notes 2011
Pharmacotherapy
Anxiolytics • indications short term treatment of transient forms of anxiety disorders, insomnia, alcohol withdrawal (especially delirium tremens), barbiturate withdrawal, organic brain syndrome (agitation in dementia), EPS and akathisia due to antipsychotics, seizure disorders, musculoskeletal disorders • relative contraindications major depression (except as an adjunct to other treatment), history of drug/alcohol abuse, pregnancy, breast feeding • mechanism of action benzodiazepines: potentiate binding of GABA to its receptors; results in decreased neuronal activity buspirone: partial agonist of 5-HT type IA receptors Rational Use of Anxiolytics (see Table 22) • anxiolytics mask or alleviate symptoms; they do not cure them Benzodiazepines • should be used for limited periods (weeks-months) to avoid dependence • all benzodiazepines are sedating • have similar efficacy, so choice depends on half-life, metabolites and route of administration, OD or BID • taper slowly over weeks-months because they can cause withdrawal reactions low dose withdrawal: tachycardia, hypertension, panic, insomnia, anxiety, impaired memory and concentration, perceptual disturbances high dose withdrawal: hyperpyrexia, seizures, psychosis, death • avoid alcohol because of potentiation of CNS depression; caution with drinking and use of machinery • side effects CNS: drowsiness, cognitive impairment, reduced motor coordination, memory impairment physical dependence, tolerance develops • withdrawal symptoms: anxiety, insomnia, dysperceptions, autonomic hyperactivity (less common) onset: 1-2 days (short-acting), 2-4 days (long-acting) duration: weeks/months complications: above 50 mg diazepam/day: seizures, delirium, arrhythmias, psychosis management: taper with long-acting benzodiazepine similar to, but less severe than alcohol withdrawal; can be fatal • overdose commonly used drug in overdose overdose is rarely fatal benzodiazepines are more dangerous and may cause death when combined with alcohol, other CNS depressants or TCAs Benzodiazepine Antagonist – Flumazenil (Anexate®) • use for suspected benzodiazepine overdose • specific antagonist at the benzodiazepine receptor site Buspirone (Buspar®) • primary use: generalized anxiety disorder • may be preferred over benzodiazepines because: non-sedating no interaction with alcohol does not alter seizure threshold not prone to abuse • onset of action: 2 weeks • side effects: dizziness, drowsiness, nausea, headache, nervousness, extrapyramidal
Psychiatry PS49
PS50 Psychiatry
Pharmacotherapy
Toronto Notes 2011
Table 22. Common Anxiolytics Class
Drug
Dose Range (mg/day)
t1/2 (hours) Appropriate Use
clonazepam (Rivotril®)
0.25-4
18-50
diazepam (Valium®)
2-40
30-100
chlordiazepoxide (Librium®) flurazepam (Dalmane®)
5-300 15-30
30-100 50-160
alprazolam (Xanax®) lorazepam (Ativan®)
0.25-4.0 0.5-6.0
6-20 10-20
oxazepam (Serax®) temazepam (Restoril®) triazolam (Halcion®)
10-120 7.5-30 0.125-0.5
8-12 8-20 1.5-5
Panic disorder, high dependency rate Sleep, generalized anxiety, akathisia, alcohol withdrawal, sublingual available for very rapid action Sleep, generalized anxiety, alcohol withdrawal Sleep Shortest t1/2, rapid sleep, but rebound insomnia
buspirone (Buspar®) zopiclone (Imovane®)
20-60 5-7.5
2-11 3.8-6.5
Generalized anxiety Sleep
Geriatric Benzodiazepines LOT Lorazepam Oxazepam Temazepam Also safe in liver disease because not metabolized by liver
Benzodiazepines Long-acting
Short-acting Benzos used for Alcohol Withdrawal Diazepam 20 mg PO/IV q1h prn Lorazepam 2-5 mg PO/IV/SL for patients with liver disease, chronic lung disease, or elderly
Azapirones
ECT in Society Prior to the 1940’s, ECT was performed without the use of muscle relaxants, resulting in seizures with full-scale convulsions and rare but serious complications such as vertebral and long-bone fractures. This practice may have led to negative societal perceptions of ECT, further perpetuated by barbaric depictions in popular culture. Despite ongoing stigmatization, ECT as it is practiced today is an effective and safe option for patients struggling with mental illness.
Efficacy of ECT in Depression: A Meta-Analytic Review J of ECT 2004; 20:13-20 Study: Meta-analysis of randomized and nonrandomized control trials. Patients: Individuals with unipolar and bipolar depression. Methods: MEDLINE search for relevant papers from 1966-2003. Main Outcomes: The Hamilton Depression Rating scale was used to determine response to treatment. Results: ECT was found to be superior to simulated ECT, placebo, TCAs, MAOIs, and anti-depressants in general. Summary: ECT is an efficacious treatment modality, particularly in severe and treatment-resistant depression.
Akathisia, generalized anxiety seizure prevention, panic disorder Generalized anxiety, seizure prevention, muscle relaxant, alcohol withdrawal Sleep, anxiety, alcohol withdrawal Sleep
Electroconvulsive Therapy • induction of a grand mal seizure using an electrical pulse through the brain while the patient is under general anesthesia and a muscle relaxant • unilateral vs. bilateral electrode placement • indications depression refractory to adequate pharmacological trial high suicide risk medical risk in addition to depression (dehydration, electrolytes, pregnancy) previous good response to ECT familial response to ECT elderly psychotic depression catatonic features marked vegetative features acute schizophrenia mania unresponsive to meds • side effects: risk of anesthesia, memory loss (may be retrograde and/or anterograde, tends to resolve by 6 to 9 months, permanent impairment controversial), headaches, myalgias • evidence that unilateral ECT causes less memory loss than bilateral but may not be as effective • contraindications: increased intracranial pressure
Experimental Therapies Deep Brain Stimulation (DBS) • constant electrical stimulation of neuroanatomical targets that have been identified in the biological model of depression • areas identified include the nucleus accumbens, internal capsule and subgenual cingulate cortex • parameters such as active electrode location, pulse width, frequency and voltage may be manipulated Transcranial Magnetic Stimulation (TMS) • non-invasive magnetic stimulation of superficial neurons in the frontal cortex (main target: dorsolateral prefrontal cortex) hypothesized to normalize cortical activity in depressed patients • meta-analyses show modest acute efficacy
Toronto Notes 2011
Canadian Legal Issues
Psychiatry PS51
Canadian Legal Issues Common Forms Table 23. Common Forms Under the Mental Health Act (in Ontario) Form
Who Signs
Form 1: Any MD Application by physician to hospitalize a patient for psychiatric assessment against his/her will to a schedule 1 facility (Form 42 given to patient)
Form 2: Order for hospitalization and medical examination against his/her will by Justice of the Peace Form 3: Certificate of involuntary admission to a schedule 1 facility (Form 30 given to patient, notice to rights advisor) Form 4: Certificate of renewal of involuntary admission to a schedule 1 facility (Form 30 given to patient, notice to rights advisor) Form 5: Change to informal/voluntary status
When
Expiration Date
Right of Patient to Review Board Hearing
Options Before Form Expires
Within 7 days after examination
72 hours after hospitalization Void if not implemented within 7 days
No
Form 3 or Voluntary admission (Form 5) or Send home ± Follow-up
Justice of the Peace
No statutory time restriction
7 days from when No completed Purpose of form is complete once patient brought to hospital
Form 1 or Send home ± Follow-up
Attending MD (different than MD who completed Form 1)
Before expiration 14 days of Form 1 Any time to change status of an informal patient
Attending MD Prior to expiration following patient of Form 3 on Form 3
Yes (within 48 hours)
Form 4 or Form 5
First: 1 month Yes Second: 2 months (within 48 hours) Third: 3 months (max)
Form 4 or Form 5
Attending MD Whenever deemed N/A following patient appropriate on Form 3/4
Form 33: Notice to patient that patient Attending MD is incompetent to consent to treatment of mental disorder and/or management of property
Whenever deemed N/A appropriate
N/A
N/A
N/A
N/A
Consent • see Ethical, Legal and Organizational Aspects of Medicine, ELOAM2, ELOAM8
Community Treatment Order (CTO) • known as “Brian’s Law,” Ontario passed legislature regarding CTOs on December 1, 2000 • similar CTOs have been implemented in Saskatchewan (1995), Manitoba (1997) and British Columbia (1999) • purpose: to provide a person who suffers from a serious mental disorder with a comprehensive plan of community-based treatment and supervision that is less restrictive than being detained in a psychiatric facility • intended for those who: as a result of their serious mental disorder, experience a pattern of admission to a psychiatric facility where their condition is usually stabilized after being released, these patients often lack supervision and stop treatment due to the destabilization of their condition, these patients usually require re-admission to hospital
Form 1: A Primer • Filled out when a patient is suspected of being an imminent harm to themselves (suicide) or others (homicide) or when they are incapable of self-care (e.g. not dressed for freezing weather) and are suffering from an apparent mental disorder. • Based on any combination of the physician’s own observations and facts communicated by others. • Box A or Box B completed. • Box A: Serious Harm Test • The Past/Present Test assesses current behaviours/threats/ attempts. • The Future Test assesses the likelihood of serious harm occurring as a result of the presenting mental disorder. • Box B: Patients with a known mental disorder, who are incapable of consenting to treatment (existing substitute decision-maker), have previously received treatment and improved, and are currently at risk of serious harm due to the same mental disorder.
PS52 Psychiatry
Canadian Legal Issues/References
Toronto Notes 2011
• criteria for a physician to issue a CTO patient with a prior history of hospitalization a community treatment plan for the person has been made examination by a physician within the previous 72 hours before entering into the CTO plan ability of the person subject to the CTO to comply with it consultation with a rights adviser and consent of the person and the person’s substitute decision maker, if any • CTOs are valid for six months unless they are renewed or terminated at an earlier date where the person fails to comply with the CTO when the person or his/her substitute decision-maker withdraws consent to the community treatment plan • CTO process is consent-based and all statutory protections governing informed consent apply • the rights of a person subject to a CTO include: the right to a review by the Consent and Capacity Board with appeal to the courts each time a CTO is issued or renewed a mandatory review by the Consent and Capacity Board every second time a CTO is renewed the right to request a re-examination by the issuing physician to determine if the CTO is still necessary for the person to live in the community the right to review findings of incapacity to consent to treatment provisions for rights advice
Duty to Inform/Warn • see Ethical, Legal, and Organizational Aspects of Medicine, ELOAM5
References American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders-Frouth Edition, Text Revision. Washington, DC, American Psychiatric Publishing Inc., 2000. Ball JR, Mitchell PB, Corry JC, Skilecorn A, Smith M, Malhi GS. A randomized controlled trial of cognitive therapy for bipolar disorder: focus on long-term change. J Clin Psychiatry. 67(2): 277-86, 2006. Clinical Practice Guidelines: Treatment of Schizophrenia in Can J Psychiatry vol 50, suppl 1 Nov 2005, p19-28. Conley RR, Kelly DL. Pharmacologic Treatment of Schizophrenia. First Edition. Professional Communications Inc., U.S.A., 2000. DeRubeis RJ et al. Cognitive therapy vs. medications in the treatment of moderate to severe depression. Arch Gen Psychiatry. 62(4): 409-16, 2005. Ditto KE. SSRI discontinuation syndrome. Awareness as an approach to prevention. Postgrad Med. 2003 Aug;114(2):79-84. Folstein MF, Folstein SE and McHugh PR (1975). Mini-Mental State: A practical method for grading the state of patients for the cllinician. Journal of Psychiatric Research. 12: 189-198. Gliatto MF, Rai AK. “Evaluation and Treatment of Patients With Suicidal Intention.” American Family Physician, Volume 59, Number 6, 1999 pp. 1500-14. Hembree EA, Foa, EB. “Posttraumatic Stress Disorder: Psychological Factors and Psychosocial Interventions.” Journal of Clinical Psychiatry, Volume 61, Supplement 7, 2000, pp. 33-9. Herrmann N. “Recommendations for the Management of Behavioural and Psychological Symptoms of Dementia.” Canadian Journal of Neurological Sciences, Volume 28, Supplement 1, 2001, pp. S96-107. Howland RH. Sequenced Treatment Alternatives to Relieve Depression (STAR*D). Part 2: Study outcomes. J Psychosoc Nurs Ment Health Serv. 2008;46(10):21-4. Kahan, M, Wilson, L. Managing Alcohol, Tobacco and other Drug Problems: A pocket guide for physicians and nurses. Toronto, ON. Centre for Addiction and Mental Health. 2002. Kapur S, Zipursky RB, Remington G. “Clinical and Theoretical Implications of 5-HT2 and D2 Receptor Occupancy of Clozapine, Risperidone, and Olanzapine in Schizophrenia.” American Journal of Psychiatry, Volume 156, Number 2, 1999, pp. 286-93. Koch, T. “A Tour of the Psychotropics.” 4th ed. Mental Health Service-St-Michael’s Hospital. Toronto, Canada. Krupnick JL et al. The role of the therapeutic alliance in psychotherapy and pharmacotherapy outcome: findings in the National Institute of Mental Health Treatment of Depression Collaborative Research Program. J Consult Clin Psychol. 64(3): 532-9, 1996. Linde K, Berner MM, Kriston L. St John’s wort for major depression. Cochrane Database of Systematic Reviews 2008, Issue 4. Lineham MM et al. Two-year randomized controlled trial and follow-up of dialectical behaviour therapy vs. therapy by experts for suicidal behaviours and boderline personality disorder. Arch Gen Psychiatry. 63(7): 757-66, 2006. Long. P. www.mentalhealth.com. 2003. Lopez M, Torpac MG. “The Texas Children’s Medication Algorithm Project: Report of the Texas Consensus Conference Panel on Medication Treatment of Childhood Attention-Deficit/Hyperactivity Disorder. Part I.” American Academy of Child and Adolescent Psychiatry, Volume 39, Number 7, 2000, pp. 908-19. March J, Silva S, Petrycki S, Curry J, Wells K, Fairbank J, Burns B, Domino M, McNulty S, Vitiello B, Severe J. Fluoxetine, cognitive-behavioral therapy, and their combination for adolescents with depression: Treatment for Adolescents With Depression Study (TADS) randomized controlled trial. JAMA 2004;292(7):807-20. Moore TH, Zammit S, Lingford-Hughes A, Barnes TR, Jones PB, Burke M, Lewis G. Cannabis use and risk of psychotic or affective mental health outcomes: a systematic review. Lancet 2007;370(9584):319-28. MTA Cooperative Group. A 14-month randomized clinical trial of treatment strategies for attention-deficit/hyperactivity disorder. 1999;56(12):1073-86. National Institute on Drug Abuse Research Report Series, Methamphetamine Abuse and Addiction, NIH Publication Number 02-4210 reprinted January 2002. Noble. Textbook of Primary Care Medicine, p.466, 470. 3rd edition. Pagnin D, de Queiroz V, Pini S, Cassano GB. Efficacy of ECT in depression: a meta-analytic review. J ECT 2004;20(1):13-20. Patterson CJ, Gauthier S, Bergman H, Cohen C, Freightner JW, Feldman H, Hogan D. “Canadian Consensus Conference on Dementia: A Physician’s Guide to Using the Recommendations.” CMAJ, Volume 160, Number 12, 1999, pp. 1738-42. Perkins DO, Gu H, Boteva K, Lieberman JA. Relationship between duration of untreated psychosis and outcome in first-episode schizophrenia: a critical review and meta-analysis. Am J Psychiatry 2005;162(10):1785-804. Pliszka SR, Greenhill LL, Crismon ML, Sedillo A, Carlson C, Conners CK, McCracken JT, Swanson JM, Hughes CW, Llana ME. Rees L, Lipsedge M, Ball C, ed. (1997). Textbook of Psychiatry. London: Oxford University Press, P. 109. Schneider LS, Dagerman KS, Insel P. Risk of death with atypical antipsychotic drug treatment for dementia: meta-analysis of randomized placebo-controlled trials. JAMA 2005;294(15):1934-43. Stahl SM. Psychopharmacology of Antidepressants. London: Martin Dunitz, 1998. Stahl SM. Psychopharmacology of Antipsychotics. London: Martin Dunitz, 1999. Szewczyk M. “Women’s Health: Depression and Related Disorders.” Primary Care, Volume 24, Number 1, 1997, pp. 83-101. Tasman. Psychiatry 1st ed. 1997. p.1208. Troiden, 1989, Journal of Homosexuality. 17: 253-267. Warneke L. “Breaking the urges of obsessive-compulsive disorder.” Canadian Journal of Diagnosis, December 1996, h. page. Weller EB, Weller RA, Fristad MA. “Bipolar Disorder in Children: Misdiagnosis, Underdiagnosis, and Future Directions.” Journal of the American Academy of Child and Adolescent Psychiatry, Volume 34, Number 6, 1995, pp. 709-714. Whittington CJ, Kendall T, Fonagy P, Cottrell D, Cotgrove A, Boddington E. Selective serotonin reuptake inhibitors in childhood depression: systematic review of published versus unpublished data. Lancet 2004;363(9418):1341-5. Zimmerman, M. Interview Guide for Evaluating DSM-IV Psychiatric Disorders and the Mental Status Examination. East Greenwich, RI. Psych Products Press. 1994.
R
Respirology Helen Cheung, Yehoshua Gleicher and Lorraine Jensen, chapter editors Doreen Ezeife and Nigel Tan, associate editors Steven Wong, EBM editor Dr. Matthew Binnie, staff editor Approach to the Respiratory Patient. . . . . . . . . 2 Basic Anatomy Review Differential Diagnoses of Common Presentations Pulmonary Function Tests (PFTs) Chest X-Rays Arterial Blood Gases Diseases of Airway Obstruction . . . . . . . . . . . . 7 Asthma Chronic Obstructive Pulmonary Disease (COPD) Bronchiectasis Cystic Fibrosis (CF) Interstitial Lung Disease (ILD) . . . . . . . . . . . . . 12 Unknown Etiologic Agents Idiopathic Pulmonary Fibrosis (IPF) Sarcoidosis Known Etiologic Agents Hypersensitivity Pneumonitis Pneumoconioses ILD Associated with Drugs or Treatments
Introduction to Intensive Care. . . . . . . . . . . . . 33 ICU Basics ICU Approach to Management Organ Failure Shock Infection/Sepsis Common Drug Overdoses Common Medications. . . . . . . . . . . . . . . . . . . . 37 Landmark Respirology Trials . . . . . . . . . . . . . . 38 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
Pulmonary Vascular Disease. . . . . . . . . . . . . . . 16 Pulmonary Hypertension Pulmonary Embolism (PE) Pulmonary Vasculitis Diseases of the Mediastinum and Pleura . . . . 21 Mediastinal Masses Mediastinitis Pleural Effusions Complicated Effusion Empyema Pneumothorax Asbestos-Related Pleural Disease and Mesothelioma Pulmonary Edema Respiratory Failure . . . . . . . . . . . . . . . . . . . . . . 25 Hypoxemic Respiratory Failure Hypercapnic Respiratory Failure Acute Respiratory Distress Syndrome (ARDS) Mechanical Ventilation Neoplasms. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 Approach to the Solitary Pulmonary Nodule Benign Lung Tumours Malignant Lung Tumours Sleep-Related Breathing Disorders . . . . . . . . . 32 Sleep Apnea
Toronto Notes 2011
Respirology R1
R2 Respirology
Approach to the Respiratory Patient
Toronto Notes 2011
Respiration Pattern
Approach to the Respiratory Patient
Normal (inspiration and expiration)
Basic Anatomy Review
Obstructive (prolonged expiration) • Asthma, COPD
Right upper lobe
B1
1
B2
B1+2
B3 B6
Right middle lobe Kussmaul’s Breathing (fast and deep) • Metabolic acidosis • Exercise • Anxiety
B4 B5
2 Right and left main bronchi
B8
Right lower lobe Biot’s/Ataxic (irregular with long apneic periods) • Drug-induced respiratory depression • Increased ICP • Brain damage, especially medullary
B4 B5
B6
3
B7
Left upper lobe
B3
B7+8 B9
B10
B9
Left lower lobe Lingula
© Hyun Joo Lee 2005
Bradypnea (slow respiratory rate) • Drug-induced respiratory depression • Diabetic coma (nonketotic) • Increased ICP
Figure 1. Lung Lobes and Bronchi Cheyne-Stokes Breathing (changing rates and depths with apneic periods) • Drug-induced respiratory depression • Brain damage (especially cerebral) • CHF • Uremia
Differential Diagnoses of Common Presentations Table 1. Differential Diagnosis of Dyspnea
Apneustic (prolonged inspiratory pause) • Pontine lesion
Figure 2. Respiration Patterns in Normal and Disease States
Kussmaul’s Breathing Cheyne-Stokes Breathing
Table 2. Differential Diagnosis of Chest Pain
Acute dyspnea (min-hrs) Nonpleuritic Pleuritic Cardiac causes Pulmonary Pulmonary Ischemic heart disease Pneumonia Pneumonia CHF exacerbation PE PE LVF with pulmonary edema Neoplastic Pneumothorax Cardiac tamponade Cardiac Hemothorax Pulmonary causes MI Neoplasm Upper airway obstruction (anaphylaxis, foreign body) Myocarditis/pericarditis TB Airway disease (asthma, COPD exacerbation, bronchitis) Esophageal Empyema Parenchymal lung disease (ARDS, pneumonia) GERD Cardiac Pulmonary vascular disease (PE, vasculitis) Spasm Pericarditis Pleural disease (pneumothorax, tension pneumothorax) Esophagitis Dressler’s syndrome Respiratory control (metabolic acidosis, ASA, toxicity) Ulceration GI Psychiatric Achalasia Subphrenic abscess Anxiety/psychosomatic Neoplasm Pancreatitis Esophageal rupture MSK Chronic dyspnea (wks-mos) Mediastinal Costochondritis Cardiac causes Lymphoma Fractured rib Valvular heart disease Thymoma Myositis Decreased CO Subdiaphragmatic Herpes zoster Respiratory causes PUD Parenchymal lung disease (interstitial disease) Gastritis Pulmonary vascular disease (pulmonary HTN, vasculitis) Biliary colic Pleural disease (effusion) Pancreatic Gas exchange (infection, emphysema, fibrosis, etc.) Vascular Airway disease – asthma, COPD Dissecting aortic aneurysm Hematologic causes MSK Severe anemia Costochondritis Neuromuscular and chest wall disorders Skin Deconditioning, obesity, pregnancy Breast Ribs
Toronto Notes 2011
Approach to the Respiratory Patient
Respirology R3
Table 3. Differential Diagnosis of Hemoptysis
Table 4. Differential Diagnosis of Cough
Airway Disease Acute or chronic bronchitis Bronchiectasis Bronchogenic CA Bronchial carcinoid tumour Parenchymal Disease Pneumonia TB Lung abscess Miscellaneous: Goodpasture’s syndrome Idiopathic pulmonary hemosiderosis Vascular Disease PE Elevated pulmonary venous pressure: LVF Mitral stenosis Vascular malformation Miscellaneous Impaired coagulation Pulmonary endometriosis
Airway Irritants Inhaled smoke, dusts, fumes Aspiration Gastric contents (GERD) Oral secretions Foreign body Postnasal drip Airway Disease URTI including postnasal drip and sinusitis Acute or chronic bronchitis Bronchiectasis Neoplasm External compression by node or mass lesion Asthma COPD Parenchymal Disease Pneumonia Lung abscess Interstitial lung disease CHF Drug-induced (e.g. ACE inhibitor)
Most Common Causes of Chronic Cough in Healthy-appearing Patient (cough >3 months with normal CXR) • GERD • Asthma • Post-nasal drip • Post-viral • ACE inhibitor
Hemoptysis • Most common cause is bronchitis • 90% of massive hemoptysis is from the bronchial arteries • Considered “massive” if >600 cc/24 hours
Adapted from Principles of Pulmonary Medicine, 5th edition, SE Weinberger, Copyright (2008), with permission from Elsevier.
Table 5. Differential Diagnosis of Clubbing Pulmonary Cystic fbrosis Pulmonary fibrosis Chronic pus in the lung (bronchiectasis, abscess, infections, etc.) Lung CA (primary or mets) A-V fistula Solitary fibrous tumour of pleura
Gastrointestinal IBD (UC, CD) Chronic infections Laxative abuse Polyposis Malignant tumours Cirrhosis Hepatocellular carcinoma Cardiac Cyanotic congenital heart disease Infective endocarditis
Mediastinal Esophageal CA Thymoma Other Graves Disease Thalassemia Other malignancies Primary hypertrophic osteoarthropathy
Normal
Clubbed
ÀABC 192º
IPD>DPD
DPD>IPD © Sherry H. Lai 2006
Figure 3. Three Signs of Clubbing
1. Profile Angle (ABC >176º) 2. Hyponychial Angle (ABD >192º) 3. Phalangeal Depth Ratio (DPD:IPD >1)
Adapted from JAMA 2001; 286(3):341-7.
Clubbing is not seen in COPD – if present, think malignancy.
Pulmonary Function Tests (PFTs) • useful in differentiating the pattern of lung disease (obstructive vs. restrictive) (Table 6) • assess lung volumes, flow rates, and diffusion capacity (see Figures 4a and 4b below) • note: normal values for FEV1 are approximately ±20% of the predicted values (for age, sex and height); race may affect predicted values
FEV1
IC VC
VT 1 SEC
FRC
TLC
ERV
RV
TIME
Figure 4A. Subcompartments of Lung Volumes
FLOW RATE (L/Sec)
VOLUME
8 6 4
Normal Restrictive
Obstructive
2 0
FEV 1 VC 8 6
1 second time marker 4
2
LUNG VOLUME (L)
0
Figure 4B. Expiratory Flow Volume Curves
Adapted from Principles of Pulmonary Medicine, 5th edition, SE Weinberger, Copyright (2008), with permission from Elsevier.
Obstructive Lung Disease • characterized by decreased flow rates (most marked during expiration), air trapping (increased RV/TLC), and hyperinflation (increased FRC, TLC) • differential diagnosis includes asthma, chronic obstructive pulmonary disease (COPD), cystic fibrosis (CF), bronchiolitis and bronchiectasis Restrictive Lung Disease • characterized by decreased lung compliance and lung volumes • differential diagnosis includes interstitial lung disease, pleural disease (e.g. pulmonary fibrosis), neuromuscular disease, chest wall disease
Lung Volumes FEV1 – Forced Expiratory Volume in one second MMFR – Maximal Mid-Expiratory Flow Rate FVC – Forced Vital Capacity FEF – Forced Expiratory Flow Rate FRC – Functional Residual Capacity TLC – Total Lung Capacity VC – Vital Capacity RV – Residual Volume Dco – Diffusion Capacity of Carbon Monoxide
R4 Respirology
Approach to the Respiratory Patient
Toronto Notes 2011
Table 6. Comparison of Lung Flow and Volume Parameters in Obstructive vs. Restrictive Lung Disease Diffusion Capacity of Carbon Monoxide (DCO) DCO decreases with: 1. Decreased surface area (e.g. emphysema) 2. Decreased hemoglobin 3. Interstitial lung disease 4. Pulmonary vascular disease
Obstructive
Restrictive
Flow Rates
FEV1/FVC
or N
Lung Volumes
TLC RV RV/TLC
or N
N
Diffusion Capacity
DCO
or N
or N
DCO increases with: 1. Asthma 2. Pulmonary hemorrhage 3. Polycythemia 4. Increased pulmonary blood volume
Pulmonary Function Tests (PFTs)
Reduced FEV1 (80% predicted Non Obstructive Defect (Restrictive)
FEV1/FVC 12% or positive metacholine challenge test
DCO
Low
FEV1/FVC normal
Normal
ANEMIA, PULMONARY VASCULAR DISEASE
No change in FEV1 Flow volume loop, Lung volumes, DCO
ASTHMA
INTERSTITAL LUNG DISEASE
Decreased TLC and FRC + increased RV
Decreased TLC and FRC + normal RV
NEUROMUSCULAR DISEASE
CHEST WALL DISEASE
Normal TLC and DCO
High TLC + low DCO
CHRONIC BRONCHITIS
EMPHYSEMA
Figure 5. Interpreting PFTs
Chest X-Rays • see also Diagnostic Medical Imaging, DM4 Table 7. CXR Patterns and Differential Diagnosis Pattern
Signs
Common DDx
Consolidation (“Airspace disease”)
Air bronchogram Silhouette sign Less visible blood vessels
Acute: water (pulmonary edema), pus (pneumonia), blood (hemorrhage) Chronic: neoplasm (lymphoma), inflammatory (eosinophilic pneumonia), granuloma (TB, fungal, sarcoid)
Reticular (“Interstitial disease”)
Increased pulmonary markings Honeycombing (IPF)
Interstitial lung disease (IPF, CVD, asbestos, drugs)
Nodular
Cavitary vs. non-cavitary
Cavitary: neoplasm (primary vs. metastatic lung cancer), infectious (TB, fungal), inflammatory (Wegener’s, RA) Non-cavitary: above + sarcoid (in HIV), Kaposi’s sarcoma
Toronto Notes 2011
Approach to the Respiratory Patient
Respirology R5
Arterial Blood Gases • provides information on acid-base and oxygenation status • see also Nephrology, NP16 Approach to Acid-Base Status 1. Is the pH acidemic (pH 7.45), or normal (pH 7.35-7.45)? 2. What is the primary disturbance? metabolic: change in HCO3 and pH in same direction respiratory: change in HCO3 and pH in opposite direction 3. Has there been appropriate compensation? (Table 8) metabolic compensation occurs over 2-3 days reflecting altered renal HCO3 production and excretion respiratory compensation through ventilation control of PaCO2 occurs immediately inadequate compensation may indicate a second acid-base disorder Table 8. Expected Compensation for Specific Acid-Base Disorders Disturbance
PaCO2 (mmHg)
HCO3 (mmHg)
Respiratory Acidosis Acute Chronic
10 10
1 3
Respiratory Alkalosis Acute Chronic
10 10
2 5
Metabolic Acidosis
1
1
Metabolic Alkalosis
5-7
10
4. If there is metabolic acidosis, what is the anion gap and osmolar gap? anion gap = [Na]–([Cl]+[HCO3]); normal ≤10-15 mmol/L osmolar gap = measured osmolarity – calculated osmolarity = measured – (2[Na] + glucose + urea); normal ≤10 5. If anion gap is increased, is the change in bicarbonate the same as the change in anion gap? if not, consider a mixed metabolic picture DIFFERENTIAL DIAGNOSIS OF RESPIRATORY ACIDOSIS characterized by increased PaCO2 secondary to hypoventilation • respiratory centre depression (decreased RR) drugs (anesthesia, sedatives, narcotics) trauma increased ICP encephalitis stroke central apnea supplemental O2 in chronic CO2 retainers (i.e. COPD) • neuromuscular disorders (decrease TV) myasthenia gravis Guillain-Barré syndrome poliomyelitis muscular dystrophies ALS myopathies chest wall disease (obesity, kyphoscoliosis) • airway obstruction (asthma, COPD) • parenchymal disease COPD pulmonary edema pneumothorax pneumonia interstitial lung disease (late stage) acute respiratory distress syndrome (ARDS) • mechanical hypoventilation (inadequate mechanical ventilation) DIFFERENTIAL DIAGNOSIS OF RESPIRATORY ALKALOSIS characterized by decreased PaCO2 secondary to hyperventilation • hypoxemia pulmonary disease (pneumonia, edema, PE, interstitial fibrosis) severe anemia heart failure high altitude
Note: Mixed acid-base disturbances can still have a “normal pH”
Ventilation Failure Think “Can’t Breathe” vs. “Won’t Breathe” (increased PaCO2) Won’t Breathe • Respiratory centre depression • Hypothyroidism • Sleep apnea Can’t Breathe • Neuromuscular disorders • Airway obstruction • Parenchymal disease
Anion Gap Metabolic Acidosis KARMEL Ketoacidosis ASA Renal failure (uremia) Methanol Ethylene glycol Lactic acidosis
Acidosis Hyperkalemia Alkalosis Hypokalemia
R6 Respirology
Approach to the Respiratory Patient
Toronto Notes 2011
• respiratory centre stimulation CNS disorders hepatic failure Gram-negative sepsis drugs (ASA, progesterone, theophylline, catecholamines, psychotropics) pregnancy anxiety pain • mechanical hyperventilation (excessive mechanical ventilation) • see Nephrology, NP16 for differential diagnosis of metabolic acidosis and alkalosis
At Sea Level A-aDO2 Gradient on Room Air A-aDO2 = {150 - 1.25 (PaCO2)} – PaO2
100
CO pCO2 temperature pH
60
pCO2 temperature pH 2,3,-DPG
PaO2 15 mmHg) = lung disease • Decreased DCO
20 0
0
20
40
60
pO2 (mmHg)
80
100
Increased PaCO2 • Hypoventilation
Give 100% O2
Figure 6. Oxygen-Hb Dissociation Curve
PaO2 improves • V/Q mismatch • Airway disease (asthma, COPD) • Interstitial lung disease • Alveolar disease • Pulmonary vascular disease
Lung normal A-a gradient (20% with methacholine challenge (to confirm diagnosis when bronchodilator response not significant) peak flow
Red Flag A good predictor of a potential life-threatening attack is excessive consumption of short-acting beta2-agonists.
Central cyanosis is not detectable until the SaO2 is beta2 agonists) Experimental (surgery, roflumilast) Rehabilitation
Diseases of Airway Obstruction
Toronto Notes 2011
Treatment • environmental control: avoid triggers • patient education: features of the disease, goals of treatment, self-monitoring • pharmacological therapy symptomatic relief in acute episodes: short-acting beta2-agonist, anticholinergic bronchodilators, oral steroids, addition of a long acting beta2-agonist long-term prevention of acute episodes: inhaled/oral corticosteroids, anti-allergic agent, long-acting beta2-agonist, methylxanthine, leukotriene receptor antagonists (LTRA) Guidelines for Asthma Management
led nha
oc
id ero ost µg c i t or 00
gluc
(d
100
5
I 0
e) dos aily 0 µg
g
0µ
200
+ Prednisone
R8 Respirology
Additional therapy
µg
(e.g. CABA)
Short-acting β2-agonist on demand Environmental control and education Severity of asthma Very mild
Mild
Symptom characteristics Subclinical
Intermittent
Moderate Moderately Severe Severe Persistent
Figure 9. Guidelines for Asthma Management Adapted from CMAJ 1999; 161(11 Suppl):S1-61
Emergency Management of Asthma (see also Emergency Medicine, ER31) 1. inhaled beta2-agonist first line (MDI route and spacer device recommended) 2. add anticholinergic therapy 3. ketamine and succinylcholine for rapid sequence intubation in life-threatening cases 4. SC/IV adrenaline, IV salbutamol if unresponsive 5. all patients admitted to ER for asthma exacerbations should be considered for corticosteroid therapy at discharge
Chronic Obstructive Pulmonary Disease (COPD) Definition • characterized by progressive development of irreversible airway obstruction • 2 subtypes (chronic bronchitis, emphysema): usually coexist to variable degrees in most patients • course: gradual decrease in FEV1 over time with episodes of acute exacerbations Table 12. Clinical and Pathologic features of COPD Chronic Bronchitis
Emphysema
Defined clinically as: Productive cough on most days for at least 3 consecutive months in 2 successive years Obstruction is due to narrowing of the airway lumen by mucosal thickening and excess mucus
Defined pathologically as: Dilation and destruction of air spaces distal to the terminal bronchiole without obvious fibrosis Decreased elastic recoil of lung parenchyma causes decreased expiratory driving pressure, airway collapse, and air trapping 2 types: 1) Centriacinar (respiratory bronchioles predominantly affected) • Typical form seen in smokers, primarily affects upper lung zones 2) Panacinar (respiratory bronchioles, alveolar ducts, and alveolar sacs affected) • Responsible for less than 1% of emphysema cases, (alpha-1-antitrypsin deficiency), primarily affects lower lobes
Toronto Notes 2011
Diseases of Airway Obstruction
Risk Factors • smoking is the most important risk factor (likelihood ratio of 8.3) • other risk factors include: environmental factors: air pollution, occupational exposure treatable factors: alpha-1-antitrypsin deficiency, bronchial hyperactivity demographic factors: age, family history, male sex, history of childhood respiratory infections, and low socioeconomic status Signs and Symptoms Table 13. Clinical Presentation and Investigations for Chronic Bronchitis and Emphysema Symptoms
Signs
Investigations
Respirology R9 The Accuracy of Patient History, Wheezing, and Laryngeal Measurements in Diagnosing Obstructive Airway Disease JAMA 2000; 238(14):1853-57. Study: Multi-center case-control study. Population: 309 patients with known chronic obstructive pulmonary disease (COPD); suspected COPD; and controls. Intervention: Various aspects of history and physical exam versus spirometry (FEV1 and FEV1/ FVC 15 hrs/day indications: PaO2 lower lobes Coal: upper > lower lobes
Remember to involve occupational health at place of work for data collection and treatment plan. Also counsel re: worker’s insurance as per jurisdiction (e.g. WSIB in Ontario).
R16 Respirology
Interstitial Lung Disease (ILD)/Pulmonary Vascular Disease
Toronto Notes 2011
Silicosis • at risk population: sandblasters, rock miners, stone cutters, quarry and highway workers • etiology: generally requires >20 years exposure; may develop with much shorter but heavier exposure • signs and symptoms: dyspnea, cough and wheezing • investigations: CXR upper > lower lobe early: nodular disease (simple pneumoconiosis), lung function usually normal late: nodules coalesce into masses (progressive massive fibrosis) • when nodules become larger and coalesce into masses, disease has changed from simple silicosis to complicated silicosis (progressive massive fibrosis) • possible hilar lymph node enlargement (frequently calcified), especially “egg shell” calcification • complications: mycobacterial infection (e.g. TB) • treatment: prevention, removal from exposure, treat associated TB if present, supportive measures (oxygen, bronchodilators), lung transplant Coal Worker’s Pneumoconiosis (CWP) • at risk population: coal workers, graphite workers • etiology: coal and silica, coal is less fibrogenic than silica • pathologic hallmark is coal macule: coal dust surrounded by minimal tissue reaction and focal emphysema found around respiratory bronchioles • simple CWP no signs or symptoms CXR: multiple nodular opacities, mostly upper lobe respiratory function well preserved • complicated CWP (also known as progressive massive fibrosis) dyspnea CXR: opacities larger and coalesce • course: few patients progress to complicated CWP • Caplan’s syndrome: rheumatoid arthritis and CWP present as larger nodules • treatment: minimize future exposure, cardiopulmonary rehabilitation, follow periodically
ILD Associated with Drugs or Treatments Drug-Induced • antineoplastic agents: bleomycin, mitomycin, busulfan, cyclophosphamide, methotrexate, chlorambucil, BCNU (carmustine) • antibiotics: nitrofurantoin, penicillin, sulfonamide • cardiovascular drugs: amiodarone, tocainide • anti-inflammatory agents • gold salts • illicit drugs (heroin, methadone) Radiation-Induced • early pneumonitis: approximately 6 weeks post-exposure • late fibrosis: 6-12 months post-exposure • infiltration conforms to the shape and field of the irradiation
Pulmonary Vascular Disease Pulmonary Hypertension Definition • mean pulmonary arterial pressure >25 mmHg at rest and >30 mmHg with exercise, or a systolic pulmonary artery pressure of >40 mmHg at rest • in the past, pulmonary hypertension was classified as primary or secondary pulmonary hypertension, but this classification was modified to a more clinically useful, treatment based classification (Table 17)
Toronto Notes 2011
Pulmonary Vascular Disease
Respirology R17
Table 17. Diagnostic Classification of Pulmonary Hypertension (WHO 1998) Classification
Causes
Pulmonary arterial hypertension
Primary pulmonary hypertension – sporadic vs. familial related to: Collagen vascular disease (scleroderma, SLE, RA) Congenital systemic-to-pulmonary shunts (Eisenmenger syndrome) Portopulmonary hypertension HIV infection Drugs and toxins (e.g. anorexigens)
Pulmonary venous hypertension
Left-sided atrial or ventricular heart disease (e.g. LV dysfunction) Left-sided valvular heart disease (e.g. aortic stenosis, mitral stenosis) Pulmonary veno-occlusive disease Extrinsic compression of central pulmonary veins (tumour, adenopathy, fibrosing mediastinitis)
Associated with disorders of the respiratory system and/or hypoxemia
Parenchymal lung disease (COPD, interstitial fibrosis, cystic fibrosis) Chronic alveolar hypoxia (chronic high altitude, alveolar hypoventilation disorders, sleep disordered breathing)
Due to chronic thrombotic and/or embolic disease
Thromboembolic obstruction of proximal pulmonary arteries Obstruction of distal pulmonary arteries – PE (thrombus, foreign material, tumour, schistosomiasis, in situ thrombosis, sickle cell disease)
Due to disorders directly affecting the pulmonary vasculature
Inflammatory (sarcoidosis, schistosomiasis) Pulmonary capillary hemangiomatosis
Mechanisms of Pulmonary Hypertension • the approach is simplified as some causes could fall under more than one mechanism • hypoxic vasoconstriction chronic hypoxia causes pulmonary vasoconstriction by a variety of actions on the pulmonary artery endothelium and smooth muscle cells, such as: down regulation of endothelial nitric oxide synthase and alteration of voltage gated potassium channels leading to vasoconstriction causes: COPD, chronic alveolar hypoxia • decreased area of pulmonary vascular bed leads to a rise in resting pulmonary arterial pressure causes: collagen vascular disease, HIV infection, drugs and toxins, thrombotic or embolic disease, inflammatory, pulmonary capillary hemangiomatosis, interstitial fibrosis, cystic fibrosis • volume and pressure overload significant hypertension only occurs with excessive volume overload, since pulmonary artery pressure will not rise in otherwise normal lung until pulmonary blood flow exceeds 2.5 times the basal rate causes: congenital systemic to pulmonary shunts (e.g. VSD, ASD, PDA), portopulmonary hypertension, left-sided heart conditions, pulmonary veno-occlusive disease, extrinsic compression of central pulmonary veins IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION [AKA PRIMARY PULMONARY HYPERTENSION (PPH)] Definition • pulmonary hypertension in the absence of a demonstrable cause (exclude left-sided cardiac valvular disease, myocardial disease, congenital heart disease, and any clinically significant parenchymal lung disease, systemic connective-tissue, or chronic thromboembolic disease) Epidemiology • disease of young women (20-40 years); mean age of diagnosis is 36 years • most cases are sporadic; familial predisposition in 10% of cases, linked to mutations in BMPR2 • may be associated with the use of anorexic drugs (e.g. Aminorex®, Fenfluramine®), also amphetamines and cocaine Signs and Symptoms • exertional dyspnea, fatigue, syncope, exertional chest pain, Raynaud’s phenomenon • see Table 18 Prognosis • 2-3 year mean survival from time of diagnosis • survival decreases to approximately 1 year if severe pulmonary HTN or right-heart failure
Guildelines for Vasodilator Response in Pulmonary Arterial HTN 1. Evidence for the use of CCBs after a positive vasodilator challenge is limited to patients with IPAH. 2. The precise definition about what constitutes a “positive” result is controversial. The latest consensus according to the European Society of Cardiology was that a positive result constitutes a fall of mean PAP pressure of 10 mmHg to less than or equal to 40 WITHOUT a change in cardiac output. 3. Best agents to use: NO or epoprostenol (prostacyclin) analogue (best safety profile). 4. Those who have a “significant” response (as determined by the criteria in #2) should be treated cautiously with a CCB (nifedipine, diltiazem, amlodipine are good choices, NOT verapamil). Evidence suggests these patients will have improved survivals. Reference: Badesch et al. Medical Therapy For Pulmonary Arterial Hypertension. ACCP EvidenceBased Clinical Practice Guidelines. Chest 126, Supplement, July, 2004.
R18 Respirology
Pulmonary Vascular Disease
Toronto Notes 2011
Table 18. Signs and Symptoms of Pulmonary Hypertension Symptoms
Signs
Dyspnea Fatigue Substernal chest pain Syncope Symptoms of underlying disease
Loud, palpable P2 RV heave Right-sided S4 (due to RVH) Systolic murmur (TR) If RV failure: right sided S3, increased JVP, positive HJR, peripheral edema, TR
Investigations • CXR: enlarged central pulmonary arteries, cardiac changes due to RV enlargement (filling of retrosternal air space) • ECG RVH/right-sided strain and RA enlargement, rightward axis deviation R/S ratio >1 in V1 increased P wave amplitude in lead II incomplete or complete R bundle branch block (RVSP) • 2-D echo doppler assessment of right ventricular systolic pressure • cardiac catheterization: direct measurement of pulmonary artery pressures • PFTs to rule out lung disease – DLco usually reduced • spiral CT to assess lung parenchyma and possible PE • V/Q scan ± pulmonary angiogram to rule out thromboembolic disease • serology: ANA positive in 30% of patients with primary pulmonary hypertension Treatment • for primary pulmonary hypertension anticoagulation in patients at increased risk for intrapulmonary thrombosis and thromboembolism (anticoagulation of choice is warfarin, target INR approximately 2.0) calcium channel blockers: nifedipine, diltiazem, NOT verapamil vasodilators: oral (sildenafil, bosentan), parenteral (epoprostenol, treprostanil) lung transplantation • for other forms of pulmonary hypertension continuous oxygen therapy for patients who are hypoxic treat underlying condition before irreversible damage occurs phlebotomy for polycythemia (rarely required) treatment of exacerbating factors: smoking, infection, sleep apnea epoprostenol – beneficial in cardiomyopathy, and NYHA class III-IV symptoms endothelin receptor antagonists (bosentan, sitaxentan) phosphodiesterase inhibitors (sildenafil)
Pulmonary Embolism (PE) Definition • lodging of a blood clot in the pulmonary arterial tree with subsequent increase in pulmonary vascular resistance and possible obstruction of blood supply to the lung parenchyma
Virchow’s Triad • Venous stasis • Endothelial cell damage • Hypercoagulable states
Clinical Prediction Rule for Pulmonary Embolism Thrombosis and Hemostasis 2000; 83(3):416-20 Wells Criteria Risk Factors
Points
Clinical signs of DVT No more likely alternative diagnosis (using H&P, CXR, ECG) Immobilization or surgery in the previous 4 weeks Previous PE/DVT Heart rate >100 beats/min Hemoptysis Malignancy
3.0 3.0 1.5 1.5 1.5 1.0 1.0
Clinical probability Low 3% Intermediate 28% High 78% Simplified Wells: >4 likely; ≤4 unlikely for PE JAMA 2006
Etiology and Pathophysiology • one of the most common causes of preventable death in the hospital • proximal leg thrombi (popliteal, femoral or iliac veins) are the source of most clinically recognized pulmonary emboli • thrombi often start in calf, but must propagate into proximal veins to create a sufficiently large thrombus for a clinically significant PE • fewer than 30% of patients have clinical evidence of DVT (e.g. leg swelling, pain or tenderness) • always suspect PE if patient suddenly collapses 1-2 weeks after surgery Risk Factors (Virchow’s Triad) • stasis immobilization: paralysis, stroke, bed rest, prolonged sitting during travel, immobilization of an extremity after fracture obesity, CHF chronic venous insufficiency • endothelial cell damage post-operative injury, trauma • hypercoagulable states underlying CA (particularly adenocarcinoma) cancer treatment (chemotherapy, hormonal) exogenous estrogen administration (OCP, HRT) pregnancy, post-partum prior history of DVT/PE, family history nephrotic syndrome
Toronto Notes 2011
Pulmonary Vascular Disease
coagulopathies: Factor V Leiden, Prothrombin 20210A variant, inherited deficiencies of antithrombin/protein C/protein S, antiphospholipid antibody, hyperhomocysteinemia, increased Factor VIII levels, and myeloproliferative disease • increasing age Investigations (if highly suspicious, go straight to spiral CT) • see Emergency Medicine, Figure 13, ER35 • D-dimer (products of thrombotic/fibrinolytic process) ELISA better than latex agglutination D-dimer results alone do not rule in or out DVT/PE consider only in out-patients with low pretest probability need to use in conjunction with leg Dopplers • spiral CT scan with contrast is both sensitive and specific for PE diagnosis and management uncertain for small filling defects spiral CT may identify an alternative diagnosis if PE is not present CT scanning of the proximal leg and pelvic veins can be done at the same time and may be helpful • venous duplex ultrasound or doppler with leg symptoms positive test rules in proximal DVT negative test rules out proximal DVT without leg symptoms positive test rules in proximal DVT negative test does not rule out a DVT – patient may have non-occlusive or calf DVT • ECG findings not sensitive or specific sinus tachycardia most common; may see non-specific ST segment and T wave changes RV strain, RAD, RBBB, S1-Q3-T3 with massive embolization • CXR frequently normal; no specific features atelectasis (subsegmental), elevation of a hemidiaphragm pleural effusion – usually small Hampton’s hump – cone-shaped area of peripheral opacification representing infarction Westermark’s sign – dilated proximal pulmonary artery with distal oligemia/decreased vascular markings (difficult to assess without prior films) dilatation of proximal PA – rare • V/Q scan (very sensitive but low specificity) order scan if CXR normal, no COPD contraindication to CT (contrast allergy, renal dysfunction) avoid V/Q scan if CXR abnormal or COPD inpatient suspect massive PE results normal – excludes the diagnosis of PE high probability – most likely means PE present, unless pre-test probability is low 60% of V/Q scans are nondiagnostic • echocardiogram little diagnostic value increased RVSP, RV hypokinesis, seen in massive PE • ABG of NO diagnostic use in PE (insensitive and nonspecific) respiratory alkalosis (due to hyperventilation) Treatment • admit for observation (patients with DVT only are often sent home on LMWH) • oxygen: provide supplemental O2 if hypoxemic or short of breath • pain relief: analgesics if chest pain – narcotics or NSAIDs • acute anticoagulation: therapeutic-dose SC LMWH or IV heparin – start ASAP anticoagulation stops clot propagation, prevents new clots and allows endogenous fibrinolytic system to dissolve existing thromboemboli over months get baseline CBC, INR, aPTT ± renal function ± liver function for SC LMWH: dalteparin 200 U/kg once daily or enoxaparin 1 mg/kg bid – no lab monitoring – avoid or reduce dose in renal dysfunction for IV heparin: bolus of 75 U/kg (usually 5,000 U) followed by infusion starting at 20 U/kg/hr – aim for aPTT 2-3 times control
Respirology R19
Evaluation of a Suspected Pulmonary Embolism Low clinical probability of embolism: D-dimer (+ve) CT scan (+ve) ruled in (–ve) decreased (–ve) decreased ruled out ruled out Intermediate or high probability: CT scan (–ve) ruled out (+ve) decreased ruled in Notes: • Use D-dimers only if low clinical probability, otherwise, go straight to spiral CT • If using V/Q scans (CT contrast allergy or renal failure): • Negative V/Q scan rules out the diagnosis • High probability V/Q scan only rules in the diagnosis if have high clinical suspicion • Inconclusive V/Q scan requires leg ultrasound to look for DVT or spiral CT
Classic ECG finding of PE is S1-Q3-T3 (inverted T3), but most commonly see only sinus tachycardia.
Workup for Idiopathic VTE Throbophilia Workup: recurrent or idiopathic DVT/PE; age 50 ml leads to blunting of posterior costophrenic angle PA: blunting of lateral costophrenic angle dense opacification of lung fields with concave meniscus decubitus: fluid will shift unless it is loculated supine: fluid will appear as general haziness • thoracentesis: indicated if pleural effusion is a new finding risk of re-expansion pulmonary edema if >1.5 L of fluid is removed inspect for colour, character, and odour of fluid analyze fluid (see Tables 21 and 22) • pleural biopsy: indicated if suspect TB, mesothelioma, or other malignancy (and if cytology negative) • ± U/S: detects small effusions and can guide thoracentesis • treatment depends on cause, ± drainage if symptomatic • CT can be helpful in differentiating parenchymal from pleural abnormalities Table 22. Analysis of Pleural Effusion Measure
Purpose
Protein, LDH
Transudate vs. exudate (see Table 21)
Gram stain, Ziehl-Nielsen stain (TB), culture
Looking for specific organisms
Cell count differential
Neutrophils vs. lymphocytes (lymphocytic effusion in TB, cancer, lymphoma, serositis)
Cytology
Malignancy, infection
Glucose (low)
RA, TB, empyema, malignancy, esophageal rupture
Rheumatoid factor, ANA, complement
Collagen vascular disease
Amylase
Pancreatitis, esophageal perforation, malignancy
pH
Empyema 20/min or PaCO2 12000 cells/mL or 10% bands Sepsis: clinical syndrome defined by the presence of both infection and SIRS and is classified by severity (see Table 31): severe sepsis: sepsis associated with organ dysfunction, hypoperfusion or hypotension septic shock: sepsis with arterial hypotension despite adequate fluid resuscitation multiorgan dysfunction syndrome: sepsis in the presence of altered organ function such that homeostasis cannot be maintained without intervention Table 31. Clinical Manifestations of Sepsis General Variables
Organ dysfunction variables
Fever (>38°C) or Hypothermia (90/min sBP 20 mL/kg over 24 hrs) Hyperglycemia (BG >7.7 mmol/L) in the absence of diabetes Leukopenia (WBC 10% immature forms Plasma C-reactive protein >2 SD above the normal value
Arterial hypoxemia (PaO2/FiO2 1.5 or aPTT >60 secs) Ileus (absent bowel sounds) Thrombocytopenia (platelet count 4 mg/dL or 70 mmol/L) Leukocytosis (WBC >12,000/L) Tissue perfusion variables Hyperlactatemia (>1 mmol/L) Decreased capillary refill or mottling
Table adapted with permission from Levy MM, Fink MP, Marshall JC, Abraham E, Angus D, Cook D, Cohen J, Opal SM, Vincent J-L, Ramsay G. 2001 SCCM/ESICM/ACCP/ ATS/SIS International Sepsis Definitions Conference. Critical Care Medicine 2003; 31(4):1250-6
Basic Principles for the Management of Sepsis • identify the cause and source of infection: blood, sputum, urine Gram stain and C&S • initiate empiric antibiotic therapy • monitor, restore and maintain hemodynamic function
Effect of Treatment with Low Doses of Hydrocortisone (HC) and Fludrocortisone (FC) on Mortality in Patients with Septic Shock JAMA 2002; 288:862-71 Study: Placebo-controlled, randomized, double-blind outcome study. Patients: 300 adult patients admitted to ICU with septic shock. Intervention: Patients were randomly assigned to receive either HC (50 mg q6h) and FC (50 mg q24h) or placebo for 7 days. Primary Outcome: 28-day survival in patients with relative adrenal insufficiency (nonresponders to corticotropin stimulation test). Results: Of the 229 nonresponders, 53% of patients died in the steroid group versus 63% in the placebo group. This corresponds to a 15.9% relative risk reduction (P=0.02). There was no significant difference between groups in the responders. Conclusion: Corticosteroid therapy in the ICU reduces mortality without increasing adverse events.
Corticosteroids for Treating Severe Sepsis and Septic Shock Cochrane Database of Systematic Reviews 2004, Issue 1 Study: Meta-analysis of 15 randomized and quasi randomized control trials examining the efficacy of corticosteroids on death at one month in patients with severe sepsis and septic shock. Results: Overall, there was no difference in 28-day all cause mortality. However, a subgroup of five trials that used long-term low dose corticosteroids (200-300 mg IV) showed a significant benefit in 28 day mortality (RR=0.80 95% CI 0.67-0.95). Data from this subgroup also showed a significant reduction of in-hospital mortality and increased shock reversal by day seven. Conclusions: The lack of an overall benefit to corticosteroids in sepsis was attributed to significant study heterogeneity. Low dose and long-term corticosteroids appear to have significant benefit for patients with sepsis. However, further research is needed to identify patients with sepsis who have adrenal insufficiency. Moreover, the optimal time to start treatment and the optimal dose require further trials.
R36 Respirology Early Goal-directed Therapy in the Treatment of Severe Sepsis and Septic Shock NEJM 2001; 345(19):1368-77 Study: RCT of patients with severe sepsis or septic shock who arrived at an emergency department were randomized to receive early-goal directed therapy or standard therapy in the emergency department. Intervention: Early-goal directed therapy consisted of specific treatment goals in the initial six hours of treatment. Specific goals were as follows: CVP: 8-12 mmHg (Rx’d with either crystalloid or colloids) MAP: >65 mmHg and 70% (Rx’d with transfusion of pRBCs until hemotacrit >30, then inotropic agents). Standard therapy in the emergency department consisted of ensuring MAP >65, CVP between 8-12 mmHg, and urine output >0.5 mg/kg/hr. Results: The in-hospital mortality for patients assigned to early-goal directed therapy was 30.5% compared to 46.5% in the standard medical care group (p= 0.009). Patients in the early-goal directed therapy group had improved APACHE II scores, higher central venous oxygen saturation, lower lactate concentrations, higher pH, and a lower base deficit. Conclusions: Early-goal directed therapy provides significant benefits to patients presenting to hospital with severe sepsis or septic shock. These patients had improved mortality and improved physiologic function as measured between 7 and 72 hours.
Introduction to Intensive Care
Toronto Notes 2011
Early Goal Directed Therapy • early goal-directed therapy that involves adjustments of cardiac preload, afterload and contractility to balance oxygen delivery with demand provides significant outcome benefits • should be started immediately and completed within 6 hours of recognition of severe sepsis or septic shock • patient should meet SIRS criteria and sBP 4 mmol/L 1. supplemental oxygen ± intubation and mechanical ventilation 2. central venous and arterial catheterization 3. if CVP F
Peripheral Arthritis
Symmetrical Small and large joints DIP less involved
Usually asymmetrical Usually larger joints, lower extremities (psoriatic arthritis may be the exception) DIP in psoriatic arthritis Dactylitis (“sausage digit”) Enthesitis
Pelvic/Axial Disease
No (except for C-spine)
Yes
Enthesitis
No
Yes
Extra-Articular
Nodules Vasculitis Sicca Raynaud’s phenomenon
Iritis (= anterior uveitis) Oral ulcers GI GU
Causes of Joint Pain SOFTER TISSUE Sepsis OA Fracture Tendon/muscle Epiphyseal Referred Tumour Ischemia Seropositive arthritides Seronegative arthritides Urate (gout)/other crystal Extra-articular rheumatism (PMR/ fibromyalgia)
Patterns of Joint Involvement Symmetrical vs. asymmetrical Small vs. large Mono vs. oligo vs. polyarticular Axial vs. peripheral
RH4 Rheumatology
Septic Arthritis/Degenerative Arthritis: Osteoarthritis
Toronto Notes 2011
Seropositive Rheumatic Diseases Connective Tissue Disease Rheumatoid Arthritis (RA) Systemic Lupus Erythematosus (SLE) Antiphospholipid Antibody Syndrome (APS) Scleroderma Polymyositis/Dermatomyositis (PM/DM) Sjogren’s Syndrome (SS) Mixed Connective Tissue Disease (MCTD)
Vasculitides
Small Vessel Non-ANCA-associated ANCA-associated (e.g. Wegener’s Granulomatosis)
Medium Vessel Polyarteritis Nodosa Kawasaki’s
Large Vessel Giant Cell Arteritis (GCA) Takayasu’s
Figure 3. Seropositive Rheumatic Diseases
Septic Arthritis Septic arthritis is a medical emergency!
• for any monoarticular arthritis one must rule out septic etiology; consider empiric antibiotic treatment until septic arthritis is excluded by history, physical exam and synovial fluid analysis (see Infectious Diseases, ID24/Orthopaedics, OR8)
Degenerative Arthritis: Osteoarthritis (OA)
Hand (DIP, PIP, 1st CMC) Hip Knee 1st MTP L-spine (L4-L5, L5-S1) C-spine Uncommon: ankle, shoulder, elbow, MCP, rest of wrist
© Linda Colati
• • • • • • •
Definition • primary (idiopathic) most common, of unknown etiology • secondary post-traumatic or mechanical post-inflammatory (e.g. RA) or post-infectious heritable skeletal disorders (e.g. scoliosis) endocrine disorders (e.g. acromegaly, hyperparathyroidism, hypothyroidism) metabolic disorders (e.g. gout, pseudogout, hemochromatosis, Wilson’s disease, ochronosis) neuropathic (also known as Charcot joints) atypical joint trauma due to loss of proprioceptive senses (e.g. diabetes, syphilis) avascular necrosis (e.g. fracture, steroids, alcohol, gout, sickle cell) other (e.g. congenital malformation)
Figure 4. Common Sites of Involvement in OA Intraarticular Corticosteroid for Treatment of Osteoarthritis of the Knee Cochrane Database of Syst Rev 2006; 2:CD005328. Study: Cochrane systematic review. 28 RCT and quasi-RCT trials. Population: Patients with osteoarthritis (OA) of the knee. Intervention: Intraarticular (IA) corticosteroid injection. Results: IA corticosteroids were more effective than placebo for pain reduction and global assessment at one week post-injection. There was significant pain reduction at 2 and 3 weeks, but no benefit for pain and function beyond 4 weeks postinjection. There was no benefit for global function beyond 1 week post-injection. There were higher rates of pain reduction at 4 weeks post-injection for triamcinolone hexacetonide versus betamethasone. There was no difference between IA corticosteroids and joint lavage in outcomes or safety. Hyaluronic acid (HA) injections showed better response than IA corticosteroids between 5 and 13 weeks post-injection. Conclusion: IA corticosteroid injection is effective for the short-term treatment of OA of the knee with few side effects. HA therapy can provide more durable results.
Etiology and Pathophysiology • altered joint function and damage • primary event is deterioration of articular cartilage due to local biomechanical factors and release of proteolytic and collagenolytic enzymes OA develops when cartilage catabolism > synthesis loss of proteoglycans and water exposes underlying bone • abnormal local bone metabolism further damages joint • synovitis is secondary to cartilage damage therefore may see small effusions in OA Epidemiology • most common arthropathy (12% of age 25-74) • increased prevalence with increasing age (35% of 30-year olds, 85% of 80-year olds) Risk Factors • genetic predisposition, advanced age, obesity (for knee OA), female, trauma Signs and Symptoms • localized to affected joints (not a systemic disease) • pain is often insidious, gradually progressive, with intermittent flare-ups and remissions Table 5. Signs and Symptoms of OA Signs
Symptoms
Joint line tenderness; stress pain Bony enlargement at affected joints Malalignment/deformity (angulation) Limited ROM Crepitus on passive ROM Inflammation (mild if present) Periarticular muscle atrophy
Joint pain with motion; relieved with rest Short duration of stiffness ( lateral standing x-rays must be done (not supine) • foot common in first MTP • lumbar spine very common especially L4-L5, L5-S1 degeneration of intervertebral discs and facet joints reactive bone growth can contribute to neurological impingement (e.g. sciatica, neurogenic claudication) or spondylolisthesis (displacement of vertebrae) • cervical spine commonly presents with neck pain, especially in lower cervical area Investigations • blood work normal CBC and ESR negative RF and ANA • radiology: 4 hallmark findings (see sidebar) • synovial fluid: non-inflammatory (see Table 25)
Rheumatology RH5 2 3
1 1. Thumb squaring 2. Heberden’s nodes 3. Bouchard’s nodes
© Tabby Lulham 2011
Toronto Notes 2011
Figure 5. Hand Findings in OA
OA of MCP joints can be seen in hemochromatosis or chondrocalcinosis.
Hint: Bouchard’s is closer to the Body
The Radiographic Hallmarks of OA 1. Joint space narrowing 2. Subchondral sclerosis 3. Subchondral cysts 4. Osteophytes
Treatment • presently no treatment alters the natural history of OA • non-pharmacological therapy weight loss (minimum 5-10 lb loss) if overweight rest/low-impact exercise physiotherapy: heat/cold, exercise programs occupational therapy: aids, splints, cane, walker, bracing • pharmacological therapy (see Table 28) oral: acetaminophen, NSAIDs joint injections: hyaluronic acid, corticosteroid topical: capsaicin, NSAIDs glucosamine ± chondroitin • surgical treatment joint replacement
Seropositive Rheumatic Disease: Connective Tissue Disorders Table 6. Features of Seropositive Arthropathies RA
SLE
Scleroderma
Dermatomyositis
History
Symmetrical Polyarthritis (small joint involvement) AM stiffness (>1 hr)
Multisystemic disease: rash, photosensitivity, Raynaud’s, alopecia, cardiac and pulmonary serositis, CNS symptoms, glomerulonephritis
Skin tightness, stiffness of fingers, Raynaud’s, heartburn/dysphagia, pulmonary hypertension, renal dysfunction
Heliotrope rash (eyelids), Gottron’s papules, macular erythema and poikiloderma (shoulders, neck and chest), proximal muscle weakness ± pain
Physical Examination
Effused joints Tenosynovitis Nodules Joint deformities Bone-on-bone crepitus
Confirm historical findings (rash, serositis, etc.) ± effused (typically small) joints (can be minimal, look for soft tissue swelling)
Skin tightness on dorsum Rash, proximal muscle of hand, facial skin weakness tightening, telangiectasia, calcinosis, non-effused joint
Clinical Features
Glucosamine Therapy for Treating Osteoarthritis Cochrane Database of Systemic Reviews 2005; 2:CD002946. DOI: 10,1002/14651858. CD002946. pub2 Study: Meta-analysis of 20 RCTs (n=2750) examining the efficacy of glucosamine on OA. Results: Overall analysis of 15 RCTs favoured glucosamine over placebo for total reduction in pain (measured by a variety of methods). Significant differences between glucosamine and placebo were also observed when compared to Levesque Index scores. Only the glucosamine containing Rotta preparation was found to be significant. No significant differences in WOMAC (pain, stiffness and function subscales) were found between glucosamine and placebo when only studies with adequate allocation concealment were included. There was evidence to suggest that glucosamine may slow the radiologic progression of OA at 3 years. Glucosamine had an excellent safety profile. Conclusion: Glucosamine appears helpful for pain when all studies (low quality and older studies) are included. However, when only the higher quality studies are included, there is no longer a difference between glucosamine and placebo. Glucosamine was very well tolerated with low toxicity. Rotta preparation of glucosamine may be of some benefit.
Meta-analysis: Chondroitin for Osteoarthritis of the Knee and Hip Annals of Internal Medicine 2007; 146(8):580-590 Study: Meta-analysis of 20 RCTs (n=3846) examining the efficacy of chondroitin on OA. Results: The analysis of this review was hampered by significant trial heterogeneity. Trials with poor methodology (small numbers, inadequate randomization concealment, no intention to treat analysis) showed larger effect sizes in favour of glucosamine than more recent trials. When the authors analyzed only the newer and more robust trials, an effect size of -0.3 (CI 95%: -0.13 to 0.07) was generated. Conclusion: There is high quality evidence to suggest there is no difference between chondroitin and placebo. Chondroitin should be disregarded from routine use in clinical practice.
RH6 Rheumatology
Seropositive Rheumatic Disease: Connective Tissue Disorders
Toronto Notes 2011
Table 6. Features of Seropositive Arthropathies (continued) RA
SLE
Scleroderma
Dermatomyositis
Increased ESR Decreased platelets (autoimmune) Decreased Hb (autoimmune) Decreased WBC (leukopenia, lymphopenia)
Increased ESR Increased platelets Decreased Hb Normal WBC
Possible increased ESR Normal platelets Decreased Hb Normal WBC
ANA +ve in 98% Anti-dsDNA +ve in 50-70% Anti-SM +ve in 30% Decreased C3, C4, total hemolytic complement False positive VDRL (in lupus subtypes) Increased PTT (in lupus subtypes; e.g. antiphospholipid Ab)
ANA +ve in >90% Anti-topoisomerase 1 (diffuse) Anti-centromere (usually in CREST, see RH11)
CK elevated in 80% ANA +ve in 33% anti-Jo-1, anti-Mi-2 Muscle biopsy EMG MRI
Laboratory Non-specific Increased ESR in 50-60% Increased platelets Decreased Hb Decreased WBC (Felty’s) Specific
RF +ve in ~80%
Mild inflammation with +ve ANA Not specific Synovial Fluid Inflammation Leukocytosis (>10,000) Radiographs
Periarticular osteopenia Joint space narrowing Erosions Absence of bone repair Symmetric/concentric
Nonerosive ± osteopenia ± soft tissue swelling
Not specific
± pulmonary fibrosis ± esophageal dysmotility ± esophageal dysmotility ± interstitial lung disease ± calcinosis ± calcifications
Rheumatoid Arthritis (RA)
Common Presentation • Morning stiffness >1 hr, improves with use • Symmetric joint involvement • Initially involves small joints of hands and feet • Constitutional symptoms
Definition • chronic, symmetric, erosive synovitis of peripheral joints (i.e. wrists, MCPs, MTPs) • characterized by a number of extra-articular features Table 7. Classification Criteria for RA: total score of 6 or more classifies patient as definite RA. Patient must have at least 1 joint with definite clinical swelling, not better explained by another disease
1987 American Rheumatism Association RA criteria At least 4 of: • Morning stiffness >1 hour for >6 weeks • Arthritis ≥3 joints for >6 weeks • Arthritis of hand joints for >6 weeks • Symmetric arthritis for >6 weeks • Rheumatoid nodules • Serum RF positive • Radiographic changes (erosions or periarticular osteopenia) Criteria are 91-94% sensitive and 89% specific for RA.
Criteria
Score
1. Joint involvement (swollen or tender) 1 large joint (shoulders, elbows, hips, knees, and ankles) 2-10 large joints 1-3 small joints (MCPs, PIPs, wrists, 2nd-5th MTPs) 4-10 small joints >10 joints (at least 1 small joint)
0 1 2 3 5
2. Serology Negative RF and negative Anti-CCP Low-positive RF or low-positive Anti-CCP (3x ULN)
0 2 3
3. Acute phase reactants Normal CRP and normal ESR Abnormal CRP and abnormal ESR
0 1
4. Duration of symptoms 1 hr, improves with use, aggravated by rest • symmetric joint involvement (see Figure 6) • joint swelling, tender joints • signs of mechanical joint damage: loss of motion, instability, deformity, crepitus • constitutional symptoms: profound fatigue; rarely myalgia or weight loss • extra-articular features (EAF) (see Table 8) • limitation of function and decrease in global functional status • complications of chronic synovitis joint deformities (see Figure 8) swan neck deformity, boutonnière deformity ulnar deviation of MCP; radial deviation of wrist joint hammer toe, mallet toe, claw toe flexion contractures atlanto-axial and subaxial subluxation C-spine instability neurological impingement (long tract signs) difficult intubation limited shoulder mobility, spontaneous tears of the rotator cuff leading to chronic spasm tenosynovitis g may cause rupture of tendons carpal tunnel syndrome ruptured Baker’s cyst (outpouching of synovium behind the knee); presentation similar to acute DVT Table 8. Extra-Articular Features of RA Classified by Underlying Pathophysiology System
Vasculitic
Skin
Periungal infarction, cutaneous ulcers, Rheumatoid nodules palpable purpura
Lymphocytic Infiltrate
Ocular
Episcleritis, scleritis
Keratoconjunctivitis sicca (see sidebar)
Head and Neck
Xerostomia (see sidebar), Hashimoto’s thyroiditis (see Endocrinology, E27)
Cardiac
Peri-/myocarditis, valvular disease, conduction defects Pulmonary fibrosis, pleural effusion, pleuritis, pulmonary nodules
Pulmonary Neurologic Hematologic
Peripheral neuropathy: sensory stocking-glove, mononeuritis multiplex Splenomegaly, neutropenia (Felty’s, see sidebar)
Mallet Toe
© Elisheva Marcus and Gloria Situ
Chondrocyte
© Dr. Lori Albert
Claw Toe Osteoclast
Figure 8. Joint Deformities
Syndromes in RA 1. Sjögren’s syndrome (common): keratoconjunctivitis sicca and xerostomia (dry eyes and mouth) 2. Caplan’s syndrome (rare): multiple pulmonary nodules and pneumoconiosis 3. Felty’s syndrome (rare): arthritis, splenomegaly, neutropenia
Poor prognostic features of RA include young age of onset, high RF titer, elevated ESR, activity of >20 joints, and presence of EAF.
RH8 Rheumatology
Side Effects of Steroids • Weight gain • Osteoporosis, avascular necrosis (AVN) • Cataracts, glaucoma • Peptic ulcer disease (PUD) • Susceptibility to infection • Easy bruising • Acne • Hypertension • Hyperlipidemia • Hypokalemia, hyperglycemia • Mood swings
Only DMARDs (not analgesics or NSAIDs) alter the course of RA!
Comparison of Treatment Strategies in Early Rheumatoid Arthritis Ann Intern Med 2007; 146(6) Study: RCT of 508 patients comparing 4 different treatment strategies for early rheumatoid arthritis (known as the BEST trial). Intervention: Group 1: Sequential Monotherapy with traditional DMARDs Group 2: Step-Up Combination Therapy Group 3: Initial Combination Therapy with prednisone (high dose) Group 4: Initial Combination Therapy with infliximab Results: Patients in groups 3 and 4 responded faster and had significantly greater overall change in physical function scores after the first year of treatment. By end of the second year, groups 1 and 2 had achieved a similar response to groups 3 and 4. Groups 3 and 4 also showed significantly less radiologic progression of their disease over 2 years than groups 1 and 2. There were no significant differences in toxicity levels between the 4 groups. Conclusions: Initial combination therapy with prednisone or infliximab results in faster response rates. Whether faster initial response rates leads to better long-term disease outcomes has not yet been studied. The Safety of Infliximab, Combined with Background Treatments, among Patients with Rheumatoid Arthritis and Various Comorbidities (START) Arthritis Rheum 2006; 54:1075-86 Study: Randomized, placebo-controlled multicentre trial. Patients: 1084 patients (mean age 52 yrs, 80% female) with active moderate to severe rheumatoid arthritis despite treatment with methotrexate. Intervention: Patients were randomized to receive infusions of placebo, infliximab dosed at 3 mg/kg, or infliximab dosed at 10 mg/kg at 0, 2, 6, and 14 weeks, in addition to methotrexate therapy. Primary Outcome: Incidence of serious infection within 22 weeks of randomization. Results: Compared with the placebo group, the relative risk of developing serious infection was 1.0 (95%CI 0.3-3.1, P=0.995) in patients receiving infliximab at 3 mg/kg and 3.1 (95%CI 1.2-7.9, P=0.013) in patients receiving infliximab at 10 mg/ kg. In addition, 31% of patients receiving infliximab at 3 mg/kg and 32% of patients receiving infliximab at 10 mg/kg were able to achieve remission at 22 weeks compared with only 14% of those receiving placebo (P3 months at >7.5 mg/day cautions/contraindications: active infection, osteoporosis, hypertension, gastric ulcer, diabetes, TB 2. Disease Modifying Anti-Rheumatic Drugs (DMARDs) (see Table 29) • DMARDs are the standard of care • start DMARDs when diagnosis is confirmed to decrease disease progression, symptoms and signs • DMARDs reduce or prevent joint damage, and are associated with better long-term disability index • delayed onset of action (may take 8-12 weeks) • many DMARDs have potential toxicities that require periodic monitoring • if repetitive flares, progressive joint damage, or ongoing disease activity after 3 months of maximal therapy g change or add other DMARDs • non-biologics choice depends on: disease duration, disease activity, presence of poor prognostic features methotrexate is the gold standard others: hydroxychloroquine, sulfasalazine, leflunomide combination therapy with methotrexate is now standard of care • biologics: indicated if persistent disease activity commonly used after failure of other DMARDs; however, evidence suggests benefit of use in early RA as well (e.g. infliximab, etanercept, etc.)
Toronto Notes 2011
Seropositive Rheumatic Disease: Connective Tissue Disorders
Rheumatology RH9
Surgical Therapy • surgery indicated for structural joint damage • synovectomy: debridement and/or removal of inflamed synovium from individual joints (surgical or radioactive) • joint replacement (hip, shoulder, knee, less commonly MCP, ankle, elbow) • joint fusion (wrist, thumb, ankle, C-spine) • reconstruction (tendon repair)
Systemic Lupus Erythematosus (SLE) Definition • chronic inflammatory multisystem disease of unknown etiology, characterized by production of autoantibodies and diverse clinical manifestations Table 9. Diagnostic Criteria of SLE: 4 or more of 11 must be present serially or simultaneously Criteria
Description Diagnostic Criteria of SLE
Clinical Malar rash
Classic “butterfly rash”, sparing of nasolabial folds, no scarring
Discoid rash
May cause scarring due to invasion of basement membrane
Photosensitivity
Skin rash in reaction to sunlight
Oral/nasal ulcers
Usually painless
Arthritis
Symmetric, involving ≥2 small or large peripheral joints, non-erosive
Serositis
Pleuritis or pericarditis
Neurologic disorder
Seizures or psychosis
Laboratory Renal disorder
Proteinuria (>0.5 g/day or 3+) Cellular casts (RBC, Hb, granular, tubular, mixed)
Hematologic disorder
Hemolytic anemia, leukopenia, lymphopenia, thromboctyopenia
Immunologic disorder
Anti-dsDNA, anti-Sm Antiphospholipid antibodies based on the finding of serum anticardiolipin Ab, lupus anticoagulant, or false positive VDRL
Antinuclear antibody (ANA)
Most sensitive test (98%), not specific
MD SOAP BRAIN Malar rash Discoid rash Serositis Oral ulcers ANA Photosensitivity
Blood Renal Arthritis Immune Neurologic
Radiographically, the arthritis of SLE is non-erosive (unlike RA).
Consider SLE in a patient who has involvement of 2 or more organ systems.
Note: “4, 7, 11” rule 4 out of 11 criteria (4 lab, 7 clinical) for diagnosis American College of Rheumatology, 1997 update
Etiology and Pathophysiology • production of autoantibodies causing multi-organ inflammation • multifactorial etiology (see Figure 9) • genetics common association with HLA-B8/-DR3; ~10% have positive family history • estrogen prepubertal and postmenopausal women have similar incidence to men men with SLE have higher concentration of estrogenic metabolites • infection viral (nonspecific stimulant of immune response) • drugs anticonvulsants (phenytoin) antihypertensives (hydralazine) antiarrhythmics (procainamide) isoniazid (INH) anti-histone antibodies are commonly seen in drug-induced lupus oral contraceptive pills associated with exacerbation biologic response modifiers Epidemiology • prevalence: 0.05% overall • F:M = 10:1; age of onset in reproductive years, 13-40 • more common and severe in African-Americans and Asians • bimodal mortality pattern early (within 2 years) active SLE, active nephritis, infection secondary to steroid use late (>10 years) inactive SLE, inactive nephritis, atherosclerosis likely due to chronic inflammation
Environment Stress, viruses, sun Genetic Hormonal HLA Drugs
T-cells
Formation of Auto-Ab Cytotoxic Ab
Immune complexes
Cell damage/death
Inflammation
Figure 9. Multifactorial Etiology of SLE
Drug-Induced SLE Often presents atypically with systemic features and serositis; usually associated with anti-histone antibodies.
RH10 Rheumatology
Raynaud’s Phenomenon Vasospastic disorder characteristically causing discolouration of fingers and toes (white blue red). Classic triggers: cold and emotional stress.
Seropositive Rheumatic Disease: Connective Tissue Disorders
Toronto Notes 2011
Signs and Symptoms • characterized by periods of exacerbation and remission • systemic fatigue, malaise, weight loss, fever, lymphadenopathy • vascular Raynaud’s phenomenon, livedo reticularis (mottled discolouration of skin due to narrowing of blood vessels, characteristic lacy or net-like appearance), thrombosis, vasculitis • renal glomerulonephritis, renal failure • dermatologic photosensitivity, malar rash, discoid rash, oral ulcers, alopecia (hair loss), purpura, panniculitis (inflammation of subcutaneous fat and muscle tissue), urticaria • musculoskeletal polyarthralgias, polyarthritis, myalgias, avascular necrosis • ophthalmic keratoconjunctivitis sicca, episcleritis, scleritis, cytoid bodies (cotton wool exudates on fundoscopy = infarction of nerve cell layer of retina) • cardiac pericarditis, coronary artery disease • pulmonary pleuritis, interstitial lung disease, pulmonary hypertension, PE, alveolar hemorrhage • gastrointestinal pancreatitis, lupus enteropathy, hepatitis, hepatomegaly • neurologic headache, depression, psychosis, seizures, cerebritis, transverse myelitis, peripheral neuropathy, stroke Investigations • ANA has high sensitivity (98%), but poor specificity used as a screening test • anti-dsDNA and anti-Sm are specific for SLE (95-99%) • anti-dsDNA titer and serum complement (C3, C4) are useful to monitor treatment response in patients who are clinically and serologically concordant anti-dsDNA increases and C3 and C4 decrease with disease activity • anticardiolipin Ab and lupus anticoagulant in antiphospholipid antibody syndrome Treatment • principles of therapy treat early and avoid long term steroid use, if possible if high doses of steroids necessary for long-term control, add steroid sparing agents and taper when possible treatment is tailored to organ system involved and severity of disease all medications used to treat SLE require periodic monitoring for potential toxicity • dermatologic preventative: use sunscreen, avoid UV light and estrogens topical steroids, hydroxychloroquine • musculoskeletal NSAIDs ± gastroprotective agent for arthritis (also beneficial for pleuritis and pericarditis) hydroxychloroquine improves long term control and prevents flares bisphosphonates, calcium, vitamin D to combat osteoporosis • organ threatening disease high-dose oral prednisone or IV methylprednisolone in severe disease steroid sparing agents: azathioprine, methotrexate, mycophenolate IV cyclophosphamide for serious organ involvement (e.g. cerebritis or SLE nephritis)
Antiphospholipid Antibody Syndrome (APS)
Manifestations of APS Thromboembolic events Spontaneous abortions Thrombocytopenia
Definition • multisystem vasculopathy manifested by recurrent thromboembolic events, spontaneous abortions and thrombocytopenia • often presents with migraine type headaches • circulating antiphospholipid autoantibodies interfere with coagulation cascade • primary APS: occurs in the absence of other disease • secondary APS: occurs in the setting of a connective tissue disease (including SLE), malignancy, drugs (hydralazine, procainamide, phenytoin, interferon, quinidine), and infections (HIV, TB, hepatitis C, infectious mononucleosis) • catastrophic APS: development within 1 week of small vessel thrombotic occlusion in ≥3 organ systems with positive antiphospholipid antibodies (high mortality)
Toronto Notes 2011
Seropositive Rheumatic Disease: Connective Tissue Disorders
Table 10. Classification Criteria of APS: 1 clinical and 1 laboratory criteria must be present Criteria
Description
Clinical Vascular thrombosis
Arterial: stroke/TIA, multi-infarct dementia, MI, valvular incompetence, limb ischemia Venous: DVT, PE, renal and retinal vein thrombosis Must be confirmed by imaging or histopathology
Pregnancy morbidity
Fetal death (>10 wks GA), recurrent spontaneous abortions (15) that focused on the secondary thromboprophylaxis in patients with aPL were selected. Results: Sixteen studies were selected. Patients with venous events and a single test for aPL showed a low recurrence rate while receiving oral anticoagulation at a target international normalized ratio (INR) of 2.0-3.0. Patients with stroke and a single positive aPL test had no increased risk compared with those without aPL. Recurrence rates in patients with definite antiphospholipid syndrome (APS) and previous venous thromboembolism were lower than in patients with arterial and/or recurrent events, both with and without therapy. Only 3.8% of recurrent events occurred at an actual INR >3.0. Mortality due to recurrent thrombosis was higher than mortality due to bleeding (18 patients versus 1 patient reported). Conclusion: For patients with definite APS, the authors recommend prolonged warfarin therapy at a target INR of 2.0-3.0 in APS patients with first venous events and >3.0 for those with recurrent and/or arterial events. For patients with venous thromboembolism or stroke and a single positive aPL test, the authors recommend further testing to determine if they have a persisting antibody. If they do not, the same therapy as for the general population should be used (warfarin at a target INR of 2.0-3.0 and low-dose aspirin, respectively).
Scleroderma Definition • a non-inflammatory disorder characterized by widespread small vessel vasculopathy and fibrosis, which occurs in the setting of immune system activation and autoimmunity Scleroderma Generalized (systemic sclerosis)
Localized (no involvement of internal organs) • Mostly children and young adults
Morphea • Hard oval patches on the skin
Linear • Line of thickened skin
Limited systemic sclerosis • Skin sclerosis restricted to hands, face, neck • 3rd to 4th decade • Pulmonary hypertension common • CREST (see sidebar)
Diffuse systemic sclerosis • Widespread skin disease (proximal to wrist, can involve trunk), tendons • Early visceral involvement (renal, pulmonary fibrosis)
Figure 10. Forms of Scleroderma Table 11. Classification Criteria of Systemic Sclerosis: 1 major or 2 minor criteria must be present Criteria
Description
Major Scleroderma proximal to MCPs
Skin tightness, thickening, non-pitting induration
Minor Sclerodactyly Digital pitting scars or loss of substance from finger pad Bibasilar pulmonary fibrosis American Rheumatism Association, 1980
Skin changes limited to digits
CREST Syndrome Calcinosis – calcium deposits on skin Raynaud’s phenomenon Esophageal dysfunction – acid reflux Sclerodactyly – tightening of skin on digits Telangiectasia – superficial dilated blood vessels
RH12 Rheumatology
Seropositive Rheumatic Disease: Connective Tissue Disorders
Toronto Notes 2011
Etiology and Pathophysiology • idiopathic vasculopathy (not vasculitis) leading to atrophy and fibrosis of tissues intimal proliferation and media mucinous degeneration progressive obliteration of vessel lumen fibrotic tissue resembles malignant hypertension Epidemiology • F:M = 3-4:1, peaking in 5th and 6th decades • associated with HLA-DR1 • associated environmental exposure (silica, epoxy resins, toxic oil, aromatic hydrocarbons, polyvinyl chloride) Signs and Symptoms Table 12. Clinical Manifestations of Scleroderma
Scleroderma is the most common cause of secondary Raynaud’s phenomenon.
Lung disease is the most common cause of morbidity and mortality.
System
Features
Dermatologic
Painless non-pitting edema skin tightening Ulcerations, calcinosis, periungual erythema, hypo/hyperpigmentation, pruritus, telangiectasias Characteristic face: mask-like facies with tight lip, beak nose, radial perioral furrows
Vascular
Raynaud’s phenomenon digital pits, gangrene
Gastrointestinal (~90%)
Distal esophageal hypomotility dysphagia Loss of lower esophageal sphincter function GERD, ulcerations, strictures Small bowel hypomotility bacterial overgrowth, diarrhea, bloating, cramps, malabsorption, weight loss Large bowel hypomotility wide mouth diverticuli are pathognomonic radiographic finding on barium study
Renal
Mild proteinuria, creatinine elevation, hypertension “Scleroderma renal crisis” (10-15%) may lead to malignant arterial hypertension, oliguria and microangiopathic hemolytic anemia
Pulmonary
Interstitial fibrosis, pulmonary HTN, pleurisy, pleural effusions
Cardiac
Left ventricular dysfunction, pericarditis, pericardial effusion, arrhythmias
Musculoskeletal
Polyarthralgias “Resorption of distal tufts” (radiological finding) Proximal weakness 2o to disuse, atrophy, low grade myopathy
Endocrine
Hypothyroidism
Investigations • bloodwork CBC, Cr, ANA anti-topoisomerase 1/anti-Scl-70: specific but not sensitive for diffuse systemic sclerosis anti-centromere: favours diagnosis of CREST variant (limited systemic sclerosis) • imaging CXR for fibrosis, echo for pulmonary HTN Treatment • dermatologic good skin hygiene low dose prednisone, methotrexate (limited evidence) • vascular patient education on cold avoidance vasodilators (CCBs, local nitroglycerine cream, systemic PGE2 inhibitors) • gastrointestinal GERD: PPIs are first line, then H2-receptor agonists small bowel bacterial overgrowth: broad spectrum antibiotics (tetracycline, metronidazole) • renal disease ACE inhibitors • pulmonary early interstitial disease: cyclophosphamide pulmonary hypertension: vasodilators e.g. bosentan (Tracleer®), epoprostenol (Flolan®) • cardiac pericarditis: systemic steroids • musculoskeletal arthritis: NSAIDs, myositis: systemic steroids
Toronto Notes 2011
Seropositive Rheumatic Disease: Connective Tissue Disorders
Rheumatology RH13
Idiopathic Inflammatory Myopathy Definition • autoimmune diseases characterized by proximal muscle weakness ± pain • muscle becomes damaged by a non-suppurative lymphocytic inflammatory process • classification adult polymyositis (PM)/dermatomyositis (DM) (see Table 13) juvenile DM (usually with vasculitis) PM/DM associated with malignancy increased risk of malignancy: age >50, DM>PM, normal CK, refractory disease 2.4-6.5 fold increased risk of underlying malignancy usually in internal organs PM/DM associated with connective tissue disease inclusion body myositis (IBM) age >50, M>F, slowly progressive, vacuoles in cells on biopsy suspect when patient unresponsive to treatment distal as well as proximal muscle weakness muscle biopsy positive for inclusion bodies POLYMYOSITIS (PM)/DERMATOMYOSITIS (DM) Table 13. Classification Criteria for PM/DM. Definite if 4 present, probable if 3 present. Criteria
Description
1. Symmetric proximal muscle weakness Typical involvement of shoulders and hips 2. Elevated muscle enzymes
Increased CK, aldolase, LDH, AST, ALT
3. EMG changes
Short polyphasic motor units, high frequency repetitive discharge, insertional irritability
4. Muscle biopsy
Segmental fibre necrosis, basophilic regeneration, perivascular inflammation and atrophy
5. Typical rash of dermatomyositis
Required for diagnosis of DM (see below)
NEJM 1975; 292:403-7
Etiology and Pathophysiology • PM is CD8 cell-mediated muscle necrosis, found in adults • DM is b-cell and CD4 immune complex-mediated perifasicular vasculitis Signs and Symptoms • progressive symmetrical proximal muscle weakness (shoulder and hip) developing over weeks to months difficulty lifting head off pillow, arising from chair, climbing stairs • dermatological DM has characteristic dermatological features (F>M, children and adults) Gottron’s papules – pink-violaceous, flat-topped papules overlying the dorsal surface of the interphalangeal joints Gottron’s sign – erythematous, smooth or scaly patches over the dorsal IPs, MCPs, elbows, knees, or medial malleoli heliotropic rash: purple rash over the eyelids; usually with edema shawl sign: erythematous rash over neck, upper chest, and shoulders mechanic’s hands: dark, dry, thick scale on palmar and lateral surface of digits periungal erythema • cardiac dysrhythmias, CHF, conduction defect, ventricular hypertrophy, pericarditis • gastrointestinal oropharyngeal and lower esophageal dysphagia, reflux • pulmonary weakness of respiratory muscles, interstitial lung disease, aspiration pneumonia Investigations • bloodwork: CK, ANA, anti-Jo-1 (DM), anti-Mi-2, anti-SRP • imaging: MRI may be used to localize biopsy site • EMG, muscle biopsy
Signs of DM Gottron’s papules and Gottron’s sign are pathognomonic of DM (occur in 70% of patients).
RH14 Rheumatology
Malignancies Associated with DM • Breast • Lung • Colon • Ovarian
Seropositive Rheumatic Disease: Connective Tissue Disorders
Toronto Notes 2011
Treatment • physical therapy and occupational therapy • medical high dose corticosteroid (1-2 mg/kg/day) and slow taper add immunosuppressive agents (azathioprine, methotrexate, cyclosporine) intravenous immunoglobulin for severe or refractory hydroxychloroquine for DM rash • malignancy surveillance detailed history and physical (breast, pelvic and rectal exam) CXR, abdominal and pelvic ultrasound, stool occult blood, Pap test, mammogram ± CT scan (thoracic, abdominal, pelvic)
Sjögren’s Syndrome (SS) Definition • autoimmune condition characterized by dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), caused by lymphocytic infiltration of salivary and lacrimal glands • may evolve into systemic disorder with diminished exocrine gland activity in respiratory tract and skin • primary and secondary form (associated with RA, SLE, DM and HIV) • incidence estimated at 4/100,000 people • 90% of cases are among females • mean age of diagnosis is 40-60 yrs Table 14. Classification Criteria for SS. Need 4 present, one of which includes salivary gland biopsy or autoantibodies Classic Triad (identifies 93% of Sjögren’s patients) • Dry eyes • Dry mouth (xerostomia) dysphagia • Arthritis (small joint, asymmetrical, nonerosive)
Criteria
Description
1. Dry eye symptoms
Dry >3 months, foreign body sensation, or requiring tear substitutes
2. Dry mouth symptoms
Dry >3 months, swollen salivary glands, or requiring liquids to swallow food
3. Dry eye signs
Schirmer test (to assess tear flow) or slit lamp exam with Rose Bengal stsin
4. Dry mouth signs
Low salivary flow, sialography
5. Salivary gland biopsy
Focal lymphocytic sialoadenitis
6. Autoantibodies
anti-Ro and/or anti-La
Ann Rheum Dis 2002; 61:554-8
Patients with Sjögren’s syndrome are at higher risk of non-Hodgkin’s lymphoma.
Signs and Symptoms • “sicca complex”: dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia) • staphylococcus blepharitis • dental caries, oral candidiasis, angular cheilitis (inflammation and fissuring at the commissures of the mouth) • systemic complications sinusitis, autoimmune thyroid dysfunction arthralgias, arthritis subclinical diffuse interstitial lung disease, xerotrachea leading to chronic dry cough renal disease, glomerulonephritis palpable purpura, vasculitis peripheral neuropathy lymphoma Treatment • ocular artificial tears or surgical punctal occlusion for dry eyes • oral good dental hygiene, hydration agents that stimulate salivary flow (e.g. pilocarpine) topical nystatin or clotrimazole x 4-6 weeks for oral candidiasis • systemic hydroxychloroquine, corticosteroids, immunosuppressive agents
Toronto Notes 2011
Seropositive Rheumatic Disease: Connective Tissue Disorders/Vasculitides
Rheumatology RH15
Mixed Connective Tissue Disease (MCTD)/ Overlap Syndrome • syndrome with features of 2 different CTD (e.g. SLE, scleroderma, PM) • common symptoms: Raynaud’s phenomenon, swollen fingers • bloodwork: anti-RNP (see Table 19) • prognosis 50-60% will evolve into SLE 40% will evolve into scleroderma only 10% will remain as MCTD for the rest of their lives
Seropositive Rheumatic Disease: Vasculitides • inflammation and subsequent necrosis of blood vessels leading to tissue ischemia or infarction • any organ system can be involved • keys to diagnosis clinical suspicion: suspect in cases of unexplained multiple organ ischemia or systemic illness with no evidence of malignancy or infection labs non-specific: anemia, increased WBC and ESR, abnormal urinalysis biopsy if tissue accessible angiography if tissue inaccessible • treatment generally involves corticosteroids and/or immunosuppressives
c-ANCA = circulating anti-neutrophil cytoplasmic antibody p-ANCA = perinuclear anti-neutrophil cytoplasmic antibody
Table 15. Classification of Vasculitis and Characteristic Features Classification
Characteristic Features
SMALL VESSEL • Non-ANCA-associated
Immune complex mediated (most common mechanism)
Predominantly cutaneous vasculitis
Also known as hypersensitivity/leukocytoclastic vasculitis
Henoch-Schönlein purpura (see Pediatrics, P98) Vascular deposition of IgA causing systemic vasculitis (skin, GI, renal), usually self-limiting, most common in childhood Essential cryoglobulinemic vasculitis
Systemic vasculitis caused by circulating cryoproteins
• ANCA-associated Wegener’s granulomatosis (c-ANCA > p-ANCA)
Granulomatous inflammation of vessels of respiratory tract and kidneys, initially have URTI symptoms, most common in middle age
Churg-Strauss syndrome (50% ANCA positive) Granulomatous inflammation of vessels with hypereosinophilia and eosinophilic tissue infiltration, can be associated with p-ANCA or c-ANCA, other manifestations include coronary arteritis, myocarditis and neuropathy, average age 40’s Microscopic polyangiitis (70% ANCA positive, usually p-ANCA) MEDIUM VESSEL Polyarteritis nodosa Kawasaki’s (see Pediatrics, P98) LARGE VESSEL Giant cell arteritis (GCA) /Temporal Arteritis Takayasu’s arteritis OTHER VASCULITIDES Buerger’s disease
Features of Small Vessel Vasculitis • Palpable purpura • Vesicles • Chronic urticaria • Superficial ulcers
Churg-Strauss Triad • Allergic rhinitis and asthma • Eosinophilic infiltrative disease resembling pneumonia • Systemic vasculitis
Pauci-immune necrotizing vasculitis, affecting kidneys (necrotizing glomerulonephritis), lungs (capillaritis and alveolar hemorrhage), skin Most common in middle age Segmental non-granulomatous necrotizing inflammation Unknown etiology in most cases, any age (average 40-50’s), M > F T-lymphocyte response and granuloma formation Inflammation predominantly of the aorta and arteries originating from it Over 50 years of age, F>M “Pulseless disease”, chronic inflammation, most often the aorta and its branches Usually young adults of Asian descent, F>M Also known as thromboangiitis obliterans, inflammation secondary to pathological clotting, affects small and medium-sized vessels of distal extremities, may lead to distal claudication and gangrene, most important etiologic factor is cigarette smoking Most common in young Asian males
Behçet’s disease
Pathology: leukocytoclastic vasculitis, multisystem disorder presenting with ocular involvement, recurrent oral and genital ulceration, venous thrombosis, skin and joint involvement, more common in Mediterranean and Asia, average age 30’s, M > F
Vasculitis mimicry
Cholesterol emboli, atrial myxoma
Features of Medium Vessel Vasculitis • Livedo reticularis • Erythema nodosum • Raynaud’s phenomenon • Nodules • Digital infarcts • Ulcers
RH16 Rheumatology
Vasculitides
Toronto Notes 2011
Predominantly Cutaneous Vasculitis SMALL VESSEL NON-ANCA ASSOCIATED VASCULITIS • subdivided into drug-induced vasculitis serum sickness reaction vasculitis associated with other underlying primary diseases Etiology and Pathophysiology • cutaneous vasculitis following drug exposure (allopurinol, gold, sulfonamides, penicillin, phenytoin) viral or bacterial infection idiopathic causes • small vessels involved (post-capillary vessels most frequently) • usually causes a leukocytoclastic vasculitis: debris from neutrophils around vessels • sometimes due to cryoglobulins which precipitate in cold temperatures Signs and Symptoms • palpable purpura ± vesicles and ulceration, urticaria, macules, papules, bullae, subcutaneous nodules Investigations • vascular involvement (both arteriole and venule) established by skin biopsy Treatment • stop possible offending drug • corticosteroids ± immunosuppressive agents • usually self-limiting
Wegener’s Granulomatosis SMALL VESSEL ANCA-ASSOCIATED VASCULITIS Definition • granulomatous inflammation of vessels that may affect the upper airways (rhinitis, sinusitis), lungs (pulmonary nodules, infiltrates), and kidneys (glomerulonephritis, renal failure) • highly associated with c-ANCA • incidence 5 per 100,000; more common in Northern latitudes Table 16. Classification Criteria: Wegener’s diagnosed if 2 or more of the following 4 criteria present Criteria
Definition
1. Nasal or oral involvement
Inflammation, ulcers, epistaxis
2. Abnormal findings on CXR
E.g. nodules, cavitations
3. Urinary sediment
Protein, RBC casts
4. Biopsy of involved tissue
Lungs show granulomas, kidneys show necrotizing segmental glomerulonephritis
American College of Rheumatology, 1990
Etiology and Pathophysiology • transformation from inflammatory prodrome (serous otitis media and sinusitis) to full-blown vasculitic syndrome
Classic Features • Necrotizing granulomatous vasculitis of lower and upper respiratory tract • Focal segmental glomerulonephritis
Signs and Symptoms • systemic malaise, fever, weakness, weight loss • ENT sinusitis or rhinitis, nasal crusting and bloody nasal discharge, nasoseptal perforation, saddle nose deformity inflammation/vasculitis involving extra-ocular muscles, retrobulbar space occupying lesions or direct extension of masses from the upper respiratory tract resulting in clinical finding of proptosis hearing loss due to involvement of CN VIII • pulmonary cough, hemoptysis • other joint, skin, eye complaints, vasculitic neuropathy
Toronto Notes 2011
Vasculitides
Rheumatology RH17
Investigations • bloodwork: anemia (normal MCV), increased WBC, increased Cr, increased ESR, ANCA (c-ANCA > p-ANCA) • urinalysis: proteinuria, hematuria • CXR: pneumonitis, lung nodules, infiltrations, cavitary lesions • biopsy: renal (segmental necrotizing glomerulonephritis), lung (tracheobronchial erosion) • possible decline in c-ANCA and ESR used to monitor response to treatment in some patients Treatment • prednisone 1 mg/kg/day PO for 3-6 months ± cyclophosphamide 2 mg/kg/day PO for 3-6 months followed by high dose methotrexate (20-25 mg PO/SC weekly) or azathioprine (2 mg/kg/day PO OD) • consider biologic agents (infliximab, rituximab, IVIG) and plasmapheresis in systemic disease resistant to corticosteroids and cyclophosphamide
Polyarteritis Nodosa (PAN) MEDIUM VESSEL VASCULITIS Definition • pauci-immune necrotizing vasculitis of medium to small vessels, without associated glomerulonephritis or pulmonary capillaritis (as seen in microscopic polyangiitis) • incidence 0.7 per 100,000; affects individuals between 40-60 yrs; M:F = 2:1 Table 17. Classification Criteria: PAN diagnosed if 3 or more of the following 10 criteria present Criteria
Definition
1. Weight loss
>4 kg, not due to dieting or other factors
2. Myalgias, weakness or leg tenderness
Diffuse myalgias or weakness
3. Livedo reticularis
Mottled, reticular pattern over skin
4. Neuropathy
Mononeuropathy, multiple mononeuropathies or polyneuropathy
5. Testicular pain or tenderness
Not due to infection, trauma or other causes
6. Diastolic BP >90 mmHg
Development of hypertension with dBP >90 mmHg
7. Elevated Cr or BUN
Cr >1.5 mg/dL (132.6 µmol/L), BUN >40 mg/dL (14.3 mmol/L)
8. Hepatitis B positive
Presence of Hepatitis B surface antigen or antibody
9. Arteriographic abnormality
Commonly aneurysms
10. Biopsy of artery
Presence of granulocytes and / or mononuclear leukocytes in the artery wall
American College of Rheumatology, 1990
Etiology and Pathophysiology • focal panmural necrotizing inflammatory lesions in small and medium-sized arteries • thrombosis, aneurysm or dilatation at lesion site may occur • healed lesions show proliferation of fibrous tissue and endothelial cells that may lead to luminal occlusion Treatment • prednisone 1 mg/kg/day PO and cyclophosphamide 2 mg/kg/day PO • ± anti-viral therapy to enhance clearance of HBV
There is an association between Hepatitis B surface antigen (HBsAg) positivity and PAN.
Consider PAN in a non-diabetic patient with mononeuritis multiplex.
Giant Cell Arteritis (GCA)/Temporal Arteritis LARGE VESSEL VASCULITIS Table 18. Classification Criteria: GCA diagnosed if 3 or more of the following 5 criteria present Criteria
Definition
1. Age at onset >50 2. New headache 3. Temporal artery abnormality
Temporal artery tenderness or decreased pulse, not due to arteriosclerosis
4. Elevated ESR
ESR >50 mm/hour
5. Abnormal artery biopsy
Mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells
American College of Rheumatology, 1990
GCA Criteria Presence of 3 or more criteria yields sensitivity of 94%, specificity of 91%.
RH18 Rheumatology
Medical Emergency Untreated, GCA can lead to permanent blindness in 20-25% of patients!
Seropositive Rheumatic Disease: Vasculitides/Investigations
Toronto Notes 2011
Signs and Symptoms • temporal headaches ± scalp tenderness due to inflammation of involved portion of the temporal or occipital arteries • sudden, painless loss of vision and/or diplopia due to narrowing of the ophthalmic or posterior ciliary arteries • tongue and jaw claudication (pain in muscles of mastication on chewing) • polymyalgia rheumatica (proximal myalgia, constitutional symptoms, elevated ESR) occurs in 30% of patients • aortic arch syndrome (involvement of subclavian and brachial branches of aorta result in pulseless disease), aortic aneurysm ± rupture Investigations • diagnosis made by clinical suspicion, increased ESR, increased CRP, temporal artery biopsy within 14 days of starting steroids, angiography Treatment • if suspect GCA, immediately start high dose prednisone 1 mg/kg in divided doses, tapering prednisone as symptoms resolve; highly effective in treatment and in prevention of blindness and other vascular complications • consider low dose ASA
Seropositive Rheumatic Disease: Investigations Differential Diagnosis of Elevated ESR • RA, PMR, GCA • Hypoalbuminemia, anemia, multiple myeloma • Bacterial infections • Malignancy ESR (and CRP) is insensitive for PM/DM, AS, scleroderma, SLE, viral infections
Bloodwork, Urinalysis, Synovial Fluid Analysis • general: CBC, BUN, creatinine • acute phase reactants: complement (C3 and C4), fibrinogen, CRP, ferritin, albumin • ESR increases with the increase of acute phase reactants, and chronically, with increase in gamma globulins • C3, C4 often decrease in active SLE • urinalysis to detect disease complications (proteinuria, active sediment) • serology: autoantibodies (see Table 19) • synovial fluid analysis (see Table 25) • radiology (plain film, CT, MRI, ultrasound, bone densitometry, angiography, bone scan) Table 19. Autoantibodies and their Prevalence in Rheumatic Diseases Autoantibody
Disease
Normal
Comments
RF
RA 80% SS 50% SLE 20%
65
Autoantibodies (IgM>IgG>IgA) directed against Fc domain of IgG Present in most seropositive diseases Levels correlate with disease severity in RA Non-specific; may be present in IE, TB, Hep C, silicosis, sarcoidosis
Anti-CCP
RA 80%
ANA
SLE 98% MCTD 95% SS 70-90% CREST 80%
80%
0%
Anti-topoisomerase I Diffuse systemic sclerosis 26-76% (formerly Scl-70)
Specific for CREST variant of systemic sclerosis
0%
c-ANCA
Active Wegener’s >90%
0%
Specific and sensitive
p-ANCA
Wegener’s 10% Other vasculitis
0%
Nonspecific and poor sensitivity (found in ulcerative colitis, polyarteritis nodosa, microscopic polyangiitis, Churg-Strauss, rapidly progressive glomerulonephritis)
Anti-Mi-2
DM 15-20%
Specific but not sensitive
Antibodies against RBCs, WBCs, or platelets
SLE
Perform direct Coomb’s test Test hemoglobin, reticulocyte, leukocyte and platelet count, antiplatelet Abs
Seronegative Rheumatic Disease: Spondyloarthropathies Table 20. A Comparison of the Spondyloarthropathies (inflammatory joint disease of the vertebral column) Feature
AS
PsA
ReA
EA
M:F
5:1
1:1
8:1
1:1
Age of onset
20’s
35-45
20’s
Any
Peripheral arthritis
25%
96%
90%
Common
Distribution
Axial, LE
Any
LE
LE
Sacroiliitis
100%
40%
80%
20%
Dactylitis
Uncommon
Occasional
Common
Uncommon
Enthesitis
Common
Common
Common
Less Common
Skin lesions
Rare
100% Psoriasis
Common Keratoderma
Occasional Pyoderma, Erythema Nodosum
Uveitis
30%
Occasional
20%
Rare
Urethritis
Rare
Occasional
Common
Rare
Aortic Regurgitation
Occasional
Rare
Occasional
Occasional
HLA-B27
90%
40%
80%
30%
LE= Lower extremities
ReA
AS
PsA
EA U-SpA
Figure 11. Spondyloarthropathy Subsets (U-SpA = undifferentiated spondyloarthropathy)
Ankylosing Spondylitis (AS) Definition • chronic inflammatory arthritis involving the sacroiliac joints and vertebrae (see Figure 12) • prototype of the spondyloarthropathies
Criteria
Definition
Clinical 1. Low back pain 2. Limitation of lumbar spine motion 3. Limitation of chest expansion
>3 months, improved by exercise, but not relieved by rest In both sagittal and frontal planes Relative to normal values for age and sex
Radiologic 1. Sacroiliitis on radiographs
Sacroiliitis grade ≥2 bilaterally or grade 3-4 unilaterally
Modified New York Critieria, Arthritis Rheum 1984; 27:361
• • • •
SI Spondylitis Hip Shoulder
Figure 12. Common Sites of Involvement of AS
© Linda Colati
Table 21. Classification Criteria of AS: Definite AS if radiologic criterion and ≥1 clinical criterion are present. Probable AS if 3 clinical criteria alone or if the radiologic criterion is present
RH20 Rheumatology
Seronegative Rheumatic Disease: Spondyloarthropathies
Toronto Notes 2011
Etiology and Pathophysiology • enthesitis (inflammation of tendon or ligament at site of attachment to bone) • inflammation osteopenia erosion ossification osteoproliferation (syndesmophytes) Rule of 2s AS occurs in 0.2% of the general population 2% of HLA-B27 positive individuals 20% of HLA-B27 positive individuals with affected family member
Extra-articular Manifestations of AS 6 As Atlanto-axial subluxation Anterior uveitis Apical lung fibrosis Aortic incompetence Amyloidosis (kidneys) Autoimmune bowel disease (UC)
Consider AS in the differential for causes of aortic regurgitation.
The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) is often used to measure and evaluate patient-reported disease activity in AS.
Epidemiology • M:F = 5:1; females have milder disease which may be under-recognized • 95% of patients have HLA-B27 (9% HLA-B27 positive in general population) Table 22. Types of Back Pain Parameter
Mechanical
Inflammatory
Past History
±
++
Family History
–
+
Onset
Acute
Insidious
Age (years)
15-90
5 cm at T4), cervical (global decrease, often extension first) postural changes: decreased lumbar lordosis + increased thoracic kyphosis + increased cervical flexion = increased occiput to wall distance • peripheral asymmetrical large joint arthritis, most often involving lower limb enthesitis: tenderness over tibial tuberosity, or Achilles tendon and plantar fascia insertions into the calcaneum • extra-articular manifestations ophthalmic: acute anterior uveitis (25-30% patients) renal: amyloidosis and IgA nephropathy gastrointestinal: inflammatory bowel disease cardiac: aortitis, aortic regurgitation, pericarditis, conduction disturbances, heart failure (rare) respiratory: apical fibrosis (rare) neurologic: cauda equina syndrome (rare) Investigations • x-ray of SI joint: “pseudowidening” of joint due to erosion with joint sclerosis bony fusion (late), symmetric sacroiliitis • x-ray of spine: “squaring of edges” from erosion and sclerosis on corners of vertebral bodies leading to ossification of outer fibres of annulus fibrosis (bridging syndesmophytes) “bamboo spine” radiographically • MRI of spine: assess activity in early disease; detection of cartilage changes, bone marrow edema, bone erosions, and subchondral bone changes Treatment • conservative/non-pharmacologic prevent fusion from poor posture and disability through: exercise (e.g. swimming), postural and deep breathing exercises, outpatient PT, smoking cessation • medical NSAIDs glucocorticoids (topical eye drops, local injections) DMARDs for peripheral arthritis (sulfasalazine, methotrexate) biologics for axial and peripheral involvement manage extra-articular manifestations • surgical hip replacement, vertebral osteotomy for marked deformity
Toronto Notes 2011
Seronegative Rheumatic Disease: Spondyloarthropathies
Rheumatology RH21
Prognosis • spontaneous remissions and relapses are common and can occur at any age • function may be excellent despite spinal deformity • favourable prognosis if female and age of onset >40 years • early onset with hip disease may lead to severe disability; may require arthroplasty
Enteropathic Arthritis (EA) • see Gastroenterology, Inflammatory Bowel Disease, G19 • manifestations of ulcerative colitis (UC) and Crohn’s disease (CD) include peripheral arthritis (large joint, asymmetrical), spondylitis, and hypertrophic osteoarthropathy • arthralgia, myalgia, osteoporosis and aseptic necrosis of bone 2º to steroid treatment of bowel inflammation • NSAIDs should be used cautiously as they may exacerbate bowel disease
Both AS and EA feature symmetric sacroiliitis.
Table 23. Comparing Features of Spondylitis vs. Peripheral Arthritis in EA Parameter
Spondylitis
Peripheral Arthritis
HLA-B27 association
Yes
No
Gender
M>F
M=F
Onset before IBD
Yes
No
Parallels IBD course
No
Yes
Type of IBD
UC = CD
CD
Psoriatic Arthritis (PsA) Etiology and Pathophysiology • unclear but many genetic, immunologic and some environmental factors involved (e.g. psoriatic plaque flora, particularly Group A Streptococcus, and trauma) Epidemiology • psoriasis affects 1% of population • arthropathy in 10% of patients with psoriasis • 15-20% of patients will develop joint disease before skin lesions appear Signs and Symptoms • dermatologic well-demarcated erythematous plaques with silvery scale nail involvement: pitting, transverse or longitudinal ridging, discolouration, subungual hyperkeratosis, onycholysis and oil drops • musculoskeletal 5 general patterns asymmetric oligoarthritis (most common – 70%) arthritis of DIP joints with nail changes destructive (mutilans) arthritis (5%) symmetric polyarthritis (similar to RA) sacroiliitis and spondylitis (usually older, male patients) other findings: dactylitis, enthesopathy • ophthalmic conjunctivitis, iritis (uveitis) • cardiac and respiratory (late findings) aortic insufficiency apical lung fibrosis • neurologic cauda equina syndrome • radiologic floating syndesmophytes pencil in cup appearance at IP joints osteolysis, periostitis Treatment • treat skin lesions (e.g. steroid cream, salicylic and/or retinoic acid, tar, UV light) • NSAIDs or intra-articular steroids • DMARDs, biologic therapies to minimize erosive disease (use early if peripheral joint involvement)
Check “hidden” areas for psoriatic lesions (ears, hair line, umbilicus, gluteal cleft, nails).
Risks and Benefits of Tumour Necrosis Factoralpha Inhibitors in the Management of Psoriatic Arthritis: Systematic Review and Meta-analysis of Randomized Controlled Trials J Rheumatol 2008; 35:883-90 Purpose: To evaluate the efficacy and safety of tumour necrosis factor-alpha (TNF-alpha) inhibitors in the management of psoriatic arthritis (PsA). Study Selection: Randomized controlled trials (RCT) of adalimumab, etanercept, and infliximab used in patients with PsA. Results: Six RCT met the inclusion criteria, including 982 patients. All 3 TNF-alpha inhibitors were significantly more effective than placebo on the basis of Psoriatic Arthritis Response Criteria (PsARC) and American College of Rheumatology response criteria ACR20, ACR50, and ACR70 ratings. There were no significant differences between TNF-alpha inhibitors and placebo in the proportions of patients who withdrew for any reason (RR 0.48, 95% CI 0.20-1.18), or withdrawal due to adverse events (RR 2.14, 95% CI 0.73-6.27), serious adverse events (RR 0.98, 95% CI 0.55-1.77), or upper respiratory tract infections (RR 0.91, 95% CI 0.65-1.28). Pooled rates for injection site reactions were significantly higher for adalimumab and etanercept than for placebo (RR 2.48, 95% CI 1.16-5.29), but there was no significant difference in the proportion of patients experiencing infusion reactions with infliximab (RR 1.03, 95% CI 0.482.20) compared to placebo. Indirect analysis did not demonstrate any significant differences between the TNF-alpha inhibitors. Conclusions: TNF-alpha inhibitors are effective treatments for PsA with no important added risks associated with their short-term use. There is still a need for longterm risk-benefit assessment of using these drugs for the management of PsA.
RH22 Rheumatology
Seronegative Rheumatic Disease: Spondyloarthropathies
Toronto Notes 2011
Reactive Arthritis (ReA) Definition • two meanings 1. reactive arthritis: a sterile arthritis following an infection (e.g. rheumatic fever, post viral arthritis etc.) 2. Reactive Arthritis (ReA): formerly known as Reiter’s Syndrome; one of the seronegative spondyloarthropathies in which patients have a peripheral arthritis (of ≥1 month duration) accompanied by one or more extra-articular manifestations that appears shortly after certain infections of the GI or GU tracts Etiology • onset following an infectious episode either involving the GI or GU tract GI: Shigella, Salmonella, Campylobacter, Yersinia species GU: Chlamydia (isolated in 16-44% of ReA cases), Mycoplasma species • acute clinical course 1-4 weeks post-infection lasts weeks to years with 1/3 chronic often recurring spinal involvement persists Look for genetic predisposition (HLA-B27) and infection.
“Can’t see, can’t pee, can’t climb a tree”: Triad of conjunctivitis, urethritis and arthritis is 99% specific (but 51% sensitive) for ReA.
Epidemiology • in HLA-B27 patients, axial > peripheral involvement • M>F Signs and Symptoms • musculoskeletal peripheral arthritis, asymmetric pattern, spondylitis (thick and skipped syndesmophytes), Achilles tendinitis, plantar fasciitis, dactylitis • ophthalmic iritis (anterior uveitis), conjunctivitis • dermatologic keratoderma blenorrhagicum (hyperkeratotic skin lesions on palms and soles) and balanitis circinata (small, shallow, painless ulcers of glans penis and urethral meatus) are diagnostic • gastrointestinal oral ulcers, diarrhea • urethritis and cervicitis sterile cultures; presence not related to site of initiating infection Investigations • diagnosis is clinical plus laboratory • bloodwork: normocytic, normochromic anemia and leukocytosis; sterile cultures • serology: HLA-B27 positive Treatment • antibiotics for non-articular infections • NSAIDs, physical therapy, exercise • local therapy joint protection intra-articular steroid injection topical steroid for ocular involvement • systemic therapy corticosteroids, sulfasalazine, methotrexate (for peripheral joint involvement only) TNF inhibitors for spinal inflammation
Toronto Notes 2011
Crystal-Induced Arthropathies
Rheumatology RH23
Crystal-Induced Arthropathies Table 24. Gout vs. Pseudogout Parameter
Gout
Pseudogout
Gender
M>F
M=F
Age
Middle-aged males Post-menopausal females
Older
Onset of disease
Acute
Acute/insidious
Crystal type
Monosodium urate (MSU) Negative birefringence (yellow when parallel), needle-shaped
Calcium pyrophosphate dihydrate (CPPD) Positive birefringence (blue when parallel), rhomboid-shaped
Distribution
First MTP
Knee, wrist, polyarticular
Radiology
“Holes in bones”
Chondrocalcinosis OA (knee, wrist, 2nd and 3rd MCP)
Treatment
NSAIDS, corticosteroids, colchicine, allopurinol
NSAIDS, corticosteroids
Gout Definition • derangement in purine metabolism resulting in hyperuricemia; monosodium urate crystal deposits in tissues (tophi) and synovium (microtophi)
Hyperuricemia • primary or genetic mostly due to idiopathic renal underexcretion (90%) also idiopathic overproduction or abnormal enzyme production/function • secondary dietary excess underexcretion (>90%) – renal failure, drugs, systemic conditions overproduction (45 years old • extremely rare in premenopausal female Signs and Symptoms • recurrent episodes of acute inflammatory arthritis • acute gouty arthritis severe pain, redness, joint swelling, usually involving lower extremities (see Figure 13) joint mobility may be limited attack will subside spontaneously within several days to weeks; may recur • tophi urate deposits on cartilage, tendons, bursae, soft tissues, and synovial membranes common sites: first MTP, ear helix, olecranon bursae, tendon insertions (common in Achilles tendon) • kidney gouty nephropathy uric acid calculi Investigations • joint aspirate: >90% of joint aspirates show crystals of monosodium urate (see Table 24) (negatively birefringent, needle-shaped) • x-rays may show tophi as soft tissue swelling, punched-out lesions
• 1st MTP=Podagra • Ankle • Knee
Figure 13. Common Sites of Involvement in Gout (asymmetric joint involvement)
An acute gout attack may mimic cellulitis. However, joint mobility is preserved in cellulitis.
Precipitants of Gout Drugs are FACT Furosemide Aspirin/Alcohol Cytotoxic drugs Thiazide diuretics Foods are SALTS Shellfish Anchovies Liver and Kidney Turkey Sardines
The majority of people with hyperuricemia do not have gout, and normal or low uric acid levels do not rule out gout
© Linda Colati
Etiology and Pathogenesis • sources of uric acid: diet and endogenous • synthesis hypoxanthine xanthine uric acid both steps catalyzed by xanthine oxidase
RH24 Rheumatology
Crystal-Induced Arthropathies
Toronto Notes 2011
Treatment • acute gout NSAIDs: high dose, then taper as symptoms improve corticosteroids: intra-articular, oral or intra-muscular (if renal, cardiovascular or GI disease and/or if NSAIDs contraindicated or failed) colchicine within first 24 hours but effectiveness limited by narrow therapeutic range allopurinol can worsen an acute attack (therefore do not start during acute flare) • chronic gout conservative avoid foods with high purine content (e.g. visceral meats, sardines, shellfish, beans, peas) avoid drugs with hyperuricemic effects (e.g. pyrazinamide, ethambutol, thiazide, alcohol) medical antihyperuricemic drugs (allopurinol and febuxostat): decrease uric acid production by inhibiting xanthine oxidase uricosuric drugs (probenecid, sulfinpyrazone): use if failure on or intolerant to allopurinol; do not use in renal failure prophylaxis prior to starting antihyperuricemic drugs (colchicine/low-dose NSAID) in renal disease secondary to hyperuricemia, use low-dose allopurinol and monitor creatinine
Pseudogout Etiology and Pathophysiology • acute inflammatory arthritis due to phagocytosis of IgG-coated calcium pyrophosphate dihydrate (CPPD) crystals by neutrophils and subsequent release of inflammatory mediators within joint space
• • • • •
Knee Polyarticular wrist Hand (MCP) Foot (1st MTP) Hip
Figure 14. Common Sites of Involvement in CPPD
© Linda Colati
Epidemiology • more frequently polyarticular, slower in onset in comparison to gout, lasts up to 3 weeks but is self-limited • risk factors: old age, advanced OA, neuropathic joints • other associated conditions: hyperparathyroidism, hypothyroidism, hypomagnesemia, hypophosphatasia (low ALP), diabetes, hemochromatosis Signs and Symptoms • affects knees, wrists, MCPs, hips, shoulders, elbows, ankles, big toe (see Figure 14) • episodes of acute inflammatory arthritis • may present as chronic arthritis with acute exacerbations • 5% will mimic rheumatoid arthritis (symmetrical polyarticular pattern with morning stiffness and constitutional symptoms) • may be triggered by dehydration, acute illness, surgery, trauma • 50% of the patients will develop degenerative joint changes Investigations • must aspirate joint to rule out septic arthritis, gout • CPPD crystals: present in 60% of patients, often only a few crystals • x-rays show chondrocalcinosis in 75%: radiodensities in fibrocartilaginous structures (e.g. knee menisci) or linear radiodensities in hyaline articular cartilage Treatment • joint aspiration, rest, and protection • NSAIDs – also used for maintenance therapy • prophylactic colchicine PO (controversial) • intra-articular or oral steroids to relieve inflammation
Synovial Fluid Analysis • synovial fluid is an ultrafiltrate of plasma plus hyaluronic acid; it lubricates joint surfaces and nourishes articular cartilage Three Most Important Tests of Synovial Fluid (3 C’s) 1. Cell count and differential 2. Culture and Gram stain (bacteria, mycobacteria, fungi) 3. Crystal examination (microscopy with polarized light) gout (monosodium urate) needle-shaped, negatively birefringent (yellow) pseudogout (calcium pyrophosphate dihydrate) rhomboid-shaped, positively birefringent (blue) • chemistry – protein, LDH, glucose less helpful
Toronto Notes 2011
Crystal-Induced Arthropathies/Pediatric Rheumatology/Non-Articular Rheumatism
Rheumatology RH25
Table 25. Synovial Fluid Analysis Parameter
Normal
Non-Inflammatory
Inflammatory
Infectious
Hemorrhagic
Colour
Clear
Clear
Opaque
Opaque
Sanguinous
Viscosity
High (due to hyaluronic acid)
High
Low
Low
Variable
WBC/mm3
50,000
Variable
% PMN
50%
Variable
Trauma Osteoarthritis Neuropathy Hypertrophic – arthropathy
Seropositives Seronegatives Crystal arthropathies
Septic arthritis
Trauma Hemophilia
Pediatric Rheumatology • see Pediatrics, P95
Non-Articular Rheumatism Definition • disorders that primarily affect soft tissues or periarticular structures • includes bursitis, tendinitis, tenosynovitis, fibromyalgia and polymyalgia rheumatica
Polymyalgia Rheumatica (PMR) Definition • characterized by profound pain and stiffness of the proximal extremities (girdle area) • closely related to giant cell arteritis (15% of patients with PMR develop GCA) • no muscle weakness Table 26. Headley’s Diagnostic Criteria: All of the following are required for diagnosis of PMR
PMR Criteria 1. Age >50 2. Bilateral aching/morning stiffness >1 month 3. ESR >40 mm/hr 4. Prompt response to low-dose corticosteroids
1. Age >50 at onset 2. Pain for >1 month in at least 2 of the following 3 areas: neck, shoulders or pelvic girdle 3. Increased ESR (>40 mm/h) 4. Rapid response to corticosteroids (50, F:M = 2:1 Signs and Symptoms • constitutional symptoms prominent (fever, weight loss, malaise) • pain and stiffness of symmetrical proximal muscles (neck, shoulder and hip girdles, thighs), gel phenomenon (stiffness after prolonged activity) • physical examination reveals tender muscles but no weakness or atrophy Investigations • bloodwork: often shows anemia, elevated platelets, ESR and CRP; normal CK Treatment • goal of therapy: symptom relief • start with steroid dose of 15-20 mg PO daily • taper slowly over 2-year period monitoring ESR and symptoms closely • treat relapses aggressively (50% relapse rate)
Prednisone Plus Methotrexate for Polymyalgia Rheumatica: A Randomized, Double-blind, Placebo-controlled Trial Ann Intern Med 2004; 141:493-500 Study: Multicenter randomized, double-blind, placebo-controlled trial. Patients: Patients with newly diagnosed polymyalgia rheumatica. Intervention: Prednisone dosage (25 mg/d) was tapered to 0 mg/d within 24 weeks and was adjusted if flare-ups occurred. Oral methotrexate (10 mg) or placebo, with folic acid supplementation (7.5 mg), was given weekly for 48 weeks. Primary Outcome: The proportion of patients no longer taking prednisone, the number of flare-ups, and the cumulative prednisone dose after 76 weeks. Results: Twenty-eight of 32 patients in the methotrexate group and 16 of 30 patients in the placebo group were no longer taking prednisone at 76 weeks (P = 0.003). The risk difference was 34 percentage points (95% CI, 11 to 53 percentage points). Similar results were obtained after adjustment for C-reactive protein level and duration of symptoms in a multivariate model. Fifteen of 32 patients in the methotrexate group and 22 of 30 patients in the placebo group had at least 1 flare-up by the end of follow-up (P = 0.04). The median prednisone dose was 2.1 g in the methotrexate group and 2.97 g in the placebo group (P = 0.03). The rate and severity of adverse events were similar. Limitations: Follow-up was short, and a high dose of folic acid and a relatively high starting dosage of prednisone were used. Ten of 72 patients (14%) discontinued treatment or were lost to follow-up. Conclusions: Prednisone plus methotrexate is associated with shorter prednisone treatment and steroid sparing. It may be useful in patients at high risk for steroid-related toxicity.
RH26 Rheumatology
Non-Articular Rheumatism
Toronto Notes 2011
Fibromyalgia Definition • chronic, widespread pain with characteristic tender points Table 27. Classification Critieria for Fibromyalgia: Need criterion 1 and 2. Note, the presence of another clinical disorder does not exclude the diagnosis of fibromyalgia 1. History of widespread pain
At least 3 months in 4 quadrants of the body
2. Pain in ≥11/18 tender point sites
With approximate force of 4 kg by digital palpation See location of tender points in Figure 15
Arthritis Rheum. 1990;33:160-72. A 14-week, Randomized, Double-Blinded, Placebo-Controlled Monotherapy Trial of Pregabalin in Patients With Fibromyalgia J Pain 2008; Jun 2 Study: Multicentre, randomized, double-blinded, placebo-controlled trial. Patients: Patients (n=750) meeting American College of Rheumatology criteria for fibromyalgia and who had a pain score of at least 40 mm on the 100-mm pain visual analog scale (VAS). Intervention: Patients were randomly assigned to placebo or pregabalin (300 mg/d, 450 mg/d, or 600 mg/d) given twice daily in equally divided doses for 12 weeks. Primary Outcome: Change in the mean pain score derived from the subject’s daily pain diary as measured at the patient’s baseline to the end point of the study. Results: Patients in 2 pregabalin treatment groups (450 and 600 mg/d pregabalin) showed a statistically significant improvement in the end point mean pain score compared with placebo-treated subjects (mean difference, -0.50; P = .0147 [450 mg/d] and -0.45, P = .0287 [600 mg/d]). The =30% responder rate was 30% (56/184) in the placebo arm and 42% (76/183) in the 300 mg/d, 50% (94/190) in the 450 mg/d, and 48% (88/188) in the 600 mg/d pregabalin arms (P = .0172, P = .0002, P = .0006, respectively), whereas the =50% responder rate was 15% (28/184) for placebo, 24% (44/183) for 300 mg/d, 27% (52/190) for 450 mg/d, and 30% (57/188) for 600 mg/d (P = .0372, P = .0038, P = .0010, respectively). The number needed to treat (NNT) for the =30% response rate was 9.01 for 300 mg/d, 5.25 for 450 mg/d, and 5.73 for 600 mg/d. The NNT for the =50% responder rate was 11.33 for 300 mg/d, 8.23 for 450 mg/d, and 6.62 for 600 mg/d. Discontinuations due to adverse events were 12%, 16%, 22%, and 26% in placebo and pregabalin 300, 450, and 600 mg/d groups, respectively. The 450 and 600 mg/d groups were significantly different from placebo (P = .0001). Conclusions: Pregabalin at 300 mg/d, 450 mg/d, and 600 mg/d showed statistically significant response rates as compared to placebo although discontinuation rates for the 450 mg/d and 600 mg/d regimens were significantly higher as compared to placebo. Exercise for Treating Fibromyalgia Syndrome Cochrane Database Syst Rev 2007; 4:CDE003786 Study: Cochrane systematic review of 34 randomized controlled trials. Population: 2276 patients diagnosed with fibromyalgia. Intervention: Exercise training including cardiorespiratory endurance, muscle strengthening, and flexibility. Primary Outcome: Global well-being, selected signs and symptoms, and physical function. Result: Aerobic-only excerises improve global well-being, physical function, and possibly pain and tender points. There was insufficient data to evaluate the effect of strength and flexibility on the primary outcomes. Conclusions: Moderate aerobic cardiorespiratory exercise improves function and well-being in patients with FM. Benefits from strength and flexibility require additional research to delineate benefits.
Low cervical Second rib
Occiput Trapezius Supraspinatus
Paired Tender Points Occiput: at suboccipital muscle insertion Low cervical: C5-C7 Trapezius: midpoint of upper border Supraspinatus: above scapular spine near medial border
Medial fat pad of knee
Second rib: 2nd costochondral junction Lateral epicondyle: 2cm below this point Lateral epicondyle Gluteal Greater trochanter
Gluteal: upper outer quadrants Greater trochanter: posterior to trochanteric prominence Knee: at medial fat pad
Figure 15. Characteristic Tender Points in Fibromyalgia
Epidemiology • F:M = 3:1 • primarily ages 25-45, some adolescents • prevalence of 2-5% in general population • overlaps with chronic fatigue syndrome and myofascial pain syndrome • strong association with psychiatric illness Signs and Symptoms • widespread aching, stiffness and reproducible tender points (see Figure 15) • easy fatiguability • sleep disturbance: non-restorative sleep, difficulty falling asleep, and frequent wakening • symptoms aggravated by physical activity, poor sleep, emotional stress • patient feels that joints are diffusely swollen although joint examination is normal • neurologic symptoms of hyperalgesia, paresthesias • associated with irritable bowel or bladder syndrome, migraines, tension headaches, restless legs syndrome, obesity, depression, and anxiety Investigations • bloodwork: includes TSH and ESR; all typically normal unless underlying illness present • serology: do not order ANA or RF unless there is clinical suspicion for a connective tissue disease • laboratory sleep assessment Treatment • conservative education exercise program (walking, aquatic exercises), physical therapy (good posture, stretching, muscle strengthening, massage) stress reduction, CBT biofeedback, meditation, acupuncture may be helpful • medical low dose tricyclic antidepressant (e.g. amitriptyline) for sleep restoration select those with lower anticholinergic side effects SNRI: duloxetine, milnacipran anticonvulsant: pregabalin analgesics may be beneficial for pain that interferes with sleep
Toronto Notes 2011
Common Medications
Rheumatology RH27
Common Medications Table 28. Common Medications for Osteoarthritis Class
NSAIDs
COX-2 Inhibitors
Generic Drug Name
Trade Name
Dosing (PO)
Indications
acetaminophen
Tylenol®
Contraindications
Adverse Effects
500 mg tid
1st line
ECASA ibuprofen diclofenac diclofenac/misoprostol naproxen meloxicam
325-975 mg qid 200-600 mg tid 25-50 mg tid 50-75/200 mg tid 125-500 mg bid 7.5-15 mg OD
2nd line
Advil,® Motrin® Voltaren® Arthrotec® Naprosyn®, Aleve® Mobicox®
GI bleed Renal impairment Allergy to ASA, NSAIDs Pregnancy (T3)
Nausea, tinnitus, vertigo, rash, dyspepsia, GI bleed, PUD, hepatitis, renal failure, HTN, nephrotic syndrome
celecoxib
Celebrex®
200 mg OD
High risk for GI bleed: age >65 hx of GI bleed, PUD
Renal impairment Sulfa allergy (celecoxib) Cardiovascular disease
Delayed ulcer healing Renal/hepatic impairment Rash
Hepatotoxicity Overdose >10 g Potentiates warfarin
Other treatments
Comments
Combination analgesics (acetaminophen + codeine)
Enhanced short term effect compared to acetaminophen alone More adverse effects: sedation, constipation, nausea, GI upset
Intra-articular corticosteroid injection
Short-term (weeks-months) decrease in pain and improvement in function
Intra-articular hyaluronic acid q6months Modest decrease in pain Used for mild-moderate OA of the knees Precaution with chicken/egg allergy Topical NSAIDs
1.5% wt/wt topical diclofenac (Pennsaid®) May use for patients who fail acetaminophen treatment and who wish to avoid systemic therapy
Capsaicin cream
Mild decrease in pain
Glucosamine sulfate ± chondroitin
Limited clinical studies No regulation by Health Canada
Table 29. Disease Modifying Anti-Rheumatic Drugs (DMARDs) Generic Drug Name Trade Name
Dosing
Contraindications
Adverse Effects
COMMONLY USED hydroxychloroquine $
Plaquenil®
400 mg PO OD initially 200-400 mg PO OD maintenance
Retinal disease, G6PD deficiency
GI symptoms, macular damage, neuromyopathy, skin rash
sulfasalazine $
Salazopyrim® Azulfidine® (US)
1000 mg PO bid-tid
Sulfa/ASA allergy, kidney disease, G6PD deficiency
GI symptoms, headache, leukopenia, rash
methotrexate $
Rheumatrex® Folex/Mexate®
7.5-25 mg PO/IM/SC qweekly
Bone marrow suppression, liver disease, Urticaria, GI symptoms, tubular necrosis, significant lung disease, immunodeficiency, myelosuppression, cirrhosis, pneumonitis, oral ulcers pregnancy, EtOH abuse
leflunomide $$
Arava®
10-20 mg PO OD
Liver disease
Kidney/liver disease, infection, hypertension Bleeding, hypertension, decreased renal function, hair growth, tremors
Alopecia, GI symptoms, pulmonary infiltrates, liver dysfunction
NOT COMMONLY USED cyclosporine $$
Neoral®
2.5-3 mg/kg/d divided and given in 2 doses PO
gold (injectable) $
Solganal® Myocrysine®
10 mg IM q1week increasing IBD, kidney/liver disease to response
Rash, mouth soreness/ulcers, proteinuria, marrow suppression
azathioprine $
Imuran®
1-2/5 mg/kg/d PO OD
Kidney/liver disease TPMT deficiency
Pancytopenia, biliary stasis, rash, hair loss, vomiting, diarrhea
cyclophosphamide $
Cytoxan®
1 g/m2/month IV as per protocol
Kidney/liver disease
Cardiotoxicity, GI symptoms, hemorrhagic cystitis, nephrotoxicity, bone marrow suppression, sterility
RH28 Rheumatology
Common Medications/References
Toronto Notes 2011
Table 29. DMARDs (continued) Generic Drug Name Trade Name
Dosing
Mechanism of Action
NEWER DMARDs (Biologics) etanercept $$$
Enbrel®
25 mg biweekly or 50 mg weekly SC
Fusion protein of TNF receptor and Fc portion of lgG
infliximab $$$
Remicade®
3-5 mg/kg IV q 8 weeks
Chimeric mouse/human monoclonal anti-TNF-alpha
anakinra $$$
Kineret®
100 mg SC OD
Interleukin-1 receptor antagonist
adalimumab $$$
Humira®
40 mg SC q 2 weeks
Monoclonal anti-TNF-alpha
abatacept $$$
Orencia®
IV infusion
Costimulation modulator of T-cell activation
rituximab $$$
Rituxan®
2 IV infusions, 2 weeks apart
Causes B-cell depletion, binds to CD20
certolizumab $$$
Cimzia®
400 mg SC q 2 weeks x3 PEGylated monoclonal anti-TNF-alpha then 200 mg SC q 4 weeks
golimumab $$$
Simponi®
50 mg SC q month
Monoclonal anti-TNF-alpha
tocilizumab $$$
Actemra®
4-8 mg/kg IV q 4 weeks
Interleukin-6 receptor antagonist
References ACR. Guidelines for the Medical Management of Osteoarthritis of the Hip — 11/95. ACR. Guidelines for the Medical Management of Osteoarthritis of the Knee — 11/95. ACR. Guidelines for Referral and Management of Systemic Lupus Erythematosus in Adults – 9/99. ACR Subcommittee on Rheumatoid Arthritis Guidelines, 2002. Guidelines for the Management of Rheumatoid Arthritis: 2002 Update. Aletaha D, Neogi T, Silman AJ, et al. 2010 Rheumatoid Arthritis Classification Criteria. Arthritis and Rheumatism 2010;62(9)2569-2581 Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988;31:315-24. Arthritis & Rheumatism 46(2):328-346. Bathon JM, Martin RW, Fleischmann RM, et al. A comparison of etanercept and methotrexate in patients with early rheumatoid arthritis. NEJM 2000;343:1586-93. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). NEJM 1975;292:403-7. Bombardier C, Laine L, Reicin A, et al. Comparison of upper gastrointestinal toxicity of rofecoxib and naproxen in patients with rheumatoid arthritis. The VIGOR Study Group. NEJM 2000;343:1520-28. Bookman AM. Clinical Evaluation of Arthritis. University of Toronto Foundations of Medical Practice Lecture. 2006. Brady OH, Masri BA, Garbuz DS, Duncan CP. 10. Joint replacement of the hip and knee – when to refer and what to expect. CMAJ 2000;163(10):1285-91. Brater DC, Harris C, Redfern JS, Gertz BJ. Renal effects of COX-2-selective inhibitors. Amer J Nephrology 2001;21(1):1-15. CMAJ Clinical Basics Rheumatology Series. Cibere J. 4. Acute monoarthritis. CMAJ 2000;162(11):1577-83. Clark BM 9. Physical & occupational therapy in the management of arthritis. CMAJ 2000;163(8):999-1005. Ensworth S. 1. Is it arthritis? CMAJ 2000;162(7):1011-6. Healey LA. Long-term follow-up of polymyalgia rheumatica: evidence for synovitis. Semin Arthritis Rheum 1984;13:322-8. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus [letter]. Arthritis Rheum 1997;40:1725. Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33:1122-8. Huang SHK. 7. Basics of therapy. CMAJ 2000;163(4):417-23 Klippel JH, Weyand CM, and Wortmann RL. Primer on Rheumatic Diseases, 11th ed. Arthritis Foundation, 1997. Klinkhoff A. 5. Diagnosis and management of inflammatory polyarthritis. CMAJ 2000;162(13):1833-38. Kremer, JM. Rational use of new and existing disease-modifying agents in rheumatoid arthritis. Ann Intern Med 2001:134: 695-706. Lacaille D. 8. Advanced therapy. CMAJ 2000;163(6):721-8. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990;33:1101-7. Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990;33:1088-93. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006;4:295-306. Musculoskeletal Injury; (OPOT). Queen’s Printer of Ontario, June 2000. www.opot.org Ontario Musculoskeletal Therapeutics Review Panel. Ontario Treatment Guidelines for Osteoarthritis, Rheumatoid Arthritis, and Acute Price GE. 6. Localized therapy. CMAJ 2000;163(2):176-83. Puttick MPE. 11. Evaluation of the patient with pain all over. CMAJ 2001;164(2):223-27. Reid G, Esdaile JM. 3. Getting the most out of radiology. CMAJ 2000;162(9):1318-25. Shojania K. 2. What laboratory tests are needed? CMAJ 2000;162(8):1157-63. Smetana, GW and Shmerling RH. Does this patient have temporal arteritis? JAMA 2002; 287:92-101. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23:581-90. Taunton JE, Wilkinson M. 14. Diagnosis and management of anterior knee pain. CMAJ 2001;164(11):1595-601. Tsang I. 12. Pain in the neck. CMAJ 2001;164(8):1182-7. Van der Linden. A proposal for modification of the New York criteria. Arthritis Rheum, 1984; 27:361. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-8. Wade, J.P. 15. Osteoporosis. CMAJ 2001;165(1):45-50. Wing PC. 13. Minimizing disability in patients with low-back pain. CMAJ 2001;164(19):1459-68. Wolfe F, Smythe HA, Yunus MB, et al. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia: report of the multicenter criteria committee. Arthritis Rheum 1990; 33:160-72.
U
Urology Samir Bidnur, Yonah Krakowsky and Deborah Sasges, chapter editors Alaina Garbens and Modupe Oyeumi, associate editors Adam Gladwish, EBM editor Dr. Armando Lorenzo, Dr. Keith Jarvi and Dr. Sender Herschorn, staff editors Basic Anatomy Review. . . . . . . . . . . . . . . . . . . . 2 Abdominal Wall Anatomy of Scrotum Genito-Urinary Tract Anatomy Penis Anatomy Common Presenting Problems. . . . . . . . . . . . . . 3 Hematuria Scrotal Complaints Urinary Retention Dysuria Voiding Dysfunction . . . . . . . . . . . . . . . . . . . . . . 5 Voiding Failure to Store: Urinary Incontinence Failure to Void: Urinary Retention Benign Prostatic Hyperplasia (BPH) Urethral Stricture Neurogenic Bladder Post Obstructive Diuresis (POD) Infectious and Inflammatory Diseases. . . . . . . 10 Urinary Tract Infections (UTI) Recurrent/Chronic Cystitis Interstitial Cystitis (Painful Bladder Syndrome) Acute Pyelonephritis Prostatitis/Prostatodynia Epididymitis and Orchitis Urethritis Urethral Syndrome Stone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Approach to Renal Stones Calcium Stones Uric Acid Stones Struvite Stones Cystine Stones Urological Neoplasms. . . . . . . . . . . . . . . . . . . . 18 Approach to Renal Mass Benign Renal Neoplasms Malignant Renal Neoplasms Carcinoma of the Renal Pelvis and Ureter Bladder Carcinoma Prostatic Carcinoma (CaP) Prostate Specific Antigen (PSA) Testicular Tumours Penile Tumours
Scrotal Mass. . . . . . . . . . . . . . . . . . . . . . . . . . . 27 Varicocele Spermatocele Hydrocele Testicular Torsion Inguinal Hernia Hematocele Penile Complaints. . . . . . . . . . . . . . . . . . . . . . . 29 Peyronie’s Disease Priapism Paraphimosis Phimosis Erectile Dysfunction (ED) Premature Ejaculation Trauma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Renal Trauma Bladder Trauma Urethral Injuries Infertility. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 Female Factors Male Factors Pediatric Urology. . . . . . . . . . . . . . . . . . . . . . . . 36 Congenital Abnormalities Nephroblastoma (Wilm’s Tumour) Cryptorchidism/Ectopic Testes Disorders of Sexual Differentiation Circumcision Enuresis Selected Urological Procedures. . . . . . . . . . . . 40 Bladder Catheterization Cystoscopy Radical Prostatectomy Transurethral Resection of the Prostate (TURP) Extracorporeal Shock Wave Lithotripsy (ESWL) Common Medications. . . . . . . . . . . . . . . . . . . . 43 Antibiotics Erectile Dysfunction Benign Prostatic Hyperplasia Prostatic Carcinoma Continence Agents References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44
Toronto Notes 2011
Urology U1
U2 Urology
Basic Anatomy Review
Toronto Notes 2011
Basic Anatomy Review Above Arcuate Line
Below Arcuate Line
Skin Superficial Fascia External Oblique Internal Oblique Transversus Abdominus Transversalis Fascia Extraperitoneal Fat Peritoneum Inferior Epigastric Artery Skin Superficial Fascia External Oblique Internal Oblique Transversus Abdominus Transversalis Fascia Extraperitoneal Fat Peritoneum
Testicular artery Vas Deferens Pampiniform plexus
External spermatic fascia Cremaster muscle Internal spermatic fascia
Testis
Tunica vaginalis Dartos fascia
Skin
© Bryce Hough
© Hyun Joo Lee 2006
Figure 1. Midline Cross-Section of Abdominal Wall Figure 2. Anatomy of Scrotum
Minor calyx Major calyx Pararenal fat Renal sinus Renal pelvis
Renal vein R Renal artery R Abdominal aorta A IVC IV
Renal cortex Renal medulla Renal papilla Renal column Renal pyramid Renal capsule (Gerota’s fascia)
Ureter
U Ureter G Gonadal artery and vein Internal iliac artery and vein In External iliac artery and vein Ex Internal pudenal artery In Common penile artery C
© Krista Shapton 2010
Male Pelvic Vasculature
Detrusor Trigone Base detrusor Urethral muscularis (internal sphincter, smooth muscle) Periurethral striated muscle Rhabdosphincter (external sphincter, striated muscle)
Uretero-vesicular junction Bladder neck Prostate Prostatic urethra Membranous urethra Bulbar urethra
Posterior urethra Anterior urethra
Spongy (penile) urethra Corpus cavernosum Corpus spongiosum Meatus
© Sandra Tavares 2007
Figure 3. Essential Genito-Urinary Tract Anatomy
Dorsal artery Dorsal vein Corpus cavernosum Deep artery Corpus spongiosum Urethra
Figure 4. Cross Section of the Penis
© June Li 2010
Toronto Notes 2011
Common Presenting Problems
Urology U3
Common Presenting Problems Hematuria Classification (see Nephrology, NP6) Table 1. Etiology of Hematuria by Age Group Age (years)
Etiology (in order of decreasing frequency)
0-20
Glomerulonephritis, UTI, congenital anomalies
20-40
UTI, stones, bladder tumour
40-60
Male: bladder tumour, stones, UTI
Female: UTI, stones, bladder tumour
>60
Male: BPH, bladder tumour, UTI
Female: bladder tumour, UTI
Etiology Table 2. Etiology of Hematuria by Type Pseudohematuria
Pre-renal
Renal
Post-renal
Vaginal bleeding Dyes (beets, rhodamine B in candy and juices) Hemoglobin (hemolytic anemia) Myoglobin (rhabdomyolysis) Drugs (rifampin, phenazopyridine, pyridium, phenytoin) Porphyria Laxatives (phenolphthalein)
Anticoagulants Coagulation defects Sickle cell disease Neoplasms Leukemia Thromboembolism
Stone Trauma Renal cell carcinoma Transitional cell carcinoma Wilm’s tumour Pyelonephritis Glomerulonephritis Interstitial nephritis Tuberculosis Infarct Polycystic kidneys Arteriovenous malformation
Stone Tumour Cystitis Urethritis Polyps Foreign body Urethral stricture
History • full history, inquire about timing of macroscopic hematuria in urinary stream initial: anterior urethra terminal: bladder neck and prostatic urethra total: bladder and/or above Investigations • gross hematuria and symptomatic hematuria require full workup CBC (rule out anemia, leukocytosis), electrolytes, creatinine, BUN urine studies: urinalysis (casts, crystals, cells) culture and sensitivity cytology imaging: CT/IVP to investigate upper tracts (ultrasound alone is not sufficient) cystoscopy to investigate lower tract (possible retrograde pyelogram) • microscopic hematuria defined as more than two red blood cells (RBC) per high-power field (HPF) (see Figure 5) Acute Management of Severe Bladder Hemorrhage • manual irrigation via catheter with normal saline to remove clots • continuous bladder irrigation (CBI) using large (22-26 Fr) 3-way Foley to help prevent clot formation • cystoscopy if bleeding quite active: identify resectable tumours coagulate obvious sites of bleeding • refractory bleeding: continuous intravesical irrigation with 1% alum (aluminum potassium sulfate) solution as needed intravesical instillation of 1% silver nitrate solution intravesical instillation of 1-4% formalin (need general anesthesia) embolization or ligation of iliac arteries cystectomy and diversion rarely
Common urologic causes of hematuria can be grossly classified as: Trauma Infection Tumours Stones
Gross painless hematuria is bladder cancer until proven otherwise.
U4 Urology
Common Presenting Problems
Toronto Notes 2011
>2 RBC/HPF The CUA guidelines advise: Repeat initial urine microscopy if history of urethral trauma, exercise, or menses. Immediate referral to nephrology if any of: proteinuria, creatinine, red cell casts or dysmorphic RBCs
Urinalysis and urine C&S 1. Rule out and treat benign causes (i.e. UTI) 2. If accompanied by dysmorphic RBC, or Cr, evaluate for primary renal disease
If neither 1 or 2, urologic evaluation required Upper Tract Imaging Options
Urothelial Cell Carcinoma (UCC) Risk Stratification
Intravenous Pyelogram – Traditional option and widely available, but use is decreasing. Reasonable sensitivity for UCC, but poor sensitivity for RCC.
HIGH RISK Smoking history Occupational chemical exposure Gross hematuria >40 yrs old Hx of storage voiding symptoms Hx of recurrent UTI’s
Ultrasound – Superior to IVP for evaluation of renal parenchyma and renal cysts. Limited sensitivity for UCC and small renal masses. U/S alone is not sufficient for upper tract imaging. CT – Optimal test for renal parenchyma, calculi and infections, but less available and more expensive than ultrasound. Involves exposure to radiation and intravenous contrast.
LOW RISK 1. Urine cytology 2. Upper tract imaging +ve +ve
Complete evaluation 1. Urine cytology 2. Upper tract imaging 3. Cystoscopy
3. Cystoscopy
Treat UCC
-ve
-ve
+ve -ve
Follow up Urinalysis, cytology, and BP at 6, 12, 24, 36 months
Figure 5. Workup of Asymptomatic Microscopic Hematuria Based on AUA Guidelines
Scrotal Complaints • see Scrotal Mass, U27
Urinary Retention • see Failure to Void, U6
Dysuria Differential Diagnosis Table 3. Differential Diagnosis of Dysuria Infectious
Cystitis, urethritis, prostatitis, epididymitis, orchitis, cervicitis, vulvovaginitis, perineal inflammation/infection, TB, vestibulitis
Neoplasm
Renal cell, bladder, prostate, penis, vagina/vulva, BPH
Calculi
Bladder stone, ureteral stone, kidney stone
Inflammatory
Seronegative arthropathies (reactive arthritis: arthritis, uveitis, urethritis), drug side effects, autoimmune disorders, chronic pelvic pain syndrome (CPPS), interstitial cystitis
Hormonal
Endometriosis, hypoestrogenism
Trauma
Catheter insertion, post-coital cystitis (honeymoon cystitis)
Psychogenic
Somatization disorder, MDD, stress/anxiety disorder
Other
Contact sensitivity, foreign body
Approach • focused history and physical to determine cause (fever, discharge, CVA tenderness, conjunctivitis, back/joint pain) • urine dip, C&S, R&M • any discharge (urethral, vaginal, cervical) should be sent for gonococcus/chlamydia testing; wet mount if vaginal discharge • if suspect infection, may start empiric antibiotic treatment • ± imaging of urinary tract (tumour, stones)
Toronto Notes 2011
Voiding Dysfunction
Urology U5
Voiding Dysfunction • see Gynecology, GY36 for relevant female topics
Voiding • two phases of lower urinary tract function: 1. Storage phase – bladder filling and urine storage accommodation and compliance no involuntary contraction 2. Voiding phase – bladder emptying coordinated detrusor contraction synchronous relaxation of outlet sphincters no anatomic obstruction • voiding dysfunction can therefore be classified as: failure to store – due to bladder or outlet failure to void – due to bladder or outlet • three types of symptoms: storage (formerly known as irritative), voiding (formerly known as obstructive), post-void
Failure to Store: Urinary Incontinence Definition • involuntary leakage of urine Etiology • urgency incontinence: detrusor overactivity: CNS lesion, inflammation/infection (cystitis, stone, tumour), bladder neck obstruction (tumour, stone), BPH decreased compliance of bladder wall: CNS lesion, fibrosis sphincter/urethral problem • stress urinary incontinence (SUI): urethral hypermobility weakened pelvic floor allows bladder neck and urethra to descend with increased intra-abdominal pressure urethra is pulled open by greater motion of posterior wall of outlet relative to anterior wall associated with childbirth, pelvic surgery, aging, levator muscle weakness intrinsic sphincter deficiency (ISD) pelvic surgery, neurologic problem, aging and hypoestrogen state intrinsic sphincter deficiency and urethral hypermobility can co-exist Epidemiology • variable prevalence in women: 25-45% • F:M = 2:1 • more frequent in the elderly, affecting 5-15% of those living in the community and 50% of nursing home residents Table 4. Urinary Incontinence: Types and Treatments Type
Urge
Stress
Overflow
Mixed
Definition
Involuntary leakage of urine Involuntary leakage of urine preceded by a strong, with sudden increases in sudden urge to void intra-abdominal pressure
Involuntary leakage of urine when intravesical pressure exceeds urethral pressure
Urinary leakage associated with urgency and increased intra-abdominal pressure
Etiology
Bladder (detrusor overactivity)
Urethra/Sphincter weakness, Obstruction, neuropathy post-partum pelvic (diabetes, MS, musculature weakness anticholinergic drugs)
Combination of bladder and sphincter issues
Diagnosis
History Urodynamics
History Stress Test (have patient bear down/cough)
History Urodynamics Stress Test
History Urodynamics
Failure to Store Lower Urinary Tract Symptoms (LUTS) (irritative) • Frequency • Urgency • Nocturia • Dysuria Think Frequent Urgent Nighttime Discomfort
Causes of Reversible Urinary Incontinence DIAPERS Delirium Inflammation/Infection Atrophic vaginitis/urethritis Pharmaceuticals/Psychological Excess urine output Restricted mobility/Retention Stool impaction
U6 Urology
Voiding Dysfunction
Toronto Notes 2011
Table 4. Urinary Incontinence: Types and Treatments (continued)
Urge Incontinence Treatment Beware of anticholinergic side effects including delirium and urinary retention.
Type
Urge
Stress
Overflow
Mixed
Treatment
Lifestyle Bladder habit training Botox Medications: Anticholinergics (tolterodine (Detrol®), oxybutynin (Ditropan®), trospium (Trosec®), solifenacin (Vesicare®), TCA’s Neuromodulation
Weight loss, Kegel’s exercises Bulking agents Surgery (slings, TVOT, artificial sphincters)
Lifestyle Catheterization to avoid organ damage Treat underlying cause
Combination of management of urge and stress incontinence
Failure to Void: Urinary Retention
Acute vs. Chronic Retention Acute retention is a medical emergency charaterized by pain and anuria with normal bladder volume and architecture. Acute overdistention can lead to bladder rupture. Chronic retention can be asymptomatic with greatly increased bladder volume and detrusor hypertrophy followed by atony (late).
Etiology • outflow obstruction: bladder neck or urethra – calculus, clot, foreign body, or neoplasm prostate – BPH, prostate cancer, prostatitis urethra – stricture, phimosis, traumatic disruption • bladder innervation: spinal cord – injury, disc herniation, multiple sclerosis stroke DM post-pelvic surgery • pharmacologic: anticholinergics narcotics antihypertensives (ganglionic blockers, methyldopa) over-the-counter cold medications containing ephedrine or pseudoephedrine (e.g. Sudafed®) antihistamines (e.g. Benadryl®, Nytol®, Sominex®) psychosomatic substances (e.g. ecstasy) Clinical Features • palpable and/or percussible bladder (suprapubic) • possible purulent/bloody meatal discharge • DRE – size of prostate, anal sphincter tone • neurological – presence of abnormal deep tendon reflexes, “anal wink”, saddle sensation, etc. Investigations • CBC, electrolytes, Cr, BUN, urine R&M, C&S, ultrasound, cystoscopy, urodynamic studies, post void residual (PVR) scan Treatment • guiding principles are to treat underlying cause of retention and use least invasive treatment possible • catheterization: contraindicated in trauma patient unless urethral disruption has been ruled out acute retention: immediate catheterization to relieve retention, leave Foley in to drain bladder, follow up to determine cause chronic retention: intermittent catheterization by patient is commonly used; definitive treatment depends on etiology • suprapubic cystotomy • for post-operative patients with retention: encourage ambulation alpha-blockers to relax bladder neck may need catheterization definitive treatment will depend on etiology
Toronto Notes 2011
Voiding Dysfunction
Urology U7
Benign Prostatic Hyperplasia (BPH) Definition • hyperplasia of stroma and epithelium in periurethral area of prostate (transition zone) – see Figure 6 • tone of prostatic smooth muscle cells plays a role in addition to hyperplasia Etiology • etiology unknown androgen dihydrotestosterone (DHT) required (converted from testosterone by 5-alpha reductase) possible role of impaired apoptosis, estrogens, other growth factors Epidemiology • age-related, extremely common (50% of 50 year olds, 80% of 80 year olds) • 25% of men will require treatment Clinical Features • result from outlet obstruction and compensatory changes in detrusor function • voiding symptoms: hesitancy, straining, weak/interrupted stream, incomplete bladder emptying decreased flow rates may be seen on uroflowmetry due to outflow obstruction and/or impaired detrusor contractility • storage symptoms: urgency, frequency, nocturia, urgency incontinence thought to be due to detrusor overactivity and decreased compliance • prostate is smooth, rubbery and symmetrically enlarged on DRE • complications: retention overflow incontinence hydronephrosis and renal compromise infection gross hematuria bladder stones Investigations • history assess LUTS and effect on quality of life, may include self-administered questionnaires (AUA symptom and impact score) • physical exam: DRE • urinalysis to exclude UTI • creatinine to assess renal function ± renal ultrasound to assess for hydronephrosis • prostate-specific antigen (PSA) to rule out malignancy (if life expectancy >10 years) • uroflowmetry to measure flow rate (optional) • bladder ultrasound to determine post-void residual urine (optional) • cystoscopy prior to potential surgical management • biopsy if suspicious for malignancy Treatment • conservative for those with mild symptoms: watchful waiting – 50% of patients improve spontaneously includes lifestyle changes (e.g. evening fluid restriction, planned voiding) • medical treatment: a-adrenergic antagonists – reduce stromal smooth muscle tone [e.g. terazosin (Hytrin®), doxazosin (Cardura®), tamsulosin (Flomax®), alfuzosin (Xatral®)] 5-a reductase inhibitor – blocks conversion of testosterone to DHT; acts on the epithelial component of the prostate – reduces prostate size [e.g. finasteride (Proscar®), dutasteride (Avodart®)] combination shown to be synergistic (see sidebar) • transurethral resection of prostate (TURP): see Selected Urological Procedures, U40 • open prostatectomy: for large prostates or associated problems (e.g. bladder stones) suprapubic (transvesically to deal with bladder pathology) retropubic (through the prostatic capsule) • minimally invasive therapy: prostatic stents, microwave therapy, laser ablation, water-induced thermotherapy, cryotherapy, high intensity focused ultrasound (HIFU) and transurethral needle ablation (TUNA)
Anterior fibromuscular area
Transition zone
Urethra Central zone
Urethral zone Peripheral zone
Ejaculatory zone © Meaghan Brierley
Figure 6. Cross-Section of Prostate
AUA Prostate Symptom Score FUNWISE Frequency Urgency Nocturia Weak stream Intermittency Straining Emptying, incomplete feeling of Each symptom graded out of 5. 0-7 – Mildly symptomatic 8-19 – Moderately symptomatic 20-35 – Severely symptomatic Note: Dysuria not included in score but is commonly associated with BPH
Prostate size does not correlate well with symptoms in BPH.
Approximate Prostate Sizes 20 cc – chestnut 25 cc – plum 50 cc – lemon 75 cc – orange 100 cc – grapefruit
Absolute Indications for BPH Surgery • Refractory urinary retention • Recurrent UTIs • Recurrent hematuria refractory to medical treatment • Renal insufficiency (rule out other causes) • Bladder stones
U8 Urology
Voiding Dysfunction
Toronto Notes 2011
Urethral Stricture The Long-Term Effect of Doxazosin, Finasteride, and Combination Therapy on the Clinical Progression of Benign Prostatic Hyperplasia (Medical Therapy of Prostatic Symptoms (MTOPS) Trial) NEJM 2003; 349:2387-2398 Study: Randomized, double-blinded, controlled trial with mean follow-up of 4.5 years. Patients: 3047 patients with symptomatic BPH (AUA symptom score 8 to 35) were randomly assigned to placebo (n=737), doxazosin (n=756), finasteride (n=768), or combination therapy (n=786). Mean age 62.6. Intervention: Conservative treatment vs. doxazosin vs. finasteride vs. combination therapy. Main Outcomes: Clinical progression defined as: first occurrence of an increase over base line of at least four points in the AUA symptom score, acute urinary retention, renal insufficiency, recurrent urinary tract infection, or urinary incontinence. Results: The 6-yr absolute reduction in cumulative incidence of clinical progression of symptomatic BPH compared to placebo for doxazosin was 39% (P3 or 4 episodes per year in females Etiology • unknown: theories: increased epithelial permeability, autoimmune, neurogenic associations: severe allergies, irritable bowel syndrome (IBS), fibromyalgia Treatment • daily low-dose prophylaxis (nitrofurantoin, TMP/SMX) • lifestyle changes (limit caffeine intake, increase fluid/water intake, smoking cessation) • post-menopausal women: consider topical or systemic estrogen therapy • no treatment for asymptomatic UTI except in pregnant women or patients undergoing urinary tract instrumentation
Interstitial Cystitis (Painful Bladder Syndrome) Definition • chronic urgency, frequency ± pain without other reasonable causation Etiology • unknown: theories: increased epithelial permeability, autoimmune, neurogenic, defective glycosaminoglycan (GAG) layer overlying mucosa associations: severe allergies, irritable bowel syndrome (IBS), fibromyalgia Epidemiology • prevalence: ~20/100,000 • 90% of cases are in females • mean age at onset is 40 years Classification • non-ulcerative (more common) – younger to middle-aged • ulcerative – middle-aged to older
Urology U11
U12 Urology
Infectious and Inflammatory Diseases
Toronto Notes 2011
Diagnosis
• required criteria:
glomerulations (submucosal petechiae) or Hunner’s ulcers on cystoscopic examination pain associated with the bladder or urinary urgency negative urinalysis, C&S
Differential Diagnosis
• UTI, vaginitis, bladder tumour • radiation/chemical cystitis • eosinophilic/TB cystitis • bladder calculi
Treatment
• patient empowerment (diet, lifestyle) • pentosan polysulfate (Elmiron®) • low dose amitriptyline • bladder hydrodistention (also diagnostic) under general anesthesia • intravesical dimethylsulfoxide (DMSO) or Cystistat® • surgery (augmentation cystoplasty and urinary diversion ± cystectomy)
Acute Pyelonephritis • see Infectious Diseases, ID21 Definition • infection of the renal parenchyma with local and systemic manifestations Etiology • ascending (usually GN bacilli) or hematogenous route (usually GP cocci) • causative microorganisms: E. coli (most common), Klebsiella, Proteus, Pseudomonas, Enterococcus faecalis, Enterobacter, S. Aureus, S. saphrophyticus • common underlying causes of pyelonephritis: stones, strictures, prostatic obstruction, vesicoureteric reflux, neurogenic bladder, catheters, DM, sickle-cell disease, PCKD, immunosuppression, post-renal transplant, instrumentation, pregnancy Clinical Features • rapid onset (hours – day) • LUTS including frequency, urgency, hematuria • fever, chills, nausea, vomiting, myalgia, malaise • CVA tenderness or exquisite flank pain • dysuria is not a symptom of pyelonephritis without concurrent cystitis Investigations • urine R&M, C&S (see Urinary Tract Infections, U10) • blood CBC + differential: leukocytosis, left shift • imaging – indicated if suspect complicated pyelonephritis or symptoms do not improve with 72 hours of treatment Abdo/pelvic U/S IVP Cystoscopy CT
MacroBID has poor tissue penetration and therefore is not used to treat pyelonephritis (requires post-renal uroconcentration).
Treatment • may treat as outpatient if hemodynamically stable, ciprofloxacin PO x 7-14 days or cotrimoxazole (TMP/SMX) PO x 14 days • severe or non-resolving: admit, hydrate and treat with ampicillin IV and gentamycin IV • emphysematous pyelonephritis: emergency nephrectomy • stone obstruction: admit and emergency stenting or percutaneous nephrostomy tube
Toronto Notes 2011
Infectious and Inflammatory Diseases
Urology U13
Prostatitis/Prostatodynia Prostatic massage may cause extreme tenderness and increased risk of inducing sepsis, abscess or epididymoorchitis.
• most common urologic diagnosis in men 6 hrs has poor prognosis.
U14 Urology
Infectious and Inflammatory Diseases
Toronto Notes 2011
Clinical Features • sudden onset scrotal pain and swelling ± radiation along cord to flank Prehn’s sign: pain may be relieved with elevation of testicles in epididymitis but not in testicular torsion. Poor sensitivity, especially in children.
• scrotal erythema and tenderness
• fever • storage symptoms, purulent discharge • reactive hydrocele Investigations • urinalysis (pyuria), urine C&S • ± urethral discharge: Gram stain/culture • if diagnosis uncertain, must do: colour-flow Doppler ultrasound nuclear medicine scan examination under anesthesia Treatment • rule out torsion • antibiotics: N. gonorrheae or C. trachomatis – cefixime 400 mg PO once followed by azithromycin 1 g single dose or doxycycline 100 mg bid x 10 days coliforms – broad spectrum antibiotics (Septra®, Cipro®) x 14 days • scrotal support, ice, analgesia Complications • if severe testicular atrophy • 30% have persistent infertility problems
Urethritis • common causes: infectious, inflammatory (e.g. reactive arthritis) Table 7. Infectious Urethritis: Gonococcal vs. Non-Gonococcal
Reactive Arthritis (formerly known as Reiter’s Syndrome) Urethritis, Uveitis and Arthritis (can’t pee, can’t see, can’t climb a tree)
Gonococcal
Non-gonococcal
Causative organism
Neisseria gonorrheae
Usually Chlamydia trachomatis
Diagnosis
History of sexual contact, yellow purulent discharge, irritative LUTS Gram stain (GN diplococci), urine PCR and/or culture from urethral specimen
History of sexual contact, mucoid whitish purulent discharge, ± irritative LUTS Gram stain demonstrates >4 PMN/oil immersion field, no evidence of N. gonorrheae, urine PCR and/or culture from urethral specimen
Treatment
Cefixime 400 mg PO once or Ceftrixone 125 mg IM once AND treat for Chlamydia trachomatis
Azithromycin 1 g PO once or doxycycline 100 mg PO bid x 7 days
Urethral Syndrome • dysuria in females with consistently sterile urine cultures or low bacterial counts • some have bacterial urethrocystitis (C. trachomatis or other organisms) and require antimicrobial treatment • treat: tetracycline or erythromycin • rule out: vaginitis, cancer, interstitial cystitis, psychological etiologies
Toronto Notes 2011
Stone Disease
Urology U15
Stone Disease Incidence • prevalance of 2-3% • male:female = 3:1, peak incidence 30-50 years of age • recurrence rate: 10% at one year, 50% at 5 years, 60-80% lifetime Clinical Features • urinary obstruction g upstream distention g pain flank pain from renal capsular distention (non-colicky) severe waxing and waning pain radiating from flank to groin, testis, or tip of penis due to stretching of collecting system or ureter (ureteral colic) • writhing, never comfortable, nausea, vomiting, hematuria (90% microscopic), diaphoresis, tachycardia, tachypnea • occasionally symptoms of trigonal irritation (frequency, urgency) • bladder stones result in: storage and voiding LUTS, terminal hematuria, suprapubic pain • If fever, rule out concurrent pyelonephritis or obstruction Differential Diagnosis of Renal Colic • acute ureteral obstruction (other causes): UPJ obstruction sloughed papillae clot colic from gross hematuria • acute abdominal crisis – biliary, bowel, pancreas, abdominal aortic aneurysm (AAA) • gynecological – ectopic pregnancy, torsion/rupture of ovarian cyst, pelvic inflammatory disease (PID) • pyelonephritis (fever, chills, pyuria) • radiculitis (L1) – herpes zoster, nerve root compression Location of Stones • calyx may cause flank discomfort, recurrent infection or persistent hematuria may remain asymptomatic for years and not require treatment • pelvis tend to cause obstruction at ureteropelvic junction (UPJ) staghorn calculi (renal pelvis and one or more calyces) often associated with infection that will not resolve until stone is cleared • ureter 2.5 cm • Staghorn • UPJ obstruction • Calyceal diverticulum • Cystine stones (poorly fragmented with ESWL)
Investigations • screening labs CBC g elevated WBC in presence of fever suggests infection electrolytes, Cr, BUN g to assess renal function urinalysis: R&M (WBCs, RBCs, crystals), C&S • imaging kidneys, ureters, bladders (KUB) x-ray to differentiate opaque from non-opaque stones (e.g. uric acid, indinavir) 90% of stones are radiopaque CT scan no contrast; good to distinguish radiolucent stone from soft tissue filling defect abdominal ultrasound may demonstrate stone (difficult in ureter) may demonstrate hydronephrosis IVP (not usually done) anatomy of urine collecting system, degree of obstruction, extravasation • cystoscopy for suspected bladder stone • strain all urine g stone analysis • if recurrent stone formers, conduct metabolic studies serum electrolytes, Ca, PO4, uric acid, creatinine and urea PTH if hypercalcemic 24 hour urine x 2 for creatinine, Ca, PO4, uric acid, Mg, oxalate, citrate Treatment – Acute • medical analgesic (Tylenol #3®, Percocet®, Demerol®, morphine) ± antiemetic NSAIDs help lower intra-ureteral pressure (e.g. Ketoralac) alpha-blockers: increase rate of spontaneous passage in distal ureteral stones ± antibiotics for UTI IV fluids if vomiting (note: IV fluids do NOT promote stone passage) • interventional: if obstruction endangers patient (i.e. sepsis, renal failure) ureteric stent (via cystoscopy) percutaneous nephrostomy (image-guided) • admit if necessary – see sidebar Treatment – Elective • medical conservative if stone 2 L/day (3-4 L if cystine) specific to stone type (Table 8)
Toronto Notes 2011
Stone Disease
Urology U17
• interventional kidney stent if stone is 1.5-2.5 cm extracorporeal shockwave lithotripsy (ESWL) if stone 2.5 cm (see sidebar U16) ureter ESWL is the primary modality of treatment ureteroscopy (extraction or fragmentation) if – failed ESWL – ureteric stricture – reasonable alternative for distal 1/3 of ureter open ureterolithotomy (very rare) bladder transurethral cystolitholapaxy remove outflow obstruction (TURP or stricture dilatation)
Efficacy of α-Blockers for the Treatment of Ureteral Stones J. Urol 2007; 1779:983-987 Study: Meta-analysis of prospective randomized trials comparing α-blockers to conservative therapy. Data Sources: MEDLINE (January 1966 to October 2005), the Cochrane Central Search library, EMBASE (1980 to 2005), and the electronic database of abstracts presented at the Annual Meeting of the American Urological Association (2002 to 2005) were searched for literature published in English. Patients: 11 studies met selection criteria (n=911). Treatment ranged from 8 days to 6 weeks. Main Outcome: Incidence of distal ureteral stone expulsion. Results: Administration of an α-blocker with conservative treatment increased incidence of stone expulsion over conservative treatment alone by 44% (95% CI 1.31-1.59, p2 L/day), potassium intake reduce animal protein, oxalate, sodium, sucrose, and fructose intake avoid high-dose vitamin C supplements • medications: thiazide diuretics for hypercalciuria allopurinol for hyperuricosuria potassium citrate for hypocitraturia
Although hypercalciuria is a risk factor for stone formation, decreasing dietary calcium is NOT recommended to prevent stone formation. Low dietary calcium leads to increased oxalate absorption and higher urine levels of calcium oxalate.
Table 8. Stone Classification Type of Stone
Calcium (75-85%)
Uric Acid (5-10%)
Struvite (5-10%)
Cystine (1%)
Etiology
Hypercalciuria Hyperuricosuria (25% of patients with Ca stones) Hyperoxaluria (2 L/day Medical if stone 5 mm or presence of Calcium struvite – antibiotics (stone complications (see U16) must be removed to treat infection) Treatment
U18 Urology
Urological Neoplasms
Toronto Notes 2011
Urological Neoplasms Approach to Renal Mass Ultrasound
Cystic
Solid
Hypoechoic No calcification Thin wall
Dense Calcified Septated
Stop
CT* Angiography Possible aspiration or biopsy
Surgery
CT (exclude angiomyolipoma)
Surveillance
Large mass (>1.5 cm)
Small mass (M 20% associated with tuberous sclerosis (especially if multiple, recurrent)
3-7% of renal tumours. More common in males
Incidence increases with age Found in 7-23% of all autopies M:F = 3:1
Characteristics
Clonal neoplasm consisting of fat, smooth muscle and blood vessels May extend into renal vein and become symptomatic
Spherical, capsulated with possible central scar Histologically organized aggregates of eosinophilic cells originating from intercalated cells of collecting duct
Small cortical lesions 3cm due to increased risk of metastasis
Malignant Renal Neoplasms RENAL ADENOCARCINOMA [Renal Cell Carcinoma (RCC)] Etiology • cause unknown • originates from proximal convoluted tubule epithelial cells • risk factors: smoking (results in 2x increased relative risk), cadmium exposure, employment in leather industry • familial incidence seen with von Hippel-Lindau syndrome Epidemiology • eighth most common malignancy (accounts for 3% of all newly diagnosed cancers) • 85% of primary malignant tumours in kidney • male:female = 3:1 • peak incidence at 50-60 years of age Pathology • histological subtypes: clear, granular, spindle cell, papillary, chromophobe Clinical Features • usually asymptomatic – frequently diagnosed incidentally by U/S or CT • poor prognostic indicators: weight loss, weakness, anemia, bone pain • local effects: classic “too late triad” found in 10-15%: gross hematuria 50% flank pain
