Radiologic Pathology: 2006-2007

Radiologic Pathology Fifth Edition VOLUME 1 Chest, Gastrointestinal, and Genitourinary Radiologic Pathology Correlation...

Author: Ellen M. Chung | Jeffrey R. Galvin | Kelly K. Koeller | Mark D. Murphey | Paula J. Woodward Angela D. Levy

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Radiologic Pathology Fifth Edition

VOLUME 1 Chest, Gastrointestinal, and Genitourinary Radiologic Pathology Correlation Editors

Angela D. Levy, COL, MC, USA Chairman and Registrar Chief, Gastrointestinal Radiology

Ellen M. Chung, LTC, MC, USA Chief, Pediatric Radiology

Jeffrey R. Galvin, MD Chief, Chest Radiology

Kelly K. Koeller, MD Chief, Neuroradiology

Mark D. Murphey, MD

Six Week Course Director Chief, Musculoskeletal Radiology

Paula J. Woodward, MD

Chief, Genitourinary Radiology

Associate Editor Jean-Claude Kurdziel, MD

2006 2007

Illustrators Aletta A. Frazier, MD Dianne D. Engelby, MAMS, RDMS Heike Blum, MFA

Department of Radiologic Pathology Armed Forces Institute of Pathology Washington DC, USA

American Registry of Pathology Armed Forces Institute of Pathology Washington, DC 20306-6000 _____________________________________

© Copyright 2006 by the American Registry of Pathology.

All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means: electronic, mechanical, photocopy, recording, or any other information storage and retrieval system without written permission of the publisher. Made in the United States of America _____________________________________

Great care has been taken to guarantee the accuracy of the information contained in this volume. However, neither the American Registry of Pathology, Armed Forces Institute of Pathology, nor the editors and contributors can be held responsible for errors or for any consequences arising from the use of the information contained herein.

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as representing the views of the Departments of the Army, Air Force, Navy, or Defense. 987654321

Library of Congress Cataloging-in-publication Data [in process] ISBN 1-933477-00-8

Preface

The Armed Forces Institute of Pathology’s Radiologic Pathologic Correlation course presented by the Department of Radiologic Pathology enters its 59th year of educating radiology residents worldwide. For the fifth year, our staff and visiting lecturers have contributed their lecture material and images to compile Radiologic Pathology 2006 – 2007, continuing the tradition of presenting richly illustrated material that teaches the pathologic basis of disease to improve our understanding of the imaging appearance of disease. We hope the efforts of our authors and editors have once again accomplished our goal of bringing the outstanding and unique Radiologic Pathologic Correlation course to your fingertips.

Acknowledgements The annual production of the Radiologic Pathologic Correlation course and syllabus is made possible through the tremendous support, dedication, and selfless service of countless individuals who work in the AFIP and the various institutions and organizations throughout the world that believe in the importance of teaching the principles of disease through radiologic pathologic correlation.

The Department of Radiologic Pathology of the Armed Forces Institute of Pathology expresses our deepest appreciation and sincerest gratitude to: - All radiologists and radiology residents who have contributed case material to the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of Pathology, - All pathologists in the AFIP who have donated their time and expertise to radiologic pathologic correlation, - All of our outstanding authors, illustrators, and department staff members who make the course and the syllabus happen effortlessly year after year, - And, to the extraordinary efforts of our production team, headed by JeanClaude Kurdziel, MD, who have tirelessly dedicated the spring and summer of the last five years to the production of this syllabus.

iii

Faculty – VOLUME 1 Chest Radiology

Marc S. Levine, MD

Jeffrey R. Galvin, MD

Professor of Radiology Hospital of the University of Pennsylvania Advisory Dean University of Pennsylvania School of Medicine Philadelphia, PA and Former Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Chief, Pulmonary and Mediastinal Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Professor of Radiology and Pulmonary Medicine University of Maryland Baltimore, MD

Gerald F. Abbott, MD

Director of Chest Radiology Rhode Island Hospital and Assistant Professor of Radiology Brown University School of Medicine Providence, RI

Deborah Rubens, MD Professor and Associate Chair Department of Imaging Sciences University of Rochester Medical Center Rochester, NY and Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Aletta A. Frazier, MD

Staff Radiologist and Medical Illustrator Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Clinical Associate Professor of Radiology University of Maryland School of Medicine Baltimore, MD

Francis J. Scholz, MD

Staff Radiologist Lahey Clinic Medical Center Burlington, MA and Clinical Professor of Radiology Tufts University School of Medicine Boston, MA

Leonard M. Glassman, MD

Washington Radiology Associates, PC Washington, DC and Clinical Professor Department of Radiology George Washington University Medical Center Washington, DC

Robert K. Zeman, MD

Chairman and Professor of Radiology George Washington University Washington, DC

Genitourinary Radiology

Melissa L. Rosado de Christenson, MD, FACR Clinical Professor of Radiology The Ohio State University Columbus, OH and Adjunct Professor of Radiology Uniformed Services University of the Health Sciences Bethesda, MD


Acting Chief, Genitourinary Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Adjunct Professor of Radiology University of Utah School of Medicine Salt Lake City UT

Rosita M. Shah, MD

Clinical Associate Professor of Radiology Hospital of the University of Pennsylvania Philadelphia, PA

Peter L. Choyke, MD

Gastrointestinal Radiology

Chief Molecular Imaging Program National Cancer Institute Bethesda, MD and Professor of Radiology and Nuclear Medicine Uniformed University of the Health Sciences Bethesda, MD

Angela D. Levy, COL, MC, USA

Chairman and Gastrointestinal Radiology Section Chief Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Associate Professor of Radiology and Nuclear Medicine Uniformed Services University of the Health Sciences Bethesda, MD

William D. Craig, MD

Chief, Genitourinary Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Bruce P. Brown, MD

Associate Professor of Radiology University of Iowa Iowa City, IA iv

David S. Hartman, MD

Professor of Radiology Department of Radiology Pennsylvania State University M. S. Hershey Medical Center Hershey, PA

Deborah J. Rubens, MD Professor and Associate Chair Department of Imaging Sciences University of Rochester Medical Center Rochester, NY and Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC Brent J. Wagner, MD

Chairman, Department of Radiology The Reading Hospital and Medical Center West Reading Radiology Associates West Reading, PA

Jade J. Wong-You-Cheong, MD

Associate Professor of Diagnostic Radiology Director of Ultrasound University of Maryland School of Medicine Baltimore, MD

v

Table of Contents – VOLUME 1 Chest Radiology Jeffrey R. Galvin, MD

An Approach to Diffuse Lung Disease, Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .3 The Idiopathic Interstitial Pneumonias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .14 Airways Disease: The Movement from Anatomic to Physiologic Assessment . . . . . . . . . . . . . . . . . . . . . . . .26 Inhalational Lung Disease (Asbestosis and Silicosis) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .44 Pulmonary Lymphoid Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .54 Angiitis and Granulomatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .63 The Pulmonary Complications of Organ Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .75 The Diagnosis of Pulmonary Embolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .82 Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .93 Fungal Disease in the Thorax: Opportunistic and Primary Pathogens . . . . . . . . . . . . . . . . . . . . . . . . . . . . .100 Bronchogenic Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .110 Chest Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .121 Chest Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .126

Aletta A. Frazier, MD

Pulmonary Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .131 Pulmonary Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .138

Melissa L. Rosado de Christenson, MD, FACR

Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148 Chest Seminar: Where is the lesion? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .168 Chest Seminar: Differential Diagnosis of Mediastinal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .173

Rosita M. Shah, MD

Pneumonia: Usual and Unusual Organisms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .178

Gerald F. Abbott, MD

Uncommon Malignant Tumors of the Lung . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .192 Benign Tumors of the Lung and Tumor-like Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .199 Pleural Disease I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .205 Pleural Disease II and Chest Wall . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .213

Leonard M. Glassman, MD (Mammography)

Classic Breast Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .220 Basic Breast Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .229 Ductal Carcinoma in Situ (DCIS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .238 Breast Abnormalities in Young Women . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .246 The Male Breast . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .257

Gastrointestinal Radiology Angela D. Levy, COL, MC, USA

Benign Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .267 Malignant Hepatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .275 Hepatic Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .284 Imaging of Chronic Liver Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .293 Benign Biliary Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .303 Biliary Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .313 Pancreatic Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .321 Gastric Malignancies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .332 Abdominal Non Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .344 Small Intestinal Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .353 Colorectal Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .361 Mesenteric Masses and Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .372 Idiopathic Inflammatory Bowel Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .382 Approach to Inflammatory Diseases of the Colon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .391 GI Seminar 1: Abdominal Gas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .400 GI Seminar 2: Nonneoplastic Disease of the Stomach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .405 GI Seminar 3: Pancreatic Duct . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .411 GI Seminar 4: Hepatic Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .417 GI Seminar 5: Complications of Meckel Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .422 vi

GI Seminar 6: Beyond Appendicitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .427 GI Seminar 7: Tumors and Tumor-Like Lesions of the Gallbladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .432

Robert K. Zeman, MD

Cholelithiasis and Cholecystitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .438

Marc S. Levine, MD

Inflammatory Diseases of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .444 Tumors of the Esophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .450 Radiology of Peptic Ulcer Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .455

Bruce Brown, MD

Pancreatitis: Imaging Has Made a Difference . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .460 Gastrointestinal Bleeding In The Age of the Endoscope. What Does a Radiologist Have To Contribute? . .468

Francis J. Scholz, MD

Small Bowel Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .475 Acute Mesenteric Ischemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .487 Malabsorption . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .505 Familial Polyposis and Other Such . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .519

Deborah J. Rubens, MD

The Spleen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .531 Portal Venous Doppler . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .542

Genitourinary Radiology Paula J. Woodward, MD

Imaging of Uterine Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .551 Approach to Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .561 Urinary Tract Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .573 Retroperitoneum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .579 Radiologic Evaluation of the Scrotum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .585 First Trimester Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .594 Fetal CNS Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .602 Fetal Body Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .607

Peter L. Choyke, MD

Cystic Diseases of the Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .614 Imaging of Prostate Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .620

William D. Craig, MD

Radiographic Evaluation of Urinary Stone Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .624

Deborah J. Rubens, MD

Testicular Torsion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .630

Brent J. Wagner, MD

Imaging of Ovarian Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .637 Adrenal Imaging in Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .645 Imaging of the Urinary Bladder and Urethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .649

Jade Wong You Cheong, MD

Non-Neoplastic Disorders Of The Ovary And Adnexae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .653 Imaging of Solid Organ Transplants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .664

David S. Hartman, MD

The Neglected Nephrogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .674 Problem Renal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .681


GU Seminar 1: MSAFP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .691 GU Seminar 2: Renal Calcifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .693

vii

Chest Radiology

An Approach to Diffuse Lung Disease, Sarcoidosis Jeffrey R. Galvin, MD

Describing Diffuse Lung Disease • •

The Alveolar vs. Interstitial Problem Alveolar or Interstitial ?

•

Radiograph ➢ Lung volumes ➢ Opacity ➢ Distribution ➢ Ancillary findings Computed tomography ➢ Opacity ➢ Distribution

An Approach to Diffuse Lung Disease

•

Figure 1-1-2

Figure 1-1-1

Radiology and pathology form a continuum of visualization Fibrosis results in reduced lung volumes

Lung Volumes •

•

Reduced [Figure 1-1-2] ➢ Pathology distal to the airway ➢ Fibrosis ➢ IPF, asbestosis, sarcoidosis, chronic hypersensitivity pneumonitis Increased [Figure 1-1-3] ➢ Pathology of the airway ➢ Emphysema, asthma, bronchitis, constrictive bronchiolitis, LAM

Figure 1-1-3

Airways disease results in increased lung volumes Chest Radiology

3


Distribution: Upper vs Lower Plain Film and CT Opacities

Figure 1-1-4

[Figure 1-1-4]

• • • • •

Nodules Reticulation and Lines Ground glass Consolidation Cystic airspaces

•

Nodules ➢ Sarcoid ➢ Silicosis, coal-workers ➢ Hypersensitivity Pneumonitis ➢ Metastasis Reticulation and Lines ➢ Fibrosis ✧ IPF-lower, subpleural ✧ Asbestosis-lower, subpleural ✧ Sarcoidosis-peribronchovascular ✧ Chronic hypersensitivity pneumonitis-mid and upper lung zone Ground glass [Shah and Miller AJR 2003] ➢ Non-specific ✧ Airspace, interstitial, combined – DIP, NSIP, AIP, DAD (32%) ✧ Infection (32%) ✧ Drug toxicity (11%) ✧ Hemorrhage (3%) ✧ Ground glass with reticulation Chronic diseases tend to involve the upper lung – Fibrosis Consolidation ➢ Organizing Pneumonia (BOOP) ➢ Chronic eosinophilic pneumonia ➢ Lymphoma ➢ Bronchoalveolar cell carcinoma ➢ Infection ➢ Hemorrhage Cystic airspaces ➢ Mimics reticulation on plain radiographs ➢ Fibrosis and honeycombing ✧ IPF-Lower, subpleural ✧ LAM-Diffuse ✧ LCH-Upper

Plain Film and CT Opacities

•

•

•

•

Radiologic-Pathology Continuum Anatomy - Secondary Lobule •

•

As defined by Miller ➢ Polygonal ➢ 1-2.5 cm ➢ Smallest unit demarcated by connective tissue septa Most useful diagnostically ➢ Readily identified on: ✧ HRCT ✧ Gross examination ✧ Histologic section ➢ Explains HRCT appearance ✧ Broad range of lung diseases ✧ Especially interstitial disease


4

Chest Radiology

Anatomy - Secondary Lobule [Figure 1-1-5] •

• •

Core structures ➢ Axial interstitium ➢ Bronchiole ➢ Pulmonary artery ➢ Lymphatics Septal structures ➢ Peripheral interstitium ➢ Pulmonary veins ➢ Lymphatics Parenchyma ➢ Alveolar interstitium ➢ Alveoli ➢ Pulmonary capillary bed

Figure 1-1-5

The secondary lobule with lymphatics in the interloblular septa and along the bronchovascular bundle

Figure 1-1-6

Septal pattern on HRCT and gross specimen Chest Radiology

5


Abnormal Patterns •

• • •

•

Bronchovascular ➢ Bronchus ✧ Asthma, CF, bronchitis, bronchiectasis [Figure 1-1-7] ➢ Lymphatic ✧ CA, lymphoma, sarcoidosis ✧ Edema Centrilobular [Figure 1-1-8] ➢ Airway related Panlobular [Figure 1-1-9] ➢ Nonspecific Septal ➢ Lymphatic ✧ CA, lymphoma, sarcoidosis [Figure 1-1-10and 1-1-11]

Edema Random [Figure 1-1-12] ➢ Hematogenous spread of tumor ➢ TB ✧

Sarcoidosis • • • •

• •

Figure 1-1-7

Bronchovascular pattern

Figure 1-1-9

Figure 1-1-8

Centrilobular pattern

Figure 1-1-10

Multisystem granulomatous disorder Unknown etiology Young and middle aged adults Bilateral hilar lymphadenopathy, pulmonary infiltration, eye and skin lesions Clinical and radiologic findings supported by evidence of noncaseating epithelioid granulomas Exclusion of granulomas of unknown cause and local sarcoid reactions

ATS Statement on Sarcoidosis 1999

Sarcoidosis: Epidemiology •

• •

•

Worldwide ➢ both sexes, all races, all ages Predilection for adults ➢ under 40 years ➢ peak 20-29 years U.S. prevalence ➢ 10 per 100,000 exams Highest disease ➢ African-American women

Panlobular pattern

Figure 1-1-11

Septal pattern

Figure 1-1-12

Sarcoidosis: Clinical Features •

• •

• •

•

Asymptomatic ➢ 15-50% Constitutional symptoms ➢ 33% Dyspnea, cough, chest pain ➢ 33-50% Palpable lymph nodes ➢ 33-75% Ocular involvement ➢ 11-83% Cutaneous involvement ➢ 20-30% Erythema nodosum, Lupus pernio


Combined septal and bronchovascular pattern 6

Random nodule pattern Chest Radiology

Sarcoidosis: Laboratory Abnormalities •

• • • • •

Figure 1-1-13

BAL ➢ ↑ macrophages, ↓ proportions; ↑ CD4 helper cells Angiotensin-Converting Enzyme ➢ Nonspecific Produced by granuloma/macrophage ➢ ↑ 33-90% Hypercalcemia 10% Hypercalciuria 30% ➢ Macrophage/granuloma extrarenal sources of 1-25 Dihydroxyvitamin D Anergy Hypergammaglobulinemia

Sarcoidosis: Respiratory System [Figure 1-1-13]

• •

•

100% lung involvement Portal of entry ➢ Local lymph nodes ➢ Distant organs Disease distribution ➢ Alveolar wall ➢ Secondary lobule, ➢ Axial CT ➢ Radiograph

Non-Caseating Granuloma and Fibrosis

Sarcoidosis pathogenesis

Alveolar Distribution

Sarcoidosis and the Secondary Lobule [Figure 1-1-14]

Figure 1-1-14

Bronchovascular distribution of granulomas in Sarcoidosis

Chest Radiology

7


Figure 1-1-15

Distribution of nodules in sarcoidosis

Masses in Sarcoidosis

Ground glass in Sarcoidosis

Conglomerate masses and fibrosis in sarcoidosis

Sarcoidosis: Computed Tomography [Figure 1-1-15] • • • •

• • •

Nodules Masses Ground Glass Fibrosis ➢ Conglomeration ➢ Distortion Emphysema Bulla Honeycombing


8

Chest Radiology

Parenchymal Disease: Radiography • • • • • • • • •

Bilateral Symmetrical Nodules Reticulonodular Masses Ground Glass Hilar Retraction Bulla Honeycombing

• • • • • • •

Node Group Hilar R. Paratracheal A-P Window Subcarinal Ant. Med. Post. Med.

Sarcoidosis: Adenopathy CXR 84 76 72 12 12 0

Figure 1-1-16

[Figures 1-1-16 and 1-1-17]

CT 88 100 92 64 48 16

Lymph node involvement is a hallmark of sarciodosis

Sarcoidosis: Staging based on Adenopathy and Parenchyma Stage 0 ➢ Normal Stage 1 ➢ Adenopathy Stage 2 ➢ Adenopathy & Parenchyma Stage 3 ➢ Parenchyma

Presentation 8

Resolution –

51

65

12

20

29

Figure 1-1-17

49

20% develop fibrosis or Stage 4 disease

Sarcoidosis Stage I

Sarcoidosis Stage II

Sarcoidosis Stage III

Sarcoidosis Stage IV

Bilateral calcified lymph nodes are common

Sarcoidosis Progression

Sarcoidosis and the Parenchyma: Computed Tomography • •

• • •

Thickened Bronchovascular Bundles Nodules ➢ Peribronchovascular ➢ Pleural, subpleural and septal Consolidation and Large Nodules Ground-Glass Opacities Fibrosis

Chest Radiology

9


Thickened Bronchovascular Bundles [Figure 1-1-18] Figure 1-1-18

Peribronchovascular opacities in sarcoidosis

Peribronchovascular Nodules

Peribronchovascular and Pleural Nodules Septal Lines

Ground Glass Opacities

Consolidation and Large Nodules Fibrosis

Bronchovascular Bundle Distortion [Figure 1-1-14] Conglomerate Mass

Fibrosis and Emphysema

Fibrosis and Honeycombing Sarcoidosis: Diagnosis • • • •

Typical clinical and radiologic manifestations Non-caseating granulomas Transbronchial Bx Endobronchial Bx

•

Infection ➢ Tuberculosis, Fungal (Histoplasmosis) Pneumoconiosis ➢ Silica, Beryllium Hypersensitivity Pneumonitis Malignancy ➢ Lymphoma

Sarcoidosis: Differential Diagnosis • • •


10

Chest Radiology

Miliary Tuberculosis

Transbronchial Spread of Tuberculosis Histoplasmosis Silicosis

Berylliosis

Extrinsic Allergic Alveolitis Sarcoidosis: Mortality • • • •

Mortality range 5-10% Cor Pulmonale related to fibrosis Cardiac Arrhythmia Pulmonary Hemorrhage ➢ Aspergilloma

Cor Pulmonale

Sarcoidosis: Cardiac Involvement •

• •

Clinical involvement 5% ➢ Heart block, arrhythmia, mitral regurgitation, CHF (dilated cardiomyopathy) and sudden death Autopsy involvement 20-30% Localized wall motion abnormalities ➢ Anterior and apical ➢ MRI, Echocardiograph, Thallium-201

Vignaux AJR 184 Jan 2005

Cardiac Sarcoidosis [Figure 1-1-19] Figure 1-1-19

Sarcoid infiltration on MRI is represented as focal zones of increased signal on T2 and early gadolinium images

Dilated Cardiomyopathy

Chest Radiology

11


Sarcoidosis: Mycetoma •

• •

Figure 1-1-20

Present in 40-50% of cystic lesions ➢ Bullae, cavities or bronchiectasis Hemorrhage Steroids may convert to invasive process

Mycetoma [Figure 1-1-20]

Sarcoidosis: Therapy • • •

• • •

Cardiac, CNS, eye involvement Hypercalcemia Corticosteroids ➢ Relief of symptoms; resolution of radiologic abnormalities; improved function Cytotoxic agents ➢ Methotrexate, Azathioprine Chlorambucil, cyclophosphamide, antimalarials Risk of recurrence

Sarcoidosis: Resolution Sarcoidosis: Prognosis •

•

Favorable ➢ Acute onset, erythema nodosum, ➢ > 80% spontaneous remission ➢ Löfgren syndrome ➢ Low stage Poor ➢ Chronic course, Lupus pernio ➢ Older age at presentation ➢ Hypercalcemia/nephrocalcinosis ➢ Black race, Extrathoracic involvement

Mycetoma in a cystic space caused by sarcoidosis

Sarcoidosis Conclusion References

General 1. Akira M, Hara H, Sakatani M. Interstitial lung disease in association with polymyositis- dermatomyositis: longterm follow-up CT evaluation in seven patients. Radiology 1999; 210(2):333-8. 2. Bergin CJ, Muller NL. CT of interstitial lung disease: a diagnostic approach. American Jounal of Roentgenology 1987; 148:8-15. 3. Bergin C, Roggli V, Coblentz C, Chiles C. The secondary pulmonary lobule:normal and abnormal CT appearances. American Journal of Roentgenology 1988; 15:21-25. 4. Epler GR, McLoud TC, Gaensler EA, Mikus JR Carrington CB. Normal chest roentgenograms in chronic diffuse infiltrative lung disease. N Engl I Med 1978:298(17):934-9. 5. Epler GR. Chest films: underused tool in interstial lung disease. Journal of Respiratory Diseases 1987; 8(6):1 4-24. 6. Felson B. A new look at pattern recognition of diffuse pulmonary disease. American Journal of Roentgenology 1979; 133:183-189. 7. Calvin JR. Mon M, Stanford W. High-resolution computed tomography and diffuse lung disease. Curr Probl Diagn Radiol 1992; 21(2):31-74. 8. Grenier P. Valeyre D, Cluze I R Brauner MW, Lenoir 5, Chastang C. Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high- resolution CT. Radiology 1991; 179:123-132. 9. Gruden JF, Webb WR, Naidich DR, McGuinness G. Multinodular disease: anatomic localization at thin-section CT—multireader evaluation of a simple algorithm. Radiology 1999; 210(3):711-20. 10. Gurney JW, Schroeder BA. Upper lobe lung disease: physiologic correlates. Radiology 1988; 167:359-366. 11. Heitzman ER. The lung. Second ed. St. Louis: C.V. Mosby, 1984. 12. Johkoh T, Muller NL, Cartier Y, Kavanagh PV, Hartman TE, Akira M, lchikado K, Ando M, Nakamura H. Idiopathic interstitial pneumonias: diagnostic accuracy of thin-section CT in 129 patients. Radiology 1999; 211 (2):555-60. 13. Mathieson JR. Mayo JR. Staples CA, Muller NL. Chronic diffuse infiltrative lung disease: comparison of dianostic accuracy of CT and chest radiography. Radiology 1989; 171:111-116. An Approach to Diffuse Lung Disease

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14. Mayo JR. Webb WR, Gould R, Stein MG, Bass I, Gamsu G, Goldberg H. High- resolution CT of the lungs: an optimal approach. Radiology 1987; 163:507-510. 15. McLoud TC, Carrington CB, Gaensler EA. Diffuse Infiltrative lung disease: a new scheme for description. Radiology 1983; 149(2):353-363. 16. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part 2. Am Rev Respir Dis 1990; 142(6 Pt 1 ):1440-8. 17. Muller NE, Miller RR. Computed tomography of chronic diffuse infiltrative lung disease. Part lAm Rev Respir Dis 1990; 142(5):1206-15. 18. Muller NE, Coiby TV. Idiopathic interstitial pneumonias: high-resolution CT and histologic findings. Radiographics 1997; 17(4): 1016-22. 19. Murata K, Itoh H, Todo G, Kanaoka M, Noma 5, Itoh T, Furuta M, Asamoto H, Torizuka K. Centrilobular lesions of the lung: demonstration by high-resolution CT and pathologic correlation. Radiology 1986; 161 :641-645. 20. Murata K, Khan A, Rojas KA, Herman PG. Optimization of computed tomography technique to demonstrate the fine structure of the lung. Investigative Radiology 1988; 23:170-175. 21. Murata K, Khan A, Herman R Pulmonary parenchymal disease: evaluation with high-resolution CT. Radiology 1989; 170:629-635. 22. Muller NI, Miller RR. Computed tomography of chronic diffuse lung disease. American Review of Respiratory Disease 1990; 142:1206-1215, 1440-1448. 23. Staples CA, Muller NE, Vedal S, Abboud R, Ostrow D, Miller RR. Usual interstitial Pneumonia: correlation of CT with clinical, functional, and radiologic findings. Radiology 1987; 162:377-381. 24. Webb WR. High resolution CT of lung parenchyma. Radiologic Clinics of North America 1989; 27(6):1085-1097. 25. Weibel ER. Looking into the lung: what can it tell us? American Journal of Roentgenology 1979; 133:1021-1031. 26. Weibel ER, Bachofen H. The Fiber Scaffold of Lung Parenchyma. In: Crystal RG, West JB, eds. The Lung. New York: Raven Press, 1991; 787-794. 27. Weibel ER, Crystal RG. Structural Organization of the Pulmonary Interstitium. In: Crystal RG, West JB, eds. The Lung. New York: Raven Press, 1991; 369-380. Sarcoidosis 1. Bergin CJ, Bell DY, Coblentz CL, Chiles C, Gamsu C, Maclntyre NR, Coleman RE, Putman CE. Sarcoidosis: correlation of pulmonary parenchymal pattern at CT with results of pulmonary function tests. Radiology 1989; 171(3):619-24. 2. Gawne-Cain ML, Hansell CM. The pattern and distribution of calcified mediastinal lymph nodes in sarcoidosis and tuberculosis: a CT study. Clin Radiol 1996; 51(4):263-7. 3. Gleeson FV, Traill ZC, Hansell CM. Evidence of expiratory CT scans of small- airway obstruction in sarcoidosis. AJRAm J Roentgenol 1996; 166(5):1052-4. 4. Hansell DM, Milne DC, Wilsher ME, Wells AU. Pulmonary sarcoidosis:morphologic associations of airflow obstruction at thin-section CT. Radiology 1998; 209(3):697-704. 5. Kuhlman JE, Fishman EK, Hamper UM, Knowles M, Siegelman SS. The computed tomographic spectrum of thoracic sarcoidosis. Radiographics 1989; 9(3):449-66. 6. Miller WT Jr, Shah RM. Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations. AJR Am J Roentgenol. 2005 Feb;184(2):613-22. 7. Muller NE, Kullnig P, Miller RR. The CT findings of pulmonary sarcoidosis: analysis of 25 patients. AJR Am J Roentgenol 1989; 152(6):1179-82. 8. Muller NE, Mawson JB, Mathieson JR. Abboud R, Ostrow DN, Champion P Sarcoidosis: correlation of extent of disease at CT with clinical, functional, and radiographic findings. Radiology 1989; 171 (3):61 3-8. 9. Murdoch J, Muller NE. Pulmonary sarcoidosis: changes on follow-up CT examination. AJR Am J Roentgenol 1992; 159(3):473-7. 10. Newman ES, Rose CS, Maier LA. Sarcoidosis [published erratum appears in N Engl J Med 1997 Jul 10;337(2):1391 [see comments]. N Engl J Med 1997; 336(17):1224-34. 11. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. Pulmonary sarcoidosis: correlation of CT and histopathologic findings [published erratum appears in Radiology 1994 Mar;190(3):907]. Radiology 1993; 189(1):105-9. 12. Nishimura K, Itoh H, Kitaichi M, Nagai S, Izumi T. CT and pathological correlation of pulmonary sarcoidosis. Semin Ultrasound CT MR 1995; 16(5):361-70. 13. Padley SP, Padhani AR, Nicholson A, Hansell DM. Pulmonary sarcoidosis mimicking cryptogenic fibrosing alveolitis on CT. Clin Radio! 1996; 51(11):807-10. 14. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis. AJR Am J Roentgenol 1985; 144(3):51328. 15. Thomas PD, Hunninghake GW. Current concepts of the pathogenesis of sarcoidosis. Am Rev Respir Dis 1987; 135(3):747-60. 16. Vignaux O. Pictorial Essay: Cardiac sarcoidosis: spectrum of MRI features. AJR Am J Roentgenol 2005 Jan;184(1):249-54. 17. Winterbauer RH, Belic N, Moores KD. Clinical interpretation of bilateral hilar adenopathy. Ann Intern Med 1973; 78(1 ):65-71. Chest Radiology

13


The Idiopathic Interstitial Pneumonias Jeffrey R. Galvin, MD

The Idiopathic Interstitial Pneumonias Chronic Diffuse Lung Disease [Figure 1-2-1 and 1-2-2] •

• • • •

Alveolar involvement ➢ Surrounding airways ➢ Fibrosis and/or cells ✧ Alveolar wall ✧ Alveolar space Restrictive physiology Decreased lung volumes Increased attenuation Subacute or chronic ➢ Weeks to months

Figure 1-2-1

Figure 1-2-2

The Idiopathic Interstitial Pneumonias involve the alveolar walls and spaces

The lung volumes are low and there are areas of increased density

The Idiopathic Interstitial Pneumonias • •

• •

Liebow 1975 Supporting lung structures ➢ Inflammation ➢ Fibrosis Not confined to interstitium Initiated within the airspace

Liebow, Prog Reps Dis 1975

The Idiopathic Interstitial Pneumonias Current List-ATS/ERS Consensus Classification • • • • • •

Idiopathic Pulmonary Fibrosis (IPF) ➢ Usual Interstitial Pneumonia (UIP) Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) Desquamative Interstitial Pneumonia (DIP) Acute Interstitial Pneumonia (AIP) Cryptogenic Organizing Pneumonia (COP) NonSpecific Interstitial Pneumonia (NSIP)

Travis et al. Am J Respir Crit Care 2002 The Idiopathic Interstitial Pneumonias

14

Chest Radiology

Idiopathic Pulmonary Fibrosis • •

• •

•

Usual Interstitial Pneumonia: histologic pattern 5th-7th decade ➢ 66% > 60 years ➢ 7/100,000 women and 10/100,000 men Insidious onset of dyspnea ➢ 6 months before diagnosis ➢ Restrictive ventilatory defect ➢ Rales and clubbing Associations: ➢ Cigarette smoke ➢ Dusty environments: farming, wood dust, metal dust ➢ GE reflux ➢ Autoantibodies common (ANA, RA) Median survival 2.5-3.5 years

Figure 1-2-3

Usual Interstitial Pneumonia: Histology • •

• • •

Geographic variation Temporal variation ➢ Fibroblast foci ➢ Mature fibrous tissue Extensive fibrosis Inflammation ➢ Minimal ➢ No correlation outcome Abnormal wound healing Prognosis ➢ Fibroblast foci ✧ Presence and extent

Katzenstein, Am J Respir Crit Care Med 1998 Selman, Ann Int Med 2001 King, Am J Respir Crit Care Med 2001

Idiopathic Pulmonary Fibrosis Imaging [Figure 1-2-3] •

•

Radiograph abnormal-95% ➢ Volume loss ➢ Reticulonodular opacities ➢ Lower lobe ➢ Honeycombing Computed tomography ➢ Peripheral and lower lobe ➢ Reticulation and ground glass ✧ Progress to honeycombing ➢ Ground glass in areas of traction bronchiectasis

The abnormalities are predominantly peripheral and lower lung field. There is progressive volume loss

Figure 1-2-4

Hartman, Chest 1996

IPF-Progressive Volume Loss

Idiopathic Pulmonary Fibrosis [Figure 1-2-4] IPF and Emphysema

Typical peripheral reticulation and honeycombing and traction bronchiectasis in a patient with IPF

Chest Radiology

15

The Idiopathic Interstitial Pneumonias

Utilility of Biopsy for Diagnosis of IPF •

• • •

• • •

Prospective, multi-center study ➢ 91 patients suspected of IPF Clinical diagnosis ➢ Positive predictive value with a confident diagnosis-87% Imaging diagnosis ➢ Positive predictive value with a confident diagnosis-96% ➢ CT always abnormal in patients with proven IPF Histologic diagnosis ➢ Agreement regarding the presence or absence of IPF-85% ➢ Agreement in patients without IPF-48% ✧ Relevance to NSIP Uncertain diagnosis Discordant data Disease other than IPF ➢ Hypersensitivity pneumonitis ➢ Collagen-vascular disease ➢ Infection

Figure 1-2-5

Hunninghake, Am J Respir Crit Care Med 2001

IPF Rad-Path Discord

Smoking Related ILD Interstitial Lung Disease [Figure 1-2-5] •

• •

Respiratory bronchiolitis ➢ RB Respiratory bronchiolitis-interstitial lung disease ➢ RB-ILD Desquamative interstitial lung disease ➢ DIP

Smoking Related ILD RB • • •

Clinical ➢ Cigarette smoke or equivalent ➢ Asymptomatic Pathology ➢ Peribronchiolar macrophages ➢ Peribronchiolar fibrosis Imaging ➢ Centrilobular nodules ✧ Poorly defined 2-3 mm ✧ Uper lobe predominance ➢ Ground glass opacity ➢ Bronchial wall thickening ➢ Decreased attenuation ➢ Emphysema ➢ Air trapping ➢ Reticulation

Smoker’s macrophages

Niewoehner, NEJM 1974 ; Remy-Jardin, Radiology 1993


16

Chest Radiology

Figure 1-2-6

Smoking Related ILD [Figure 1-2-6] RB-ILD •

• •

Clinical ➢ Cigarette smoke or equivalent ➢ Dyspnea ➢ Restrictive or mixed PFT’s ➢ Good prognosis Pathology ➢ Peribronchiolar macrophages ➢ Peribronchiolar fibrosis Imaging ➢ Centrilobular nodules ✧ Poorly defined 2-3 mm ✧ Uper lobe predominance ➢ Ground glass opacity ➢ Bronchial wall thickening ➢ Decreased attenuation ➢ Emphysema ➢ Air trapping ➢ Reticulation

Meyers, Am Rev Respir Dis 1987 Park, J Comput Assist Tomogr 2002

Small centrilobular nodules with an upper lobe predominance in RB-ILD

Smoking Related ILD DIP •

•

•

Clinical ➢ Cigarette smoke ➢ 4th and 5th decade ➢ Uncommon ➢ 70% survival-10 years ➢ Steroids Pathology ➢ Pigmented macrophages ➢ Interstitial infiltrate ✧ Plasma cells and eosinophils ➢ Fibrosis Imaging ➢ Ground glass ✧ Symmetrical ✧ Basal predominance ➢ Reticulation ➢ Cysts ✧ Alveolar ducts ✧ Bronchioles ✧ Emphysematous spaces

Figure 1-2-7

Carrington, NEJM 1978 ; Hartman, Radiology 1993

Desquamative Interstitial Pneumonia [Figure 1-2-7

Ground glass opacities in DIP Chest Radiology

17


Dependent Density

Figure 1-2-8

Desquamative Interstitial Pneumonia RB and DIP

Smoking Related ILD [Figure 1-2-8] Acute Interstitial Pneumonia AIP • • • • • •

Hammon-Rich disease Rapidly progressive Days-weeks Antecedent flu-like syndrome Mean age 50 years 50% fatal at least

Vourlekis, Medicine 2000

Acute Interstitial Pneumonia Histology •

• • •

Exudative phase ➢ Hyaline membranes ➢ Edema ➢ Inflammation Collapse of alveoli Organizing phase ➢ Type II hyperplasia ➢ Loose fibrosis Diffuse Alveolar Damage

Smoking related interstitial lung disease with upper lobe indistinct nodules, reticulation and well defined emphysematous spaces combined with lowerlobe ground glass

Figure 1-2-9

Katzenstein, Am J Pathol 1986 ; Ichikado, AJR 1997

Diffuse Alveolar Damage

Acute Interstitial Pneumonia Radiography [Figure 1-2-9 and 1-2-10] • • • • •

Diffuse Airspace opacification Costal sparing Mechanical ventilation Resembles ARDS

Figure 1-2-10

AIP involves all 5 lobes

Most patients are intubated with diffuse opacities The Idiopathic Interstitial Pneumonias

18

Chest Radiology

Acute Interstitial Pneumonia Computed Tomography • Exudative phase •

Figure 1-2-11

➢ Consolidation ➢ Bilateral ➢ Focal sparing Organizing phase ➢ Distortion ➢ Traction bronchiectasis ➢ Ground glass

Johkoh, Radiology 1999; Ichikado et al. Am J Respr Crit Care Med 2002

Acute Interstitial Pneumonia [Figure 1-2-11] Cryptogenic Organizing Pneumonia • • • • •

•

Non-specific inflammatory response Pattern of repair Self-perpetuating Cryptogenic Secondary ➢ Connective tissues disease, hematologic malignancy, drugs or organ transplantation Focal ➢ Bacteria, legionella, mycoplasma, mycobacterial, or infarction

Lohr, Arch Int Med 1997

Cryptogenic Organizing Pneumonia •

• • • • • •

Focal areas of sparing are common in AIP

Terminology problem ➢ Bronchiolitis obliterans OP (BOOP), bronchiolitis obliterans (BO), bronchiolitis interstitial pneumonia (BIP) Subacute presentation (3 months) M=F Cough, dyspnea, weight loss, fever Restrictive PFT’s Steroid responsive Relapse common

Figure 1-2-12

Epler, NEJM 1985

Cryptogenic Organizing Pneumonia Histology • • •

Fibroblastic plugs in alveoli Fibrosis in the alveolar space May be airway centered ➢ Bronchiolitis

Cryptogenic Organizing Pneumonia [Figure 1-2-12] Radiography •

• •

Consolidation ➢ Unlateral or bilateral Small Nodules ➢ 10-50% Lung volumes ➢ normal in 75%

Cryptogenic organizing pneumonia is characterized by focal areas of consolidation more common in the lower lung fields Chest Radiology

19


Cryptogenic Organizing Pneumonia Computed Tomography • • • • • •

Figure 1-2-13

Consolidation 90% Ground glass 75% Bronchial thickening and dilatation Small nodules along bronchvascular bundles Large nodules (15%) ➢ Irregular margins ➢ Air bronchograms Reverse halo

Cryptogenic Organizing Pneumonia [Figure 1-2-13 and 1-2-14]

Figure 1-2-14 Typical findings in COP with peripheral areas of consolidation. The differential includes chronic eosinophilic pneumonia, bronchoalveolar cell carcinoma, lymphoma and infection

Diffuse nodules may also be seen in COP

Nonspecific Interstitial Pneumonia •

• • • •

•

Katzenstein ➢ Described in 1994 Does not fit definition of other IIPs ➢ UIP, RB-ILD, DIP, OP, AIP Represents a variety of etiologies ➢ Collagen vascular disease, drug reaction, inhaled antigen ➢ Inadequately sampled UIP or OP Median age 45 Onset gradual with wide range ➢ 6 months to 3 years Better prognosis

Katzenstein, Am J Resp Crit Care 1994 Nicholson, Am J Respir Crit Care Med 2001


20

Chest Radiology

Nonspecific Interstitial Pneumonitis Histology •

• • •

3 categories ➢ Cellular ➢ Fibrosing ➢ Mixed Prognosis=fibrosis OP common Temporally uniform

Nonspecific Interstitial Pneumonitis Imaging • •

• •

Few reports on chest radiography Wide variety of CT patterns ➢ Ground glass, consolidation, reticular and honeycombing Traction bronchiectasis=fibrosis CT pattern indistinguishable ➢ UIP 32% ➢ Hypersensitivity 20% ➢ OP 14% ➢ Other 12%

Hartman, Radiology 2000

Nonspecific Interstitial Pneumonia NSIP ATS Consensus Conference • • • • •

Pathologists Radiologists Pulmonalogists 300 cases submitted ➢ 11 cases agreed to be NSIP by all pathologists Imaging ➢ Lower lobe ➢ Peribronchiolar reticulation and distortion ➢ Subpleural clearing

NSIP Current View

NSIP Fibrosis with IPF Imaging • •

•

Areas of NSIP commonly found in proven cases of UIP NSIP and UIP ➢ Different severity of injury? ➢ Different mechanism of injury? Prognosis in these cases is driven by the imaging

Katzenstein AA et al, Amer J of Surg Path 2002

Figure 1-2-15

Hypersensitivity Pneumonitis NSIP in Cigarette Smokers

Nonspecific Interstitial Pneumonia OP-NSIP

[Figure 1-2-15]

OP-NSIP is peribronchovascular with UIP is peripheral Chest Radiology

21


The Idiopathic Interstitial Pneumonias Current List •

• • • • •

Figure 1-2-16

Idiopathic Pulmonary Fibrosis (IPF) ➢ Usual Interstitial Pneumonia (UIP) Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD) Desquamative Interstitial Pneumonia (DIP) Acute Interstitial Pneumonia (AIP) Cryptogenic Organizing Pneumonia (COP) NonSpecific Interstitial Pneumonia (NSIP)

Idiopathic Pulmonary Fibrosis [Figure 1-2-16]

RB/RB-ILD [Figure 1-2-17]

RB-ILD/DIP [Figure 1-2-18]

IPF

Figure 1-2-17

RB-ILD

Figure 1-2-18

DIP

Acute Interstitial Pneumonia [Figure 1-2-19 and 1-2-20] Figure 1-2-19

Figure 1-2-20

AIP early phase


AIP late phase with organization and fibrosis 22

Chest Radiology

Organizing Pneumonia NSIP in the Literature

Figure 1-2-21

[Figure 1-2-21]

NSIP-IPF

NSIP-Cigarette Smokers [Figure 1-2-22] Figure 1-2-22

Organizing pneumonia

Patients with smoking related fibrosis may have a biopsy that demonstrates NSIP

NSIP-Hypersensitivity Pneumonitis

NSIP-Organizing Pneumonia [Figure 1-2-23] Figure 1-2-23

Patients with organizing pneumonia may have a biopsy that demonstrates NSIP

Chest Radiology

23


References

General 1. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165:277-304 2. Wittram C, Mark EJ, McLoud TC. CT-histologic correlation of the ATS/ERS 2002 classification of idiopathic interstitial pneumonias. Radiographics. 2003 Sep-Oct;23(5):1057-71. IPF/UIP 1. Hansell DM, Wells AU. CT evaluation of fibrosing alveolitis—applications and insights. J Thorac Imaging 1996; 11(4):231-49. 2. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am U Respir Crit Care Med 1998; 157(4 Pt 1):1301-15. 3. Kondoh Y, Taniguchi H, Kawabata Y, Yokoi T, Suzuki K, Takagi K. Acute exacerbation in idiopathic pulmonary fibrosis. Analysis of clinical and pathologic findings in three cases. Chest 1993; 103(6):1808-12. 4. Liebow AA. Definition and classification of interstitial pneumonias in human pathology. Prog Resp Res 1975; 8:133. 5. Tobin RW, Pope CE, 2nd, Pellegrini CA, Emond MJ, Sillery J, Raghu G. Increased prevalence of gastroesophageal reflux in patients with idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 158(6): 1804-8. 6. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, Tscholakoff D. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology 1990; 176(755759). 7. Coxson HO, Hogg JC, Mayo JR, Behzad H, Whittall KP, Schwait DA, Hartley PC, Galvin JR, Wilson JS, Hunninghake SW. Quantification of idiopathic pulmonary fibrosis using computed tomography and histology. Am J Respir Crit Care Med 1997; 155(5):1649-56. 8. Gay SE, Kazerooni EA, Toews GB, Lynch UP, 3rd, Gross BH, Cascade PN, Spizarny DL, Flint A, Schork MA, Whyte RI, Popovich U, Hyzy R, Martinez FJ. Idiopathic pulmonary fibrosis: predicting response to therapy and survival. Am U Respir Crit Care Med 1998; 157(4 Pt 1):1063-72. 9. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar Ho, Schroeder DR, Offord KR. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am U Respir Crit Care Med 1998; 157(1):1 99-203. DIP 1. Gaensler EA, Goff AM, Prowse CM. Desquamative interstitial pneumonia. N Engl U Med 1966; 274(3)113-28. 2. Ryu JH, Myers JL, Capizzi SA, Douglas WW, Vassallo R, Decker PA.Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease. Chest. 2005 Jan;127(1):178-84.

DAD/AIP 1. Bone RC. The ARDS lung. New insights from computed tomography [editorial; comment]. Jama 1993; 269(1 6):21 34-5. 2. Desai SR, Wells AU, Rubens MB, Evans TW, Hansell DM. Acute respiratory distress syndrome: CT abnormalities at long-term follow-up. Radiology 1999; 210(1):29-35. 3. Greene R. Adult respiratory distress syndrome: acute alveolar damage. Radiology 1987; 163(1):57-66. 4. Ichikado K, Johkoh T, Ikezoe U, Takeuchi N, Kohno N, Arisawa U, Nakamura H, Nagareda T, Itoh H, Ando M. Acute interstitial pneumonia: high-resolution CT findings correlated with pathology. AUR Am U Roentgenol 1997; 1 68(2):333-8. 5. Johkoh T, Muller NL, Taniguchi H, Kondoh Y, Akira M, Ichikado K, Ando M, Honda 0, Tomiyama N, Nakamura H. Acute interstitial pneumonia: thin-section CT findings in 36 patients. Radiology 1999; 211(3):859-63. 6. Katzenstein AL, Myers UL, Mazur MT. Acute interstitial pneumonia. A clinicopathologic, ultrastructural, and cell kinetic study. Am U Surg Pathol 1986; 10(4):256-67. 7. Olson U, Colby TV, Elliott CG. Hamman-Rich syndrome revisited [see comments]. Mayo Clin Proc 1990; 65(12):1538-48. 8. Primack SL, Hartman TE, Ikezoe U, Akira M, Sakatani M, Muller NL. Acute interstitial pneumonia: radiographic and CT findings in nine patients [see comments]. Radiology 1993; 188(3):817-20.

NSIP 1. Cottin V, Donsbeck AV, Revel D, Loire R, Cordier JR Nonspecific interstitial pneumonia. Individualization of a clinicopathologic entity in a series of 12 patients. Am J Respir Crit Care Med 1998; 158(4):1286-93. 2. Katzenstein AL, Fiorelli RF. Nonspecific interstitial pneumonia/fibrosis. Histologic features and clinical significance. Am J Surg Pathol 1994; 18(2):136-47. The Idiopathic Interstitial Pneumonias

24

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3.

Kim TS, Lee KS, Chung MP, Han J, Park JS, Hwang JH, Kwon OJ, Rhee OH. Nonspecific interstitial pneumonia with fibrosis: high-resolution CT and pathologic findings. AJR Am J Roentgenol 1998; 171(6): 1645-50.

BOOP/Organizing Pneumonia 1. Akira M, Yamamoto S, Sakatani M. Bronchiolitis obliterans organizing pneumonia manifesting as multiple large nodules or masses. AJR Am J Roentgenol 1998; 170(2):291-5. 2. Carlson BA, Swensen SJ, O’Connell EJ, Edell ES. High-resolution computed tomography for obliterative bronchiolitis. Mayo Clin Proc 1993; 68(3):307-8. 3. Chandler PW, Shin MS, Friedman SE, Myers JL, Katzenstein AL. Radiographic manifestations of bronchiolitis obliterans with organizing pneumonia vs usual interstitial pneumonia. AJR Am J Roentgenol 1986; 147(5):899906. 4. Epler GR, Colby TV, McLoud TC, Carrington CB, Oaensler EA. Bronchiolitis obliterans organizing pneumonia. N Engl J Med 1985; 312(3):152-8. 5. Gosink RB, Friedman Pd, Liebow AA. Bronchiolitis obliterans. Roentgenologic¬pathologic correlation. Am J Roentgenol Radium Ther Nucl Med 1973; 11 7(4):81 6-32. 6. Haddock JA, Hansell DM. The radiology and terminology of cryptogenic organizing pneumonia. Br J Radiol 1992; 65(776):674-80. 7. Katzenstein AL, Myers JL, Prophet WD, Corley LS, 3rd, Shin MS. Bronchiolitis obliterans and usual interstitial pneumonia. A comparative clinicopathologic study. Am J Surg Pathol 1986; 10(6):373-81. 8. Lau DM, Siegel MJ, Hildebolt CF, Cohen AH. Bronchiolitis obliterans syndrome: thin-section CT diagnosis of obstructive changes in infants and young children after lung transplantation. Radiology 1998; 208(3):783-8. 9. Lee KS, Kullnig P, Hartman TE, Muller NL. Cryptogenic organizing pneumonia: CT findings in 43 patients. AJR Am J Roentgenol 1994; 162(3):543-6. 10. Lohr RH, Boland BJ, Douglas WW, Dockrell DH, Colby TV, Swensen SJ, Wollan PC, Silverstein MD. Organizing pneumonia. Features and prognosis of cryptogenic, secondary, and focal variants. Arch Intern Med 1997; 157(12):1323-9. 11. McLoud TC, Epler GR, Colby TV, Gaensler EA, Carrington CB. Bronchiolitis obliterans. Radiology 1986; 159(1)1-8. 12. Muller NL, Cuerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, Pare P, Hogg JC. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional, and radiologic findings. Radiology 1987; 162(1 Pt 1):151-6. 13. Muller NL, Staples CA, Miller RR. Bronchiolitis obliterans organizing pneumonia: CT features in 14 patients. AJR Am J Roentgenol 1990; 154(5):983-7.

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Airways Disease: The Movement from Anatomic to Physiologic Assessment Jeffrey R. Galvin, MD

Assessment of Dyspnea A Common Clinical Problem • • • •

55 million adult smokers 15 million meet criteria for bronchitis 5 million with airway obstruction 10 million with asthma

Figure 1-3-1

Gordon Snyder

Differential Diagnosis of Airways Obstruction •

•

Common Emphysema, bronchitis, bronchiectasis, asthma Uncommon ➢ LAM, BO, panbronchiolitis, sarcoid, alpha-1 deficiency, ABPA ➢

Diseases with Obstructive Physiology The Changing Role of Imaging • •

Diagnosis Functional assessment

Why Pulmonary Functions are Insensitive •

• •

PFT’s based on wide range of normal 80-120% predicted Diseases with opposing physiologic processes The “silent zone” of the lungs

➢

Small airway tethered to the pleural surface by alveolar walls

The “Silent Zone” of the Lungs [Figure 1-3-1 and 1-3-2]

“Small Airways” •

• •

Figure 1-3-2

Peter Macklem ➢ 1970’s No cartilage ➢ 1cm Radiography is insensitive ➢ 41% of moderate disease ➢ 66% of severe disease ➢ ➢ ➢

Saber Trachea [Figure 1-3-6]

Emphysema and Computed Tomography [Figure 1-3-7]

Typical low attenuation lesions of emphysema

The Diagnosis of Mild Emphysema Correlation of CT and Pathology Scores •

HRCT detects emphysema Before there is airflow limitation on PFT’s HRCT excludes emphysema ➢ Patients with moderate to severe airflow limitation ➢

•

Kuwano et al, Am Rev Respir Dis 1990

Early Emphysema

Airways Disease

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Chest Radiology

Respiratory Bronchiolitis [Figure 1-3-8] “Smoker’s Bronchiolitis” •

• • •

Figure 1-3-8

Common change all smokers Pigmented macrophages ➢ In respiratory bronchioles ➢ Surrounding alveoli Upper lobe predominance Usually asymptomatic ➢ May cause symptoms ➢

Relationship of RB and Emphysema • • •

•

Prospective study 111 subjects ➢ Followed for 5 years ✧ Imaged at inception TO and 5 years T1 ➢ Smokers, nonsmokers and quitters Micronodules at TO predisposes to the development of emphysema at T1 Micronodules and emphysema at TO predicts more rapid decline in lung function

Remy-Jardin, Radiology 2001

Relationship of RB and Emphysema Remy-Jardin, Radiology 2001

Respiratory bronchiolitis

Emphysema and Fibrosis • • •

14 patients Scanning electron microscopy Thick and thin walls ➢ Both fibrotic

Figure 1-3-9

Nagai & Thurlbeck, Am Rev Resp Dis 1985

Emphysema and Fibrosis

•

Normal lung volumes and Normal flow rates Reduced diffusing capacity ➢ Severe Minimal pulmonary reserve

• • • • •

TLC 119% VC 126% RV 109% FEV1/FVC 88% D/Va 28%

• • •

Emphysema and Fibrosis

Emphysema and Fibrosis [Figure 1-3-9]

Emphysema and fibrosis

Idiopathic Pulmonary Fibrosis Langerhans Cell Histiocytosis Clinical Presentation • • • • •

Almost exclusively cigarette smokers Slight male preponderance Cough and dyspnea most common May be asymptomatic Occasional bone lesion

Chest Radiology

29

Airways Disease

Langerhans Cell Histiocytosis Histology [Figure 1-3-10] • • •

•

Figure 1-3-10

Nodular Interstitial lesions Located near bronchioles Histiocytes, eosinophils, plasma cells and lymphocytes Diagnosis requires Langerhans cells ➢ Large histiocytes ➢ Folded nuclei ➢ Eosinophilic cytoplasm Path DDX ➢ Eos pneumonia, DIP,UIP ➢ ➢

Langherhans Cell Histiocytosis [Figures 1-3-11 and 1-3-12]

Figure 1-3-11

The range of findings in Langerhans Cell Histiocytosis

Figure 1-3-12

Cystic lesions in LCH

Typical nodules in LCH

Langherhans Cell Histiocytosis Radiographic Features [Figure 1-3-13] • •

• • • • •

Figure 1-3-13

Varies over time Upper lobe ➢ Predominance Nodules ➢ 0.5-1.0 cm in upper lobes ➢ Early Cysts replace nodules ➢ Later Honeycomb lung Pneumothorax 15% Adenopathy and effusion are unusual

LCH is characterized by low attenuation areas with bizarre shapes Airways Disease

30

Chest Radiology

Langerhans Cell Histiocytosis EM and Immunohistochemistry •

• •

Immunoperoxidase staining CD1a, S-100 protein Cells in clusters in interstitium EM ➢ X-bodies ➢ Langerhans cell granules ➢ Birbeck granules ➢

Figure 1-3-14

Langerhans Cell Histiocytosis Clinical Course •

• • •

Clinical resolution Common Radiographic abnormalities ➢ Persist Occasional progression ➢ Fibrosis and honeycombing May be fatal ➢ Rapid progression ➢

Langherhans Cell Histiocytosis Alpha-1 Antitrypsin Deficiency Pathophysiology

• • • • • •

1-2% of emphysema in the US Alpha-1 antitrypsin inactivates neutrophil elastase Production controlled by 2 genes Level of antitrypsin dependent on allele ZZ homozygotes most severe Smoking accelerates the destruction

• • • •

Radiograph may be normal Lower lobe predominance Panacinar emphysema CT ➢ Upper lobe involvement ➢ Bronchiectasis ➢ Airway thickening common CT more sensitive

Alpha-1 Antitrypsin Deficiency Imaging Features

•

Alpha-1 antitrypsin deficiency

Alpha-1 Antitrypsin Deficiency [Figure 1-3-14]

Figure 1-3-15

Bronchiectasis Pathophysiology • • • • • •

Dilatation of bronchi Reversible form ➢ Infection ➢ Atelectasis Congenital ➢ tracheobronchomalacia Post-inflammatory Postobstructive Fibrotic ➢ IPF ➢ Sarcoid

Williams-Campbell [Figure 1-3-15] Chest Radiology

Williams-Campbell 31

Airways Disease

Mounier-Kuhn Syndrome

Figure 1-3-16

Bronchiectasis Postinflammatory •

• • • •

Primary Ciliary Dyskinesia Kartagener’s Immunodeficiency Postinfectious ➢ TB, Measles, pertussis, viral Post-toxic bronchitis ➢ gastric acid aspiration Immunologic ➢ ABPA ➢

Primary Ciliary Dyskinesia

Post Obstructive Bronchiectasis [Figure 1-3-16] • • • •

Neoplasm Foreign body Broncholith Lymph node enlargement

• • • • •

Cough Purulent sputum Hemoptysis (50%) Dyspnea Rare ➢ clubbing, brain abscess, amyloidosis

Figure 1-3-17

Bronchiectasis Clinical Presentation

Bronchiectasis Radiographic Features

• • • • •

Prominent markings Crowding of Vessels “Tram Tracks” Loss of volume Cystic spaces

• • • •

Bronchi in the periphery “Signet Rings” “Tram Tracks” Sensitivity ➢ Collimation

Post obstructive bronchiectasis in a patient with mucoepidermoid carcinoma

Upper lobe smoking related emphysema

Figure 1-3-18

Bronchiectasis CT Features

RB-ILD

Emphysema [Figure 1-3-17]

Figure 1-3-19

RB/RB-ILD [Figure 1-3-18]

Emphysema and Fibrosis [Figure 1-3-19]

Emphysema and fibrosis Airways Disease

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Chest Radiology

Langherhans Cell Histiocytosis [Figures 1-3-20 to 1-3-22] Alpha-1 Antitrypsin Deficiency [Figure 1-3-23]

Figure 1-3-21

Figure 1-3-20

Early LCH nodules

Late LCH Cysts and nodules

End-stage LCH

Lower lobe predominance in Alpha-1 antitrypsin

Figure 1-3-22

Figure 1-3-23

Bronchiectasis

Diffuse Lung Disease Airways • • • •

Airways involvement Obstructive physiology Increased lung volumes Decreased attenuation

• • •

Reversible airway disease Increased airway responsiveness Persistent airflow obstruction occurs in chronic asthmatic ➢ Why? 6% in the American population ➢ Rate has doubled in 20 years ➢ Higher incidence in large cities

Asthma ATS Definition

•

Asthma Extrinsic • • • • • •

Family history atopy Early onset 30y ➢ Age >65 ➢ CHF,DM ➢ Alcoholism ➢ Thrombocytopenia ➢ Renal dysfunction ➢ Number of lobes

Chest 1993; 103:1152-56

S. pneumoniae: Pathology · · · ·

Aspiration to peripheral air spaces Alveolus represents site of initial inflammatory lesion Spread occurs by contignous involvement of adjacent alveoli 3 pathologic stages

S. pneumoniae: Pathology ·

· ·

ACUTE RESPONSE ➢ Increased capillary permeability ➢ Protein rich edema ➢ Contiguous alveolar filling via Pores of Kohn and Canals of Lambert RED HEPATIZATION ➢ PMN infiltration and intra-alveolar hemorrhage GRAY HEPATIZATION ➢ Macrophage infiltration and uptake of blood products

Chest Radiology

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Pneumonia

S. pneumoniae: Radiology [Figure 1-19-1] ·

LOBAR pattern ➢ Homogeneous, confluent density ➢ Nonsegmental distributions

S. pneumoniae: Radiology · ·

Spread at alveolar level results in nonsegmental distributions characteristic of early lobar pneumonia Round pneumonia ➢ Manifestation of nonsegmental distribution ➢ Most common in pediatric infection with S.pneumoniae

Figure 1-19-1

S. pneumoniae: Radiology ·

LOBAR pattern ➢ Prominent air bronchograms ➢ Preserved volume

S. pneumoniae: Radiology · · · ·

48% of consecutive hospitalized pts demonstrated focal lobar patterns 33%, multifocal lobar patterns 16% lobular pattern Dominant pattern did not vary with immune status or disease severity

AJR 2000;175:1533

Lobar pattern consolidation due to S. pneumoniae

S. pneumoniae: Radiology · ·

·

Small pleural effusions up to 60% Infrequent cavitation ➢ Associated with serotype 3 Most frequent organism in pulmonary gangrene ➢ Vascular thrombosis from severe necrosis ➢ Intracavitary mass (sloughed lung)

M. pneumoniae: Demographics · · · ·

15-35% of CAP ➢ 50% of CAP during summer months Peak age 5-25 yo Self limited ➢ Few fatal cases associated with ARDS ➢ Increased severity in sickle cell anemia Most frequent etiology in Atypical Pneumonia Syndrome ➢ Atypical radiographic features ➢ Prominent extrapulmonary complaints

M. pneumoniae: Pathology ·

·

Eaton agent-1944 ➢ Gram -- filamentous rod ➢ Absent cell wall Acute cellular bronchiolitis ➢ Superficial inflammation involving luminal surface of bronchi, bronchioles ➢ Associated interstitial infiltrates

Pneumonia

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Chest Radiology

M. pneumoniae: Radiology [Figure 1-19-2] · ·

· · ·

Figure 1-19-2

LOBULAR pattern Bronchopneumonia Heterogeneous, patchy consolidation ➢ Minimal exudate into centrilobular alveoli Segmental distribution ➢ Spread at bronchiolar level Volume loss Minimal air bronchograms ➢ Peribronchial thickening

M. pneumoniae: Radiology ·

CT Findings [Figure 1-19-3] ➢ 86% centrilobular nodules ➢ 82% bronchovascular thickening ➢ 59% consolidation with lobular distribution

Reittner, AJR 2000; 174:37

Bronchopneumonia pattern due to M. pneumoniae

Respiratory Viruses · · · · · ·

Influenzae A,B,C Para-influenzae Respiratory syncytial virus Adenovirus Herpes viruses SARS

Figure 1-19-3

Influenzae A: Demographics · · ·

·

10-20% CAP 10,000-40,000 deaths/ influenzae epidemic Peak incidence ➢ Pediatric population Highest mortality-adult and aged ➢ Superinfection ➢ S.aureus ➢ S.pneumoniae

Influenzae A: Pathology · · ·

St 1 infection of epithelial cells, proliferation and necrosis St 2 bronchial and alveolar wall edema,hemorrhage Ulceration, bacterial infection

HRCT of M. pneumoniae

Influenzae A: Radiology ·

INTERSTITIAL pattern ➢ Reticular ➢ Nodular ➢ Peribronchial thickening ➢ Subpleural edema ➢ Hilar haze

Figure 1-19-4

Influenzae A: Radiology [Figure 1-19-4]

· · ·

Bilateral, parahilar, lower lobe Air trapping Prominent GGO

(left) CXR (right) HRCT Influenzae pneumonia mimicking edema

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Pneumonia

Influenzae A: Radiology · ·

Pleural effusions, cavitation uncommon without bacterial superinfection Rapid deterioration should suggest superinfection

Adenovirus [Figure 1-19-5] · ·

·

Interstitial pneumonia with prominent necrotizing bronchiolitis Potential infection in immune competent and suppressed hosts with high mortality ➢ Pediatric population ➢ Military epidemics ➢ Transplant recipients Swyer James, Macleod’s syndrome ➢ Bronchiolitis obliterans following viral infection in early childhood

Figure 1-19-5

Respiratory Herpesviruses ·

·

HSV-1, HSV-2, VZV, EBV, CMV ➢ Primary infection, latency, reactivation ➢ Up to 40% mortality Risk factors ➢ Immune-suppression, lung transplantation, airway management, pregnancy

Swyer James Syndrome due to pediatric viral pneumonia

Varicella Pneumonia ·

·

Figure 1-19-6

Complication of adult chickenpox ➢ 5-50% incidence Prominent acinar opacities ➢ 5-10mm nodules, coalescence ➢ Patchy GGO

Kim AJR 1999;172:113 · May heal with miliary calcifications

Varicella Pneumonia [Figure 1-19-6] ·

Prominent acinar opacities

Severe Acute Respiratory Syndrome · · · · ·

SARS-CoV (corona virus) Initial cases Nov 2002-June 2003, rapid spread from Asia 20-50% require mechanical ventilation 10% mortality, age dependant Severe DAD

Severe Acute Respiratory Syndrome ·

· · · ·

Acinar nodules in varicella pneumonia

Predominant consolidation 1-2weeks ➢ Focal (39%), multifocal (28%), diffuse (14%) Ground glass opacity Reticulation Bronchiolar dilation Residual changes in 50% at 4wks

Ooi GC. Radiology 2004;230:836; Paul NS. AJR 2003;182:493

Severe Community-acquired Pneumonia: Definition · · ·

Impending respiratory failure Hemodynamic instability Radiographic assessment ➢ Bilateral or multilobar involvemnt ➢ 50% increase in size of opacity within 48hr

Pneumonia

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Severe Community-acquired Pneumonia: Etiology

· · · ·

S. pneumoniae L. pneumophila S. aureus P. aeruginosa in patients with bronchiectasis

L. pneumophila: Demographics ·

· ·

15% of CAP ➢ Epidemic and sporadic forms ➢ Legionnaire’s disease= pneumonic form ➢ Peak summer Aerobic Gram -- bacillus Proliferates in warm, humid environments

Figure 1-19-7

L. pneumophila: Pathology ·

Bronchocentric inflammation

L. pneumophila: Demographics · ·

· ·

Acute onset Prominent extrapulmonary symptoms ➢ Neurologic manifestations, diarrhea, renal insufficiency 10% mechanical ventilation 15% mortality in cases requiring hospitalization

L. pneumophila: Radiology [Figure 1-19-7] · · · · · ·

Bronchopneumonia pattern Pleural effusions in 2/3 Bilateral and multifocal in 50% May produce lobar or mass-like consolidation Cavitation uncommon without immunosupression Delayed resolution

K. pneumoniae: Demographics · · · · ·

Nosocomial or community acquired 5-10% lobar pneumonias 25% bacteremic, 50% mortality Males, >60yo Risk factors: alcoholism, COPD, DM

K. pneumoniae: Pathology ·

Gram -- bacillus ➢ Abundant PMN infiltration of alveoli, edema ➢ Lobar expansion - Friedlander’s pneumonia ➢ Massive necrosis ➢ Common association with gangrene

HRCT in Legionella pneumonia demonstrating bronchocentric nodules and pleural effusion

K. pneumoniae: Radiology ·

· ·

Lobar pattern ➢ Bulging fissures Abscess 30-50% Necrotizing pneumonia at CT ➢ Low density areas with small cavities

Moon JCAT 1995;19:176

Chest Radiology

183

Pneumonia

S.aureus: Demographics ·

· · · · ·

Figure 1-19-8

30-50% colonization rates in healthy adults ➢ DM ➢ IVDA ➢ HIV ➢ Surgical pts Methicillin resistance 1944 ➢ Increasing incidence of resistant community-acq infection Antecedant viral pneumonia Frequent cause of nosocomial infection Extremes of age ➢ Nursing home population Risk factors ➢ Debilitated states, mechanical ventilation, burns, indwelling catheters, IVDA

S. aureus: Radiology · · · · ·

Aerogenous infection [Figures 1-19-8 and 1-19-9] ➢ Multifocal Broncho-pneumonia Hematogenous infection [Figure 1-19-10] ➢ Multifocal, discrete nodular or wedge shaped abnormality with normal intervening lung Cavitation / abscess (25-75%) Pneumatoceles (60% ped infection) Pleural effusions / empyema (50%)

CXR and HRCT bronchopneumonia pattern due to S. aureus

Figure 1-19-9

Necrotizing bronchopneumonia due to S. aureus

Pneumonia

184

Chest Radiology

P. aeruginosa in Cystic Fibrosis ·

Figure 1-19-10

Chronic colonization with P.aeruginosa ➢ Mucoid variant ➢ ABX resistance ➢ Elastase production ➢ Bronchiectasis

The Practical Points · · · · ·

S.pneumoniae and K.pneumoniae most commonly associated with lobar pattern and pulmonary gangrene M.pneumoniae, L.pneumophilus most commonly associated with broncho-pneumonia pattern and atypical pneumonia syndrome Viral pneumonias associated with interstitial pattern Pathologic in immune-competent and suppressed hosts Prominent bronchiolitis seen with mycoplasma, adeno and other respiratory viruses

Septic emboli

Nosocomial Pneumonia · ·

Rising incidence parallels usage of antibiotics Gram negative infections ➢ 40-50% increase 1950-60 ➢ 55-65% nosocomial infections ➢ 50% nosocomial pneumonia ➢ 75% ICU pneumonia

Nosocomial Pneumonia: Definition ·

Pneumonia developing >48hr sp admission, intubation or discharge

Diagnosis ·

Quantitative cultures ➢ Tracheal aspirate 10 5-6 cfu/ml ➢ BAL 10 4 cfu/ml ➢ Protected specimen brush 10 3 cfu/ml ➢ False negative and false positive rates 20-30%

Baughman Chest 2000

Pathophysiology · · ·

Direct inhalation Hematogenous spread Aspiration ➢ 45% incidence in sleep ➢ Altered gag reflex, consciousness, GI motility ➢ NG / ET

Pathophysiology ·

· · ·

Abnormal gram negative airway colonization ➢ 25% 24hr ➢ 40% 7d Gastric alkalinization Serious illness Antibiotic TX

Johanson Ann Intern Med 1972

Pathophysiology · · ·

Repetitive aspiration leads to Bronchiolitis Lobular (broncho) pneumonia ➢ Peribronchiolar neutrophilic infiltrate (104 cfu/g)

Chest Radiology

185

Pneumonia

Nosocomial Pneumonia in the ICU · ·

·

Ventilator-associated Pneumonia (VAP) Most common nosocomial infection in ICU ➢ 3-21x greater incidence in intubated patients ✧ > 1 intubation, >3 days ➢ 10-65% ICU patients acquire VAP ➢ 1/3-3/4 ARDS patients acquire VAP 20-80% mortality rate

Figure 1-19-11

Ventilator-associated Pneumonia ·

Prognosis depends: ➢ Organism ✧ Highest mortality: P.aeruginosa, MRSA ➢ Population ✧ Highest mortality: medical ICU ✧ 10-20% mortality: trauma ICU ➢ Late onset ✧ MDR

Nosocomial Pneumonia and Aspiration [Figure 1-19-11] ·

Pneumonitis (Mendelson Syndrome) ➢ pH < 2.5, >0.3 ml/kg ➢ Biphasic inflammatory response ✧ 1-2 hr permeability edema ✧ 4-6 hr acute inflammation ➢ 30% mortality ➢ Rapid clearing

Nosocomial Pneumonia and Aspiration · · · · ·

Sterile vs Normal flora (5d) Anaerobes seen in late aspiration Aspiration accounts for upto 15% of CAP

Dependent aspiration pneumonia complicated by ARDS

Marik NEJM 2001

Microbiology · · · · · ·

Normal flora Gram – bacilli S. aureus Anaerobes Legionella Respiratory viruses

Microbiology ·

·

Early 5d ➢ S. aureus ➢ P. aeruginosa ➢ Enterobacteriaceae ➢ Acinetobacter spp. ➢ Stenotrophomonas maltophilia

Pneumonia

186

Chest Radiology

P. aeruginosa • • • •

Most common ICU isolate 70% TX failure rate Distal airway colonization, hematogenous dissemination Increased severity in neutropenia, bacteremia

• •

Micro-abscesses Necrotic vasculits ➢ Sm-med pulmonary arteries Hemorrhage

P. aeruginosa: Pathology •

P. aeruginosa: Radiology •

• •

Bronchopneumonia pattern ➢ Discrete nodules may be indicative of vasculitis Frequent cavitation Pleural effusions/empyema

Nosocomial Viral Pneumonia • • • •

Rate of infection assoc with length of hospitalization Hospital worker as carrier Frequently unsuspected High mortality rates ➢ RSV 30-100% ➢ Parainfluenzae 15-30%

Nodular or Mass-like Consolidations • •

Nonsegmental distribution ➢ ‘round’ pneumonia Granulomatous infection ➢ M. tuberculosis ➢ Fungi ➢ Actinomycosis ➢ Nocardia

A. Israelii; Demographics • •

•

Figure 1-19-12

Normal oral flora Sites of infection: ➢ Cervicofacial 55% ➢ Abdomen 20% ➢ Pulmonary 25% Risk factors: poor oral hygiene, aspiration

Smego RA. Clin Infec Dis 1998;26:1255

A. Israelii: Pathology • • •

Multifocal abscesses Interconnecting sinus tracts Sulphur granule ➢ Spoke-wheel arrangement of neutrophils surrounding filamentous organism

A. Israelii: Radiology [Figure 1-19-12] •

•

Pneumonia with chest wall involvement due to A. israelli

Consolidation ➢ Mass-like ➢ Cavitary Pleural, chest wall and osseous involvement ➢ Up to 50%

Chest Radiology

187

Pneumonia

N. Asteroides: Demographics

· · ·

Ubiquitous distribution 50% of patients are immunocompetent Risk factors: ➢ Neutropenia ✧ Steroids, late HIV, hemetologic malignancy, alveolar proteinosis

N. Asteroides: Pathology · ·

Peribronchial abscesses, granulomatous inflammation Extensive necrosis ➢ May mimic M.TB or fungal infection

N. Asteroides: Radiology ·

Extrapulmonary disease 50% with 40-90% mortality ➢ CNS 25% ➢ Skin and subcutaneous abscesses

N. Asteroides: Radiology ·

· ·

Consolidation ➢ Mass-like ➢ Cavitary Pleural and chest wall involvement 30-50% Adenopathy 40%

Cavitary Pneumonia in AIDS ·

N. asteroides

Alveolar Proteinosis and N. asteroides Parasitic Infection ·

Pulmonary involvement due to hypersensitivity or direct invasion ➢ Echinococcosis ➢ Paragonimiasis ➢ Ascariasis ➢ Strongyloidiasis

Figure 1-19-13

Parasitic Infection ·

Radiographic findings may overlap with other infections ➢ Fleeting, patchy infiltrates ➢ Reticulonodular opacities ➢ Bronchopneumonia ➢ Atelectasis

Echinococcus granulosus · ·

Cestode (tapeworm), endemic to S.America, Australia, Middle East, Africa and Mediterranean Definitive host - dog,wolf Intermediate host - sheep, cow, deer, moose

Echinococcus granulosus ·

Duodenum - portal venous system liver ➢ 45-75% isolated liver involvement ➢ 15-35% pulmonary involvement

Intact (right lung) and ruptured (left lung) echinococcal cysts

Echinococcus granulosus [Figure 1-19-13] · · · ·

Pulmonary cysts acquired in childhood Diagnosis 30-40yo Intact cyst - asymptomatic Eosinophilia 25-40%

Pneumonia

188

Chest Radiology

Echinococcus granulosus: Pathology ·

Hydatid cyst consists of 3 layers ➢ Pericyst – host inflammatory cells ➢ Exocyst – acellular laminated membrane ➢ Endocyst – fluid-filled germinal center, daughter cysts

Echinococcus granulosus: Radiology ·

Intact cyst ➢ Well demarcated, homogeneous mass ➢ Spherical when central, ovoid when peripheral ➢ Multiple 20-30% ➢ Lower lobes 60%

Echinococcus granulosus: Radiology ·

·

Impending Rupture ➢ Crescent sign - air between pericyst and laminated membrane Ruptured cyst ➢ Water lily sign – rupture of endocyst

Paragonimiasis westermani ·

· ·

Trematode (lung fluke) ➢ endemic to Asia ➢ Contaminated freshwater crab Jejunum – peritoneal cavity – diaphragm – pleura – lung Chronic granulomatous reaction

Paragonimiasis westermani: Radiology ·

·

Pulmonary findings dependant on stage of infection ➢ PTX and pleural infection during pleural penetration by juvenile worms ➢ Transient, patchy consolidation and linear tracts during larval migration Peribronchial cysts associated with mature worm

Ascariasis lumbricoides · ·

· ·

Roundworm infection Most common parasitic infection ➢ Endemic worldwide ➢ 25-95% prevalence ➢ Highest incidence in children Large iingestion associated with pneumonitis Small bowel – systemic circulation – alveoli - trachea – small bowel

Strongyloides stercoralis · ·

Round worm Skin – systemic circulation – alveoli – trachea – small bowel

Ascariasis Strongyloides: Radiology · · ·

Bronchopneumonia Patchy, transient consolidation Eosinophilic pneumonia

B. Anthracis: Anthrax ·

· ·

Gram+ spore forming rod ➢ Dormant spores are virulent Infection typical in livestock Exotoxin production associated with hemorrhagic mediastinitis, edema and pleuritis

Earls Radiology:222:305, 2001

Chest Radiology

189

Pneumonia

Complications of Pneumonia [Figure 1-19-14] · ·

Figure 1-19-14

Pleural Infection Empyema ➢ Purulent exudate ➢ WBC>25,000 ➢ pH90% Reiter Disease – 63%–75% Psoriasis – not increased without arthritis ➢ with peripheral arthritis – 20% ➢ with axial arthritis – 50% IBD with axial arthritis – 50%

Figure 4-24-16

Psoriatic Arthritis

• • •

Peak ages – 20–40 years M:F – 1:1 ➢ Spine and DIP – M>F ➢ Symmetric polyarthritis – F>M Arthritis in 5%–8% of patients with psoriasis Skin dz before arthritis in 75% Arthritis before skin dz in 10%

Psoriatic Arthritis: Radiographic Manifestations • • • • •

•

Fusiform soft tissue swelling Maintenance of mineralization Dramatic joint space loss Bone proliferation Marginal erosions predominate ➢ “Pencil-in-cup” erosions Bilateral asymmetric dz

Psoriatic arthropathy of the hands involving the interphalangeal joints of both hands in a bilateral but asymmetric pattern

Figure 4-24-17

Psoriatic Arthritis: Radiographic Manifestations •

[Figures 4-24-16 and 4-24-17]

•

Hand/Feet ➢ Distribution ❖ IP joints – asymmetric ❖ Ray distribution ❖ RA distribution ➢ Acroosteolysis ➢ Ankylosis – ~ 15% ➢ Calcaneal erosion – plantar bone proliferation Wrist – pancarpal

Typical central erosion in patient with erosive osteoarthritis compared to marginal erosions seen in patient with psoriatic arthritis Approach to the Inflammatory Arthropathies

916

Musculoskeletal Radiology

• • • • • • •

Erosive Osteoarthritis

Figure 4-24-18

Asymmetrical soft tissues around joint Normal mineralization Nonuniform loss of joint space Central “sea gull” erosions Osteophytes Subchondral sclerosis Distribution – symmetrical

Psoriatic Arthritis: Radiographic Manifestations •

•

• • • •

SI Joints – 30%–50% ➢ Bilateral asymmetrical (symmetrical) ➢ Erosion (iliac > sacral) and repair Spine – 17% ➢ Large, bulky, lateral bone outgrowths ➢ Unilateral or bilateral, asymmetrical ➢ Infrequent apophyseal involvement in lumbar spine

Reactive Arthritis: (Reiter Disease) Young adults M:F – 50–1:1 Annual incidence – 30–40/100,000 Frequently associated with infection ➢ Urethritis/cervicitis ➢ Diarrhea – Shigella, Salmonella, Campylobacter

Typical fluffy inflammatory plantar calcaneal enthesophyte that parallels the undersurface of the calcaneus in patient with reactive arthritis. The bone is dense and an erosion is present in the posterior superior aspect of the calcaneus

Figure 4-24-19

Reiter Disease/Reactive Arthritis: Radiographic Manifestations • • • • • • • •

Diffuse soft tissue swelling Early – juxta-articular osteoporosis Late – normal mineralization Uniform joint space loss Aggressive marginal erosions Bone production Bilateral asymmetrical distribution Feet, ankles, knees and SI joints

25 year old man with reactive arthritis. Erosions and subluxation are seen in the metatarsal phalangeal joints in a bilateral but asymmetric pattern. Subtle bone formation is present along the medial aspect of the navicular and the medial cuneiform of the left foot as a manifestation of the asymmetric nature of this disease

Reiter Disease/Reactive Arthritis: Radiographic Manifestations •

[Figures 4-24-18 to 4-24-20]

• •

• •

Figure 4-24-20

Feet – 40%–55% ➢ IP’s and MTP’s ➢ Erosions with repair ➢ Periostitis along diaphyses Calcaneus – 25%–50% ➢ May be “sole” sight of disease ➢ Plantar and posterior erosions ➢ Enthesophytes Ankle – 30%–50% ➢ Joint space loss and periostitis Knee ➢ Effusion ➢ Joint space loss and periostitis SI joints ➢ Bilateral asymmetric ➢ Erosions and repair

Bone formation at the posteromedial aspect of the distal tibia in a patient with psoriatic arthritis


917

Approach to the Inflammatory Arthropathies

• • • • •

Reiter vs. Psoriatic

• • • •

Figure 4-24-21

Juxtaarticular osteoporosis Periostitis without joint findings Less ankylosis of IP joints Tendency to involve MTP vs. IP joints Lower extremity involvement predominates

Ankylosing Spondylitis

•

Peak age of onset – 15–35 years M:F – 3–5:1 Incidence ~ 6.6/100,000 Strong association with HLA B27 Rare in blacks Predilection for axial involvement

AS-Radiographic Manifestations •

Typical presentation of ankylosing spondylitis with erosions and sclerosis involving the inferior aspect of the SI joints in a bilateral and symmetric pattern

[Figures 4-24-21 and 4-24-22]

•

• • • • • • •

Sacroiliac disease ➢ Bilateral symmetric – same as enteropathic ➢ Erosions predominate iliac vs. sacrum ➢ Sclerosis ➢ Ankylosis Other pelvic dz ➢ Pubic symphysis – 16%–23 % erosion and ankylosis ➢ Enthesitis – ilium and ischium

Figure 4-24-22

Sacroiliitis: Differential Diagnosis Ankylosing Spondylitis Enteropathic Arthropathy Psoriasis Reactive Arthritis Hyperparathyroidism Osteiitis Condensans Infection Ferguson view of the pelvis with bilateral symmetric sacroillitis in patient with ankylosing spondylitis

AS-Radiographic Manifestations •

[Figures 4-24-23 to 4-24-25]

Spine Disease – ascends from lumbar to cervical ➢ Discovertebral destruction ❖ Romanus and Andersson lesion ➢ Shiny corner sign ➢ Squaring of vertebral body ➢ Syndesmophyte ➢ Bamboo spine ➢ Trolley track and Dagger signs ➢ Atlantoaxial disease

Figure 4-24-23

Density confined to the anterior superior and inferior end plates of the lumbar spine resulting in the classic “shiny corner” presentation of ankylosing spondylitis. Note the lack of anterior concavity of the vertebral bodies that contributes to its “squared” appearance


918


Figure 4-24-24

Figure 4-24-25

Lateral radiograph of the cervical spine in patient with ankylosing spondylitis shows thin posterior and anterior syndesmophytes and fusion of the facet joints. Lateral radiograph of the thoracic spine also shows thin posterior and anterior syndesmophytes

The normal lumbar vertebral body is concave anteriorally. The 41 year old patient with ankylosing spondylitis shows bone formation at the anterior aspect of the vertebral bodies resulting is a squared appearance

Figure 4-24-26

DISH: Diffuse Idiopathic Skeletal Hyperostosis [Figure 4-24-26] • •

• • •

Common disease – 12% of elderly population Flowing bulky paravertebral ossification ➢ Four contiguous vertebral bodies Thoracic>lumbar>cervical Enthesophytes – particularly pelvis Absence of erosions/ joint abnormality

• • • •

Uveitis Ascending aortitis/ aortic valve disease Cardiac conduction abnormalities Interstitial lung dz - upper lobes

AS-Extraskeletal Manifestations

•

Juvenile Chronic Arthritis • • • • •

JRA (seronegative) – 70% ➢ Still Disease, pauci/monarticular, polyarticular Juvenile-onset adult type RA – 10% Juvenile-onset ankylosing spondylitis Psoriatic arthritis Enteropathic arthritis Reactive/ Reiter arthritis

• • • • • • •

M:F = 1:1 Age usually less than 5 years Acute febrile illness Rash Generalized adenopathy/hepatosplenomegaly Pericarditis Mild joint findings – arthralgias/mild arthritis

Bulky paravertebral ossification in patients with DISH is usually easily distinguished from the thin anterior syndesmophytes of ankylosing spondylitis that typically have no horizontal component

Still Disease: Systemic Disease (Classic)


919


• •

JRA-Still Disease: Pauci or Monoarticular

• • • • • • •

Figure 4-24-27

Females more commonly than males Large joint disease ➢ Knees, ankles, elbows, and wrists

JRA: Polyarticular M:F = 1:1 Symmetric arthritis Hands – MCP, PIP Wrists Knees/Ankles Feet – intertarsal, MTT, MTP, IP’s Cervical spine

Juvenile-onset adult type RA (Seropositive JRA) • • • • •

Classic manifestation of mono or pauciarticular JRA. The left knee is enlarged and shows advanced bone age in comparison to asymptomatic right knee. Note the lack of erosions and joint space narrowing

F>M >10 years of age at onset Polyarticular Subcutaneous nodules Vasculitis

Figure 4-24-28

JRA: Radiographic Manifestations • • •

[Figures 4-24-27 to 4-24-31]

• • • • • • • •

Fusiform soft tissue swelling Osteoporosis Joint space loss ➢ Not prominent in monoarticular ➢ May be rapid in sero(+) JRA ➢ Ankylosis – hands, wrists, cervical spine Bone erosion – not prominent finding Periostitis ➢ Phalanges, metacarpals, metatarsals Ballooned epiphyses Accelerated skeletal growth Premature fusion of physes

17 year old man with polyarticular JRA and left hip pain. The femoral heads are enlarged resulting in “ballooned” appearance of the epiphyses

Osteoarthritis

• • •

Most common arthropathy ~ 80% of patients over 75 years Second only to CHD as cause of work disability for men > 50 years of age Primary – no underlying abnormality Secondary -preexisting metabolic, anatomic, traumatic, or inflammatory condition

Figure 4-24-29

Osteoarthritis: Definition American College of Rheumatology “ a heterogeneous group of conditions that lead to joint symptoms and signs which are associated with defective integrity of articular cartilage, in addition to related changes in the underlying bone at the joint margins.”

Coronal SPGR image of the left hip in patient with JRA showing marked irregularity in the articular cartilage


920


Figure 4-24-30

Figure 4-24-31

Polyarticular JRA of the hands. Generalized osteoporosis and joint space narrowing is present with striking lack of erosions

• • • •

Adult patient with JRA as a child. Note ballooned appearance of the metacarpal heads right greater than left. Wrist involvement was also aymmetric in this patient. Minimal erosions are seen

Osteoarthritis: Clinical

• •

Increasing prevalence with age over 40 Pain, stiffness, and loss of range of motion Symptoms may regress or be cyclic Risk Factors ➢ Heredity – AD trait with Heberden nodes ➢ Obesity – risk factor for knee and possibly hand ❖ Not risk factor for hip ➢ Hypermobility – increases risk ➢ Occupation – increased risk in heavy manual labor ❖ No increased risk from recreational sports ➢ Diabetes – increases risk ➢ OA in one joint increases risk for other joints ➢ Osteoporosis – protective effect in hip OA ➢ Cigarette smoking – protective effect

Osteoarthritis and Pain

• • •

• • • • • • •

Most common and important complaint Source ➢ raised intraosseous pressure ➢ synovitis/bursitis/tenosynovitis ➢ periosteal elevation ➢ muscular imbalance Less common in very old or young Psychosocial factors Radiographic predictors ➢ Osteophytes in knee good predictor ➢ Joint space narrowing in hip predictor ➢ Good in first CMC joint ➢ Poor in hand IP joints

Osteoarthritis: Radiographic Manifestations Normal mineralization Nonuniform joint space loss Absence of erosions Subchondral new bone formation Osteophyte formation Subchondral cysts Subluxations


921


• •

Osteoarthritis: Subchondral “Cysts” [Figure 4-24-32]

• • •

Figure 4-24-32

Not true cysts Intrusion ➢ Defect in cartilage leads herniation of joint fluid into bone ➢ Cyst size based on joint pressure Contusion ➢ Repeated insult to subchondral bone leads to resorption

Osteoarthritis: Osteophytes [Figures 4-24-33 to 4-24-35] • •

Tend to occur at the margins of joints Produce “enlargement” of joint ➢ attempt to stabilize joint Can be central – “button osteophyte” May not be dramatic in osteoporotic women

Figure 4-24-33

Typical subchondral lucencies in specimen radiograph of osteoarthritic femoral head

Osteophytes of the interphalangeal joints of the hands are usually best appreciated on lateral radiographs

Figure 4-24-34

Figure 4-24-35

Osteoarthritis of the hip with superior lateral joint space narrowing accompanied by subchondral sclerosis, subchondral cyst formation and osteophyte production

Large osteophytes projecting from the articular surfaces of the medial and lateral femoral condyles


922


• • •

Osteoarthritis: Subchondral Sclerosis

Figure 4-24-36

Also known as eburnation Stimulated by loss of hyaline cartilage Combination of new bone on existing trabeculae from microfracture and repair

Osteoarthritis: Radiographic Manifestations •

[Figures 4-24-36 and 4-24-37]

•

Hands ➢ DIP joints – Heberden nodes ➢ PIP joints – Bouchard nodes Wrist ➢ First metacarpal-carpal joint

Figure 4-24-37 Patient A shows Heberdon nodes from osteophytes and soft tissue swelling at the distal interphalangeal joints. Bouchard nodes are seen at the proximal interphalangeal joints of patient B

Figure 4-24-38

Hooked osteophytes are seen involving the second and third metacarpal heads. These may be seen in hemochromatosis, CPPD arthropathy or osteoarthritis

Osteoarthritis - Knee: Radiographic Manifestations • • • • •

[Figures 4-24-38 and 4-24-39]

May require weight-bearing views Medial compartment – 75% Patellofemoral joint – 48% Lateral compartment – 26% Pancompartmental ➢ Think deposition dz or prior inflammatory arthropathy

Non weight bearing AP of the knee (A) shows osteoarthritis in the medial compartment with subchondral sclerosis and osteophyte formation but the joint space appears maintained. AP weight bearing view of the knee (B) shows the expected loss of joint space in the medial aspect of the knee

Figure 4-24-39

Osteoarthritis - Hip: Radiographic Manifestations •

• •

Superolateral migration ➢ 60% ➢ M>F Medial migration ➢ 25% ➢ F>M Axial migration ➢ Think deposition dz or prior inflammatory dz

56 year old woman with acromegaly. Manifestations of osteoarthritis are seen but the joint spaces are widened rather than narrowed


923


Hip Joint Space Narrowing

• •

Osteoarthritis - Foot: Radiographic Manifestations

• •

Occurs along lines of weight bearing First MTP joint ➢ Hallux rigidus ➢ Hallux valgus First MTT joint Talonavicular joint ➢ Dorsal talar beak (coalition vs. DJD)

References 1. 2.

Brower A: Arthritis in Black and White, 2nd ed. Philadelphia, Pa: WB Saunders; 1997: 252. Resnick D ed. Diagnosis of bone and joint disorders, 4th Ed. Philadelphia: W.B.Saunders, 2002:


924


MRI of the Rotator Cuff Donald J. Flemming, MD MRI of Rotator Cuff Tears Utopia CSS FT ESS FT CCS PT ESS PT

Sensitivity 89%–100% 56% 78% 0% 71%

Specificity 88%–97% 73% 83% 68% 71%

CCS-Clinical Community; ESS-Experienced Specialist Arthroscopy 1997; 13:710–719 • • • • •

Review anatomy/positioning Review MR appearance of tears Discuss problem tears Discuss clinical mimics of rotator cuff tear Discuss the radiologic report

• • • • • • • • • • •

Rotator Cuff Disease Impingement Arthritis Adhesive Capsulitis Cervical Spine Referred Pain Instability Fracture Osteonecrosis Nerve Entrapment Syndromes Bursitis

• • • • •

Radiographs Arthrography CT Arthrography Ultrasound MRI

• •

Humeroacromial space 50% of tendon thickness

Coronal T2 weighted image through the supraspinatus tendon (left) shows a deep partial undersurface tear. Coronal T2 weighted image through the supraspinatus tendon of a different patient (right) shows fluid signal interrupting the articular surface of the supraspinatus tendon but the bursal surface is intact indicating an undersurface tear. Both patients have SLAP tears of the superior labrum

Partial Undersurface [Figure 4-25-12]

MRI of the Rotator Cuff

928


Intrasubstance vs Partial US: Value of ABER [Figure 4-25-13] • • •

Figure 4-25-13

Rim Rent Tear [Figure 4-25-14]

• •

Seen in young patients Usually anterior Intrasubstance vs. partial undersurface

Subscapularis Tears [Figure 4-25-15] • • • • •

T1 fat saturated image of the shoulder following indirect arthrography in the ABER position (left) shows the undersurface of the infraspinatus tendon is intact. The conventional coronal oblique T1 weighted image through infraspinatus tendon was suggested an undersurface tear

Abnormal lift-off test on PE Uncommon – 2% of all tears ➢ LHBT dislocation-49% Look for on axial and anterior coronals ➢ Sagittals provide more clues Devastating to surgeon if missed Easy to miss on arthroscopy

Figure 4-25-14

Long Head of Biceps Tendon

•

Abnormality frequently associated with RCT Medial Dislocation ➢ Abnormal bicipital groove ➢ Chronic impingement ➢ Usually extra-articular ➢ Intra-articular with/without subscapularis tendon injury ➢ Associated with degeneration of tendon

Long Head of Biceps Tendon •

Tendonitis ➢ Increased signal in tendon ➢ Thickening of tendon Rupture of tendon ➢ Intracapsular ➢ Extracapsular ➢ Ovoid/ heart shaped – partial tear

Coronal oblique fat sat T2 weighted image shows horizontally oriented increased signal in the insertional portion of the posterior supraspinatus tendon consistent with a tear but it is difficult to determine whether the tear is an undersurface or intrasubstance defect

Figure 4-25-15

Subscapularis Insertion [Figure 4-25-16]

Figure 4-25-16 Sagittal T2 weighted image at the level of the lesser tuberosity shows a focus of high signal in the subscapularis tendon representing a partial undersurface tear. The tear is also seen on the axial T1 weighted gradient echo image following indirect arthrography (right) but is easier to see on the sagittal image

Axial gradient echo image through the subscapularis tendon (A) that appears to only insert on the lesser tuberosity on this image. However, photo of a gross specimen and a photomicrograph through the insertion of the subscapularis show that the tendon inserts on both the lesser and greater tuberosity. The transverse ligament covering the bicipital groove is not actually a ligament but represents the portion of the subscapularis tendon that inserts on the greater tuberosity [Courtesy Tim Sanders, M.D.]


929


• • •

Rotator Cuff Atrophy

•

Figure 4-25-17

Rotator cuff tear Acute brachial neuritis Nerve entrapment syndromes ➢ Suprascapular nerve entrapment ➢ Quadrilateral space syndrome

Suprascapular Nerve Entrapment

• •

Suprascapular Notch ➢ Supraspinatus/ Infraspinatus innervation Spinoglenoid Notch ➢ Infraspinatus innervation Atrophy of SSM and ISM Look for mass in region of suprascapular notch

•

Infraspinatus atrophy

• • • • •

Axillary N. Compression Fibrous band Pain, paresthesia Atrophy of deltoid and/or teres minor Weightlifters

•

Spinoglenoid Notch Entrapment [Figure 4-25-17] Quadrilateral Space Syndrome [Figure 4-25-18]

Figure 4-25-18

Coronal oblique fat saturated T2 weighted image shows a paralabral cyst extending from a posterior superior labral tear into the spinoglenoid notch. A clinical photo (B) in the same patient shows marked atrophy of the right infraspinatus muscle belly as indicated by the loss of soft tissue inferior to the scapular spine. Sagittal T2 image (C) medial to the spinoglenoid notch shows atrophy and denervation edema in the infraspinatus muscle belly The quadrilateral space is bounded by teres minor, teres major, long head triceps and the humerus

• • •

Clinical Mimics of Rotator Cuff Tear

• •

Calcific tendonitis Adhesive capsulitis Subacromial bursitis

Calcific Tendonitis • • •

Rotator cuff most common site Primary or secondary disorder? HADD in tendon Concretion – low T1 and T2 Variable surrounding edema May erode cortex/ invade marrow


930


• • •

Adhesive Capsulitis [Figure 4-25-19]

• •

Figure 4-25-19

Clinical mimic of cuff tear Capsule thickened Abnormal enhancement — IV gad

Pectoralis Major Tear

• • •

Weight-lifters Sternal and clavicular heads ➢ Sternal head superior on humerus ➢ Clavicular head inferior Sternal head tear most common Use torso coil and coronal obliques Myotendinous vs. tendon

Myotendinous Tear of Pectoralis [Figure 4-25-20] Figure 4-25-20

Axial T1 weighted gradient echo image (A) shows marked enhancement in the anterior inferior capsule following IV contrast administration indicating adhesive capsulitis in the atraumatic setting

Coronal oblique T1 (left) and fat sat T2 (right) weighted images through the pectoralis major muscle show hemorrhage associated with a myotendinous injury. The distal tendon is intact

• • •

Radiologic Report

• •

Acromion-os acromiale Tendon – normal, tendinosis, tear Size and location of tear ➢ Massive>5cm Partial thickness tear ➢ > or < 50% thickness of tendon Retraction/Muscle atrophy

References 1. 2.

Zlatkin MB, Needell SD, Hoffman C. MRI of the Shoulder, 2nd Edition. Lippincott Williams & Wilkins, Philadelphia, PA. 2003. Steinbach LS, Peterfy CG, Tirman PFJ, Feller JF eds. Shoulder Magnetic Resonance Imaging. Lippincott Williams & Wilkins, Philadelphia, PA. September 1998


931


MR Arthrography of Glenohumeral Instability Timothy G. Sanders, MD •

Glenohumeral Joint Intrinsically Unstable joint ➢ Shallow glenoid fossa ➢ Large articular surface of the humeral head • Static Stabilizers ➢ Joint capsule ➢ Glenohumeral Ligaments ➢ Glenoid labrum • Dynamic Stabilizers ➢ Rotator cuff ➢ Long Head of the Biceps Tendon

Figure 4-26-1

•

Classification TUBS ➢ Traumatic ➢ Unidirectional ➢ Bankart ➢ Surgery • AMBRI ➢ Atraumatic ➢ Multidirectional ➢ Bilateral ➢ Rehabilitation ➢ Inferior Capsular Shift

Superior glenohumeral ligament

Figure 4-26-2

• •

Multidirectional Instability AMBRI Patient Causes of Multidirectional Instability ➢ Hypermobility or Laxity ➢ Stretching or Overuse of Support Structures • MR Imaging not usually Required ➢ MR Findings Nonspecific ➢ MR Useful if Direction Unknown to Rule Out Conventional Causes • • • • •

Anterior Stabilizers

• •

Middle glenohumeral ligament

Labrum Glenohumeral Ligaments Capsule Subscapularis Muscle Most Important Anterior Stabilizer: Inferior Glenohumeral Labroligamentous complex ➢ Anteroinferior labrum ➢ Anterior Band of the Inferior Glenohumeral Ligament

Figure 4-26-3

Normal Labrum

• •

Anterior and Posterior Labrum best seen in the Axial Plane LABRUM: ➢ Dark on all Pulse Sequences ➢ May be triangular, rounded, or blunted

Normal Superior Labrum Seen Best in the Coronal Plane Superior Labrum: ➢ Dark on all pulse sequences ➢ Triangular ➢ Extends off of Superior Glenoid

MR Arthrography of Glenohumeral Instability

Inferior glenohumeral ligament

932


• • •

Superior Glenohumeral Ligament [Figure 4-26-1]

•

Prevents inferior subluxation with arm in 0º abduction Courses from superior glenoid tubercle to lesser tuberosity Parallels Coracoid process

Figure 4-26-4

Middle Glenohumeral Ligament [Figure 4-26-2] • • • • • •

Prevents external rotation of humeral head when arm is between 45º and 60º of abduction Originates at superior glenoid tubercle Courses obliquely superficial to the anterior labrum Blends with the deep fibers of subscapularis Most variable of the glenohumeral ligaments

Inferior Glenohumeral Ligament [Figure 4-26-3] • • •

Most important GHL Prevents anterior subluxation with arm in full abduction and external rotation Extends from anterior inferior labrum to humeral neck Lax with arm in neutral position Redundant when the arm is in neutral position

• • •

Scan plane along the long axis of the humeral shaft Coronal scout with arm in ABER position Stretches anterior band of IGHL

Left: Scan plane for the ABER view Right: Normal ABER view

Figure 4-26-5

Scout Position and Scan Plane for ABER [Figure 4-26-4]

•

Normal Anatomic Variants 1. Cartilage Undermining ➢ Articular cartilage hyaline- intermediate signal intensity ➢ Labrum- fibrocartilage- low signal intensity ➢ Smooth, tapering ➢ Does not Extend Completely Beneath Labrum • 2. Sublabral Foramen (Hole) ➢ Occurs only in the anterosuperior quadrant ➢ Complete detachment of the labrum from the glenoid • 3. Sublabral Recess [Figure 4-26-5] ➢ Smooth, tapering ➢ Extends toward the glenoid ➢ No signal extends into the black triangle of the superior labrum ➢ Can mimic a SLAP tear • Buford complex [Figure 4-26-6] ➢ 1.5 % of patients ➢ Can mimic anterior labral tear ➢ Thick cord-like MGHL ➢ Absent or diminutive anterior-superior labrum • •

Sublabral recess

Figure 4-26-6

Anterior Instability 95% of all dislocations Mechanism ➢ Fall on outstretched arm ➢ Abduction and external rotation

Bufford complex Musculoskeletal Radiology

933


•

Bankart Lesions [Figure 4-26-7] • • • •

Figure 4-26-7

The most common injury following anterior dislocation First-time dislocators under 35 y.o. Anterior labro-ligamentous avulsion with disruption of the medial scapular periosteum

Osseous Bankart Lesion [Figure 4-26-8]

•

Fracture of inferior glenoid Disruption of the cortex of the anteroinferior glenoid

Hill-Sachs Lesion • • • • •

Bankart lesion

Results from impaction of humeral head against anterior-inferior glenoid Associated with Bankart lesion Normally: Top 3 images round Hill-Sachs: flattening or concavity Acute: + Edema

Figure 4-26-8

Double Axillary Pouch Sign [Figure 4-26-9]

• • • • •

Double axillary pouch: small collection of contrast in inferior labrum seen on coronal images

Perthes Lesion Bankart variation (non-displaced) Labro-ligamentous disruption Medial scapular periosteum intact May resynovialize in place Best detected on ABER view

Osseous Bankart

Figure 4-26-9

Nondisplaced Tear Anteroinferior Labrum Perthes Lesion [Figure 4-26-10] • • • •

ALPSA Lesion

•

Anterior labroligamentous periosteal sleeve avulsion Intact medial periosteal periosteum Medialized Bankart lesion Surgical repair technique differs from Bankart Double axillary pouch

Medialized Bankart Lesion [Figure 4-26-11]

• •

ALPSA- Anterior labroligamentous periosteal sleeve avulsion

Figure 4-26-10

Chronic Medialized Bankart Lesion •

Labrum scars down medially Scar tissue mounds up covering medialized labrum and resynovializes Treatment: complete Bankart and reconstruction

Perthes Lesion

Figure 4-26-11

ALPSA lesion MR Arthrography of Glenohumeral Instability

934


•

Axillary Nerve Neuropraxy [Figure 4-26-12]

Figure 4-26-12

Axillary nerve can be stretched at time of anterior dislocation resulting in denervation atrophy: Deltoid and Infraspinatus muscles • Denervation atropy: ➢ Acute: edema (high signal on T2) ➢ Chronic: fatty (high signal on T1) • • • • •

Clinical presentation can be confusing Tear supraspinatus tendon Fracture greater tuberosity Avulse subscapularis and anterior capsule from the humerus MRI can play pivotal role in directing patient therapy

• • •

First time dislocation over age 35: Bankart lesion uncommon Rotator cuff tendon becomes the weak link

•

Avulsion fracture of the greater tuberosity

First Time Dislocation Over Age 35

Tear of the Supraspinatus Tendon Denervation atrophy of Deltoid muscle

First Time Dislocation Over Age 35 •

Figure 4-26-13

Greater Tuberosity Fracture • • • •

Avulsion of the greater tuberosity is often occult radiographically Can mimic RCT Treated conservatively MRI can accurately distinguish

Avulsion of Subscapularis • •

Subscapularis muscle can avulse off of lesser tuberosity Associated with dislocation of the biceps tendon Seen best in axial plane

• •

Disruption of subscapularis at musculotendinous junction Requires surgical repair

Humeral Avulsion of the Glenohumeral Ligament (HAGL) Lesion

Disruption of Subscapularis

• • • •

Figure 4-26-14

Hagl Lesion Humeral avulsion of the glenohumeral ligament Results from dislocation No age predilection MR findings: contrast extravasation from joint capsule/ avulsion of subscapularis

• • • • •

Inferior GHL can disrupt anywhere along course Humeral attachment/ mid substance Difficult to detect with scope Cause of failed repairs Can present on MRI as avulsion of subscapularis muscle

• • • • •

2% - 4% of all traumatic dislocations 20% - 25% of shoulder instability cases in active duty military population Adduction with internal rotation Seizure, electrocution, weight lifting, swimming, lineman blocking “Reverse” Hill Sachs, Bankart

• • •

Repetitive microtrauma: non-displaced posterior labral tear Reverse Bankart Reverse Hill Sachs

Hagl Lesion [Figure 4-26-13] Reverse Bankart and Hill Sachs Lesion

Posterior Instability

Posterior Instability [Figure 4-26-14]


935


• • • •

Glenolabral articular disruption Forced adduction injury (humeral head impacts the glenoid fossa) Clinically a stable lesion Partial tear anteroinferior labrum / articular cartilage injury

• • • •

Non displaced tear anteroinferior labrum Best seen on ABER Articular Cartilage Injury Best seen on axial or coronal

Glad Lesion [Figure 4-26-15]

Figure 4-26-15

Glad Lesion [Figure 4-26-15]

• •

Posterior Superior Glenoid Impingement • • •

Also known as “Internal Impingement” Undersurface tearing of posterior rotator cuff (posterior SST or IST) Impingement between posterior labrum and greater tuberosity Throwing athletes- posterior shoulder pain Associated with anterior instability

[Figure 4-26-16]

Posterior Superior Glenoid Impingement • • •

Undersurface of posterior rotator cuff impinged between the greater tuberosity and the posterosuperior labrum Seen best on ABER view Glenolabral Articular Disruption (GLAD) Lesion

Posterior Superior Glenoid Impingement MR Findings: ➢ Undersurface tear of posterior rotator cuff ➢ Degenerative changes of posterosuperior labrum ➢ Cystic change in greater tuberosity ➢ Internal impingement seen on ABER view

Figure 4-26-16

[Figure 4-26-17] • Scarring and thickening of the posterior capsule and has recently been described as a source of potential pain in throwing athletes • MR imaging demonstrates thickening of the posterior capsule

Glenohumeral Internal Rotation Deficit (GIRD)

•

“SLAP” Tears The superior labrum, anterior-to-posterior lesion, can include biceps tendon • Mechanism ➢ Fall on outstretched arm ➢ Repetitive overhead activity (throwing, swimming) • Symptoms: pain with overhead activity, catching, popping sensation • •

Important factors to observe

Posterior Superior Glenoid Impingement

Figure 4-26-17

Abnormal signal in superior labrum Extent of lesion ➢ Posterior labrum ➢ Anteroinferior quadrant • Biceps involvement • Type of SLAP tear

Glenohumeral Internal Rotation Deficit (GIRD) MR Arthrography of Glenohumeral Instability

936


•

SLAP Lesion

Figure 4-26-18

Type I [Figure 4-26-18] ➢ Fraying and degeneration; but labrum firmly attached • Type II [Figure 4-26-19] ➢ Fraying of labrum and superior labrum avulsed from glenoid ➢ Signal extends into the triangle of the superior labrum • Type III [Figure 4-26-20] ➢ Bucket-handle tear of the superior labrum; biceps tendon remains intact ➢ On MR imaging: fragment seen displaced into superior joint space • Type 4 [Figure 4-26-21] ➢ Bucket-handle tear of superior labrum involves biceps anchor ➢ Biceps involvement best seen on axial or sagittal images

SLAP Type I: Fraying of the undersurface of labrum

Figure 4-26-19

Figure 4-26-20

SLAP Type II: Avulsion of labrum from glenoid

Figure 4-26-21

SLAP Type III: Bucket-handle tear of the superior labrum

•

Pitfalls: SLAP Tears 1. Sublabral recess ➢ Smooth, tapering ➢ No signal in superior labrum ➢ SLAP tear: any signal extending into black triangle • 2. Sublabral recess: axial images ➢ Smooth linear collection of contrast ➢ SLAP on axial images: irregular contrast collection ➢ Sublabral recess: coronal images ➢ No displacement of superior labrum ➢ Type 2 SLAP tear ➢ Labrum pulled away from glenoid •

SLAP Type IV: Bucket-handle tear with involvement of biceps anchor

Paralabral Cyst • • • • • • • • •

High association with labral tears and GH joint instability Superior labral cyst: SLAP tears Posterior labral cyst: posterior labral tears Labral tear may resynovialize Can result in shoulder pain and adjacent nerve entrapment DDX: Intramuscular cysts of rotator cuff associated with PT tear of the cuff tendon

Figure 4-26-22

Paralabral Cysts [Figure 4-26-22] SLAP tear with superior paralabral cyst Suprascapular notch Suprascapular nerve entrapment Denervation edema: supraspinatus and infraspinatus muscles


937

Left: SLAP tear with paralabral cyst Right: Denervation edema within supraspinatus and infraspinatus muscles


• • • • •

Paralabral Cysts

• • • • • •

Figure 4-26-23

Posterior paralabral cysts Extend into spinoglenoid notch Entrapment of suprascapular nerve Persistent shoulder pain for 3 years Small anterior labral tear with small adjacent paralabral cyst

Paralabral cysts

• •

Small inferior labral cyst Teres Minor normal 3 years later; persistent pain Paralabral cyst larger Axillary nerve entrapment Atrophy Teres Minor

Left: Direct arthroscopic repair of Bankart lesion. Suture anchors at the 3, 4, and 5 o’clock positions Right: Sagittal MR image shows location of suture anchors

Figure 4-26-24

Labral Repair: Surgical Approach Direct repair of labral and capsular lesions Indirect repairs ➢ Staple capsulorapphy (Du Toit & Roux) ➢ Subscapularis manipulation to tighten anterior capsule (Putti Platt/ Magnuson Stack) ➢ Movement of the coracoid process (Bristow procedure)

•

Direct Repairs Arthroscopic/ open (deltopectoral interval) ➢ Suture anchors 3-,4-,5-, o’clock position • Capsulorapphy (open/ arthroscopic) ➢ Staple redundant capsule ➢ Done in conjunction with direct repair ➢ High failure rate if done as isolated procedure • Osseous Bankart

Recurrent displace Bankart lesion

MR Findings of Bankart Repair •

Figure 4-26-25

[Figure 4-26-23]

Suture anchor artifact from repair may obscure visualization

MR Findings of Failed Bankart Repair • • •

[Figure 4-26-24]

Recurrent displaced anterior labrum

Failed Bankart Repair [Figure 4-26-25] Missed HAGL lesion In one series– up to 30% of failed repairs

Left: Missed HAGL lesion. IGHL avulsed from humeral neck Center: Missed HAGL lesion. IGHL avulsed from humeral neck Right: HAGL lesion

• •

Displaced fragment anterosuperior labrum Osteochondral defect anterosuperior glenoid

• •

Fraying and irregularity of superior labrum; no displaced fragment Partial thickness articular surface tear rotator cuff

Recurrent SLAP Following Repair [Figure 4-26-26]

Figure 4-26-26

5 Months Following SLAP Repair: Recurrent Pain

Recurrent SLAP tear with a displaced fragment


938


• •

Multidirectional Instability [Figure 4-26-27]

•

Figure 4-26-27

Treated first with rehabilitation Surgery ➢ Inferior capsular shift/plication ➢ Decrease volume of GHJ anteriorly, inferiorly, posteriorly ➢ MR capsular thickening

Hardware Complication [Figure 4-26-28] Proud suture anchor

Synovitis - Prior SA Decompression and Rotator Cuff Debridement: Recurrent Pain • •

•

Synovitis: 4 mm adhesive capsulitis Normal postop capsule: ➢ 2-4 mm after surgical procedure ➢ Thickened and nodular capsule

Postop Infection [Figure 4-26-29] • •

Infectious versus reactive synovitis difficult to differentiate with imaging Thickened enhancing capsule; effusion/ joint destruction/ cartilage loss/ cysts, erosions

. Normal postoperative MR appearance following inferior capsular shift. Thickened capsule

Acute Chondrolysis of the Glenohumeral Joint [Figure 4-26-30] • • • •

Rapid onset chondrolysis refers to a condition in which widespread chondrocyte death occurs within a joint over a relatively short period of time Devastating complication reported following arthroscopy and reconstruction of the GHJ in young individuals Rapid onset pain Marked loss of motion Treatment supportive; eventually total joint arthroplasty

Figure 4-26-28

Chondrolysis Shoulder: Proposed Etiologies • • • • • • •

Damage from use of thermal probe for capsular shrinkage Marcaine pump Left: MR appearance of a proud suture anchor Unknown infectious agent Right: CT appearance of a proud suture anchor Bioabsorbable material Mechanical trauma at time of arthroscopy Chemical trauma to the chondrocytes Event during arthroscopy that triggers an immune response and subsequent Figure 4-26-29 migration of inflammatory cells into the GH joint

Figure 4-26-30

Postoperative infection Acute Chondrolysis of the Glenohumeral Joint following Shoulder Arthroscopy


939


References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19.

Bankart ASB: Recurrent or habitual dislocation of the shoulder-joint. Br J Surg 26: 23-29, 1938 Beltran J, Rosenberg ZS, Chandnani VP, et al: Glenohumeral instability: Evaluation with MR arthrography. Radiographics 17: 657-673, 1997 Chandnani VP, Gagliardi JA, Murnane TG, et al: Glenohumeral ligaments and shoulder capsular mechanism: Evaluation with MR arthrography. Rad 196: 27-32, 1995 Cvitanic O, Tirman PFJ, Feller JF, et al: Using abduction and external rotation of the shoulder to increase the sensitivity of MR arthrography in revealing tears of the anterior glenoid labrum. AJR 169 837-844, 1997 Kaplan PA, Bryans KC, Davick JP, et al: MR imaging of the normal shoulder: Variants and pitfalls. Rad 184: 519524, 1992 Linker CS, Helms CA, Fritz RC: Quadrilateral space syndrome: Findings at MR imaging. Rad 188: 675-676, 1993 Neviaser RJ, Neviaser TJ, Neviaser JS: Concurrent rupture of the rotator cuff and anterior dislocation of the shoulder in the older patient. JBJS 70-A: 1308-1311, 1988 Neviaser TJ: The anterior labroligamentous periosteal sleeve avulsion lesion: A cause of anterior instability of the shoulder. Arthroscopy 9: 17-21, 1993 Neviaser TJ: The GLAD lesion: Another cause of anterior shoulder pain. Arthroscopy 9: 22-23, 1993 Palmer WE, Brown JH, Rosenthal DI: Labral-Ligamentous complex of the shoulder: Evaluation with MR arthrography. Rad 190: 645-651, 1994 Petersilge CA, Witte DH, Sewell BO, et al: Normal regional anatomy of the shoulder. MRI Clin North Am 5: 667681, 1997 Sanders TG, Tirman PFJ, Linares R: The Glenolabral articular disruption lesion: MR arthrography with arthroscopic correlation. AJR 172: 171-175, 1999 Schweitzer ME: MR arthrography of the labral-ligamentous complex of the shoulder. Rad 190: 641-643, 1994 Synder SJ, Karzel RP, Pizzo WD, et al: SLAP lesions of the shoulder. Arthroscopy 6: 274-279, 1990 Tirman PFJ, Bost FW, Garvin GJ, et al: Posterosuperior glenoid impingement of the shoulder: Findings at MR arthrography and MR arthrography with arthroscopic correlation. Rad 193: 431-436, 1994 Tirman PFJ, Feller JF, Jansen DL, et al: Association of glenoid labral cysts with labral tears and glenohumeral instability: Radiographic findings and clinical significance. Rad 190: 653-658, 1994 Tirman PFJ, Feller JF, Palmer WE, et al: The Buford complex—A variation of normal shoulder anatomy: MR arthrographic imaging features. AJR 166: 869-873, 1996 Tirman PFJ, Steinbach LS, Feller, FJ: Humeral avulsion of the anterior shoulder stabilizing structures after anterior shoulder dislocation: demonstration by MRI and MR arthrography. Skeletal Radiol 25: 743-748, 1996 Wolf EM, Cheng JC, Dickson K: Humeral avulsion of glenohumeral ligaments as a cause of anterior shoulder instability. Arthroscopy 11: 600-607, 1995


940


Imaging of Upper Extremity Trauma Timothy G. Sanders, MD •

Imaging of Upper Extremity Trauma

Figure 4-27-1

Anatomic locations ➢ Shoulder girdle, humerus, elbow, forearm, wrist and hand • Structures involved ➢ Bones, joints, articular cartilage, tendons, ligaments • Mechanism of injury ➢ Acute trauma, sports related, repetitive stress injury •

Sterno-clavicular Joint Dislocation most common injury ➢ Anterior more common than posterior Grade II separation of AC Joint • Best evaluated with limited CT scan • Normal distal clavicle extends above manubrium- use symmetry as guide • Plain film- 40º cephalic angulation • • • •

Clavicle Fractures Mechanism: indirect trauma- fall on outer prominence of shoulder Most common site of injury is middle third Healing may result in deformity (extensive callous) Distal third fracture must evaluate coracoacromial ligament integrity

Figure 4-27-2

• • •

Acromio-clavicular Joint Injuries Mechanism: fall on outer prominence of shoulder AC joint: weak capsule and inherently unstable Grade I injury ➢ mild strain of AC joint ➢ Ligaments intact; point tenderness over AC joint ➢ X-rays normal ➢ Treatment conservative- recovery is spontaneous • Grade II injury ➢ moderate strain [Figure 4-27-1] ➢ Disrupted AC ligament ➢ CC ligaments intact ➢ X-ray- widening of AC joint; slight uplifting of distal clavicle ➢ Treatment conservative- recovery is spontaneous • Grade III injury ➢ severe [Figure 4-27-2] ➢ Ruptured AC and CC ligaments ➢ Complete AC separation with increased distance between coracoid and clavicle • •

Grade III separation of the left AC joint

Figure 4-27-3

Surgical Repair of AC Joint Separation Internal fixation: 8-10 weeks Until CC ligaments heal

•

Osteolysis of Distal Clavicle [Figure 4-27-3] Post-traumatic osteolysis ➢ Complication of trauma (occurs within 2 months of injury, self limiting) ➢ Repetitive stress (wt. lifters) ➢ X-ray: loss of normal cortical line- distal clavicle • DDX: rheumatoid arthritis, infection, hyperparathyroid

Post-traumatic osteolysis of distal clavicle


941


• • • • • • •

Scapular Fractures Mechanism: direct trauma to scapula (MVA) Uncommon fractures Frequently missed on X-ray Intra-articular fracture important clinically Fracture of scapular body- source of considerable pain Fracture of the neck of the scapula Non-articular fractures: clinically insignificant; musculature holds fragments in place; conservative treatment

•

CT- imaging modality of choice for evaluating scapula

• • •

Acromion process fractures Direct trauma Restore active range of motion; if severely comminuted- excise fragments

•

Os Acromiale: unfused acromial ossification center

• •

Coracoid fracture: may occur in conjunction with type III AC separation Treatment conservative

•

Trap shooter’s shoulder

Scapular Fractures Scapular Fractures

Scapular Fractures: Acromion Scapular Fractures: Coracoid Figure 4-27-4

Stress Fracture of Coracoid Process • •

Imaging of Glenohumeral Joint • • • • • • •

• • • • •

Standard AP view is oblique to the GH joint Excellent osseous detail (homogeneous distribution of soft tissues) Glenohumeral “True” AP View (Grashey view) Beam tilted 45° laterally Evaluate GH joint, subluxation, loss of articular cartilage Less value for fractures of surrounding structures Axillary view ➢ Evaluate for subluxation/ dislocation; fractures of ant/ post glenoid West Point View: variant of the Axillary View ➢ Improves visualization of the anterior glenoid (Bankart lesion) Scapular “Y” View ➢ Image along plane of scapula; 60° relative to the AP view ➢ Easily acquired in setting of acute trauma ➢ Evaluate for ant/ post dislocation ➢ Poor evaluation of the osseous structures

Figure 4-27-5

Anterior Dislocation [Figure 4-27-4]

• •

AP view of the shoulder demonstrates anterior dislocation of the humeral head

Mechanism: fall on outstretched arm X-ray: humeral head displaced anterior and medial Associated lesions Under 35 y.o. Hill-Sachs defect; Bankart lesion or variant Over 35 y.o. 1. Avulsion fracture greater tuberosity 2. RCT 3. Subscapularis tear

Anterior Dislocation Axillary view Scapular “Y” view

Lesions Associated with Anterior Dislocation •

[Figure 4-27-5]

Occurs secondary to impaction of humeral head against inferior glenoid rim


942

Hill-Sachs deformity


•

Lesions Associated with Anterior Dislocation • • • •

Mechanism: results from impaction of humeral head against inferior glenoid rim Osseous Bankart lesion Fibrous Bankart lesion best evaluated with MR

Figure 4-27-6

Trauma [Figure 4-27-6]

• •

24 y.o. female: persistent painful shoulder after skiing accident Radiographically occult humeral head fractures best evaluated with MR imaging

Posterior Instability • • •

2% - 4% of all traumatic dislocations 20% - 25% of shoulder instability cases in active duty military population Adduction with internal rotation Seizure, electrocution, weight lifting, swimming, lineman blocking “Reverse” Hill-Sachs, Bankart

• • • •

Dislocates straight posterior Sometimes difficult to detect on AP view: 50% missed Locked in internal rotation; most reliable sign Very obvious on axillary view

Posterior Dislocation [Figure 4-27-7]

• •

MR appearance of a radiographically occult fracture of the greater tuberosity

“Trough” Sign • • •

Vertical line of dense cortical bone paralleling the medial cortex of the humerus Results from impaction fracture of the posterior medial aspect of humeral head “Reverse” Hills Sachs Fracture

Figure 4-27-7

“Positive Rim” Sign • •

Widening of the joint is termed the “positive rim sign” Normally the space between the anterior glenoid rim and medial humeral head is 1 cm displacement or >45° angulation; considered significantly displaced No fragment >1 cm or 45°- considered a non-displaced fracture 1 fragment displaced = 2 part fracture; 2 fragments displaced = 3 part fracture; etc

Figure 4-27-8

Humeral Shaft Fractures

• • •

Mechanism: ➢ Indirect twisting force: spiral fx ➢ Direct force: transverse fx ➢ Usually involve mid-shaft Shaft fractures heal easily Rarely require internal fixation Ball-and-socket joint tolerates some degree of angular and rotational malalignment Neer’s Four-segment Classification


943


• • •

Humeral Shaft Fractures [Figure 4-27-9]

•

Figure 4-27-9

Common location for pathologic fractures Minor trauma Children: ➢ Unicameral bone cyst (fallen fragment) ➢ Fibrous dysplasia Adults: ➢ Metastatic lesion

• • • •

Radial head: most common fracture of upper limb in young adults Mechanism: fall on outstretched arm Frequently occult; oblique views; joint effusion Treatment: non-displaced- conservative; comminuted- resection

• • • • • • • • •

Supracondylar fracture: Most common elbow fracture in childhood Risk: brachial nerve injury Mechanism: Fall on outstretched arm Posterior fat pad sign Anterior “sail” sign In setting of trauma indicates occult elbow fracture Pathologic fracture through a unicameral bone Anterior humeral line cyst Intersect the middle 1/3 of capitellum on lateral view Subtle supracondylar fracture results in posteriorly displaced capitellum

• • •

Mechanism: fall on outstretched arm- common in child and adult Posterior or posterolateral Fractures (minor): coronoid/ radial head: child- medial epicondyle

Elbow Fractures: Adult

Elbow Fractures: Child [Figure 4-27-10]

Figure 4-27-10

Elbow Dislocations

• •

Complications of Dislocation

• • •

Brachial artery or nerve damage Post-traumatic ossification: stiffness- intra and periarticular adhesions: forms in subperiosteal and capsular hematoma

Posterior Dislocations in Child

•

Associated with medial epicondyle avulsion in 50% of cases Up to 30% become entrapped in the joint following reduction Treatment: open reduction for >1 cm displacement of medial epicondyle Abnormal anterior humeral line

Radiocapitellar Line [Figure 4-27-11]

• •

Line drawn along long axis of radius should intersect capitellum in any projection

Night Stick Fracture •

Mechanism: direct force to forearm (usually middle 1/3) Isolated fracture of the mid-shaft of the ulna: Usually no displacement or angulation Must exclude associated dislocations

Figure 4-27-11

Normal radiocapitellar line Imaging of Upper Extremity Trauma

944


• • •

Isolated Fractures Ulna: Monteggia [Figure 4-27-12]

• • •

Figure 4-27-12

Ulnar fracture (usually proximal 1/3); radial head dislocation Mechanism: fall on outstretched arm (forced pronation of forearm) Treatment: ➢ Open adult/closed child ➢ Restrict pronation/ supination

Isolated Fractures Radius: Galeazzi

• •

Radius fracture/ distal radioulnar joint dislocation High incidence non-union, delayed union with closed reduction Tx: ORIF- still a tendency to dislocate after ORIF

Monteggia fracture

Essex-Lopresti [Figure 4-27-13] Comminuted radial head fracture/ DRUJ dislocation or instability Interosseous ligament: ➢ Intact: radial head resection ➢ Disrupted: radial head prosthesis

• • •

Childhood: distal radial buckle fracture Young adults: scaphoid fracture Older adults (>40): Colles fracture

• • •

Most common fracture of distal radius in patient over 40 y.o. FOOSH injury with wrist in dorsiflexion Distal fragment displaced dorsally

• • • • • •

A- Extra-articular fx radius B- (A) + ulnar styloid fx C- Intra-articular fx distal radius D- (C) + ulnar styloid fx E- Comminuted fxs of radiocarpal and radioulnar joints F- (E) + ulnar styloid fx

• • •

Volar angulation of distal fragment Younger patient High energy trauma with wrist in volar flexion

• • • •

Longitudinal shear injury of the distal radius Barton: Dorsal rim - Reverse Barton: Volar rim Transverse load with shearing forces Often requires internal fixation: unstable fracture

•

Intra-articular fracture of the radial styloid

Fall on Outstretched Hand

Figure 4-27-13

Colles Fracture

Colles Fracture Classification

Smith Fracture (Reverse Colles)

Comminuted fracture of the radial head

Barton Fracture (Reverse)

Radial Styloid Hutchinson’s/ Chauffeur’s Fracture • • •

Distal Radial Fracture in Childhood Transverse metaphyseal fracture Most common forearm fracture ages 4-10 y.o. May be complete or incomplete ➢ Torus/ Buckle fracture

Evaluation of Suspected Scaphoid Fracture Snuff Box Tenderness • 1. Additional radiographic views ➢ Oblique view ➢ Scaphoid view • 2. Cross-sectional imaging ➢ MRI ➢ CT ➢ Bone scan


945

Top: MR imaging demonstrating an intact interosseous membrane of the forearm Bottom: MR imaging demonstrating disruption of the interosseous membrane of the forearm


Scaphoid fracture: Complication rate is high • • • • • •

[Figures 4-27-14 and 4-27-15]

Figure 4-27-14

AVN Delayed/ nonunion Osteoarthritis AVN complication of scaphoid fracture Recurrent blood supply Risk factors: delayed diagnosis, displacement, proximal fracture site

Figure 4-27-15 Left: Avascular necrosis of the proximal pole of the scaphoid Right: Nonunion of a scaphoid fracture with secondary osteoarthritis

Figure 4-27-16

Recurrent blood supply to the scaphoid bone

• •

Scapholunate Ligament Disruption [Figure 4-27-16]

•

Normal scapholunate distance = 2mm Gap accentuated with “clenched” fist view

Scapholunate Ligament

•

Wrist MR arthrography can increase detection rate of subtle interosseous ligament disruption

Increased scapholunate distance indicating disruption of the scapholunate ligament

Carpal Dislocations [Figure 4-27-17] Dislocations ➢ Lunate: lunate dislocates in volar direction ➢ Perilunate: capitate dislocated dorsally/ lunate remains normal

Figure 4-27-17

Left: AP view of wrist demonstrates lunate dislocation Center: Lateral view of wrist demonstrate lunate dislocation Right: Perilunate dislocation: posterior capitate dislocation, lunate remains in normal position relative to the radius


946


•

Triquetrum Fracture [Figure 4-27-18]

Figure 4-27-18

• • • •

Shearing forces, dorsal avulsion fracture seen only on the lateral view Point tender over dorsal aspect of wrist Mechanism: fall on out-stretched hand Immobilize in plaster for 3 weeks Associated with lunate, perilunate dislocation

• •

Point tenderness over hook of hamate Direct trauma to volar aspect of wrist; surgical resection of hook

• • • • •

Oblique intra-articular fracture/subluxation of base of 1st MC The large fragment subluxes; smaller fragment maintains position Closed treatment: persistent subluxation/ traumatic arthritis Usually treat with ORIF Bennett “Bad”

Hamate Fracture [Figure 4-27-19]

Fracture of First MC- Bennett Fracture [Figure 4-27-20]

• Deforming forces ➢ Abductor pollicis longus pulls the distal fragment in a proximal/dorsal direction ➢ Adductor pollicis muscle stabilizes the volar ulnar lip of the articular surface in its normal position ➢ Thus results in distraction of the 2 fracture fragments

Bennett Fracture

• • •

Triquetrum fracture

Figure 4-27-19

Fracture of First MC- Rolando Fracture

• •

Comminuted Bennett fracture Dorsal subluxation and a separate dorsal fragment Usually treated closed as pinning does not work well on comminuted fragments Worse prognosis than the Bennett fracture Rolando “Ruined”

• • •

Disrupt Ulnar Collateral Ligament May have small avulsion fracture Pin or ORIF

Skier’s Thumb Injury (Gamekeeper’s thumb)

• •

Hook of Hamate Fracture

Figure 4-27-20

Stress Views [Figure 4-27-21] •

Stener Lesion: MR imaging Interposition of adductor pollicis apponeurosis between torn UCL and base of proximal phalanx Surgical lesion

Figure 4-27-21

Bennett Fracture

Disrupted ulnar collateral ligament


947


• • • •

Sudden resisted flexion of DIP joint Finger jammed or distal tip hit with a ball Avulsion of extensor digitorum tendon ORIF and splint in full extension 6 weeks

• •

Hyperextension avulsion injury MCP or PIP joint

Mallet Finger (Baseball Finger) [Figure 4-27-22]

Figure 4-27-22

Volar Plate Avulsion

Avulsion of Flexor Digitorum: Jersey Finger • • •

[Figure 4-27-23]

Forced hyperextension Avulsion of flexor digitorum profundus Osseous fragment displaced proximally

Figure 4-27-23

Isolated Tendon and Pulley Injuries [Figure 4-27• • •

24]

Rupture of pulley system occurs with forced extension Bowstringing of flexor tendon Common injury in rock climbers Mallet finger: avulsion of extensor digitorum tendon

Phalangeal Dislocations: Coach’s Finger • • • • • •

[Figure 4-27-25]

Usually dorsal; often associated with volar plate injury Simple: closed reduction; Complex: ST entrapment: open reduction

Boxer’s Fracture

• • • •

4th or 5th MC Dorsal angulation Treatment open >35º angulation Jersey Finger: Avulsion

Distal Tuft Fracture

injury of the flexor digitorum Fracture of distal tuft of phalanx profundus Mechanism: crushing injury Ignore the fracture and treat soft tissue injury Watch for complication of osteomyelitis with open fracture/ nail bed injury

Figure 4-27-24

Figure 4-27-25

Coach’s Finger: Interphalangeal dislocation

References 1. 2.

Manaster, BJ. Handbook of Skeletal Radiology, second edition. St Louis, MO: Mosby, 1997: 171-225 Rogers, LF. Radiology of Skeletal Trauma, third edition. Philadelphia, PA, Churchill Livingstone, 2002, 593-929 Rock Climber’s Finger: Flexor pulley disruption


948


Crystal Deposition Diseases and Neuropathic Osteoarthropathy Charles S. Resnik, MD •

Crystal Deposition Diseases • • •

Gouty arthritis ➢ Monosodium urate CPPD crystal deposition disease ➢ Calcium pyrophosphate dihydrate HA crystal deposition disease ➢ Calcium hydroxyapatite

Related Disorders •

Hemochromatosis ➢ Iron deposition Alkaptonuria ➢ Homogentisic acid

• • • •

Monosodium urate crystals Intra-articular, periarticular Acute inflammatory response Chronic granulomatous reaction

• • •

Male: Female = 20 : 1 40–50 years of age (Familial history)

• • • •

Hereditary diseases Myeloproliferative diseases Endocrine disorders Drug therapy

• • • • •

Asymptomatic hyperuricemia Acute gouty arthritis Interval phase Recurrent arthritis Chronic tophaceous gout

• • • • • •

Polyarticular Asymmetric 1st MTP joint (90%) Tarsometatarsal Carpometacarpal Olecranon bursitis

Gout

Gout: Primary (Idiopathic)

Gout: Secondary

Gout: Clinical Stages

Gouty Arthritis


949

Crystal Deposition Diseases and Neuropathic Osteoarthropathy

• • • • • •

Gouty Arthritis [Figures 4-28-1 to 4-28-3]

Figure 4-28-1

Soft tissue swelling Tophi (calcification rare) Well-defined erosions Overhanging edge Preserved joint space Extra-articular erosions

Figure 4-28-2

Gouty arthritis. Radiograph of the distal interphalangeal joint shows characteristic welldefined erosion of bone with overhanging edge of new bone (arrow). Note also preservation of joint space

Gouty arthritis. A. Pathological specimen shows tophaceous material (star) and sharp interface with adjacent bone (arrow). B. Clinical radiograph shows large well-defined erosions of the first metatarsal head with overhanging edge of new bone (arrow). Note multiple other erosions including the base of the first metatarsal

• • •

Pseudogout Chondrocalcinosis Pyrophosphate arthropathy

• • • •

Usually idiopathic Occasionally hereditary Over 50 years of age Incidence 5% to 34%

Figure 4-28-3

CPPD Crystal Deposition Disease: Terminology

CPPD Crystal Deposition Disease

CPPD Crystal Deposition Disease: Related Disorders • •

Primary hyperparathyroidism Hemochromatosis Gouty arthritis. There are multiple well-defined erosions, particularly at the bases of the metacarpals at the common carpometacarpal joint

CPPD Crystal Deposition Disease: Sites of Calcification • • • • • • •

Fibrocartilage Hyaline cartilage Synovial membrane Joint capsule Ligaments Tendons Bursae


950


• • •

Fibrocartilage Calcification

• • • •

Knee menisci Triangular fibrocartilage Symphysis pubis

Figure 4-28-4

Pyrophosphate Arthropathy Joint space narrowing Bone sclerosis Osteophyte formation Subchondral cysts

Differentiation of Pyrophosphate Arthropathy from Degenerative Joint Disease • • • • • •

Unusual articular distribution Unusual intra-articular distribution Variable osteophyte formation Prominent subchondral cysts Progressive destruction Calcification

CPPD crystal deposition disease. A. Frontal radiograph of the knee shows chondrocalcinosis including meniscal fibrocartilage calcification (white arrows) and hyaline cartilage calcification (gray arrow). B. Lateral radiograph shows complete loss of patellofemoral joint space

CPPD Crystal Deposition Disease: Knee • • • • •

[Figure 4-28-4]

Meniscal calcification Hyaline cartilage calcification Patellofemoral arthropathy Subchondral cysts Osseous bodies

CPPD Crystal Deposition Disease: Wrist and Hand [Figures 4-28-5 and 4-28-6] • • • • •

Figure 4-28-5

Triangular fibrocartilage calcification Radiocarpal arthropathy “Stepladder” configuration (SLAC) Metacarpophalangeal arthropathy Absence of erosions

Figure 4-28-6

CPPD crystal deposition disease. Frontal radiograph of the wrist shows joint space narrowing between the radius and scaphoid and between the lunate and capitate (scapholunate advanced collapse [SLAC]). There is extensive chondrocalcinosis including the triangular fibrocartilage (arrow) as well as a large subchondral cyst in the radius (star)

CPPD crystal deposition disease. Frontal radiograph shows narrowing of the second and third metacarpophalangeal joints with sclerosis, osteophytes, and subchondral cysts. Note hyaline cartilage calcification (green arrow) and probable synovial and capsular calcification (yellow arrows)


951


•

Hemochromatosis •

• • • • • • •

Primary ➢ Increased iron absorption Secondary ➢ Increased iron intake ➢ Multiple blood transfusions

Hemochromatosis: Clinical Findings Onset between 40 and 60 years Men more often than women Bronze pigmentation Cirrhosis Diabetes mellitus Cardiac failure Arthropathy

Figure 4-28-7

Hemochromatosis: Pathologic/Radiologic Findings • • • • • • •

Iron in synovioblasts CPPD crystal deposition Osteoporosis Symphysis pubis calcification Hyaline cartilage calcification Uniform MCP joint involvement Hook-like osteophytes

• • • •

Primary Secondary Periarticular Intra-articular

[Figure 4-28-7]

Hemochromatosis. Frontal radiograph shows narrowing of the second through fifth metacarpophalangeal joints as well as multiple large hook-like osteophytes (arrows)

HA Crystal Deposition Disease

Primary Periarticular HA Crystal Deposition Disease: (Calcific Tendinitis) • • • • • • •

Middle-aged Monoarticular (Asymptomatic) Localized pain Tenderness to palpation Restriction of motion (Fever and malaise)

Primary Periarticular HA Crystal Deposition Disease: (Calcific Tendinitis) [Figure 4-28-8] • • • •

Figure 4-28-8

Soft tissue swelling Poorly defined calcification Sharply defined calcification Resorption of calcification

Intra-Articular Hydroxyapatite Crystal Deposition Disease: Milwaukee Shoulder • • • • • • •

Elderly women Shoulder pain Decreased mobility HA crystal shedding Activated collagenase Neutral protease Tissue destruction


Calcific tendinitis. A. Radiograph of the shoulder in external rotation shows poorly defined calcification corresponding to the supraspinatus tendon (arrow). B. Radiograph of the shoulder of a different patient in internal rotation shows sharply defined calcification corresponding to the infraspinatus or teres minor tendon (arrow)

952


• • • • • •

Amorphous calcification Glenohumeral joint narrowing Subchondral sclerosis Bone destruction Rotator cuff disruption Acromiohumeral abutment

• • • • • • • •

Homogentisic acid oxidase deficiency Onset between 20 and 30 years Bluish-black pigmentation (ochronosis) Dark colored urine Cardiovascular Genitourinary Upper respiratory Articular

• • • • •

Connective tissue pigmentation Fibrocartilage and hyaline cartilage Fibrillation, fragmentation Granulation tissue Osseous proliferation

Milwaukee Shoulder: Radiologic Findings

Alkaptonuria: Clinical Findings

Alkaptonuria: Pathologic Findings

•

Alkaptonuria: Radiologic Findings • • •

Disc calcification ➢ Annulus fibrosus ➢ Diffuse Multilevel disc narrowing Vacuum phenomena Osseous sclerosis

• • • • •

Knees, hips, shoulders Symmetric cartilage loss Osseous sclerosis Collapse and fragmentation Intra-articular bodies

• • • • •

Charcot joint Tabetic arthropathy Neurotrophic joint Neuropathic arthropathy Neuroarthropathy

• • • • • • • •

Diabetes mellitus Alcoholism Syringomyelia Syphilis Leprosy Meningomyelocele Congenital insensitivity to pain Steroid administration (intra-articular)

Alkaptonuria: Radiologic Findings [Figure 4-28-9] Figure 4-28-9

Neuropathic Osteoarthropathy

Neuroarthropathy: Etiologies


Alkaptonuria. A. Lateral radiograph of the lumbar spine shows uniform loss of disc height and associated bone sclerosis. There is disc calcification bridging all levels anteriorly except for L4-L5, which shows large osteophytes and a vacuum phenomenon. B. Frontal radiograph of the knee shows uniform joint space loss and bone sclerosis

953


• • • •

French theory German theory Neurotraumatic Neurovascular

• • • • •

Mitchell (1831) Charcot (1868) Damage to CNS trophic centers Altered bone and joint nutrition Osseous and articular atrophy

• • •

Volkmann, Virchow Insensitivity to pain Recurrent trauma

• • • • •

Eloesser (1917) Posterior sensory nerve section Continued weightbearing Joint destruction Chemical analysis: no atrophy

Neuroarthropathy: Pathogenesis

Figure 4-28-10

Neuroarthropathy: French Theory

Neuroarthropathy: German Theory

Neuroarthropathy: Neurotraumatic Theory

• • • •

Neurally initiated vascular reflex Increased bone blood flow Osteoclastic bone resorption Fracture and repair

• • • •

Swollen, deformed joint Usually painless Detritic synovitis Rapid progression

Neuroarthropathy: Neurovascular Theory

Neuropathic osteoarthropathy. Frontal radiograph of the shoulder shows almost complete resorption of the humeral head except for some osseous debris medially (arrow). Note the extremely sharp margin of the remaining portion of the humeral shaft

Figure 4-28-12

Neuroarthropathy: Clinical/Pathologic Findings

Neuroarthropathy: Radiologic Findings • • • • • • •

[Figures 4-28-10 to 4-28-12]

Destruction (atrophy) Dislocation Disorganization Debris Detritus Density (sclerosis) Distension (effusion)

Figure 4-28-11

Neuropathic osteoarthropathy. Frontal radiograph of the foot shows characteristic destruction, disorganization, and debris around the tarsometatarsal joints

Neuropathic osteoarthropathy. A. Lateral radiograph of the foot shows extensive destruction of the bones of the midfoot with dislocation and disorganization. Note extensive vascular calcification from diabetes. B. Followup lateral radiograph less than one month later shows further bone destruction with almost complete disappearance of the talus and the anterior portion of the calcaneus


954


MRI of the Elbow Mark E. Schweitzer, MD; William B. Morrison, MD •

Figure 4-29-1

Anatomy • •

• • • •

Osseous-radius ulna humerus Ligamentous-medial collateral lateral collateral Musculotendinous ➢ Posterior: triceps ➢ Anterior: biceps, brachialis ➢ Medial: flexor-pronator ➢ Lateral: common extensor Neurovascular

Articular Anatomy

• • •

Capitellum-hemispherical-articulates with radius Trochlea-spool 300 degree arch-articulates with ulna Coronoid fossa-ant/sup to trochlea, small-articulates with coronoid Olecranon fossa 180 degrees-art. with semilunar notch Lesser sigmoid notch- radial side of proximal ulna/PRUJ

Biomechanics

• •

Three articulations: ➢ Ulnar-tochlear ➢ Radiocapitellar ➢ Proximal radioulnar ➢ 0-140 degrees flex/ext ➢ 75 pronation ➢ 80 supination

Effusion and synovitis. Note complex fluid in the joint distending the anterior and posterior fat pads (arrows). The fat pads are intracapsular but extrasynovial

Effusion [Figure 4-29-1]

• • •

Fat pads are intracapsular and extrasynovial DDx: ➢ Fracture ➢ Internal Derangement (e.g., ligament, cartilage) ➢ Arthritis (e.g., RA, OA, septic)

Figure 4-29-2

Cartilage Loss

•

•

Difficult to see directly (cartilage thin) Subchondral marrow edema best sign Phytes-posterior/medial/coronoid ➢ Confirm cart loss / cause impingement Associated with effusion and bodies

OCD [Figure 4-29-2] • • • •

Capitellum- 3rd most common site in body (after ankle, knee) Repetitive microtrauma during valgus (assoc with MCL) Symptoms = pain, locking Sequelae- bodies/OA Staging-fluid/cyst under fragment=loose (unstable)


955

Unstable OCD of the capitellum. Note contrast extending under osteochondral fragment (arrow) on this MR arthrogram

MRI of the Elbow

• • •

Pitfalls: NL variation

•

Trochlear sulcus Posterior capitellar pseudodefect DDx: location; no underlying edema

Figure 4-29-3

Intraarticular Bodies [Figure 4-29-3] • •

• •

• •

Often adherent to synovium ➢ Intraarticular, not loose Usually begin as cartilage defects and grow ➢ Often from OCDs) Variable signal characteristics ➢ Use GRE (TE>7), tend to bloom In the recesses, usually olecranon / coronoid MR imaging: arthrography, or effusion

Synovial Folds

•

Embryologic remnant Several locations ➢ Posterior/lateral catches ➢ Medial (meniscoid) most common May mimic bodies clinically and on MR

•

Lateral plica syndrome: Posterolateral catching/locking

Synovial Folds • •

Intraarticular body (arrow) surrounded by contrast in the olecranon recess in a patient with posterior impingement

Posterior Impingement • • • •

Part of tennis elbow spectrum Osteophytes- often related to chronic MCL overload, hyperextension (e.g., pitchers) Bodies in olecranon fossa Non-union of old triceps avulsion

Nerve Impingement

•

• • • • • • •

[Figure 4-29-4]

Potential locations of nerve impingement Median/radial nerve ➢ Proximal arcade of Struthers (avian spur) ➢ Impinged by fascicle of lacertus fibrosus ➢ Median nerve branch through pronator teres muscle (pronator syndrome) ➢ Supinator syndrome, radial tunnel syndrome, interosseous syndrome ➢ Look for muscle edema distally Ulnar nerve [Figure 4-29-4] ➢ Cubital tunnel ➢ Focal edema/STS/sublux/mass ➢ Look above and below ➢ Associated with epicondylitis, MCL injury

Figure 4-29-4

Epicondylitis Medial: golfer’s elbow (common flexor origin) Lateral: tennis elbow (common extensor origin) Spectrum from degeneration to partial to completetear Increased T1 signal Increased STIR, T2 signal Linear vs. diffuse Histologically- angioblastic changes/ fibrillar collagen degeneration

MRI of the Elbow

956

Enlargement of the ulnar nerve (arrow) with surrounding soft tissue inflammation in a patient with cubital tunnel syndrome


• • • • •

Repetitive overload of extensors “Tennis elbow” 35-70 years old Pain/tenderness focally, may radiate proximally Usually extensor carpi radialis

• • • • • • •

Long head: superior glenoid Short head: coracoid Two heads merge distal to the bicipital groove Insertion onto radial tuberosity at elbow Intimate with brachialis Proximal synovial sheath Distally paratenon, bicipitoradialis bursa and lacertus fibrosus

Lateral Epicondylitis [Figure 4-29-5]

Figure 4-29-5

Biceps: Anatomy

•

Biceps Pathophysiology • •

Degeneration ➢ Primary (overuse injury) ➢ Or direct frictional effect Mechanical-pronation leads to impingement between radius and ulna Hypovascular-critical zone distally like rotator cuff

• • •

Common, but rare to image Imaging/ clinically overlap with partial tear, bursitis Very distal at insertion

• • • • •

Attritional Pain No pop, usually no ecchymosis More marrow edema and bursitis Surgery not usually needed unless large

Lateral epicondylitis (tennis elbow) with focal fluid signal (arrow) at the common extensor tendon origin indicating a partial tear

Figure 4-29-6

Biceps Tendinosis

Biceps-Partial Tears

• • • • • •

Biceps Injury: Distal vs Proximal [Figure 4-29-6]

• • •

Both: muscle belly retraction “popeye arm” Fluid dissects around muscle belly Both: sudden snap, arm hematoma Distal young/ proximal older Distal sports/ proximal – chronic impingement, RCT Proximal: surgery uncommon (two heads), except for repair of cuff, resection of spur Distal: surgery common Associated marrow edema Associated bursitis

• • • • •

Distal biceps lacks a sheath Apparent fluid around is bursal Close to insertion Ddx; vessel Assoc with biceps tears (esp partial), RA, mechanical –maybe 1st

Complete distal biceps tendon tear. Note end of retracted tendon (arrow) with muscle bunched up proximally resulting in a “popeye” arm

Bicipital Radialis Bursitis


957

MRI of the Elbow

• •

Triceps Injuries • • • • • • • • •

Fairly uncommon Spectrum tendonitis (posterior tennis elbow/ posterior impingement), snapping, to tear Risk factors: steroids, SLE, CRF, RA, gout Within 2–3 cm of insertion, usually at Associated olecranon bursitis Associated soft tissue edema Look for avulsion fx

Figure 4-29-7

Olecranon Bursitis [Figure 4-29-7]

• • •

Anatomic bursa Normally no fluid visible Bursitis: Fluid, loss of subQ fat adjacent to olecranon Causes ➢ Trauma ➢ RA ➢ Gout ➢ Infection

Muscle Disorders

• • • •

Tears DOMS Neuropathy-Parsonage Turner Syndrome “edema-like” muscle signal Infection Olecranon bursitis

Osseous Injury [Figure 4-29-8]

•

Effusion on Xray: presumed fx F/U X-ray vs MR Bone marrow edema after trauma: Bone bruise vs. fracture ➢ T1: focal low signal (linear) = fracture ➢ No line, ill-defined edema = bruise

Figure 4-29-8

Avulsion •

Chronic avulsive stress ➢ Tendinopathy ➢ Usu subtle edema at enthesis Avulsion fx ➢ Ligamentous or tendinous ➢ Thin, longitudinally oriented edema at cortical margin

• • •

Analogous bruises from ACL tears Transient disloc/sublux Often both sides of joint

• • • • • • • • •

OA Bodies Capsular fibrosis Non union / malunion Associated ligament injury/instability (e.g., Essex Lopresti) AVN Pain Limited ROM Instability

Osteochondral Impaction

Fracture Complications

MRI of the Elbow

958

Occult radial neck fracture


• •

AVN

• •

Figure 4-29-9

Older: typical risk factors for AVN Younger = Panner’s disease ➢ Capitellum ➢ Boys; 4-10 years ➢ Decreased vasc to growing epiphysis ➢ Usu. spontaneously resolves ➢ If >10, higher risk of complications

Ligament Disorders

•

Medial Lateral

Medial Collateral Ligament

•

Three segments ➢ Anterior bundle ❖ Most important soft tissue static constraint to valgus stability ➢ Posterior bundle ➢ Transverse bundle

MCL • • • • •

[Figures 4-29-9 and 4-29-10]

Anatomy ➢ Anterior/posterior/transverse bands ➢ Strongest is anterior Pathophysiology ➢ Overhead throwing /valgus overuse, weakens/incompletely heals, reinjures Partial tears — “T” sign vs. complete tears Old tears show thickening +/- bowing Association with epicondylitis

Medial collateral ligament tear (arrow) and osteochondral impaction injury (arrowhead)

Figure 4-29-10

Lateral Ligaments

• •

Components ➢ Lateral collateral ligament proper (LCL) (Radial collateral ligament) ➢ Annular ligament ➢ Lateral ulnar collateral ligament (LUCL)

Annular Ligament • • • • •

Fibro-osseous ring that encircles and stabilizes the radial head Attaches on the anterior and posterior edges of the lesser sigmoid notch Anterior portion taught in supination and posterior portion taught in pronation

Medial collateral ligament tear (arrow) and lateral ulnar collateral ligament tear (arrowhead) in a patient with recent elbow dislocation

Radial Collateral Ligament

•

Extends from the lateral epicondyle and attaches to the annular ligament Immediately deep to the common extensors About half to a third size of MCL Maintains humeroradial apposition in the presence of varus stress

Lateral Ulnar Collateral Ligament • •

With annular ligament (PRUJ) and radial collateral ligament (radial head) makes up lateral lig complex Sweeps posteriorly past the radial neck and inserts on the ulna Stabilizer for rotational and varus stress


959

MRI of the Elbow

• • • •

Posterolateral Rotatory Instability Instability of the elbow manifested by painful clicking of the elbow in extension Radial head moves posteriorly in relation to the capitellum Essential lesion - tear of the LUCL Lateral pivot shift test ➢ Supination with axial and valgus stress Lateral pivot shift test - supination with axial and valgus stress

• •

Caused by a fall on an outstretched hand Iatrogenic injury during release or repair of lateral epicondylitis

• • • • •

Mimics a mass SubQ Inflammatory-like signal Cat scratch fever Assoc fasciitis

• • • •

Bodies MCL injuries OCDs Subtle cartilage loss

LUCL INJURIES

Epitrochlear Lymph Node

MR Arthrography Indications

•

Elbow MRA • •

Ligament tear ➢ Extracapsular leakage of contrast ➢ Medial or lateral collateral ligament tear IA bodies ➢ Anterior, posterior recesses OCD ➢ Same dx as knee ➢ Esp capitellum

References 1. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist, and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-379. 2. Bordalo-Rodrigues M, Rosenberg ZS. MR imaging of entrapment neuropathies at the elbow. Magn Reson Imaging Clin N Am. 2004 May;12(2):247-263. 3. Potter HG, Ho ST, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskelet Radiol. 2004 Mar;8(1):5-16 4. Chung CB, Kim HJ. Sports injuries of the elbow. Magn Reson Imaging Clin N Am. 2003 May;11(2):239-53. 5. Steinbach LS, Palmer WE, Schweitzer ME. Special focus session. MR arthrography. Radiographics. 2002 SepOct;22(5):1223-1246. 6. Zou KH, Carrino JA. Comparison of accuracy and interreader agreement in side-by side versus independent evaluations of MR imaging of the medial collateral ligament of the elbow. Acad Radiol. 2002 May9(5):520-5. 7. Jbara M, Patnana M, Kazmi F, Beltran J. MR imaging: arthropathies and infectious conditions of the elbow, wrist, and hand. Magn Reson Imaging Clin N Am. 2004 May;12(2):361-79 8. Bordalo-Rodriguez M, Rosenberg ZS. MR Imaging of entrapment neuropathies at the elbow. Magn Reson Imaging Clin N. Am. 2004 May; 12(2):247-63. 9. Chung CB, Chew FS, Steinbach L. MR imaging of tendon abnormalities of the elbow. Magn Reson Imaging Clin N. Am. 2004 May;12(2):233-45. 10. Kaplan LJ, Potter HG. MR imaging of ligament injuries to the elbow. Magn Reson Imaging Clin N. Am. 2004 May;12(2):221-32, v-vi. 11. Fowler KA, Chung CB. Normal MR imaging anatomy of the elbow. Magn Reson Imaging Clin N. Am. 2004 May;12(2):191-206, v. 12. Potter HG, Ho St, Altchek DW. Magnetic resonance imaging of the elbow. Semin Musculoskeletal Radiol. 2004 Mar;8(1):5-16. 13. Savnik A, Jensen B, Norregaard J, Egund N, Danneskiold-Samsoe B, Bliddal H. Magnetic resonance imaging in the evaluation of treatment response of lateral epicondylitis of the elbow. Eur Radiol. 2004 June;14(6):964-9. Epub 2003 Dec 11. MRI of the Elbow

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Skeletal Metastases, Myeloma, Lymphoma Michael E. Mulligan, MD Figure 4-30-1 • • • • • • • • •

Incidence of Metastases [Figure 4-30-1] 30% of all patients with Cancer Skeleton – 3rd most common site More than 80% due to PTBLK #1-Breast, #2-Prostate, #3-Lung Spine lesion – Breast 75% Femur lesion – Breast 50% Skull lesion – MM, B, L Hands/Feet – Lung P=prostate T=thyroid B=breast L=lung K=kidney

Mechanism of Spread to Bones Hematogenous/Contiguous • • •

Marrow vessels unusual, rich sinusoidal system with large endothelial gaps Batson’s plexus has direct connection to IVC/SVC with no valves Arterial – mechanism for distal mets?

Batson OV. Ann Surg 1940;112:138

48 year old man with renal cell carcinoma metastases mimicking multiple myeloma

Figure 4-30-2 • • •

Bone Metastases Different T1, T2 signal Different Gadoliniumenhancement The “holy grail” – DWI

Radiology 1998; 207:305-7

Acute Vertebral Collapse - Osteoporosis or Malignancy •

• • • • • • • •

[Figure 4-30-2]

Symptoms and Signs ➢ Pain, most common symptom, but only in 2/3 patients ➢ Pathologic fracture ❖ Common sign, esp bad in spine, femur

Pathologic Fractures [Figure 4-30-3] 5–10% of all patients with mets 50% or more cortex gone - 2/3 will develop pathologic fracture 61 year old woman with breast Less than 50% cortex gone - 1/5 will develop pathologic fracture cancer, focal depression of L2 Any lesion in femoral neck superior endplate is indicative of Avulsion of lesser trochanter metastatic disease Any lesion prox femur >2.5cm Figure 4-30-3 Mirels score - site, size, l/b, pain 3.0 cm lesion with persistent pain after XRT

Mirels, H. Clin Ortho Rel Res 1989; 249: 256-64

55 year old man with lung cancer and cortical “cookie bite” metastasis


961

Metastases, Myeloma, Lymphoma

• •

Bone Metastases: Systemic Features [Figure 4-30-4]

Figure 4-30-4

Hypercalcemia Hypertropic Osteoarthropathy, triad of ➢ Periosteal reaction ➢ Clubbing ➢ Pain

• • • •

Classic Triad Joint swelling, 30%–40% patients 5% patients with lung cancer Cause? – may be paraneoplastic, due to a growth hormone releasing factor

• • • •

Pure lysis Lysis with blowout (renal, thyroid) Mixed lytic/blastic (breast, lung, GI) Pure blastic (prostate, carcinoid, medulloblastoma)

• • • •

Stimulates clasts to synthesize collagenase Produced by normal activated leukocytes Dependent on prostaglandin E Prostaglandin inhibitors can reduce/obliterate osteolysis

Hypertrophic Osteoarthropathy

Bone Metastases: Radiologic Features

Osteoclast – Activating Factor (A Cytokine)

JBJS 68A:310, FEB 1986 • • • • • •

Bone Metastases: Radiologic Features Ivory vertebra Pathologic fx Periosteal rx – prostate, lung neuroblastoma, GI tumors Soft tissue mass (lung) Missing pedicle Intracortical – lung cancer

Ivory Vertebra(ae): Differential Diagnosis [Figure 4-30-5] • • • •

#1 Paget’s #2 Hodgkin’s #3 Metastasis

Figure 4-30-5

2 year old Irish setter with lung cancer. Foreleg bones show classic periosteal changes of H.O

Figure 4-30-6

50% 30% 20%

Breast Carcinoma • • •

The most common source of bone mets in women Spine #1 site 65% lytic, 25% mixed, 10% blastic

Prostate [Figure 4-30-6] • • •

The most common source of metastases in men More than 1/3 of patients 75% blastic, 15% mixed, 10% lytic Humoral factor stimulates blasts

59 year old woman with multiple ivory vertebrae secondary to breast cancer metastases Metastases, Myeloma, Lymphoma

962

76 year old man with florid periosteal reaction around distal fibula metastasis Musculoskeletal Radiology

• • •

15% of patients have mets to bone 80% lytic, 15% mixed, 5% blastic Small cell – 20% blastic

• •

25%–30% of patients have mets to bone 90% Lytic

Lung Cancer

Renal Cell Carcinoma [Figure 4-30-7]

Figure 4-30-7

71 year old man with blowout type metastasis from renal cell cancer. Note active hyperemia on angiogram

Figure 4-30-8

• •

8% of patients have metastases Lytic

• • • • •

1st choice any patient under 10 years old Can mimic primary malignancy Usually multiple, often symmetric Histology confused with Ewing’s Periosteal rx – aggressive

• • • •

1 – History / physical 2 – Lab studies Direct workup based on 1, 2 Primary not found in up to 60% patients

Thyroid Cancer

Neuroblastoma [Figure 4-30-8]

Workup of Patient with Metastases

3 year old boy with neuroblastoma. Metastasis in proximal radius shows permeative appearance similar to Ewing’s sarcoma Musculoskeletal Radiology

963


• • • • •

Solitary Focus Bone Scan [Figure 4-30-9]

Figure 4-30-9

Seen in 2%–15% of cancer patients % due to metastatic disease varies by site of involvement 10% solitary rib lesions 50%–60% in other locations (spine) 10% malignant even if there is “DJD” in the area

Rib – J Nucl Med 1985;26:1140–1143 All sites – Radiology 1976;121:663–667 • • • • • • •

Malignant Round Cell Tumors of Bone

•

Myeloma Lymphoma Ewing’s sarcoma Neuroblastoma Rhabdomyosarcoma Small cell carcinoma PNET

Myeloma Types • • • • • • • •

MGUS – monoclonal gammopathy of undetermined significance (1% of all SPEPs) Asymptomatic myeloma (no bone lesions) Symptomatic multiple myeloma (classic, generalized, osteosclerotic [POEMS], multiple myeloma with osteosclerosis, leukemic) Non-Secretory myeloma – 3% of all cases Solitary plasmacytoma in bone – 3%-5% of cases Extramedullary plasmacytoma – 5%

Myeloma: Pathologic Features

• • •

Plasma cells proliferate in erythropoietic areas Grossly – dark red, tan; soft Histology – sheets of malignant plasma cells obliterate the marrow Special studies – markers for light chains Amyloid – 10% Osteoclast stimulating factor

• • • • •

A cytokine (lymphotoxin alpha) Similar to O-AF Produced by myeloma cells, T cells Interferon is a cytokine antagonist Bisphosphonates used to counter osteoclastic resorption

65 year old man with solitary rib abnormality on whole body bone scan, proven to be esophageal cancer metastasis

Osteoclast-Stimulating Factor

Cancer 1997; 80:1557-63 • •

Classic Multiple Myeloma: Clinical Features • •

Signs and Symptoms: pain, bleeding diathesis, infection, renal insufficiency Lab findings: monoclonal spikes (IgG, IgA), B-J proteinuria, anemia, hypercalcemia, elevated alkaline phosphatase Imaging W/U: X-rays, CT/MRI, Nucs, PET, PET/CT MM has highly malignant course

B-J=Bence-Jones MM=multiple myeloma


964


Durie/Salmon PLUS Staging* [Figure 4-30-10] Radiologist’s role is to help determine the true tumor burden throughout the skeleton • Stage IA: normal skeletal survey or single lesion • Stage IB: < 5 focal lesions or mild diffuse disease • Stage IIA/B: 5-20 focal lesions or moderately diffuse • Stage IIIA/B: >20 focal lesions or severe diffuse disease • Subclasses A&B (A = nl renal function, B = abnl) • Stage is generally predictive of survival ➢ IA median survival = 60 months ➢ IIIB median survival = 15 months

Figure 4-30-10

*Durie et al. Myeloma management guidelines: a consensus report. The Hematology Journal 2003; 4: 379-398 • •

Myeloma: Imaging Features [Figure 4-30-11] • • • •

80% of new MM patients – abnormal skeletal survey Multiple myeloma - punched out lesions ➢ endosteal scalloping Solitary plasmacytoma: bubbly, any margin +/- soft tissue mass Generalized form- just osteopenia Sclerosing - < 3%, assoc with POEMS syndrome Spine MR – 3 patterns; mild, moderate, severe

Sclerotic Myeloma: Multiple myeloma with sclerosis or POEMS syndrome • Polyneuropathy • Organomegaly • Endocrinopathy • Monoclonal gammopathy • Skin changes

From left to right: mild, moderate, and severe. The three types of myelomatous spinal involvement for the Durie/Salmon PLUS staging system (T1-weighted images) [Figure 4-30-12]

Figure 4-30-11

NEJM 1992;327:1919–1923

Figure 4-30-12

56 year old man with myeloma. Humeral radiograph shows typical endosteal scalloping and macrosection shows plasma cells filling the marrow space with osteoclasts along the endosteal surface

Blastic or sclerotic lesions that are usually painless are typical in the POEMS syndrome Musculoskeletal Radiology

965


• •

Myeloma: Differential Diagnosis [Figure 4-30-13]

Figure 4-30-13

Figure 4-30-14

Metastatic disease B cell malignancy ➢ ALL, NHL, CLL, Waldenstrom’s

Plasmacytoma Differential Diagnosis [Figure 4-30-14] • •

Metastasis: thyroid, renal Primary: Fibrosarcoma, MFH

Primary Lymphocytic Lymphoma of Bone [Figure 4-30-15] Figure 4-30-15 56 year old man with myeloma,. skull radiograph shows typical “punched out” lytic lesions 71 year old woman with aggressive looking solitary plasmacytoma mimicking blowout type metastasis

70 year old man with PLB, skull radiograph shows multiple lytic lesions, some with central sequestra (arrow) • •

Primary Lymphoma: Path Features •

Gross – pinkish-grey, “fish-flesh” Histology – similar to nodal lymphoma round cells of various sizes (Ewing’s monotonous) Reticulin stain – meshwork of fibers around each cell

• • • • • •

Non-Hodgkin’s (94%), Hodgkin’s (6%) Rare, @ 3% malignant tumors Any age, but rare under 10 years Stage like soft tissue lymphoma Solitary and multifocal (skull, femur, tibia) Osteoclast-stimulating factor

• • • • •

151 M, 86 F, ratio 1.8:1 Average age 42 years (range: 2 to 88 years) Long bones n=162 (71%) Flat bones n=78 (22%) Other sites (including spine, small bones) n=17

Figure 4-30-16

Primary Lymphoma: Clinical Features

PLB – 237 AFIP CASES

Mulligan, et al. AJR 1999; 173: 1691-1697 • • • • •

Typical Features – PLB [Figure 4-30-16] Location: Metadiaphyseal n=120 (54%) Pattern: Permeative n=130 (55%) Cortical involvement: n=148 (62%) Periosteal reaction: interrupted or solid single layer n=57 (65.5%) Soft tissue mass: n=113 (48%)

Mulligan, et al. AJR 1999; 173: 1691-1697 Metastases, Myeloma, Lymphoma

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57 year old woman with PLB, tibia lesion shows all of the most common radiographic features Musculoskeletal Radiology

•

Variations – PLB [Figure 4-30-17]

•

•

Figure 4-30-17

Locations ➢ Epiphysis n=11 (5%) ➢ Diaphysis n=45 (19%) ➢ Intracortical n=16 (7%) Patterns ➢ Normal x-ray n=12 (5%) ➢ Geographic n=26 (11%) ➢ “Blow Out” n=2 (< 1%) ➢ Blastic n=4 (2%)

Radionuclide, CT and MRI Findings • •

•

•

Radionuclides n=56, markedly increased uptake n=36 (64%) CT n=45, MRI n=20 “Cortical holes” by CT or MR ➢ Large n=20 (31%), small n=45 (69%) Soft tissue mass ➢ Seen by CT n=36 (80%) ➢ Seen by MR n=20 (100%)

Variations – PLB • • •

Markedly abnormal bone scan and MR exam in 57 year old man with thigh pain and normal xrays. This type of extensive marrow replacement pattern with normal xrays is highly suggestive of “round blue cell tumors” like PLB and Ewing’s sarcoma

[Figure 4-30-18]

Periosteal reaction ➢ Multiple layers n=26 (10.2%) ➢ Sunburst n=4 (1.6%) Pathologic fracture n=52 (22%) Sequestra n=37 (15.6%) Crossing joint n=12 (5%)

Figure 4-30-18

AJR 1999; 173: 1691-1697 • • • • • •

Primary Lymphoma: Differential Diagnosis

•

Metastatic lymphoma Ewing’s sarcoma Neuroblastoma / PNET Rhabdomyosarcoma Osteomyelitis Eosinophilic granuloma

Summary – PLB •

Usually has an aggressive appearance ➢ CT or MRI showing large soft tissue mass without large cortical holes is typical Wide range of appearances ➢ Normal x-rays ➢ Geographic lesions ➢ “Blow out” lesions ➢ Blastic lesions

Large lytic lesions with soft tissue mass and sequestra should put PLB high up in the differential diagnosis

References 1. 2. 3. 4. 5. 6. 7.

Durie et al. Myeloma management guidelines: a consensus report. The Hematology Journal 2003; 4: 379-398 Mirels H. Metastatic disease in long bones. Clin Orthop Relat Res 1989;249:256-264 Mulligan M et al. Skeletal Metastatic Disease. In: Pope et al, Imaging of the Musculoskeletal System. Philadelphia: Elsevier, 2006 Mulligan M. Imaging techniques used in the diagnosis, staging, and follow-up of patients with myeloma. Acta Radiologica 2005;46:716-724 Mulligan M, McRae G, Murphey M. Imaging features of primary lymphoma of bone. AJR 1999; 173: 1691-1697 Roodman GD. Mechanisms of bone metastasis. N Engl J Med 2004;350:1655-1664 Weber K et al. An approach to the management of the patient with metastatic bone disease. Instr Course Lect 2004;53:663-676


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Imaging of Hematologic Disease

Figure 4-31-1

Thomas Lee Pope, Jr, MD, FACR • •

Objectives • •

To identify some of the common hematologic disorders To describe the clinical and epidemiological aspects of these entities To demonstrate the most significant MSK imaging findings

Hematologic Disease

•

• • • • •

Hereditary anemias ➢ Sickle cell anemia ➢ Thalassemia ➢ Rare anemias: Fanconi’s, thrombocytopenia with absent radii syndrome (TAR) Coagulation disorders ➢ Hemophilia ➢ Myelofibrosis

Characteristics of the Hereditary Anemias

•

Aberrations and/or abnormalities of RBC shape Molecularly distinctive Autosomal dominant Electrophoresis The Five “In’s” ➢ In sufficient ossification ➢ In farction ➢ In fection ➢ In failure (anemia) ➢ In volution (spleen)

Hand Foot Syndrome: Notice the diffuse involvement with regions of osteolysis and periosteal reaction

Figure 4-31-2

Sickle Cell Disease • • •

• • • • •

One of the most common inherited blood disorders (> 100,000 born with the disease worldwide per year) One of the most prevalent genetic disorders in the US (> 80,000 African Americans) Hemoglobin S gene (carrier state) ➢ Autosomal dominant ➢ Carried by 8% of African Americans or ~ 2 million US Blacks Hemoglobin SS disease (Sickle cell anemia) ➢ Autosomal recessive ➢ 1 birth in 400 in African Americans ➢ Occurs in 0.3%-1.3% of NA Blacks or ~ 50, 000 in the US

Economic Impact of SC Disease 75,000 hospitalizations yearly Average of $6, 300 per hospitalization $475 million in health care costs alone Does not include lost wages, productivity, etc… Likely > $1 billion yearly cost

Acute (upper image) and chronic (lower image) Salmonella osteomyelitis


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•

Characteristics of sickled and normal RBC’s

Figure 4-31-3

NORMAL ➢ Disc-shaped ➢ Soft and compressible ➢ Easily flows through vessels ➢ Life span of > 120 days • SICKLE ➢ Sickle-shaped ➢ Hard (tough and not malleable) ➢ Sticks in blood vessels ➢ Life span of < 20 days

Major Pathology-Vascular occlusion

[Figure

•

4-31-1]

Hand-Foot syndrome ➢ Dactylitis • Infarction (any site) • Infection • Marrow hyperplasia • • •

Hand foot syndrome (Dactylitis)

• • • • •

Up to 50% of “sickle cell anemia” children 2 months to 6 years Pain, low grade fever, diffuse non-pitting edema of the extremities Vaso-occlusion with osteonecrosis Infection is major DDx Distinction: Clinically

Infection

MR features of acute Salmonella osteomyelitis Courtesy of Dr. Hilary Umans New York, NY

Figure 4-31-4

[Figure 4-31-2]

50X less common than infarction Salmonella much more frequent pathogen in SCA patients • Infecting organisms: ➢ Salmonella – 70% ➢ Staph aureus – 10% ➢ Shigella sonnei, E coli, Arizona hinshawii and Serratia • Proposed mechanisms: ➢ Vascular insufficiency ➢ Decreased phagocytosis-low O ➢ Decreased splenic function Multiple hospitalizations

Classic features of avascular necrosis with areas of osteolysis and osteosclerosis with preservation of articular space

Figure 4-31-5 Figure 4-31-6

Radiographic and anatomic gross specimen correlation of the “rim” sign MR imaging of intramedullary infarcts


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Differential Diagnosis: “Bone within Bone” Appearance • • • • • •

Figure 4-31-7

S – Sickle cell disease T – Thoratrast O – Osteopetrosis P – Paget disease Heavy metal Hypervitaminosis D

http://chorus.mcw.edu

• • •

“H-Shaped” Vertebral Bodies [Figure 4-31-8]

• • • •

Another manifestation of ischemia and infarction >10 years of age Incidence: ➢ 43% of SS ➢ 36% of Sickle/Thalassemia ➢ 25% of SC

Thalassemia

• •

“Bone within a bone” apprearance in sickle cell anemia

1925 - Cooley and Lee Synonyms: Cooley’s anemia, mediterranean anemia, leptocytosis Impaired alpha or beta chain Hgb production Homozygous beta thalassemia (800-1000 US persons - NE corridor between Boston and NY) Heterozygous trait (2.5% of Italian Americans, 7%-10% of Greek Americans)

Figure 4-31-8

Types of Thalassemia

•

Alpha ➢ Least severe: ❖ Silent carrier = loss of 1 alpha globulin gene - often incidental finding ❖ Most severe: ❖ Hydops fetalis = loss of 4 alpha globulin genes - die in utero Beta: ➢ Spectrum ❖ Minor = slight anemia ❖ Major = life-threatening anemia requiring transfusions ❖ Risk of Fe++ overload “H” shaped vertebral bodies of sickle cell disease


Classic “Hair on end” appearance of Thalassemia Imaging of Hematologic Disease

Marrow expansion in Thalassemia with widening of the medullary canal and thinning of the cortices 970


Imaging Features of Thalassemia •

Figure 4-31-11

[Figures 4-31-9 to 4-31-12]

• • • • •

Diffuse marrow expansion ➢ Skull - (“hair on end”) appearance ➢ Face - (“rodent-like facies”) ➢ Long bones – “Erlenmeyer flask” deformity Extramedullary hematopoeisis Rare minor features: Growth disturbances, fractures, crystal deposition

Gaucher’s Disease

• • • • • •

Ashkenazic Jews of Eastern European descent Defect of beta glucosidase Accumulation of glycosyl ceramide in the RE cells of BM, spleen, and liver Hepatosplenomegaly, yellow skin, scleral pigmentation, acid and alkaline phosphatase elevation

Imaging features

• • • • • •

AVN of the hip and femoral head Osteoporosis Marrow expansion with cortical thinning Erlenmeyer flask deformity Lytic lesions and sometimes periostitis

Erlenmeyer flask deformity

Differential Diagnosis: “Erlenmeyer Flask” Deformity

Figure 4-31-12

Osteoporosis Chronic anemia (Sickle cell disease) Gaucher disease Niemann- Pick (enzyme deficiency) Fibrous dysplasia Metaphyseal dysplasia (Pyle’s disease)

Extramedullary Hematopoiesis •

[Figures 4-31-13 and 4-31-14]

•

Blood production in fetal regions ➢ Liver, spleen, adrenal, thymus, heart, lung, nodes, renal pelvis, GI tract, dura mater (almost anywhere !) Major causes: ➢ Hematologic disease (SS and thalassemia) ➢ Myelofibrosis ➢ Leukemia ➢ Hodgkin’s ➢ Hyperparathyroidism ➢ Rickets ➢ Carcinomatosis

Figure 4-31-14

“Erlenmyer flask” deformity in Gaucher’s disease. “Crumpled tissue paper” cytoplasm on histology

Figure 4-31-13

Extramedullary hematopoesis with hepatosplenomegaly and posterior mediastinal masses

Extramedullary hematopoesis with MR correlation Musculoskeletal Radiology

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• •

Fanconi’s Anemia [Figure 4-31-15] • •

Figure 4-31-15

Onset after first decade Severe anemia, pancytopenia, brown pigmentation Death 2–3 years after appearance Anomalies: ➢ Short stature, microcephaly, delayed ossification ➢ Hip dysplasia, renal anomalies ➢ Radius absent (50%) ➢ Thumb always absent

Thrombocytopenia with Absent Radii (TAR) [Figure 4-31-16] • • •

• • • • • • • • • • •

Fanconi’s anemia:

Congenital hypomegakaryocytic thrombocytopenia Apparent at birth or shortly thereafter Anomalies: ➢ Bilateral radial aplasia always present ➢ Thumb is present (differentiation from Fanconi’s) If kids survive for the first two years, the anemia often spontaneously resolves

Figure 4-31-16

Hemophilia

•

Oldest known bleeding disorder First noted in offspring of Queen Victoria of England Mutation in Queen Elizabeth’s X chromosomes Group of X-linked recessive disorders Gene carried by women and expressed in men All races affected 20, 000 hemophiliacs in US 400 new cases/year Severity – related to lack of clotting factor 70% have < 1% of normal clotting factor

TAR with absent radii syndrome: Note that the thumb is present

Figure 4-31-17

Major Types of Hemophilia

•

•

Hemophilia A ➢ 85% of all cases ➢ Factor VIII (antihemophiliac factor-AHF) deficiency ➢ 70% have < 1% of normal amounts of AHF Hemophilia B (Christmas disease) ➢ 15% of all cases ➢ Factor IX (Plasma thromboplastin componentPTC) deficiency

Acute hemorrhagic effusions in two patients with hemophilia

Figure 4-31-18

Joint Disease •

• • • • •

Acute hemarthroses: tense, swollen, red and tender joints, pain, LOM, fever, increase in WBC Stages of joint disease: ➢ Stage I: STS ➢ Stage II: Osteoporosis ➢ Stage III: Osseous lesions ➢ Stage IV: Cartilage destruction ➢ Stage V: Joint disorganization

Knee [Figures 4-31-17 and 4-31-18]

Marked articular space narrowing and cartilage destruction with massive subchondral cyst formation

Dense effusions Juxtaarticular osteoporosis Subchondral irregularity Epiphyseal overgrowth Squaring of inferior pole of patella (20%-30%)


972


Elbow [Figure 4-31-19]

Figure 4-31-19

Low SI synovial proliferation [Figure 4-31-20] •

Differential Diagnosis of Hemophilia

• •

Juvenile chronic arthritis ➢ Single joint tough to differentiate ➢ Distribution may be helpful: ❖ JCA – hands/feet/big joints ❖ Hemophilia - knee, ankle, elbow PVNS, infection, especially TB (monoarticular) NM diseases: CP, muscular dystrophy, Figure 4-31-20 polio Hemophiliac involvement of two elbows in two different patients

Figure 4-31-21

Low signal intensity of synovial proliferation in hemophilia.

Medial talar tilt quite characteristic of hemophilia

Kerr R: Imaging of MSK complications of hemophilia. Sem in MSK

Figure 4-31-22 •

Other Less Common Imaging Findings • • • • • • •

Ectopic ST ossification (periarticular – pelvis, thigh, paraspinal, knee) Hemophiliac pseudotumor Osteonecrosis (epiphyseal fragmentation) Fractures Chondrocalcinosis

Pseudotumor [Figures 4-31-22 to 4-31-26]

• •

2% of patients Femur, pelvis, tibia, hands and feet Locations: ➢ Soft tissue, intraosseous, and subperiosteal ST ➢ Hard palpable subcutaneous masses Intraosseous and subperiosteal ➢ Lytic, expansile, destructive, aggressive process

Soft tissue pseudotumor of hemophilia Park JS, Ryu KN: AJR 2004;

Figure 4-31-23

Subperiosteal pseudotumor of hemophilia Park JS, Ryu KN: AJR 2004; 183:55-61


973


Figure 4-31-24

Figure 4-31-25

Intraosseous pseudotumors of hemophilia Park JS, Ryu KN: AJR 2004; 183:55-61

Differential Diagnosis for Hemophiliac Pseudotumor •

• • • • •

Malignancy ➢ Osteosarcoma ➢ Chondrosarcoma ➢ Ewing tumor Metastases Infection

Intraosseous pseudotumor of the ilium in a hemophiliac Kerr R: Imaging of MSK complications of hemophilia in MSK Radiology 7:2, 2003

Figure 4-31-26

Myelofibrosis

•

• • • •

Affects progenitor (stem) cells of the bone marrow Primary (idiopathic) and secondary forms Major manifestations ➢ Fibrotic or sclerotic bone marrow ➢ Extramedullary hematopoiesis Other designations: ➢ Idiopathic myelofibrosis ➢ Myeloid metaplasia ➢ Agnogenic myeloid metaplasia

Primary (Idiopathic) Myelofibrosis Bone marrow replaced by fibrosis Unknown cause > 50 year old, incidence - 2/100,000 Findings: ➢ BM fibrosis with hepatosplenomegaly ➢ Anemia ➢ Increased nucleated RBC’s ➢ Leukocytosis or leukopenia ➢ Abnormal WBC’s

Intraosseous “blowout” lesions of hemophilia

Figure 4-31-27

Primary (Idiopathic) Myelofibrosis • • • •

[Figure 4-31-27]

• • • •

Diagnosis of exclusion Unknown cause > 50 year old, incidence - 2/100,000 Findings: ➢ BM fibrosis with hepatosplenomegaly ➢ Anemia ➢ Increased nucleated RBC’s ➢ Leukocytosis or leukopenia ➢ Abnormal WBC’s Diffusely dense bones characteristic of Diagnosis - BM aspiration myelofibrosis Rx: Transfusions, chemo, Interferon, splenectomy, radiation 50%-80% of patients have elevated serum or urinary uric acid levels Secondary gout occurs in 5-20% of patients


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•

Secondary Myelofibrosis • • • • • • •

Malignant disease ➢ Leukemias, Polycythemia vera, MM, Hodgkin’s disease, NHL, cancer Chronic infection ➢ Tuberculosis, osteomyelitis Toxins ➢ X- or gamma radiation, benzene exposure

Imaging Findings [Figure 4-31-15]

•

Generalized osteosclerosis (most common) Cortical thickening Osteopenia Rarely periostitis Extramedullary hematopoiesis

Review

•

•

•

Sickle Cell ➢ Vaso-occlusion ❖ “Hand-Foot” syndrome ❖ AVN and medullary bone infarcts ❖ “H-shaped” (“Lincoln log”) vertebral bodies ❖ “Bone within a bone” appearance ❖ Salmonella infection Thalassemia ➢ “Hair on end” ➢ “Pseudohemangiomatous” appearance ➢ “Erlenmeyer flask” deformity (differential-Gaucher’s) ➢ “Rodent” facies Hemophilia ➢ Wide intercondylar notch ➢ Erosions ➢ Medial slope of distal tibia at ankle ➢ “Pseudotumor” Myelofibrosis ➢ Primary and secondary forms ➢ Diffusely dense bones ➢ Hepatosplenomegaly ➢ BM bx to make dx

References 1.

2.

3.

4. 5. 6. 7.

"What is Sickle Cell Disease". Sickle Cell Information Center. December 16, 2003. Copyright © 1997. The Georgia Comprehensive Sickle Cell Center at Grady Health System, The Sickle Cell Foundation of Georgia, Inc., Emory University School of Medicine, Department of Pediatrics, Morehouse School of Medicine. http://www.scinfo.org/sicklept.htm Funaki B. "Sickle cell anemia: Bone manifestations", "Bone within a bone". Chorus: Collaborative Hypertext of Radiology. (Kahn CE ed). July 2004. Medical College of Wisconsin. February 1995. http://chorus.rad.mcw.edu/doc/01060.html Kahn CE. " Erlenmeyer flask deformity". Chorus: Collaborative Hypertext of Radiology. (Kahn CE ed). May 2004. Medical College of Wisconsin. < http://chorus.rad.mcw.edu/doc/00648.html> Kerr R. Imaging of musculoskeletal complications of hemophilia. Semin Musculoskelet Radiol 2003; 7:127-136. Lonergan GJ, Cline DB, Abbondanzo SL. Sickle cell anemia. Radiographics 2001; 21:971-994. Park JS, Ryu KN. Hemophilic pseudotumor involving the musculoskeletal system: spectrum of radiologic findings. AJR Am J Roentgenol 2004; 183:55-61. Wong AL, Sakamoto KM, Johnson EE. Differentiating osteomyelitis from bone infarction in sickle cell disease. Pediatr Emerg Care 2001; 17:60-63; quiz 64.


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Generalized Musculoskeletal Disorders Thomas Lee Pope, Jr, MD, FACR •

Figure 4-32-1

Learning Objectives • • • • • •

To describe a group of entities not well covered in the rest of the course To outline the imaging features of these diseases To introduce the listener to these entities so that he/she can study about them further

Outline of Diseases Osteoporosis (and its sequelae) Osteogenesis imperfecta Neurofibromatosis Collagen vascular-like diseases ➢ SLE ➢ Scleroderma ➢ Polymyositis/dermatomyositis Disuse osteoporosis in a 76 yo with left sided CVA • •

Terminology

•

Osteopenia – “paucity of bone” Osteoporosis ➢ Decreased bone mineral density ➢ Normal in quality ➢ Decreased in quantity 30%-50% of cancellous bone must be gone to recognize

• • •

Localized Regional or segmental Generalized or diffuse

Types of Osteopenia

• •

Localized Osteopenia/Osteoporosis Focal areas of bone loss Differential diagnosis: ➢ Infection ➢ Arthritides

Figure 4-32-2

Regional Osteopenia/Osteoporosis • •

Segmental area of decreased BMD Differential diagnosis: ➢ Disuse (immobilization) ➢ Chronic regional pain syndrome (CRPS) (RSD (Reflex sympathetic dystrophy) [Figure 4-32-2]

➢ Transient osteoporosis (bone marrow edema) ➢ Regional migratory osteoporosis

Generalized Musculoskeletal Disorders

Chronic regional pain syndrome (Reflex sympathetic dystrophy) Note the increased radionuclide accumulation on the early and late views of the bone scan

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•

Disuse/Immobilization Osteoporosis

• • •

Figure 4-32-3

[Figure 4-32-1]

Major cause ➢ Immobilization for traumatic injury ➢ Motor paralysis ➢ Inflammatory lesions of bones and joints Changes take 7-10 days (maximal at 23 months) Patterns: uniform, spotty, bands, cortical lamination or scalloping May appear very aggressive!!!

Chronic Regional Pain SyndromeReflex Sympathetic Dystrophy • • •

[Figure 4-32-1]

Elderly Trivial trauma Pain, swelling, temperature changes

Transient Regional Osteoporosis • •

[Figure 4-32-3]

•

• • •

Transient regional osteoporosis (bone marrow edema) of the hip: Note the osteopenia of the right hip and the proximal diffuse increased radionuclide accumulation on the bone scan Note the marrow replacement in the proximal right femur on T1 and the increased signal intensity (edema) on the T2 weighted images

General term Conditions sharing features of: ➢ Rapidly developing, self-limited, reversible osteoporosis ➢ Absence of clear cut inciting events Major types: ➢ Transient osteoporosis (bone marrow edema) of the hip ➢ Regional migratory osteoporosis

Figure 4-32-4

Regional migratory osteoporosis Rapidly developing, self-limiting and reversible Knee, ankle, foot and hip Joint nearest involved likely to be next involved

Transient Regional Osteoporosis (bone marrow edema) of the hip • • • • • • • •

1st -women in the third trimester of pregnancy Middle aged males LE > UE Osteoporosis and BM edema Differential diagnosis: AVN, infection Generally spontaneously resolves in 9-12 months

Pathology of osteoporosis (rib specimens)

Generalized Osteopenia/Osteoporosis

• • •

Diffuse decreased BMD Differential diagnosis: ➢ Senile osteoporosis ➢ Medications (Steroids, heparin) ➢ Systemic diseases (Deficiency states) ❖ Scurvy ❖ Malnutrition ❖ Calcium deficiency

Senile Osteoporosis [Figure 4-32-4]

• •

Pommer-1985 — “increased porosity” Most commonly encountered metabolic disease Reduction in bone “quantity,” normal in “quality” F>M, 4:1, (equal incidence) > 80 yo Most pain source: compression fx’s and kyphosis PE – kyphosis, shortened stature, and spinal rigidity


977


•

Epidemiological data

• • •

Figure 4-32-5

Surgeon General Report, October, 2004 ➢ Half population in US > 50 yo with low bone mass and risk for fracture ➢ 1.5 million/year osteoporosis-related fx ➢ 34 million with hip osteopenia Caucasian females > 50 yo – 40% chance of fx in lifetime (13% for males) Hip fracture: ➢ Risk of mortality within 3 months is 4X greater than normal ➢ 20% of fx victims die or wind up in nursing home within year after event Annual cost of treating osteoporosis: $18 BILLION

Pathology of osteoporosis [Figure 4-32-4] Osteoporosis “life cycle” - Fracture index [Figure 4-32-5] • • • • • •

Osteoporosis Measurement [Figure 4-32-6] Dual energy X-ray absorptiometry (DEXA) Conventional X-ray (radiogrammetry) Single photon absorptiometry (SPA) Dual photon absorptiometry (DPA) Neutron activation analysis Quantitative CT (QCT)

Figure 4-32-6

Lumbar

• • • • • •

Hip

Distal Radius

Dual Energy X-ray Absorptiometry (DEXA)

• • • •

Relative tissue attenuation from dual energy X-ray source Easy to perform Most reproducible technique with the least coefficient of variation (COV) Detects changes of 1–3% Expressed in gm/cm2 Primary indication: Estrogen deficiency to determine therapy

BMD Terms BMD measured in gm/cm2 T-score: Patient’s BMD compared to normative data (Normal = 25 yo women) Z-score: Patient’s BMD compared to her aged-matched controls World health organization uses T scores to classify a patient’s bone mineral status


978


• •

WHO Classification of BMD • • • •

STANDARD: Mean BMD of 25 yo women NORMAL: BMD from the mean to 1 standard deviation below the mean (mean to -1SD) OSTEOPENIA: T-score from 1 to 2.5 SD below mean (-1SD and -2.5 SD)) OSTEOPOROSIS: T-score below 2.5 SD below mean (> -2.5 SD) Osteoporosis also established by presence of a non-traumatic vertebral compression fracture

Figure 4-32-7

Important implications • • •

• • •

Fracture risk doubles with each drop of 0.1 below the mean of the T-score Risk of fracture also doubles for each decade the patient is > 50 yo Goal is to eventually be able to calculate an “absolute fracture risk”- more holistic measurement method Patients respond better to “you have a 70% of Photomicrograph of senile vertebral osteoporosis developing a fracture” than they do to “your T-score is whatever”

Figure 4-32-8

Senile Osteoporosis: Imaging Features

•

Increased radiolucency on X-ray (“osteopenia”) Cortical thinning Altered trabecular patterns

Senile Osteoporosis: Complications

• •

Acute fractures ➢ Spine (L>T>C) ➢ Distal radius (Colles) ➢ Proximal femur ➢ Humerus (neck) ➢ Ankle (malleoli) Insufficiency fractures ➢ Covered in “osseous stress injury” talk

Lateral radiographs of three patients with “codfish” vertebral bodies

Spinal effects of osteoporosis • •

Decreased bone density ➢ Accentuation of primary trabeculae Cortical thinning Changes in vertebral shape ➢ “Biconcave” – CODFISH (“fish”) shape ➢ Endplate deformities (Schmorl’s nodes, cortical irregularities) ➢ Wedged vertebrae ➢ Vertebrae plana (“pancake”/silver dollar)

Senile vertebral osteoporosis “Codfish” vertebral bodies

Figure 4-32-9

[Figure 4-32-7]

[Figure 4-32-8]

Femoral neck fracture locations

[Figure 4-32-9]

A = subcapital B = neck C = basicervical D = intertrochanteric E = subtrochanteric


979


• •

Garden classification [Figure 4-32-10] < II = Percutaneous pinning > II = THA (AVN risk)

Figure 4-32-10

Bohndorf, Imhoff, Pope: Musculoskeletal Imaging: A Multimodality Approach George Thieme Verlag, 2001

Figure 4-32-11

Garden Type I [Figure 4-32-11] • • • •

Intertrochanteric Fractures

• • • • • • • • •

Extracapsular Periosteum present Low incidence of nonunion or AVN (~1%) Distinction from basicervical often difficult (no clinical concern) Most comminuted, 15% severely GT/LT may be displaced by gluteus or iliopsoas May have other non-suspected injuries in pelvis

Osteogenesis Imperfecta (OI)

• •

[Figure 4-32-12]

Skeletal, skin, sclera and dentin abnormality 1/30K affected 1/50K severely 20-50K in US 85% AD Major types: ➢ Congenita ➢ Tarda

Garden I type of femoral neck fracture treated with Knowles’ pins

Figure 4-32-12

OI vs Child Abuse • •

Metaphyseal corner fx’s uncommon in OI Sternal, rib, scapular, skull and bucket handle fx’s common Fx’s continue to occur in protective custody Other non-MSK findings not present: ➢ Retinal hemorrhage ➢ Visceral intramural hematomas ➢ Intracranial bleeding ➢ Pancreatitis ➢ Splenic trauma

OI-congenital type OI-tarda

Congenital type of Osteogenesis Imperfecta

Figure 4-32-13

[Figure 4-32-12]

[Figures 4-32-13 and 4-32-14]

Tarda form of Osteogenesis Imperfecta: Note healing fractures with exuberrant callus formation


980


Figure 4-32-14

Figure 4-32-15

Note multiple fractures, intramedullary rods and dynamic hip screw and Harrington rods for scoliosis

Tarda form of Osteogenesis Imperfecta: Note exuberrant callus formation and intramedullary rod placement for fractures • •

Neurofibromatosis (NF) • • • • • • • • •

Described first by Tiresius (1773) and Smith (1849) Named for von Recklinghausen - noticed association of neural and cutaneous elements in 1882!! Defects of all three cell layers = phakomatosis 1/3000 births Estimated ~100,000 in US One of humanity’s most common genetic disorders Mutation rate is 1/10,000 gametes/generation Greater than that for ALL OTHER COMMON GENETIC DISORDERS AD with variable gene expression (FH in 60%) Equal incidence in male and female and Caucasian and non-Caucasian

Neurofibromatosis (NF)

• • •

Two distinct clinical forms: ➢ NF-1 (vonRecklinghausen’s) - Café-au-lait spots, neurofibromas, skeletal deformities ➢ NF-2 - Acoustic neuromas Can lead to disfigurement, blindness, deafness, dermal/brain/spinal tumors, loss of limbs, malignancies, learning disabilities WAS NOT “Elephant Man’s Disease”…John Merrick had Proteus Syndrome (cell growth disturbance with hemihypertrophy and macrodactyly)

Imaging features [Figures 4-32-16 to 4-32-20 overleaf]

•

•

Spinal changes: ➢ Dural ectasia ❖ Vertebral scalloping ❖ Foraminal enlargement ❖ Pedicle erosion ➢ Mesodermal dysplastic changes: ❖ Scoliosis - Typical - Dysplastic, sharply angulated, < 6 segments of lower T spine (pathognomonic of NF) ❖ Pencilling and spindling of the transverse processes Long bones (due to neurofibromas or mesodermal dysplastic changes) ➢ Pencilling ➢ Bone erosions ➢ Pseudarthrosis (characteristically of the tibia) ➢ Associated with nonossifying fibromas Ribs ➢ Scalloped and irregular (“twisted ribbons”) ➢ Erosions of inferior rib surfaces


981


Figure 4-32-16

Figure 4-32-17

Neurofibromatosis: Note accentuated scoliosis

Neurofibromatosis: Note kyphosis, vertebral anomalies and widened neural foramen


Neurofibromatosis: Note mesodermal dysplastic changes in the pelvis of two different individuals

Figure 4-32-20

Neurofibromatosis: Note posterior vertebral scalloping and dural ectasia

Neurofibromatosis with the characteristic “pseudoarthrosis” of the tibia Generalized Musculoskeletal Disorders

982


• • •

Meningoceles [Figures 4-32-21 and 4-32-22]

• • •

Figure 4-32-21

About 2/3 of patients with NF 70%-80% of all meningoceles in NF patients Most common presentation: Asymptomatic post mediastinal mass Protrusion of dura and arachnoid through IV foramen and posterior rib cage into the extrapleural thoracic cavity - nonca++ Presence of ca++ excludes meningocele

Systemic Lupus Erythematosis (SLE) • • • • • • • •

“Lupus” -Latin for wolf – malar erythema looked like the bite of a wolf Generalized connective tissue disorder F>M, second to fourth decade, rare over the age of 45 Higher incidence in AA and Hispanics Fever, anorexia, weight loss, polyarthralgias, skin rash Chronic disease with acute episodes Variable prognosis

Intrathoracic meningocele: Note the posterior mediastinal mass on the right

Figure 4-32-22

Three Categories of SLE •

Discoid-skin rash only, 20% of patients with SLE Systemic-chronic, inflammatory, multisystem disorder of the immune system Drug-induced-Chlorpromazine, hydralazine, isoniazid, methyldopa, procainamide CT and MR imaging of intrathoracic meningocele

Musculoskeletal Imaging Findings • •

Figure 4-32-23

[Figure 4-32-23]

•

Major: deforming nonerosive arthropathy Minor: ➢ Osteonecrosis ➢ Insufficiency fractures ➢ ST calcification ➢ Acroosteolysis ➢ Tendon weakening and rupture ➢ Subchondral cysts ➢ Myositis ➢ Polyarthritis ➢ Osteomyelitis and septic arthritis

Symmetric Polyarthritis • • • • • •

Articular symptoms and signs common-75%-90% of patients Non-deforming nonerosive arthropathy of SLE Frequently bilateral and symmetric (differential diagnosis is post-Streptococcal 5%-40% with disease (Jacoud’s) arthropathy Hands > knees > wrists > shoulders ST swelling, periarticular osteopenia Reversible and little functional effect !!Cartilage and osseous destruction rare without underlying osteonecrosis!!

• • • • • • •

Unknown cause F:M (4:1) 3rd to 5th decade < 20 cases/million/year Variable prognosis Up to 65% MSK involvement at presentation Death: Lung, heart and renal involvement

Scleroderma


983


• •

CREST Syndrome • • • • • • • •

Figure 4-32-24

First described by Winterbauer in 1964 as CRST Velayos added esophageal involvement to make CREST in 1979 50,000 to 100,000 in US 7 times more common in females Calcinosis Raynaud’s phenomenon Esophageal abnormalities Sclerodactyly Telangiectasia

Calcinosis [Figure 4-32-24] • • • • •

Abnormal calcium deposition in ST without calcium metabolism abnormality Fingers, forearms, and extensor surfaces of elbows and knees

Calcinosis of Scleroderma

Figure 4-32-25

Raynaud’s phenomenon [Figure 4-32-24] Often the first symptom of scleroderma Ischemia of fingers, toes and ears Numbness, tingling and burning pain Attacks precipitated by cold, vibration and emotional stimuli

Acroosteolysis

[Figure 4-32-25]

Acroosteolysis of scleroderma

• •

Fibrosis and atrophy of the smooth muscle Hypermobility, dysphasia, reflux esophagitis and strictures

• • •

Replacement of the normal connective tissue with dense collagen bundles Skin = thin, appears smooth and is tightly bound Fingers narrow and taper distally

• •

Permanent dilatation of capillaries and venules Face, lips, tongue and fingers

Gastrointestinal (esophageal) involvement

Sclerodactyly

Telangiectasia

• • • •

Idiopathic inflammatory myopathies

•

•

Dermatomyositis and Polymyositis 2:1 female to male ratio 5 cases/million/year (incidence increasing) Dermatomyositis ➢ Complement-mediated (terminal attack complex) vascular inflammation Polymyositis ➢ Direct cytotoxic effect of CD8+ lymphocytes on muscle

Idiopathic inflammatory myopathies

•

Dermatomyositis ➢ Men > 40 yo ➢ Skin rash and muscle weakness ➢ Primary malignancies: ❖ Lung, prostate, female pelvic organs, breast or GI tract ➢ Precedes detection of tumor months to years Polymyositis: ➢ Primary malignancies: Lung, NHL


984


•

Imaging Findings [Figures 4-32-26 to 4-32-29] •

Figure 4-32-26

ST abnormalities ➢ ST thickening and edema ➢ Soft and periarticular calcification (IM > SQ) Articular abnormalities ➢ Radial subluxation or dislocation of IP of thumb (“floppy thumb”) = quite characteristic ➢ Erosions of multiple sites in hands ➢ Flexion deformities (MCP) ❖ “Swan neck” deformity

Polymyositis with soft tissue calcification

Figure 4-32-28

Figure 4-32-27

“Floppy thumb” of dermatomyositis/polymyositis Dermatomyositis with extensive soft tissue calcification • •

Figure 4-32-29

“Swan neck” deformity

•

MC flexion, PIP hyperextension and flexion at the DIP Most common inrheumatoid arthritis

Other imaging Findings •

•

RBS ➢ Increased accumulation at sites of calcification (Technetium and gallium) MR Imaging ➢ Muscle atrophy ➢ Fatty replacement ➢ Decreased SI correlating with activity of disease ➢ Increased SI on T2WI and STIR

Remember! PM, DM, Scleroderma, SLE, mixed CVD and overlap syndromes may all look alike ➢ ST calcification ➢ Articular and osseous abnormalities

Dermatomyositis with increased signal intensity in the right gluteal region Musculoskeletal Radiology

985


•

Summary • •

• • •

Osteoporosis ➢ Most common metabolic disease ➢ Insufficiency fractures may mimic mets/myeloma Osteogenesis imperfecta ➢ Diagnosis of exclusion in young patient with osteopenia out of proportion to age ➢ Easily fractured and exuberant callous formation Neurofibromatosis ➢ Pencilling, “pseudarthrosis,”, posterior scalloping, thoracic meningocele Systemic lupus erythematosis ➢ Ulnar deviation without erosions (differential is Jacoud’s (poststreptococcal arthritis) Scleroderma ➢ CREST, acroosteolysis Inflammatory muscle disease ➢ Dermatomyositis and polymyositis ➢ Nonspecific findings (look like scleroderma) ➢ Must look for malignancy in these patients

References 1.

Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York, NY, Thieme Medical Publishers, 2001


986


Osseous Musculoskeletal Stress Injuries Thomas Lee Pope, Jr, MD, FACR • • • •

Outline Biomechanical considerations Historical perspective Epidemiology and clinical manifestations Anatomic approach with examples ➢ Stress injury “look alikes” • Avulsive and muscular “tug” injuries • Unusual activities causing stress injury • Recommended work-up •

“Bone Fatigue” 1922 - Muller ➢ Isolated a segment from the radius of a dog ➢ Created a fatigue fracture of the ulna • 1949/50 - Rutishauser/Majno ➢ First description of the histologic aspects of fatigue fractures Muller W. Bruns Beitr. klin. Chir. 127:251-290, 1922 R/M. Schweiz. med. Wschr. 79: 281-88, 1949. 6:333-42, 1950. •

Historical Perspective • • • • •

1855 - Breithaupt - Prussian military surgeon ➢ Clinical features of painful feet on long marches 1887 - Pauzet - army doc ➢ Exostoses on PE from bone proliferation soldiers’ feet 1897 - Stechow - Prussian guard ➢ First imaging in 36 cases of MT stress fractures 1905 – Blecher ➢ First femoral neck stress fracture 1921 – Deutschlander - German physician ➢ Comprehensive study of stress lesions in > 50 yo 1936 - Asal – German ➢ First large series of 590 stress fractures in German troops

•

Types of MSK stress injury Soft tissue injury ➢ Bruises ➢ Muscle tears ➢ Musculotendinous injury • Collagen injury ➢ Tendon and ligament strain/tears • Cartilage injury ➢ Chondral injury (hyaline and fibrocartilage) • Osseous abnormalities ➢ Stress reaction (sclerosis/lucency/periosteal reaction) ➢ Stress fracture • •

Stress injuries Most common in lower extremities Dissipation of ground reaction forces (GRF) (running, walking, marching, jumping) • Bone exposed to stress (ie load) and strain (ie deformation) with weightbearing • Factors influencing bone response: ➢ Bone geometry and bone density • Jumping and landing – GRF up to 12 X body weight


987

Osseous Musculoskeletal Stress Injuries

•

How Do Stress Injuries Develop? • • • • •

Figure 4-33-1

“Wolff’s law” ➢ Bone remodels in DIRECT reponse to the forces applied to it Normally a happy “marriage/relationship” between osteoblasts and osteoclasts Increased stresses cause increased osteoclastic activity with transient weakening Transient weakening predisposes to “microdamage” Coalescence of “microdamage” – stress reaction or injury Cascade ➢ Fissures ➢ Microfractures ➢ Osteoblastic response (periosteal reaction or cancellous “clouding”) ➢ Coalescence ➢ Fatigue reaction/injury

• • • •

Epidemiology 20% of all injuries seen in sports medicine clinics Between 4.7% and 15.6% of injuries in runners 20-25% of stress injuries in tibia, fibula or MT Females: 49% with very irregular menstruation, 39% with irregular menstruation • Study of 320 athletes with stress injury: ➢ 69% runners ➢ 8% fitness class participants ➢ 5% racket sports ➢ 4% basketball players • Track athletes have highest incidence • • • •

Clinical Features of Stress Injury Pain associated with activity Pain resolves without activity Pain in characteristic location associated with activity PE: ST swelling, point tenderness

Grade 4 Stress injury of the tibia, pathological fracture of the fibula in patient with RA on steroids Condensation of cancellous bone Perpendicular to the long axis

•

Risk Factors for Stress Injury Intrinsic ➢ Low BMD ➢ Lower limb misalignment ➢ Muscle fatigue ➢ Weakness/strength imbalance ➢ Pathologic bone ➢ Menstrual/hormonal irregularities ➢ Genetic predisposition • Extrinsic ➢ Excess volume/intensity of training ➢ Change in training surface (density or topography) ➢ Worn out training shoes ➢ Inadequate nutrition ➢ Cigarette smoking


988


•

Activities associated with Stress Injury Lower extremity ➢ Running ➢ Marching ➢ Soccer (pelvis) ➢ Basketball ➢ Skating (fibula) ➢ Jumping (pelvis) ➢ Swimming (tibia, MT) ➢ Ballet (pelvis, spine) • Upper extremity ➢ Baseball: ❖ Throwing-Humerus, scapula, olecranon, first rib ❖ Batting-Ribs ❖ Catching-Patella, tibia ➢ Javelin throwing-ulna ➢ Basketball ➢ Volleyball • • • •

Activities associated with Stress Injury Rowing, kayaking-Second through tenth ribs Running with hand held weights-Scapula Coughing-ribs Trapshooting-Coracoid process

•

Major categories of Stress Injury “Fatigue” fracture ➢ Abnormal muscular stress of torque ➢ Bone of normal elastic resistance • “Insufficiency” fracture ➢ Normal or physiologic activity ➢ Bone deficient in mineral or elastic resistance *****Imaging findings are similar • • • • • • •

Osteoporosis Metabolic disease Hyperparathyroidism Osteomalacia/Rickets Cushing’s disease Paget disease Diabetes mellitus

• • • • •

0 – Normal study 1 – Subtle periosteal edema (IR, FS T2-W images) 2 – Periosteal edema and increased marrow SI on FS T2-W images 3 – More extensive edema (T1-W and T2-W) 4 – Discrete fracture line visible on MR or on radiography

Risk factors - Insufficiency fractures

MR Grading System Stress injuries

[Figure 4-33-1]

Fredericson M, Bergman AG et al: Am J Sports Med 1995;23:472-481 • • • • • •

Anatomic approach Lower extremity: Foot (MT, navicular, calcaneus), tibia, femur Pelvis (Insufficiency and adolescent) Upper extremity Unusual causes of stress injury Mimics of stress injury Recommended workup


989


•

Metatarsal stress fractures [Figures 4-33-2 to 4-33-5]

Figure 4-33-2

Second most common stress fracture site behind the tibia • First described in military recruits ➢ Flat flexible feet = MT stress fractures ➢ Cavus feet = tibial stress fractures • Distance runners and ballet dancers • In order: 2, 3, 1, 4, 5 • •

Navicular stress fracture [Figures 4-33-6 and 4-33-7] • • • • •

0.7%-2.4% of all stress fx’s Activities: Track and field (59%), Australian football (19%), Basketball (10%) First description: 1958 in greyhounds 1970 - humans Most common in runners Increasing dorsal midfoot pain radiating down medial arch Rx: Non-weight bearing cast (86% success rate)

Figure 4-33-3

Second MT fatigue fracture

Figure 4-33-4

Right 2nd MT fatigue fracture and 1 month f/u in 42 yo male

Figure 4-33-5

MR imaging of third MT fatigue fracture, soccer player

Sesamoid necrosis in ballet dancer Osseous Musculoskeletal Stress Injuries

990



Classic navicular fatigue fracture [Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]

Figure 4-33-8

Classic fatigue fracture of calcaneus

Microangiopathic studies cadaveric feet…navicular supplied by both ant and post tibial arteries, enter at small “waist” of cortical bone and branch out to supply the medial and lateral 1/3… Central 1/3, under greatest stress with relative avascularity •

[Case courtesy of Dr. Armando Abreu, Porto Allegro, BR]

Tibia stress fracture

Figure 4-33-9

Three major types ➢ Medial tibial plateau ➢ Tibial diaphysis ➢ Anterior mid tibia

• •

Medial tibial plateau [Figure 4-33-9] Less frequent than tibial diaphysis Often misdiagnosed as pes anserinus bursitis or tendoninitis • Less critical stress injury • Treatment ➢ Rest for 4-6 weeks ➢ Then return to full activity

Medial tibial fatigue fracture

Figure 4-33-10

Medial tibial stress syndrome “Shin splints”[Figure 4-33-10]

MR features of medial tibial stress syndrome (“shin splints”)


991


Figure 4-33-11

Figure 4-33-12

“Aggressive” periosteal reaction in the tibia in fatigue fracture Diaphyseal fatigue fracture with histologic correlation from the AFIP fascicles • •

Figure 4-33-13

Tibial diaphyseal Fatigue Fracture[Figures 4-33-11 and 4-33-12] • •

Posteromedial border of tibia Tensile forces produced along anterior convex side, compressive forces along posterior concave margin 465 injuries causing exertional leg pain – 75% in posteromedial tibial diaphysis Difficult to tell stress injury from “shin splints” (medial tibial stress syndrome)

• • •

Anterior mid-tibia [Figure 4-33-13] Most common in jumping and leaping athletes Focal cortical thickening and sclerosis “Dreaded black line” ➢ Propensity to nonunion ➢ Risk of complete displaces fracture • Require more aggressive treatment

Diaphyseal fatigue fracture with the “dreaded black line”

Figure 4-33-14

Longitudinal Tibial Stress Fracture • • •

[Figure 4-33-14]

Devas 1960 Patients MAY not give h/o increased activity Saifuddin (Clin Rad 1994): ➢ Two cases ➢ Stress fx located superomedial to the nutrient foramen of the tibia ➢ Foramen weakens bone at this site? ➢ ? insufficiency fracture

Longitudinal stress fractures of the tibia

Longitudinal fatigue fracture

Craig et al, Henry Ford Hospital, Detroit, Skeletal Radiology, 2003 • Six cases • All female (age range - 15-69 yo) • Diagnosis made by finding cleft on multiple axial images • 5/6 patients had: ➢ Edema starting at level of the entrance of the nutritent vessel into the medullary cavity ➢ Vertical fx identified below this level on the anteromedial tibial cortex

Figure 4-33-15

Compression type of femoral neck fatigue fracture


992


•

Femoral neck stress injury [Figures 4-33-15 and 4-33-16]

Figure 4-33-16

Any athlete (jogger/runner) with hip, thigh or groin pain • Two types ➢ Tension type: ❖ Superior cortex ❖ Older osteoporotic patients ➢ Compression type: ❖ Younger athletic patients • Treatment: 2-3 months non-weight bearing with gradual return •

Pelvic Stress Injury [Figures 4-33-17 to 4-33-21] Running increases risk of stress lesions in sacrum and ischial rami • Sacral fractures more common in young women (sx’s mimic sacroiilitis) ➢ Fatigue: ❖ Anteroinferior sacral wing unilaterally ➢ Insufficiency: ❖ Elderly women, irradiated women ❖ Often bilateral (“Honda” sign)

Fatigue fracture which progressed to complete femoral neck fracture

Figure 4-33-17

Figure 4-33-19

Sacral insufficiency fracture

Figure 4-33-18

Bilateral sacral insufficiency fractures (the “Honda” sign)

Figure 4-33-20 CT of previous figure on left showing the classic CT findings of right sacral insufficiency fracture

Figure 4-33-21

Symphysis pubis stress reaction in soccer player Bilateral sacral and symphyseal insufficiency fractures in elderly female S/P external beam radiation for cervical cancer. Note the bilateral nature of the sacral fractures Musculoskeletal Radiology

993


•

Adolescent Stress Lesions [Figure 4-33-22] • • • •

Figure 4-33-22

Muscular “tug” (avulsive) lesions (Classic lesion: Cortical desmoid) Avulsion injury and sequelae Tendons, ligaments stronger than bone in adolescents May mimic primary soft tissue neoplasm in acute setting May mimic primary bone neoplasm after healing has occurred

Recommended Workup - Suspected Stress Injury • •

Correlate clinical situation with imaging Think “stress injury” in the correct setting (you may be the only clinician who does so!!) • Initial study is the conventional radiograph • Young patient-CT or MR imaging • Elderly patient ➢ Typical site-CT or MR ➢ Unusual site-RBS as screen, then CT or MR

16 yo with bilateral healed avulsions whose diagnosis was made at age 42!

References 1. 2. 3. 4. 5.

Chamay A. Mechanical and morphological aspects of experimental overload and fatigue in bone. J Biomech 1970; 3:263-270. Craig JG, Widman D, van Holsbeeck M. Longitudinal stress fracture: patterns of edema and the importance of the nutrient foramen. Skeletal Radiol 2003; 32:22-27. Müller W. Bruns Beitr. klin. Chir. 127:251-290, 1922 Rutishauser E, Majno G. [Lesions of normal and pathological bones due to overstrain.]. Bull Schweiz Akad Med Wiss 1950; 6:333-342. Tschantz P, Rutishauser E. [The mechanical overloading of living bone: initial plastic deformations and adaptation hypertrophy]. Ann Anat Pathol (Paris) 1967; 12:223-248.


994


Pelvis and Lower Extremity Trauma: An introduction Thomas Lee Pope, Jr, MD, FACR Figure 4-34-1

•

Outline Caveats ➢ Major highlights ➢ Not enough time to cover in depth ➢ You must read more on your own to supplement this lecture • Pelvic trauma • Acetabular trauma • Lower extremity trauma ➢ Femur ➢ Knee ➢ Ankle ➢ Foot ❖ Talus ❖ Calcaneus ❖ Classic fx’s

Radiographic anatomy [Figure 4-34-1] Major mechanisms of pelvic injury [Figure 4-34-2]

Pelvic Radiographic Anatomy, ip = iliopubic line, ii = ilioischial line, SIJ = sacroiliac joints

•

Most Popular Classifications Pelvic Ring Fractures

Figure 4-34-2

Tile ➢ Pelvic stability • Young-Burgess ➢ Degree of injury • Major divisions ➢ “Ring sparing” ➢ AP compression ➢ Lateral compression ➢ Vertical shear ➢ Complex

Major mechanisms of pelvic injury (Tony Wilson, Seattle Washington)

Young-Burgess vs Tile Classifications FRACTURE TYPE

YOUNG-BURGESS

TILE

Ring sparing

Not included

Type A

Anterior compression

AP compression Types I-III

B1 (B1, 1.1-1.3)

Lateral compression

Lateral compression Types I-III

B2 (B2, B2.1-2.2)

Vertical shear

Vertical shear

C (C1-3)


995

Pelvis-Lower Extremities Trauma

•

Young-Burgess Classification Pelvic Ring Fractures

Figure 4-34-3

Lateral compression (Most common) ➢ Types I and II • Anteroposterior (AP) compression ➢ Types I, II and III • Vertical shear • • • •

Lateral compression Most common mechanism of pelvic injury Lateral blow to the side of the pelvis Three types depending on severity “KEYS TO THIS INJURY:” ➢ Horizontal fx’s of pubic ramus/rami ➢ Crush (buckling) fx of sacrum

Lateral compression Type I injury-STABLE (Note the disruption of the sacral foraminal (arcuate) lines-arrows)

Figure 4-34-4

Lateral compression - Young-Burgess classification [Figures 4-34-3 to 4-34-5] • •

I – Ipsilateral sacral compression fx (stable) II- I + associated iliac wing fx ➢ Rotationally unstable Vertically stable • III – I + II with contralateral “open book” appearance (“windswept pelvis”)

“Windswept pelvis” - Lateral compressionipsilateral - AP compressioncontralateral [Figure 4-34-6] • • •

Lateral compression Type I injury

Severe anterior force Internal rotation of ipsilateral hemipelvis with external rotation of contralateral hemipelvis “Rolled over” look

Figure 4-34-5

Figure 4-34-6

“Windswept” pelvis Lateral compression Type I injury • • • • • • •

AP Compression Blows to front of pelvis MVA Three types depending on which ligaments involved Increases volume of pelvis Major risk = hemorrhage Often brain/abdominal injuries KEYS TO THIS INJURY: ➢ Vertical fx inf pubic rami (one or both sides) ➢ > 50% post acetabular wall ➢ < 10% sacral fx


996


AP Compression - Young-Burgess classification • •

[Figures 4-34-7 to 4-34-9]

Figure 4-34-7

Type I = SP disrupted (all ligs intact) Type II ➢ SP diastasis < 2.5 cm ➢ Torn ligaments: ❖ SP, SS, ST and ventral (anterior SI) • Type III ➢ SP diastasis > 2.5 cm ➢ Torn ligaments: ❖ SP, ST, SS and both ventral (anterior) and dorsal (posterior) SI AP compression Type II injury

Figure 4-34-8

Figure 4-34-9

AP Compression injury with vertical fracture of the sacrum

AP compression injury (Note marked widening of the symphysis pubis) •

Figure 4-34-10

AP Compression, Type II • • • • •

Disruption of sacrospinous, sacrotuberous and ventral (anterior) SIJ ligaments Diastasis of SP > 2.5 cm Diastasis of both SIJ anteriorly “Open book” appearance Rotationally unstable Vertically and posteriorly stable

• • • • •

Type I and II Disruption of all SIJ ligaments Complete separation of iliac wing from sacrum Complete pelvic instability Rotationally, vertically and posteriorly unstable

AP Compression, Type III

• • •

Vertical Shear Fall from height or head and back trauma Least common KEYS TO THIS INJURY: ➢ Disruption of SP or SIJ ➢ Cephalad or caudad displacement of pelvis (best seen on OUTLET film) • Rotationally, vertically and posteriorly UNSTABLE • •

Vertical Shear [Figures 4-34-10 and 4-34-11] Disruption of SP, ST, SS, and ant/post SIJ ligaments Characteristics ➢ Vertical pubic rami fractures ➢ SIJ disruption +/- adjacent fractures • Hemipelvis vertically (cranially) displaced


997

Vertical shear injury – Note vertical migration of left hemipelvis


•

Clues

Figure 4-34-11

Horizontally oriented pubic fracture ➢ Think lateral compression, look closely at sacral arcuate lines • Vertically oriented pubic fracture ➢ With AP displacement, think AP compression ➢ With vertical displacement, think vertical shear • Posterior wall acetabular fx ➢ Think AP compression • Central acetabular fx’s ➢ Think lateral compression •

Complications Pelvic Ring Disruption [Figure 4-34-12] Mortality 5%-50% (reflect severity) ➢ AP compression – 26% ➢ Vertical shear – 25% ➢ Complex – 17% ➢ Lateral compression – 13% • Head injury and hemorrhage (internal iliac branches or superior gluteal artery near sciatic notch) • • •

Acetabular Injury Significant trauma (MVA, falls) Associated pelvic ring fractures Pattern of acetabular injury depends on: ➢ 1. Position of femoral head at time of traumatic event ❖ FH externally rotated = anterior column ❖ FH adducted = acetabular roof ❖ FH abducted = forces transmitted inferiorly ➢ 2. Direction of force ❖ Anterior force = posterior wall and column ❖ Lateral force = medial acetabular wall (transverse type) • Therapy depends on proper classification

•

Soft tissue findings of vertical shear injury

Acetabular Columns Letournel and Judet Anterior ➢ Iliac wing to anterior acetabulum ➢ Incorporates superior pubic ramus • Posterior ➢ Sciatic notch to posterior acetabulum to ischium

Figure 4-34-12

“Inverted Y” column principle Radiographic Evaluation Acetabulum • •

[Figure 4-34-13]

AP pelvis Judet views ➢ 45 degree oblique views ➢ Right ❖ RPO = Iliac oblique ❖ RAO = Obturator oblique ➢ Left ❖ LPO = Iliac oblique ❖ LAO = Obturator oblique

Soft tissue complications of pelvic ring fractures

Figure 4-34-13

• •

Acetabular fractures Letournel and Judet classification, 1993 Ten different patterns ➢ Five elementary (run in single plane) ➢ Five associated (combination of elementary) • Difficult to remember • Most common: ➢ Posterior wall ➢ Transverse with posterior wall ➢ Both column (most common type) • Add T-shaped and transverse = 90% Pelvis-Lower Extremities Trauma

Pelvic CT anatomy (see Harris et al: AJR 2004;182:1363-75) 998


•

Letournel and Judet, 1993

Figure 4-34-14

Elementary (simple) fractures ➢ Posterior wall ➢ Posterior column ➢ Anterior wall ➢ Anterior column ➢ Transverse • Complex (associated) fractures ➢ T-shaped ➢ Posterior wall posterior column ➢ Transverse posterior wall ➢ Anterior with posterior hemitransverse ➢ Both columns

CT patterns of acetabular fractures Hunter, RCNA 1997 •

• • •

Axial CT image through roof of acetabulum ➢ Column Fracture ➢ Transverse Fracture ➢ Wall Fracture ➢ Normal

Wall Fracture [Figures 4-34-14 and 4-34-15]

• • • •

Weight-bearing columns of acetabulum not disrupted Posterior wall most common Major complications: ➢ Hip joint instability ➢ Osteonecrosis

Transverse Fractures [Figures 4-34-16 and 4-34-17] Medial and lateral components Fx line anterior to posterior Separates “walls” from “columns” CT = sagittal plane

Figure 4-34-15 Posterior wall acetabular fracture

Figure 4-34-16

CT of posterior wall fracture (Same patient )

T-type transverse fracture Musculoskeletal Radiology

999


• • • • •

Column Fracture

• •

Figure 4-34-17

Craniocaudad (coronal) direction Front and back halves Ant/post only or both Associated with other fx’s (post column, post wall) Conceptualize: Grasp ASIS could move acetabulum freely Obturator fx = column type or T-shaped fracture

Questions To Ask Yourself… • • • • • • •

Obturator ring fx? ➢ T-shaped or column fx Ilioischial line disrupted? ➢ Posterior column or transverse fx patterns Iliopectineal line disrupted? ➢ Anterior column or transverse-type fx’s Is iliac wing above acetabulum fractured? ➢ Fracture of anterior column Is the posterior wall fractured? ➢ Isolated or combo with post column or transverse fx’s Is “spur” sign present? ➢ Almost assuredly “both column” fracture

CT of T-type transverse fracture with Sagittal (lower left) and Coronal (lower right) reconstruction

Hip Trauma

• • • • •

Dislocations Hip fx’s ➢ Common injury in multi-trauma ➢ Common in the elderly ❖ Osteoporosis and cerebrovascular disease ❖ Prone to falls

Hip Dislocations 5% of all dislocations High energy trauma (MVA, MCA, etc.) ~90+% posterior Commonly associated with femoral shaft, patella and post acetabular fx’s Clinically ➢ Limb shortening, internally rotated and adducted ➢ 10-15% transient sciatic nerve palsy (direct impingement)

•

Hip Fractures Intracapsular ➢ Subcapital ➢ Mid cervical ➢ Basicervical • Extracapsular ➢ Intertrochanteric ➢ Subtrochanteric • Femoral neck fx’s 3-6X > women • Intertroch fx = frequency • • • •

Subtrochanteric Fractures Fracture line extends between LT and point 5 cm distally Direct trauma Older patient, less force required High incidence of malunion or nonunion ➢ ? secondary to greater proportion of cortical bone to trabecular bone in this region • Rx: Intramedullary rod


1000


•

Knee Injury Soft tissue signs ➢ ST swelling, lipohemarthrosis • Fractures ➢ *Supracondylar ➢ *Condylar ➢ *Tibial plateau ➢ Impaction (lateral femoral condylar notch) ➢ Tibial avulsion fractures ➢ Segond fracture ➢ Patellar fractures and dislocation • • • • • •

Valgus stress, 85% involve lateral tibial plateau Fat-fluid level Schatzker classification (6 types) Depression = cartilage thickness (3mm) Meniscal injury ~ 50% Rx: Lateral buttress plate and screw

• • • • • •

I=split fx (younger) II=split + depression of LTP (older) III=depression - splitting IV=MTP +/- depression V=split fx through MTP and LTP VI=dissociation of TP from underlying diaphysis

Tibial Plateau Fractures

Schatzker Classification

• • • •

Tibial Plafond (pilon) Fracture High energy axial loading (talus on tibial plafond) Ankle and distal tibial metaphyseal fx, intraarticular 20%-25% open Associated injuries: ➢ Compartment syndrome, vertebral compression fractures • Often require ORIF • Post-traumatic arthritis common • • • • •

Maisonneuve fracture

• • •

External rotation of ankle Fibular fx Serious injury Requires ORIF with screws Removed 8-12 weeks after injury

Ankle Fractures

•

Common injuries Soft tissue changes (STS, effusion) Classification schemes ➢ Lauge-Hansen ❖ Difficult to remember ❖ Not very reproducible ➢ Danis-Weber (AO) ❖ Easy to remember ❖ Reproducible

Danis-Weber Classification • •

Type A: Horizontal avulsion fx below mortise, stable, Rx: Closed reduction and casting (without displaced MM fx) Type B: Spiral fibular fx – level of mortise, external rotation, stable or unstable, Rx: Closed reduction unless fragments displaced Type C: Above mortise, disruption of lig attachment of tibia/fibula distal to fx, unstable, Rx: ORIF


1001


• • •

Ankle Fractures

• • •

Talar Neck Fractures [Figure • • •

Figure 4-34-18

Key is re-establishing tibiotalar joint congruence Mortise view important 1-2 mm displacement of talus in mortise dramatically changes contact area and pressure 40% decrease in contact area with 1mm lateral talar shift 4-34-18]

3%-5 % of foot fractures Dorsally directed force on braced foot (“aviator’s astragulus” – WWI pilots), now most commonly MVA Main blood supply of talar body enters neck through sinus tarsi and proceeds retrograde to supply body Neck fx’s compromise vascularity Hawkins sign (no AVN) Hawkins II talar neck fracture

Talar Neck Fractures - Hawkins classification [Figure 4-34-19] Figure 4-34-19

Figure 4-34-20

Risk of AVN: I = 10%; II = 40%; III = 90%; IV = 100% Hawkins classification of talar neck fractures (Bohndorf K, Imhoff H, Pope T: Synopsis of MSK Imaging: A Multimodality Approach, Thieme) • • • •

Snowboarder’s fx Eversion Lat process caught between LM and calcaneus May be caused by inversion and dorsiflexion

• • • • • • • • •

Most frequently fractured tarsal bone (60% of all fx’s) 2% of all fx’s in adults 5% - 9% bilateral 10% LS compression fx’s Peroneal tendon entrapment or compartment Intraarticular 70%, extra-articular 30% Most common EA = calcaneal body fx Anterior process = 15% Difficult to treat if displaced

Lateral Process of Talus fx [Figure

Calcaneal Fractures [Figure

4-34-20]

4-34-21]

Figure 4-34-21

Lateral process of talus fracture (Snowboarder’s fracture)

Coronal reconstruction of CT of calcaneal fracture

CT of calcaneal fracture


1002


• •

Classification systems Calcaneal Fractures

Figure 4-34-22

Bohler (1931) Essex-Lopresti (1952) ➢ Intraarticular vs extra-articular ➢ Types: Tongue and Joint depression • Rowe (1963) • Saunders – CT classification (1992) • Others: Hanover, Rowe, Palmer, Souer and Remy •

Sanders Classification • • • • • • •

I = Non-displaced ➢ Non-operative II = 2 parts (split) ➢ ORIF III = 3 parts (split and depression ➢ ORIF IV = Comminuted ➢ Defies open reduction Measures height of PF A = most cephalic point of tuberosity to posterior border of subtalar joint B = posterior border of subtalar joint to anterior process Normal: 20 - 40º Anatomy of the tarsal joints (Bohndorf K, Imhoff H, Pope T: Synopsis of MSK Imaging: A Multimodality Approach, Thieme)

•

Foot Injuries 5th MT ➢ Avulsion (pseudo-Jones or tennis fracture) ➢ Jones ➢ Stress fx (fatigue or insufficiency) • LisFranc • • • •

Figure 4-34-23

Jones Fracture Transverse fx 2-3 cm distally Displaces on weight bearing 35%-50% persistent non-union

Google Search: Sponsored links (Jones Fracture lawsuits-Recover medical expenses-Find attorneys and help nationwide-personal-injury-lawyer.com)

• • • • •

Lisfranc [Figures

•

4-34-22 and 4-34-23]

Napoleonic surgeon Developed quicker technique of forefoot amputation for gangrene Faster wiithout having to cut bone Injury in foot never described by him Commonly misdiagnosed

Divergent (left) and Homolateral (right) types of LisFranc injury

Summary • • • • • •

Reviewed major pelvic, acetabular and lower extremity traumatic lesions Meant as an introduction Supplement with reading and study Xerox major classifications of fractures Have readily available in MSK reading area Consult classifications frequently Supplement clinical experience with personal reading EVERY DAY

References 1. 2. 3. 4.

Bohler L: Diagnosis, pathology, and treatment of fractures of the os calcis. J Bone Joint Surg 13:75-89, 1931. Bohndorf K, Imhof H, Pope TL (eds). Musculoskeletal Imaging: A Concise Multimodality Approach. New York, NY, Thieme Medical Publishers, 2001 Borrill J, Funk L, Deakin S. Orthoteers: The guiding light in orthopaedic education. 2006.British Orthopaedic Association.. eMedicine (James WD, Adler J, Lutsep HL, Lorenzo CT, Lin EC, Ho SSW, Roy H, Gellman H, Meyers AD eds)


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5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16.

1996-2005 eMedicine.com, Inc . WedMD. Essex-Lopresti P. The mechanism, reduction technique, and results in fractures of the os calcis. Br J Surg 1952; 39:395-419. GE: Healthcare reimagined. Copyright General Electric Company 1997-2006. GE Medical Systems. 2006. Harris JH, Jr., Lee JS, Coupe KJ, Trotscher T. Acetabular fractures revisited: part 1, redefinition of the Letournel anterior column. AJR Am J Roentgenol 2004; 182:1363-1366. Hunter JC, Brandser EA, Tran KA. Pelvic and acetabular trauma. Radiol Clin North Am 1997; 35:559-590. Letournel E, Judet R. Fractures of the acetabulum, 2nd ed. Heidelberg, Germany: Springer-Verlag,1993 MacLeod M, Powell JN. Evaluation of pelvic fractures. Clinical and radiologic. Orthop Clin North Am 1997; 28:299-319. Palmer I. The mechanism and treatment of fractures of the calcaneus: open reduction with the use of cancellous grafts. J Bone Joint surg 1948;30-A(1):2-8 Perry DC, DeLong W. Acetabular fractures. Orthop Clin North Am 1997; 28:405-417. Rowe CR, Sakellarides HT, Freeman PA, et al. Fractures of the os calcis: long term follow-up study of 146 patients. JAMA 1963;184:920-923 Sanders, R., Hansen, S.T. & McReynolds, I.S.: Fractures of the calcaneus, in Jahss, M. (Ed.): Disorders of the foot and ankle, Philadelphia, W.B. Saunders, 1991. p. 2326-2354. Souer, R. & Remy, R.: Fractures of the calcaneus with displacement of the thalamic portion. J Bone Joint Surg [Br] 57: 413-421, 1975. Wheeless' Textbook of Orthopaedics. Copyright 1996-2005 Data Trace Publishing Company. Duke University Medical Center's Division of Orthopaedic Surgery. Data Trace Internet Publishing Company. 2006.


1004


Musculoskeletal Seminar I Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY 15 year old male with longstanding hindfoot pain

UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS LESIONS WITH SEQUESTRA-LIKE APPEARANCE • • • • •

Osteomyelitis Metastasis Fibrosarcoma/ Malignant Fibrous Histiocytoma (MFH) Lymphoma Osteoblastoma

UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS CORTICAL LUCENCY/CENTRAL CALCIFICATION • • • • • • •

Langerhans cell histiocytosis Osteoid osteoma Brodie abscess

UNKNOWN CASE #1: FINDINGS

• • • • • • •

Diffuse sclerosis of calcaneus CT-solid periosteal reaction causing sclerosis on radiographs Subchondral low density lesion with central calcification Diffuse edema/focal lesion related to posterior subtalar joint with joint effusion

T1

UNKNOWN CASE #1: OSTEOID OSTEOMA 10–25 years, M>F (3:1) Night pain relieved by ASA Lytic nidus (F; 4–6:1; 3–7 years old Often asymptomatic Unilateral 75–80% AVN ? Treatment-immobilization

UNKNOWN CASE #3: OSTEOCHONDROSES

UNKNOWN CASE #3: KOHLER DISEASE

Musculoskeletal Seminar I

Asymptomatic side

1006


•

13 year old female 1 year of pain now severe and worsening

• • • • •

Subacute osteomyelitis Medullary or cortical lucency surrounding sclerosis Channel-like lesion may extend to or across growth plate Staph aureus MRI or CT to evaluate soft tissue extension

•

30 year old female with progressive clubbing of fingers

UNKNOWN CASE #4: HISTORY

UNKNOWN CASE #4: BRODIE ABSCESS

T2


UNKNOWN CASE #5: ACROOSTEOLYSIS BANDLIKE: DIFFERENTIAL DIAGNOSIS • • • •

Hyperparathyroidism Polyvinyl chloride Hajdu-Cheney syndrome Post-traumatic

UNKNOWN CASE #5: WORMIAN BONES: DIFFERENTIAL DIAGNOSIS • • • • • •

Normal Cleidocranial dysplasia Cretinism Osteogenesis imperfecta Hypophosphatasia Pyknodysostosis


1007


• • • • •

Autosomal dominant; described 1948 Bathrocephaly, wormian bones, open sutures Acroosteolysis Poor dentition Osteoporosis

•

4 year old male with left hip pain

UNKNOWN CASE #5: HAJDU-CHENEY SYNDROME


UNKNOWN CASE #6: DIFFERENTIAL DIAGNOSIS EPIPHYSEAL LESIONS • • • • • • •

Chondroblastoma Giant cell tumor (GCT) Subchondral cyst/Intraosseous ganglion Infection Langerhans cell histiocytosis (LCH) Osteoid osteoma/osteoblastoma Clear cell chondrosarcoma

• • •

Lytic lesion epiphysis and metaphysis Small amount surrounding sclerosis MRI and CT – no joint fluid or calcification


UNKNOWN CASE #6: EOSINOPHILIC GRANULOMA (LCH) • • • • • •

5–15 years; M:F-2:1 95% of patients Caucasian Solitary 67% Flat bones involved-70% Lytic hole within hole appearance Diaphysis (58%), metadiaphysis (18%), metaphysis (28%), epiphysis (2%)


1008


Musculoskeletal Seminar II Mark D. Murphey, MD •

62 year old male with polyarticular joint pain

• • •

Asymmetric erosive arthritis hands and feet: MCP, and IP joints New bone formation Limited osteopenia



UNKNOWN CASE #1: SERONEGATIVE SPONDYLOARTHROPATHY CHARACTERISTICS • • • •

Asymmetry Bone Production Less juxtaarticular osteopenia Distribution

UNKNOWN CASE #1: PSORIATIC ARTHRITIS POSSIBLE PRESENTATIONS • • • • •

DIP and PIP joints Arthritis mutilans Oligoarthritis or ray distribution Rheumatoid like (rare) Sacroiliitis/spondylitis


1009

Musculoskeletal Seminar II

•

42 year old female with right low back pain

• • •

Unilateral destruction on both sides right sacroiliac joint Thickening of iliacus muscle Focal sclerotic fragments in joint

• • •

Unilateral-Infection, RA, Gout, Psoriatic, Reiter Bilateral asymmetric-RA, Gout, Psoriatic, Reiter Bilateral symmetric-AS, Enteropathic, Psoriatic, Reiter, RA

•

1 year old female with hand and foot pain



UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS SACROILIITIS


• •

UNKNOWN CASE #3: FINDINGS Soft tissue swelling about several fingers Periosteal reaction along several rays: phalanx hand, metacarpal and metatarsal

UNKNOWN CASE #3: DIFFERENTIAL DIAGNOSIS – DACTYLITIS •

• • •

Infection ➢ Pyogenic ➢ Unusual organism-TB Sickle cell anemia Thermal injury

UNKNOWN CASE #3: SICKLE CELL ANEMIA Musculoskeletal changes • Osteomyelitis (salmonella) • Avascular necrosis (AVN) • H-type vertebrae • Osteopenia • Diffuse sclerosis


1010


•

UNKNOWN CASE #4: HISTORY 11 year old male with ankle pain after previous fracture

5 months after initial fracture • • • •

25%–33% growth sequelae Only 10% important Follow for 2 years-X-rays Look for bowing/shortening

• • • •

Initial Salter-Harris IV fracture without good reduction Subsequent lateral bowing of fibula and tibia Epiphyseal plate irregular CT/conventional tomography-osseous bar bridging plate

•

38 year old male with calf pain and mass, no history of trauma

UNKNOWN CASE #4: SALTER-HARRIS FRACTURE



T2

T1


1011


•

UNKNOWN CASE #5: FINDINGS • • •

Nonspecific enhancing inflammation and edema in calf with more focal mass medially Biopsy-soft tissue osteosarcoma 3 weeks later early calcification on CT predominantly peripheral 4 weeks later thick rind of calcification peripherally

UNKNOWN CASE #5: MYOSITIS OSSIFICANS (HETEROTOPIC BONE FORMATION) • • • • •

No history of trauma 25% Soft tissue mass Subsequent calcification Zonal phenomenon X-ray and path Follow-up for maturation

•

14 year old female with enlarging right foot mass


UNKNOWN CASE #6: DIFFERENTIAL DIAGNOSIS CALCIFIED SOFT TISSUE MASS • • • • • • • •

Myositis ossificans Gout, collagen vascular disease Hyperparathyroidism, tumoral calcinosis Hemangioma Soft tissue chondro/osteosarcoma Synovial sarcoma

UNKNOWN CASE #6: FINDINGS • • • • •

Soft tissue mass plantar aspect mid to forefoot Faint calcification-CT and mag views; smooth erosion of 2nd and 3rd metatarsals Large soft tissue mass on MRI mildly heterogeneous and hyperintense T2W

UNKNOWN CASE #6: SYNOVIAL SARCOMA

• •

20–40 years old 68% lower extremity – particularly knee Most begin periarticular (< 10% intraarticular) Biphasic – epithelioid and spindle cell element on histo (also monophasic ) Radiographs – soft tissue mass, joint effusion (10–20%), calcification (30%), erosion or destruction adjacent bone Metastases-lungs and lymph nodes


1012


Musculoskeletal Seminar III Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY 63 year old woman with vague calvarial pain

UNKNOWN CASE #1: DIFFERENTIAL DIAGNOSIS – LYTIC SKULL LESIONS • • • • • • • •

Langerhans cell histiocytosis Metastases Myeloma Paget disease Brown tumor

UNKNOWN CASE #1: FINDINGS Focal skull lytic lesions: frontal and parieto-occipital Bone scan – multifocal area increased activity Radiographs – multiple lesions trabecular thickening

• • • •

Common – 3% of people over 40 years Lytic, blastic or mixed phases Most frequent to involve: spine, skull, pelvis Trabecular thickening – bone enlargement

• • • • •

Osseous deformity Fractures Neurologic symptoms Arthropaty Neoplasm

PAGET DISEASE

PAGET DISEASE: COMPLICATIONS


1013

Musculoskeletal Seminar III

•

UNKNOWN CASE #2: HISTORY 14 year old boy with thigh pain

T1

T2 • • • •

Cortical scalloping – femur Hair-on-end periosteal reaction Broad based soft tissue mass No medullary involvement

• • • •

Most chondroblastic 85% diaphysis femur/tibia Same age group as conventional osteosarcoma Better prognosis

•

55 year old man with hip pain


UNKNOWN CASE #2: PERIOSTEAL OSTEOSARCOMA

UNKNOWN CASE #3: HISTORY T1

UNKNOWN CASE #3: FINDINGS • • • • •

Osteopenia of left hip MRI – decreased intensity left proximal femur T1W, and diffuse increased signal T2W No focal defects on MRI; effusion Enhances with gado; hot on bone scan Returns to normal in several months


T2 1014


UNKNOWN CASE #3: TRANSIENT OSTEOPOROSIS HIP / BONE MARROW EDEMA SYNDROME • • • • • •

Middle aged males Spontaneous pain; worsened by weight-bearing Symptoms regress 2–6 months Migratory form may recur at nearby joint Cause unknown-bone marrow edema Relationship to AVN ?

•

82 year old man with slowly enlarging mass in the thigh


T2 T1

UNKNOWN CASE #4: DIFFERENTIAL DIAGNOSIS – CALCIFIED SOFT TISSUE MASS • • • • • • •

Myositis ossificans Aneurysm Lipoma/liposarcoma Soft tissue osteosarcoma/chondrosarcoma Synovial sarcoma


• • •

Large mass thigh with mineralization calcification/ossification MRI/CT: ➢ Fat component ➢ Hemorrhagic component ➢ Myxoid component

UNKNOWN CASE #4: MYXOID LIPOSARCOMA

•

Myxoid variety most common liposarcoma (40–50%) Intermediate grade See lipomatous components with CT/MRI (40–50%) (We believe 90%-95% by MR) Mineralization not rare in liposarcoma


1015

T1 GD


•

40 year old female with arthralgias

• • •

Interphalangeal joint subluxations No erosions Osteopenia



UNKNOWN CASE #5: DIFFERENTIAL DIAGNOSIS SUBLUXATIONS/NO EROSIONS • • • • • •

Systemic Lupus Erythematosis (SLE) Mixed Connective Tissue Disease (MCTD) Juvenile chronic arthritis Ehlers-Danlos Jaccoud arthropathy

UNKNOWN CASE #5: SYSTEMIC LUPUS ERYTHEMATOSIS Musculoskeletal changes ➢ Deforming nonerosive arthropathy ➢ Tendon rupture ➢ Avascular necrosis (AVN) ➢ Joint and bone infection ➢ Acrosclerosis


1016


•

UNKNOWN CASE #6: HISTORY 37 year old female with progressive ankle deformity ➢ What is the underlying disease? ➢ What process involves the ankle subsequently? ➢ How can the processes be correlated?

• • • • • • •

Langerhans cell histiocytosis Enchondromatosis Fibrous dysplasia Hereditary multiple exostoses Paget disease Neurofibromatosis (Type 1) Angiomatous lesions

• • •

Cause – pain sensation vs. neurovascular Destruction, debris, density increase, disorganization Diabetes, syphilis, cord-damage-syrinx

DIFFERENTIAL DIAGNOSIS – BENIGN POLYOSTOTIC LESIONS

UNKNOWN CASE #6: NEUROPATHIC JOINT

• • •


•

Lytic expansile benign appearing polyostotic lesions Fibula, femur, metatarsal Subsequently ankle-fragmentation, debris, destruction, increased density

UNKNOWN CASE #6: FIBROUS DYSPLASIA Endocrine Abnormalities • Sexual precocity • Cushings • Acromegaly 5 years after initial images • Hyperthyroidism • Diabetes mellitus (hypothalamic dysfunction) • Diagnosis case #6-fibrous dysplasia (polyostotic) with neuropathic ankle due to diabetes mellitus


1017


Musculoskeletal Seminar IV Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY Several patients with various wrist subluxation patterns: Match with pattern ➢ Lunate ➢ Perilunate ➢ Barton fracture/subluxation

Musculoskeletal Seminar IV

1018


• •

Perilunate – 75%, usually with transscaphoid fracture rest of carpus-dorsal Lunate – 25% lunate rotated volar, capitate remains aligned to radius

• •

Barton – fracture of dorsal rim of radius with dislocation of carpus Reverse Barton – fracture of volar rim of radius – with dislocation of carpus

•

31 year old female with underlying systemic disorder

• • •

Dysplastic changes thoracolumbar junction Short segment scoliosis Posterior vertebral body scalloping

UNKNOWN CASE #1: WRIST SUBLUXATIONS

UNKNOWN CASE #1: WRIST SUBLUXATIONS




1019


UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS POSTERIOR VERTEBRAL SCALLOPING • • • • • • •

Normal variant L4–5 Neurofibromatosis (Type I) Tumor/increased intraspinal pressure Achondroplasia Acromegaly Ehlers-Danlos, Marfan , Osteogenesis Imperfecta Mucopolysaccharidosis

UNKNOWN CASE #2: DIFFERENTIAL DIAGNOSIS DYSPLASTIC THORACOLUMBAR JUNCTION • • • •

Neurofibromatosis (Type 1) Cretinism Idiopathic Achondroplasia

UNKNOWN CASE #2: NEUROFIBROMATOSIS I MUSCULOSKELETAL MANIFESTATIONS • • • • • • •

Cranium-enlarged empty orbit, left lambdoid suture defect Spine – scoliosis, posterior vertebral scalloping, lateral meningocoeles Pseudoarthrosis (tibia), bowing, fractures Ribbon ribs Neurofibroma – 5% malignant degeneration Localized gigantism Multiple nonossifying fibromas

•

13 year old boy with bilateral hip pain


T1

UNKNOWN CASE #3: DIFFERENTIAL DIAGNOSIS IRREGULAR EPIPHYSES (MULTIPLE) • • • • • • •

Normal variant Avascular necrosis (AVN) Hypothyroidism Epiphyseal dysplasia Trevor disease Mucopolysaccharidosis

UNKNOWN CASE #3: FINDINGS • •

Multiple irregular epiphyses ➢ Bilateral femora ➢ Right humerus Delayed skeletal maturation Changes of slipped capital femoral epiphysis (SCFE)


1020

T2 Musculoskeletal Radiology

• • • • •

UNKNOWN CASE #3: CAUSES OF SCFE Idiopathic Rickets-renal Trauma, obesity Hypothyroidism, hypoparathyroidism Radiation

UNKNOWN CASE #3: MUSCULOSKELETAL CHANGES IN HYPOTHYROIDISM • • • • • •

Delayed skeletal maturation, Wormian bones Epiphyseal dysgenesis with osteoarthritis Thoracolumbar junction gibbus SCFE; ligamentous laxity Osteoporosis; soft tissue calcification Soft tissue edema, carpal tunnel syndrome

• • • • •

Ossification from multiple sites Femoral, humeral centers and talus Not due to vascular insufficiency May disappear with treatment May lead to premature osteoarthritis (OA)

•

45 year old man with wrist pain

UNKNOWN CASE #3: EPIPHYSEAL DYSGENESIS


UNKNOWN CASE #4: DIFFERENTIAL DIAGNOSIS CHONDROCALCINOSIS • • • •

CPPD deposition/arthropathy Hemochromatosis Hyperparathyroidism All others poor association


1021


•

UNKNOWN CASE #4: FINDINGS • •

Osteoarthritic changes – unusual locations radiocarpal and MCP joints (2nd through 5th) Chondrocalcinosis – TFFC, no scapholunate separation Hook-like osteophytes metacarpal heads

UNKNOWN CASE #4: HEMOCHROMATOSIS MUSCULOSKELETAL CHANGES • • • •

•

Osteoporosis Chondrocalcinosis (20–60%) Arthropathy (24–50%) looks like osteoarthritis Differences from CPPD arthropathy-involvement of 4th and 5th MCP joints; hook-like osteophytes metacarpal heads; less scapholunate separation; pericapitate narrowing

UNKNOWN CASE #5: HISTORY 39 year old woman with low back pain

2 weeks prior to previous radiographs


1022


UNKNOWN CASE #5: DIFFERENTIAL DIAGNOSIS NARROW DISK SPACE • • • • • •

Degenerative disk disease (DDD); herniated disk (trauma) Inflammatory arthritis Scheuermann disease Osteomyelitis Neoplasm (very rare) Amyloid– chronic renal failure (CRF)

T1

T2

• • • •

Rapid disk space narrowing L2–3 over two week interval Subtle endplate destruction L2–3 level T1W-MR: marrow replacement L2–3 with disk involvement T2W-MR: increased marrow intensity L2–3 with disk involvement

• • •

Usually starts in anterior subchondral bone then spreads rapidly to disk Bacterial vs. unusual cause (TB) Drug abusers predisposed

•

65 year old man with right pain


UNKNOWN CASE #5: INFECTIOUS SPONDYLODISCITIS



1023


UNKNOWN CASE #6: DIFFERENTIAL DIAGNOSIS CHONDROID LESION •

•

Benign ➢ Enchondroma, bone infarct, chondroblastoma, chondromyxoid fibroma (CMF), osteoblastoma Malignant ➢ Chondrosarcoma - intramedullary, juxtacortical, clear cell, mesenchymal, myxoid, dedifferentiated, extraskeletal

T2

T1 • • • •

UNKNOWN CASE #6: FINDINGS Lytic lesion proximal femur some areas of surrounding sclerosis Cortical permeation inferomedial on conventional tomography Matrix on CT and tomography-chondroid Soft tissue mass best seen on MRI

RADIOLOGIC DIFFERENTIATION OF CHONDROSARCOMATOUS LESIONS •

• •

Aggressive chondroid lesion with soft tissue mass ➢ High grade conventional chondrosarcoma ➢ Dedifferentiated chondrosarcoma ➢ Mesenchymal chondrosarcoma Large fluid component bone or soft tissue ➢ Myxoid chondrosarcoma Change in appearance or foci of more aggressive nature ➢ Diagnosis: Dedifferentiated chondrosarcoma


1024


Musculoskeletal Seminar V Mark D. Murphey, MD •

UNKNOWN CASE #1: HISTORY 60 year-old man with 6 months of knee pain ➢ Biopsied and diagnosed as myeloma. ➢ Is this a tenable diagnosis? ➢ What is the correct diagnosis and why was the initial pathology incorrect?

T1

T2 Fat Sat T1 GD

T2 FAT SAT •

UNKNOWN CASE # 1: FINDINGS •

•

Radiographs – Geographic 1A lesion with channel/tract like component inferiorly (subtle) MRI – Marrow replacement T1W ➢ Rim enhancement (fluid filled mass) ➢ Homogeneous very high signal T2W ➢ Surrounding edema ➢ Tract like component inferiorly Differential diagnosis – UBC, ABC (no expansion) intraosseous hematoma, ablated lesion, Brodie abscess


1025

Musculoskeletal Seminar V

UNKNOWN CASE #1: SUBACUTE OSTEOMYELITIS BRODIE ABSCESS • • • • • • •

Described in 1832 - chronic/subacute Walled-off with central fluid, often sterile (staph- only cultured in 50% of cases) Children (M>F), metaphysis, tibia Intramedullary; channel-like lucencies May cross growth plate or be cortical Periosteal reaction/sequestra may be seen

UNKNOWN CASE #1: BRODIE ABSCESS •

•

Biopsied at margin in reactive tissue ➢ Led to erroneous diagnosis Myeloma (untreated) not a tenable diagnosis ➢ asthis is a solid lesion ➢ Importance of radiologic/pathologic correlation

UNKNOWN CASE #1: HISTORY 59 year old veteran involved in mild MVA (first film) with progressive pain (second set of films 2 weeks later and MRI)

First Radiograph

Second radiographs 3 weeks prior to first radiograph

T2

T1


1026


• • • • •

UNKNOWN CASE # 2: FINDINGS Relatively rapid destruction of shoulder Fragments in joint Sharp “surgical” margin MRI – replacement of humeral head with high intensity on T2W History of drained syrinx 40 years ago and cervical spine MRI shows severe myelomalacia

• • •

Cause – pain sensation vs. neurovascular Radiologic- destruction, debris, density increase, disorganization Diabetes, syphilis, cord damage - syrinx

•

15 year-old boy with hip pain

UNKNOWN CASE # 2: NEUROPATHIC SHOULDER-SYRINX

UNKNOWN CASE # 3: HISTORY

T1

•

STIR

UNKNOWN CASE # 3: FINDINGS • • •

Marrow replacement right femoral neck T1W ➢ medial transcervical region High signal on STIR ➢ surrounding edema periosteum/ST ➢ horizontal low signal linear band medially Subsequent near total resolution

STRESS FRACTURE: FEMUR •

•

Medial femoral neck - fatigue type ➢ heal with symptomatic treatment (3 to 12 months) ➢ crescentic MR abnormality above lesser trochanter Lateral femoral neck - insufficiency type ➢ possible cause most subcapital fractures ➢ DO NOT HEAL: COMPLETE/DISPLACED ➢ Garden staging (< Grade 2 percutaneous pins) (> Grade 2 THA due to development of AVN) Usually horizontal/oblique rarely longitudinal ➢ thigh splints (stress reaction)


1027


•

UNKNOWN CASE # 4: HISTORY 23 year-old man with knee pain, masses and lesions on radiographs ➢ Diagnosis of bone lesions ➢ Underlying condition

T1

T1

T2

T2 WITH FAT SAT


1028


• • • • • • • • • •

NEUROFIBROMATOSIS 1: SKELETAL MANIFESTATIONS

•

•

Mesodermal dysplasia Kyphoscoliosis Facial, orbital, lambdoid suture (left) defects Multiple nonossifying fibromas Meningocele Posterior vertebral scalloping Rib deformity (ribbon ribs) Congenital pseudarthrosis (tibia) Focal hypertrophy (gigantism) Localized neurofibroma - most common ➢ least characteristic, often deep/multiple ➢ superficial lesions (fibroma molluscum) Plexiform neurofibroma - pathognomonic ➢ early childhood ➢ precedes cutaneous neurofibromas

UNKNOWN CASE # 5: HISTORY 53 year-old man with lateral knee mass and pain

T1

STIR

STIR


1029


PROTON DENSITY

STIR • •

UNKNOWN CASE # 5: FINDINGS

•

Tibiofibular/lateral tibiofemoral joint osteoarthritis High fluid content multilocular mass laterally ➢ multilocular / surrounding edema ➢ appears to arise from tibiofibular joint ➢ components in bone (tibia and femur) and soft tissue D/DX -- Ganglion/Synovial cyst, myxoid tumor


1030


• • • •

GANGLION/SYNOVIAL CYST

• • •

Etiology unknown- neoplasm, trauma, inflammatory Young adults-most common mass hand/wrist Pain-may affect adjacent nerves Location ➢ ST: Hand, foot, knee, hip, shoulder ➢ Intraosseous: medial malleolus, wrist, knee ➢ BOTH Thick walled unilocular/multilocular ➢ high protein content affects CT/T1W MR ➢ walls/septa may enhance CT/MRI/Sono - cystic mass ➢ may rupture cause surrounding edema

UNKNOWN CASE # 6: HISTORY 47 year-old woman with mid to low back pain ➢ Most likely diagnosis? ➢ Two other possible diagnoses?

T1

T2

T2

STIR


1031


•

UNKNOWN CASE # 6: FINDINGS • • • •

Multifocal bone scan areas increased radionuclide activity ➢ spine, SC joints, SI joints CT-multifocal sclerosis ➢ anterior/posterior paralleling endplates ➢ erosions/bone production costovertebral joints ➢ cause of hot bone scan MRI - multifocal areas marrow abnormality ➢ low T1W, high T2W/STIR ➢ anterior/posterior paralleling endplates ➢ no soft tissue mass Radiographs- subtle sclerosis, sacroiliitis D/DX - Metastases, myeloma, lymphoma


1032


• • • • • •

ANKYLOSING SPONDYLITIS: CLINICAL CHARACTERISTICS

• • •

Peak age of onset 15-35 years M:F 3-5:1 Incidence 6.6/100,000 HLA-B27 > 90% Rare in blacks Predilection axial involvement

ANKYLOSING SPONDYLITIS: DISTRIBUTION

• •

• • • • • • • •

Osseous ankylosis Ligament/Tendon ossification Spine/SI joints ➢ symmetric Pelvis - symphysis, ischium, iliac, hips Peripheral changes unusual early (10% - 50%) ➢ asymmetric

ANKYLOSING SPONDYLITIS: SPINE CHANGES Osteitis -”shining corners” Squared vertebral bodies Syndesmophytes Bamboo spine Calcified disc, fused facets/ligaments Pseudarthrosis/fractures Atlantoaxial disease/Cauda equina Other diagnoses- Reiter/Psoriatic, SAPHO


1033


Radiologic Pathology 2006-2007 - Volume 2 - Index 25-OH Vitamin D 902 Abscess 796 Soft Tissue Infection 827 Subperiosteal 823 Access. Navicular 880 Accessory Muscles Masses (Ankle and Foot) 886 Accessory Soleus 886 Acetabulae protrusio 816 Acetabular Columns Letournel and Judet 998 Injury 998 Trauma 995 Acetabulum 998 Achilles Tendon 883 Achondroplasia 1020 ACL 867 Post-operative 868 Tear 866, 867 Acromegaly 773 Acromial Variation 927 Acromio-clavicular Joint Injuries 941 Acromion 942 Acroosteolysis 983, 1007, 1008 Actinomyces 833 Actinomycosis 829, 833 Active infection 825 Acute Osteomyelitis 821 Adamantinoma 721, 771, 776 Adductor pollicis 947 Adenomatoid odontogenic tumor 855 Adhesive Capsulitis 931 Adolescent Stress Lesions 994 Adult Palmar Fibromatosis 779 Adult Plantar Fibromatosis 780 Aggressive “Malignant” Osteoblastoma 750 Aggressive infantile Fibromatosis 778 Aggressive Osteoblastoma 750 Alcoholism 907, 953 Alkaline phosphatase 812 Alkaptonuria 949, 953 Allograft 707 ALPSA Lesion 934 Aluminum Toxicity 716 Ameloblastic fibroma 852 Ameloblastic fibro-odontoma 857 Ameloblastoma 852, 854 Amyloid 795 Amyloidosis 716, 1006 B2 – microglobulin 716 Carpal tunnel syndrome 716 Destructive spondyloarthropathy 716 Discovertebral erosions 717 Anatomy of the tarsal joints 1003 Aneurysm 804 Aneurysmal Bone Cyst 749, 784, 787 Angioblastoma 776 Angiomatoid 780 Angiomatosis 805, 808

Angiomatous lesions 1017 Angiosarcoma 721, 805, 810 Ankle 879, 995 Fractures 1001 Ankylosing Spondylitis 912, 916, 918, 1033 Ankylosis 914 Anterior Dislocation (Glenohumeral) 942 Anterior Drawer 867 Anterior Instability (Glenohumeral) 933 Anterior talofibular 881 Anterior tibiofibular ligament 880 Anterolisthesis 840 Anteroposterior (AP) compression (Pelvic Trauma) 996 Arthritis 795, 1006 Juvenile Chronic 919 Psoriatic 916 Septic 825 Tuberculous 832 Articular cartilage 859 Aseptic necrosis 906 Aspergillosis 835 Aspirin/nonsteroidals (Osteoma) 746 Atlantoaxial subluxation 915 Atlas Fractures 843 Atypical Mycobacterium 833 Autograft Healing 706 Avascular Necrosis 719, 959, 1010, 1020 Aviator’s astragulus 1002 Avulsion 865 Avulsive cortical injury 777 Axial Compression Injury 846 Axial Osteomalacia 904 Axillary Nerve Neuropraxy 935 Axillary View 942 Bacillary angiomatosis 837 Ballooned epiphyses 920 Bamboo spine 918 Banana fracture 816 Bankart Lesion 932 Bankart Repair 938 Barton Fracture (Reverse) 945 Baseball Finger 948 Basilar invagination 816, 901 Bathrocephaly 1008 Batson’s plexus 961 Benign Bone Tumors: Age Distribution by Decade 723 Benign Fibrosis Histiocytoma 773 Benign Polyostotic Lesions 1017 Bennett Fracture 947 Biceps Injury 957 Partial Tears 957 Tendinosis 957 Biceps Femoris 869 Biceps Tendon 929 Bicipital Radialis Bursitis 957 Bilateral Facet Dislocation 842 Birbeck bodies (Eosinophilic Granuloma) 890 Blade of grass 813 I1

Blastic Disease 814 Blastic Lesions 967 Blastic Phase 812 Blastomycosis 835 Blount 911 Blow out lesions 967 BMD 978 BMD (WHO Classification) 979 Bohler 1003 Bone Autograft 706, 707, 710 Bone bruise 865, 879, 958 Bone enlargement 814 Bone Graft Complications 708 Fracture 709 Joint Instability 709 Nonunion and Pseudarthrosis 708 Resorption 709 Bone Graft Substitutes 710 Bone Infarct 769, 906, 1024 Osteonecrosis 769 Bone Island 743 Bone Metastases 961 Bone Production 914 Bone scan 744 Bone Tumors 720 Cartilage 720 Histiocytic 721 Marrow 720 Notochord 721 Osteoid 720 Unknown Origin 721 Vascular 721 Bone Tumors (Incidence) 722 Important Factors in the Diagnosis of 723 Primary Benign 722 Primary Malignant 722 Bone within Bone 970 Botryoid odontogenic cyst 852 Bouchard nodes 923 Bowing of long bones 901 Boxer’s Fracture 948 Brachial artery 944 Brachial nerve injury 944 Breast Carcinoma 962 Bristow procedure 938 Brodie abscess 748, 824, 1007, 1026 Brown tumor 711, 853, 1013 Brucella 829 Brucellosis 829 Bucket handle 861, 862 Bumpy (Soft Tissue Swelling) 912 Bursae (Knee) 870 Bursitis (Bicipital Radialis) 957 Bursitis (septic) 826 Burst Fracture 846 Button osteophyte 922 Cafe-au-lait spots 773, 981 Calcaneal Fractures 1002 Calcaneofibular 881 Calcaneus 995

Calcific Myelitis 792 Tendinitis 952 Tendonitis 930 Tendonitis (Glenohumeral) 943 Calcified falx cerebri 853 Calcified Soft Tissue Mass 1012, 1015 Calcinosis 984 Calcitonin 818 Calcium deficiency 977 Calcium hydroxyapatite 718, 792, 949 Cancellous (Osteoid Osteoma) 746 Candidiasis 835 Candle flame 813 Cap thickness 758 Capillary hemangioma 809 Capitate (Dislocation) 946 Capitellum 955 Capsulorapphy 938 Carcinomatosis 971 Carpal Dislocations 946 Carpal Stability 874 Carpal Tunnel 872, 876 Syndrome 877 Cartilage metaplasia 799 Cartilage nodules 799 Cartilaginous Lesions 757 Chondroblastoma 757 Chondromyxoid fibroma 757 Chondrosarcoma 757 Enchondroma 757 Juxtacortical chondroma 757 Osteochondroma 757 Caseating necrosis 830 Cavernous hemangiomas 761 Cavernous spaces 806 Cellulitis 826 Cementoblastoma 854 Central giant cell granuloma 852 Cervical Burst Fracture 846 Cervical Spine Trauma 839 Cervical spondylosis 848 Charcot joint 953 Chondroblastoma 720, 1008, 1024 Codman Tumor 763 Chondroblasts 764 Chondrocalcinosis 950, 1021 Chondroid 738 Chondroid Lesion 1024 Chondroid Matrix (Enchondroma) 761 Chondroid Matrix (Intramedullary Chondrosarcoma) 765 Chondromyxoid Fibroma 720, 1024 Chondrosarcoma 762, 764, 1024 Dedifferentiated 769 Extraskeletal 768 Mesenchymal 768 Myxoid 768 Chondrosarcomatous Lesions 1024 Chordoma 721, 786 Christmas disease 972 Chronic ACL Tear 867 Chronic Granulomatous Disease of Childhood 827 I2

Chronic hematoma 796 Chronic infection 818 Chronic Osteomyelitis 825, 852 Chronic Recurrent Multifocal Osteomyelitis (CRMO) 827 Chronic regional pain syndrome 976 Chronic renal failure 793 Chronic sclerosing osteomyelitis 856 Chronic symmetric plasma cell osteomyelitis 827 Clavicle Clay Shoveler Fracture 843 Clear Cell Chondrocytes 767 Clear Cell Chondrosarcoma 763, 764, 767, 1008 Clear Cell Sarcoma 792, 803 Cleidocranial dysplasia 1007 Clutton joints 834 Coach’s Finger 948 Coccidioidomycosis 835 Codman Tumor 763 Collagen Vascular Diseases 793, 907, 1012 Collagen vascular-like diseases 976 Collateral ligament 869 Collateral ligaments 859 Colles fracture 945 Column Fracture (Pelvis) 1000 Complete pelvic instability 997 Compression Fracture 841 Congenital insensitivity to pain 953 Congenital Syphilis 834 Contiguous spread 820 Contusion 865 Cooley’s anemia 970 Coracoid 942 Coronoid fossa 955 Cortical Osteoid Osteoma 746 Desmoid 777 Fibrous Dysplasia 775 Involvement 738, 739 Lucency/Central Calcification 1005 Resorption 712 Thickening (Chondrosarcoma) 765 Cotton wool 814 CPPD 718, 949 Arthropathy 792 Deposition/arthropathy 1021 Cranial Sclerosis 744 Craniotabes 901 CREST Syndrome 984 Cretinism 1007, 1020 Crohn's disease 912 Cruciate ligaments 859 Cryptococcosis 836 Crystal deposition (Thalassemia) 971 Crystal Deposition Disease 718, 949 Cushing syndrome 773 Cyst (Paralabral) 938 Cystic hygroma 808 Cystic Masses (Knee) 870 Cysticercosis 837 Dactylitis 830, 834, 969, 1010 Dagger sign 918 Danis-Weber Classification 1001

Decubitus ulcers 825 Dedifferentiated Chondrosarcoma 764, 769 Deep Endosteal Scalloping 765 Deforming nonerosive arthropathy 983 Degenerative Joint Disease 951 Dental Anatomy 849 Dentigerous cyst 852 Dentition 849 Deoxyhemoglobin 848 DeQuervain’s Syndrome 876 Dermatofibrosarcoma Protuberans (DFSP) 771, 781, 782, 802 Dermatofibrosis lenticularis disseminata 744 Dermatomyositis 976 DeSmet 860 Desmoid 746 Extraabdominal 779 Desmoplastic fibroma 771, 777 DEXA 978 Diabetes 825 Insipidus 892 Mellitus 773, 825, 953 Diffuse sclerosis 1010 Diphoshonates 818 Direct implantation 820 Discitis 828 Discography 828 Discoid 860 DISI deformity 874 Distal radial buckle fracture 945 Distal radioulnar joint 872 Distal Tuft Fracture 948 Disuse/Immobilization Osteoporosis 977 Double Axillary Pouch Sign 934 Double line sign 908 Doughnut sign 785 Drug Abusers 827 Du Toit & Roux 938 Dual Energy X-ray Absorptiometry (DEXA) 978 Dupuytren Exostosis 759 Durie/Salmon PLUS Staging (Myeloma) 965 Dysbaric disorders 907 Dysplasia Epiphysealis Hemimelica: Trevor Disease 759 Dysplastic Thoracolumbar Junction 1020 Dysprosium 165 795 Early focal cemento-osseous dysplasia 850 Echinococcus 837 ECU tendon sheath 875 Ehlers-Danlos 1016 Elbow 955 Dislocations and Fractures 944 Embolization 748, 808 Enchondroma 720, 760, 769, 1024 Enchondroma vs. Chondrosarcoma 769 Enchondromatosis 1017 Endocrinopathies 773 Endodontic procedures 854 Endosteal scalloping 770 Enostosis 720, 743 Enteropathic arthritis 912 Enthesopathy 914 Enucleation 852

I3

Eosinophilic Granuloma 721, 887, 890, 967 LCH 1008 Epicondylitis (Elbow) 956 Epidermoid Carcinoma 826, 834 Epidermoid Inclusion Cyst 784, 790 Epidural hematoma 848 Epiphyseal Lesions 1008 Dysgenesis 1021 Dysplasia 1020 Epiphysis 767 Epithelial nests 776 Epitrochlear Lymph Node 960 Erlenmeyer flask deformity 971 Erosions 913 Erosive Osteoarthritis 917 Erupting teeth 854 Essex-Lopresti 945, 1003 Ewing Sarcoma 721, 887, 964, 967 Ewing Sarcoma (Intergroup Study) 888 Exophthalmos 892 Exostoses 856 Exostosis (Subungual - Dupuytren Exostosis) 759 Extensor Carpi Ulnaris Sheath 875 Extensor tendons 859 Extent of Musculoskeletal Neoplasm 738 Extra-abdominal Desmoid Fibromatosis 778 Extra-articular erosions (Gouty Arthritis) 950 Extramedullary Hematopoiesis 971 Extraskeletal Chondrosarcoma 764, 768 Facet 840 Fallen fragment sign 787 Familial vitamin D res rickets 903 Fanconi syndromes 903, 968 Fanconi’s Anemia 972 Felon 825 Felty Syndrome 914 Femur 995 Fibrocartilage Calcification 951 Fibroma molluscum 1029 Fibromas 746 Fibromatosis 771, 778 Fibromatosis: Types 778 Fibrosarcoma 771, 780, 781, 802 Fibrosarcoma/MFH 748 Fibrosis Histiocytoma 773 Fibrous cortical defect 771 Fibrous dysplasia 771, 773, 856, 944, 1017 Fibrous Histiocytoma (Malignant) 721 Fibrous medullary defect 771 Fibroxanthoma (Nonossifying fibroma) 771 Fibular (lateral) 869 Fibular collateral lig complex 881 Fibular collateral ligament 867 Filariasis 837 Finger (Trauma) 948 First MC (Fracture) 947 Flexion Teardrop Fracture 843 Flexor Digitorum (Avulsion) 948 Flipped (meniscal tear) 862 Florid cemento-osseous dysplasia 856 Fluid - fluid level 764, 785, 802 Fluorosis 818

Focal cemento-osseous dysplasia 855, 857 Focal scerosing osteomyelitis 854 Foot 879, 995 Injuries 1003 Fracture (First MC) 947 Fractures 816 Fractures (Pathologic) 961 Frieburg 911 Full thickness (Rotator Cuff Tear) 928 Galeazzi 945 Ganglia 870 Ganglia (Knee) 870 Ganglion 792 Ganglion/Synovial Cyst 1031 Ganglion/synovial cyst/bursa 796 Gardner Syndrome 852, 856 Gardner Syndrome (Osteoma) 746 Gastrocnemius 869 Gastrocnemius/ Semimembranosus 870 Gaucher disease 818, 907, 971 GCT 769 Geodes 790 Geographic Contusion (Knee) 866 Geographic Pattern (Bone Tumors) 725 Geographic 1A: Differential Diagnosis 725 Geographic IB: Differential Diagnosis 726 Geographic IC: Differential Diagnosis 726 Giant Bone Island 743 Giant Cell (Reparative) Granuloma 780 Giant Cell Tumor 721, 784, 1008 Giant Cell Tumor Tendon Sheath (GCT-TS) 794 Giant Cells 784 Gigantism 1029 Glad Lesion 936 Glenohumeral 932 Instability 932 Joint 932 Ligaments 932 Glenohumeral Internal Rotation Deficit (GIRD) 936 Glenohumeral Labroligamentous complex 932 Glenohumeral ligament (avulsion) 935 Glenoid labrum 932 Glomus 721 Glomus Tumor 805, 808 Gnathic Osteosarcoma 754 Golfer’s elbow 956 Goltz syndrome 744, 784 Gorham 809 Gorlin syndrome 853 Gout 718, 817, 949, 1010, 1012 Gouty arthritis 949, 950 Gouty tophus 804 Gracilis 869 Granulomatous Disease of Childhood 827 Grashey view 942 Greater Tuberosity Fracture 935 Ground glass 774 Group B strep 822 Guinea worm (dracunculosis) 837 Gumma 834 Guyon’s canal 872, 877 HA Crystal Deposition Disease 952 I4

Hagl Lesion 935 Hajdu-Cheney Syndrome 1008 Hallux 924 rigidus 924 valgus 924 Hamartoma 743 Hamate Fracture 947 Hand-Foot syndrome 969 Hand-Schüller-Christian disease 887, 892 Hangee Fracture 845 Hangman Fracture 845 Hanover 1003 Hawkins classification of talar neck fractures 1002 Hawkins sign 1002 Heberden nodes 923 Hemangioendothelioma (HE) 721, 805, 810 Hemangioma 721, 804, 805, 1012 Arteriovenous 805 AV malformation 852 Capillary 805, 809 Cavernous 805 Venous 805 Hemangiopericytoma (HPC) 721, 768, 805, 810 Hematogenous Osteomyelitis: Adult 824 Hematogenous Osteomyelitis: Child 822 Hematogenous Osteomyelitis: Infant 822 Hematogenous Vascular Supply 820 Hematologic Disease 968 Hemochromatosis 949, 950, 952, 1021 Hemodialysis elbow 718 Hemoglobinopathy 907 Hemophilia 968, 972 Hemophiliac pseudotumor 973 Hemorrhage 781, 790 Hereditary Hyperphosphatasia: Juvenile Paget Disease 818 Hereditary multiple exostoses (HME) 757, 760, 1017 Heterotopic Bone Formation: Myositis Ossificans 800 High - Grade Surface 754 High output congestive failure 812 Hill-Sachs Lesion 934, 943 Hip Fractures 1000 Joint instability 999 Trauma 1000 Histoplasmosis 836 HLA B27 916 Hodgkin 966, 971 Hook of hamate 877 Hoop stresses 860 H-Shaped Vertebral Bodies 970 H-type vertebrae 1010 Human/animal bites 825 Humeral Fractures 943 Humphrey 859 Hutchinson triad 834 Hyaline cartilage cap 757, 758 Hyaluronic acid 795 Hydrops Fetalis 808 Hydroxyapatite 715 Hydroxyapatite Crystal Deposition 952 Hydroxyproline 812 Hygroma 808

Hypercementosis 854 Hyperextension 840 Hyperextension Dislocation 844 Hyperextension Injuries 843 Hyperextension: Teardrop Fracture 845 Hyperflexion 840 Hyperflexion Injuries 840 Hyperflexion Sprain 840 Hyperparathyroidism 711, 773, 852, 856, 950, 971, 1007, 1012, 1021 Hyperthyroidism 773 Hypertrophic Osteoarthropathy 962 Hypophosphatasia 904, 1007 Hypothyroidism 1020, 1021 Idiopatic osteosclerosis 854 Ifosfamide 903 Iliotibial band 867 Iliotibial tract 869 Imaging for Staging Musculoskeletal Neoplasm 738 Immature cementoblastoma 850 Immature periapical cemental dysplasia 850 Impingement Syndrome 927 anterolateral 881 Infantile dermal/digital fibromatosis 778 Infantile myofibromatosis 778 Infarction 969 Infection 1006 Infectious Spondylodiscitis 1023 Inferior Glenohumeral Labroligamentous complex 932 Inferior Glenohumeral Ligament 933 Inflammatory 780 Infrapatellar 870 Infrapatellar cleft 870 Insufficiency fractures 979, 983 Interbody Fusion 710 Intermedius 869 Interosseous ligament 880 Interosseous syndrome (Elbow) 956 Interspinous widening 840 Intertrochanteric Fractures 980 Intraarticular Bodies (Elbow) 956 Intra-Articular Hydroxyapatite Crystal Deposition Disease 952 Intracortical fibrous dysplasia 775 Intramedullary (Chondrosarcoma) 764 Intramedullary Extent 738 Intramedullary Hemorrhage 848 Intranuclear inclusions 812 Intraosseous ganglion 784, 790, 1008 Intrasubstance Tear (Rotator Cuff) 929 Invisible Margin (Bone Tumors) 728 Involucrum 820 Irradiation 907 Irregular Epiphyses (multiple) 1020 Isolated Fractures Radius: Galeazzi 945 Isolated Fractures Ulna: Monteggia 945 Isolated Tendon Injuries 948 ITB Friction Syndrome 869 Ivory vertebra 814, 962 Differential Diagnosis 962 Jaccoud arthropathy 1016 Jaffe-Campanacci syndrome 773 I5

Jaws 849 Jefferson Fracture 846 Jersey Finger 948 Joint Arthroplasty (Complications of ) 699 Dislocation / Abnormal Alignment 702 Fractures and Nonunion 702 Heterotopic Bone Formation 699, 703 Loosening and/or infection 699 Radionuclide Evaluation 700 Small Particle Disease 699, 701, 702 Joint involvement 738, 739 Abscess 741 Bursitis 741 Diabetic muscle ischemia 741 Fibromatosis 741 Gadolinium 742 Hematoma 741 intraarticular resection 739 Lymphocele 741 Muscle flap 741 Myositis ossificans 741 Myxoid liposarcoma 741 Post-Operative Imaging (Bone Neoplasm) 741 Radiation necrosis 741 Reactive lymph node 741 Seroma 741 Soft Tissue Mass - Benign 740 Soft Tissue Mass - Malignant 740 Subtraction MRI 742 Synovial cell sarcoma 741 Joint Replacement 699 Jones Fracture 1003 JRA 919 Polyarticular 920 Still Disease: Pauci or Monoarticular 920 Jumpers Knee 870 Juvenile aponeurotic fibroma 778 Juvenile Chronic Arthritis 912, 919, 973, 1016 Juvenile Paget Disease 818 Juvenile-onset adult type RA 920 Juvenile-onset ankylosing spondylitis 919 Juxtaarticular Osteoporosis 912 Juxtacortical Chondroma 720, 762, 767 Juxtacortical Chondrosarcoma 767 Juxtacortical Osteosarcoma 752 Kaplan 860 Kaposi sarcoma 837 Kasabach - Merritt 809 Keloid formation 744 Keratocyst 852 Kienböck 911 Kienböck’s Disease 911 Klippel-Trenaunay-Weber 809 Knee 995 Knee Injury 1001 Knee Stabilizers 867 Köehler 911 Kohler Disease 1006 Kyphosis 830, 840 Labral Repair 938 Labrum 932 Lamina dura 850

Laminar Fractures 843 Langerhans Cell Histiocytosis (LCH) 748, 887, 890, 1013, 1008, 1017 Laser therapy 808 Lateral compression (Pelvis Trauma) 996 Lateral Epicondylitis (Elbow) 957 Lateral inferior geniculate artery 863 Lateral Meniscus 859 Lateral Process of Talus Fractures 1002 Lateral Stabilizers 869 Lateral Tendons (Ankle and Foot) 884 Lateral Ulnar Collateral Ligament 959 Lauge-Hansen 1001 LCL-Biceps Femoris 870 Ledderhose disease 780 Legg-Calvé-Perthes 911 Leiomyosarcoma 781, 802 Leprosy 829, 833, 953 Lesion matrix 738 Letournel and Judet classification 998 Letterer-Siwe disease 887, 892 Leukemia 971 Ligament and Tendon Involvement 739 ligament(ous) injury 840 Limb Salvage Procedures 737 Lipoblastoma 896 Lipoma 720, 804, 1015 Lipoma Arborescens 798 Lipoma Intramuscular 895 Lipomatosis 897 Liposarcoma 720, 781, 802, 893, 897, 1015 Atypical 898 Dedifferentiated 898 Higher Grade Lesions 898 Myxoid Lesions 898 Pleomorphic Liposarcoma 899 Well-Differentiated 897 Loa loa 837 Location in Bone: Axial (Bone Tumors) 724 Locked facets 842 Long Head of Biceps Tendon 929 Long Head of the Biceps Tendon 932 Longitudinal Tibial Stress Fracture 992 Loose Bodies 871 Looser zones (Osteomalacia) 715 Looser’s zones 902 Lower Extremity Trauma 995 Low-Grade Chondroid Lesion 769 Low-Grade chondrosarcoma 769 Lumpy 912 Lunate 946 Lung Cancer 963 Lunotriquetral Instability 875 Lyme disease 834, 835 Lymphangioma 721, 805 Lymphoma 720, 748, 961, 964 Lymphoma of Bone (Primary Lymphocytic) 966 Lytic Patterns (Bone Tumors) 728 lytic phase 812 Lytic Phase 813 Lytic Skull Lesions 1013 Macho-Macho 852 I6

Madura Foot 837 Maffucci syndrome 760, 761, 809 Magic Angle Phenomenon 876 Magnuson Stack 938 Malignant Bone Tumors: Age Distribution by Decade 723 Malignant Fibrous Histiocytoma (MFH) 721, 771, 780, 781 Malignant melanoma of soft parts 803 Malignant myositis 801 Malignant transformation (Multiple Enchondromatosis) 761 Mallet Finger 948 Malnutrition 977 Marrow Edema (Osteoid Osteoma) 747 Marrow hyperplasia 969 Massive Osteolysis of Gorham 809 Mastocytosis 818 Matrix Formation 723 Matrix Formation (Bone Tumors) 729 Mazabraud syndrome 773 McCune Albright syndrome 773 Medial Collateral Ligament (MCL) 867 Injuries 868 Medial Collateral Ligament (Elbow) 959 Medial Meniscus 859 Medial migration (Osteoarthritis: Hip) 923 Medial Tendons (Ankle and Foot) 884 Median nerve 872, 877 Impingement 956 Melorheostosis 744 Meningoceles 983 Meningomyelocele 953 Meniscal Cyst 798, 870 Meniscal Flap 862 Meniscal tears 798, 858, 861 Menisci (Calcification) 951 Menisci (Post-surgical) 863 Meniscofemoral ligaments 863 Meniscus homologue 875 Mesenchymal cells 768 Mesenchymal Chondrosarcoma 764, 768 Metabolic Bone Disease 900 Metachronous Osteosarcoma 750 Metaphyseal chondrodysplasia 904 Metastases 748, 768, 1013 Skeletal 961 Metastatic lymphoma 967 Methemoglobin 848 MFH (Malignant Fibrous Histiocytoma) 802 MFH/ fibrosarcoma 769 Middle Glenohumeral Ligament 933 Milk - Alkali syndrome 793 Milwaukee Shoulder 952 Mithramycin 818 Mixed Connective Tissue Disease (MCTD) 1016 Mixed/Blastic Disease 814 Monostotic 773, 812 Monteggia 945 Morton’s Neuroma 885 Motheaten (Bone Tumors) - Differential Diagnosis 727 Mucoepidermoid carcinoma 852 Mucopolysaccharidosis 1020 Mucormycosis 836 Multidirectional Instability (Glenohumeral) 939

Multiple enchondromatosis 760, 761 Multiple Myeloma 964 Multiple myeloma with sclerosis or POEMS syndrome 965 Multiple tori and exostoses 856 Musculoskeletal Infection 820 Musculoskeletal Neoplasm - Extent 738 Musculoskeletal Neoplasm - Staging - Surgical Implications 737 Musculoskeletal Tumors - Staging 734 Histologically Benign 735 Histologically Malignant 736 Mycetoma 837 Mycobacteria 829 Mycobacterium 833 Mycobacterium Leprae 833 Myelitis (Calcic) 792 Myelofibrosis 818, 968, 971, 974 Myelography 840 Myeloma 720, 961, 964, 1013 Myeloma/plasmacytoma 786 Myelomalacia 848 Myeloproliferative diseases 949 Myositis 837, 983 Myositis Ossificans 792, 800, 1012 Myotendinous (Rotator Cuff Tear) 928 Myotendinous Tear of Pectoralis 931 Myxoid 780 Myxoid Chondrosarcoma 764, 768 Myxoma 773, 792 Myxomatous neoplasms 796 Narrow Disk Space 1023 Nasopalatine duct cyst 850 Navicular 880 Neck of the scapula 942 Necrosis - Avascular 719 Neoplasm (Paget Disease) 817 Nerve Impingement (Elbow) 956 Nerve root avulsion 840 Neuroarthropathy 953 Neuroblastoma 963, 964, 967 Neurofibromatosis (NF) 976, 981 Neurofibromatosis (Type 1) 773, 1017, 1020, 1029 Neurogenic tumor 786 Neuropathic 834 Joint 1017 Osteoarthropathy 949, 953 Shoulder-Syrinx 1027 Neurovascular involvement 738, 739 Nevoid basal cell carcinoma syndrome 853 NF (Neurofibromatosis) 981 NF-1 (vonRecklinghausen’s) 981 NF-2 - Acoustic neuromas 981 Nidus (Osteoid Osteoma) 746 Night Stick Fracture 944 Nocardia 833 Nodular Fasciitis 792, 801 Non-Hodgkin 966 Non-Insertional Achilles Tendon Pathology 883 Nonossifying fibroma (NOF) 771, 981 Nonosteogenic fibroma 771 Nonspecific spindle cell sarcoma 781, 802 O’Donoghue’s Triad 867 I7

Oblique meniscomeniscal ligament 863 Occult fracture 865 OCD 955 Odontogenic cyst 852 Odontogenic keratocyst 852 Odontogenic myxoma 852 Odontoid Fracture 847 Odontoma 855, 857 OI (Osteogenesis Imperfecta) 980 Olecranon bursitis 949, 958 Olecranon fossa 955 Ollier disease 760 Ollier Syndrome 762 Oncogenic osteomalacia 773 Os Acromiale 928, 942 Os odontodeum 847 Os Trigonum 880 Osgood-Schlatter 911 Osler-Weber-Rendu 809 Osseous bowing 812 Osseous deformity 816 Osseous Lesions both Sides of Joint 1006 Osseous Neoplasm 733 Ossicle 860 Ossifying fibroma 775, 855, 857 Osteitis deformans 812 Osteoarthritis 920 Osteoarthritis (secondary) 826 Osteoarthropathy (Hypertrophic) 962 Osteoarthropathy (Neuropathic) 949, 953 Osteoblastic Metastasis 743 Osteoblastoma 720, 743, 748, 763, 855, 857, 1008, 1024 Osteochondral fracture 879 Osteochondral Lesion 879 Osteochondritis 834 Osteochondritis Dissecans 799, 865, 879, 910 Osteochondroma 720, 757 Osteochondroses 911, 1006 Osteochondrosis 906 Osteoclast 962 Osteofibrous dysplasia 771, 775, 776 Osteogenesis Imperfecta (OI) 976, 980, 1007 Osteoid 738 Osteoid Osteoma 720, 743, 746, 748, 763, 1005, 1008 Osteolysis (Post-Traumatic - Clavicle) 941 Osteolysis of Gorham 809 Osteoma 720, 743, 745, 857 Osteomalacia 711, 714, 809, 900 Osteomyelitis 718, 748, 821, 824, 825, 827, 830, 856, 967, 1026 Osteomyelitis (salmonella) 1010 Osteonecrosis 769, 906, 999 Osteonecrosis (Spontaneous) 910 Osteopathia striata 744 Osteopenia 714, 900, 979, 1010 Osteopetrosis 856 Osteophyte (button) 922 Osteophyte formation 921 Osteopoikilosis 744 Osteoporosis 711, 900, 976, 979, 1008 Osteoporosis (transient) 909 Osteoporosis circumscripta 813

Osteosarcoma 743, 750, 751, 769, 857, 1012 Intramedullary 751 Juxtacortical 752 Parosteal 753 Telangiectatic 752 Osteosarcoma (Sclerosing) 754 Osteosarcoma: Low Grade Intramedullary 754 Osteosarcoma: Soft Tissue 755 Extraskeletal 755 Osteosarcoma : Intracortical 756 Osteosarcomatosis 755 Osteosclerosis 711, 714 Overhanging edge 914 Overhanging edge (Gouty Arthritis) 950 Oxalosis 718 Oxyhemoglobin 848 P V N S 793 Paget Disease 812, 1013, 1017 Paget’s disease 856 Pain (Congenital Insensitivity) 953 Palmar Fibromatosis 779 Palmer 1003 Pancreatitis 907 Panne 911 Panner’s disease 959 Paralabral Cyst 937 paramyxovirus (measles) 812 Parathormone (PTH) 711 Paravertebral soft tissue 828 Paronychia 825 Parosteal Osteosarcoma 753 Parrot Beak 862 Parsonage Turner Syndrome 958 Partial thickness (Rotator Cuff Tear) 928 Patellar Dislocation 866 Patellar Retinacula 867, 869 Patellar Tendinitis 870 Patellar tendon 869 Patellofemoral Joint 870 Patellofemoral Syndrome 870 Pathologic Fractures 961 Pattern of Bone Destruction and Lesion Margin 725 Geographic 725 Motheaten 725 Permeative 725 Transition Zone 725 PCL 867, 868 PCL Tear 868 Pectoralis Major Tear 931 Pedicle erosion 981 Pedicle sclerosis 749 Pedicolaminar Fracture-Separation 844 Pelvic Ring Disruption 998 Pelvic Ring Fractures 995 Pelvic Stress Injury 993 Pelvis Trauma 995 Pencilling (Long Bones) 981 perched facets 842 Percutaneous ablation (radiofrequency) 748 Percutaneous removal (Osteoid Osteoma) 748 Embolization 748 Radiofrequency 748 I8

Periapical cemento-osseous dysplasia 854 Periapical cyst 850 Periapical granuloma 850 Periarticular Calcification 715 pericapsular fat planes 826 Periodontal ligament 850 Periosteal Osteosarcoma 753, 1014 Periosteal reaction 723 Periosteal Reaction (Intramedullary Chondrosarcoma) 765 Periosteal reaction (PTH) 711 Periosteal Reaction: Aggressive 731 Codman triangle 731 Hair-On-End 731 Laminated 731 Sunburst 731 Periosteal Reaction: Nonaggressive 730 Buttressing 730 Expansion 730 Septation 730 Solid 730 Periosteal/juxtacortical (Chondrosarcoma) 764 Periostitis 914 Permeative (Bone Tumors) - Differential Diagnosis 727 Peroneus Brevis 884 Peroneus Brevis Split Syndrome 885 Peroneus Longus and Brevis 884 Peroneus Quartus 886 Perthes Lesion 934 Pes anserine 870 Phemister triad 832 Phlebolith 806 Picture frame 814 Pigmented Villonodular Synovitis (PVNS) 793 Pillar Fracture 844 Pisotriquetral joint 872 Plantar Fascia 882 Plantar Fasciitis 882 Plantar Fibromatosis 780, 882 Plantaris Tendon 884 Plasmacytoma 720, 966 PNET 964, 967 POEMS syndrome 964, 965 Polyarthritis 983 Polymyositis 976 Polyostotic 773, 812 Polyostotic Lesions 732 Angiomatous lesions 732 Malignant 732 Neurofibromatosis (type 1) 732 Paget disease 732 Popliteus hiatus 870 Popliteus tendon 859 Positive Rim Sign 943 Posterior Dislocation (Glenohumeral) 943 Posterior Impingement (Elbow) 956 Posterior Instability (Glenohumeral) 935, 943 Posterior recesses 870 Posterior Superior Glenoid Impingement 936 Posterior talofibular 881 Posterior Tibial Tendon 884 Posterior tibiofibular ligament 880 Posterior Vertebral Scalloping 1020

Posterolateral Rotatory Instability (Elbow) 960 Post-traumatic cyst 784, 790 Pregnancy 907 Prepatellar 870 Primary Lymphocytic Lymphoma of Bone 966 Primary Lymphoma 966 Primary Periarticular HA Crystal Deposition Disease: (Calcific Tendinitis) 952 Prostaglandin (Osteoma) 746 Prostate Carcinoma 962 Pseudarthrosis (Tibia) 981 Pseudogout 950 Pseudomonas 821 Pseudotumor (Hemophiliac) 973 Pseudoxanthoma Elasticum 792 Psoriasis 916 Psoriatic arthritis 912, 916, 1009 Psoriatic Sacroiliitis 1010 Pubic fracture 998 Pulley Injuries 948 Puncture wounds 825 Putti Platt 938 PVNS 792, 973, 1006 Pyknodysostosis 1007 Pyomyositis 826 Pyrophosphate arthropathy 950, 951 Quadriceps tendon 869 Quadrilateral Space Syndrome 930 RA 869 Rachitic rosary 901 Radial Collateral Ligament 959 Radial Fracture 945 Radial head 944 Radial head dislocation 945 Radial nerve impingement 956 Radial Styloid Hutchinson’s/ Chauffer’s Fracture 945 Radial tunnel syndrome 956 Radiation 757 Radiation - internal synovectomy 795 Radiation Induced Chondrosarcoma 764 Radiocapitellar Line 944 Radiolucent Lesions Multilocular (Macho-Macho) 852 Periapical 850 Pericoronal 852 Radiopaque and Mixed Lesions Ground Glass 856 Interradicular 855 Multifocal Confluent 856 Periapical 854 Target Lesion, Dense 857 Radioulnar ligaments 875 Raynaud’s phenomenon 984 Reactive Arthritis 917 Rectus femoris 869 Recurrent Multifocal Osteomyelitis 827 Reflex sympathetic dystrophy 976 Reiter Disease 912, 916, 917, 1010 Renal Cell Carcinoma 963 Renal disease 869 Renal Insufficiency - Chronic (MSK Manifestations) 711 Renal Osteodystrophy 711, 903 I9

Renal Tubular Disorders 903 Rhabdomyosarcoma 769, 781, 802, 964, 967 Rheumatoid Arthritis 797, 817, 912, 914, 941 Rheumatoid arthritis, JRA 748 Ribbon ribs 1029 Rickets 714, 900, 971 Acetabuli Protrusio 714 Basilar invagination 714 Triradiate pelvis 714 Rim Rent Tear (Rotator Cuff) 929 Ring sequestra 825 Rolando Fracture 947 Romanus and Andersson lesion 918 Rotator cuff 932 Atrophy 930 Tears 915, 925 Types 928 Rowe 1003 Saber shin 834 Sacral Lesions: Differential Diagnosis 786 Sacroiliac disease 918 Sacroiliitis 1010 Sacrospinous ligaments 997 Sacrotuberous ligament 997 Saddle nose 834 Salmonella 821 Salter-Harris Fracture 1011 Sanders Classification 1003 SAPHO 827 Sarcoid 837 Sarcomatous transformation 817 Sartorius 869 Saucerization (Juxtacortical Chondroma) 762 Saunders 1003 Sausage digit 912 Scaphoid fracture 945 Scapholunate Ligament 946 Disruption 946 Scapular “Y” View 942 Scapular Fractures 942 Schatzker Classification (Tibial Plateau Fractures) 1001 Scheuermann Disease 911 Sclerodactyly 984 Scleroderma 793, 976, 985 Sclerosing osteomyelitis of Garre 825 Sclerosing Osteosarcoma 754 Scoliosis 901, 981 Scurvy 900, 904, 977 Secondary chondromatosis - trauma 799 Secondary Chondrosarcoma 764 Secondary osteoarthritis 826 Segond fracture 807, 1001 Semimembranosus 869, 870 Semitendinosus 869 Senile osteoporosis 977 Septic Arthritis 718, 748, 825 Septic bursitis 826 Septic tenosynovitis 826 sequestra 824, 967 Sequestra-Like Appearance 1005 Sequestrum 820 Seronegative Spondyloarthropathy 1009

Serpentine sclerosis 907 Serpentine vessels 806 Sever 911 Shepherd’s Crook 774 Shiny corner sign 918 Sickle cell anemia 818, 968, 1010 Simple Bone Cyst 786 Sinding-Larsen-Johansson 911 Sinus lesions 745 Sinus Tarsi Syndrome 882 Sinus tracts 822 Skeletal Metastases 961 Skull: beveled edge, button sequestrum 891 SLAC Wrist 874 SLAP Tears 936 SLE 869, 976 SLE (Systemic Lupus Erythematosis) 983 Slipped Epiphyses 715, 826 Small cell carcinoma 964 Smith Fracture (Reverse Colles) 945 Soft Tissue abscess 826 Soft Tissue Chondroma 800 Soft tissue chondrosarcoma 1012 Soft Tissue Ganglion 795 Soft Tissue Hemangioma 805 Soft Tissue infection 825 Soft Tissue Lipomatous Tumors 893 Soft Tissue Masses Differential Diagnosis 804 Soft Tissue Neoplasm 733 Angiomatous lesions 733 Elastofibroma and fibromatosis 733 Lipomatous lesions 733 Neurogenic tumors 733 PVNS and ganglion 733 Soft Tissue Sarcoma Incidence 781 Solitary Focus Bone Scan 964 Souer and Remy 1003 Sphenoid 745 Spina ventosa 830 Spinal cord edema/hematoma 848 Spine Infections 828 Spinoglenoid Notch Entrapment 930 Spirochetes 834 Spondylitis 918 Spondyloarthropathies 912, 916 Spondylodiscitis 828, 830, 1023 Spondylolisithes 845 Spontaneous healing (Osteoid Osteoma) 748 Spontaneous osteonecrosis 865, 910 Sporotrichosis 836 Squamous cell carcinoma 852 Staph aureus 821, 828 Stener Lesion 947 Sterno-clavicular Joint 941 Steroid administration 953 Steroids 869, 907 Steroids (Osteopenia/Osteoporosis) 977 Stewart-Treve syndrome 810 Still Disease 919 Storioform 780 Stress fracture 748 Femur 1027 I 10

Stress Injuries 987 Subacute osteomyelitis 748, 824, 1007, 1026 Subchondral cyst 784, 790, 921, 1008 Subchondral cyst/intraosseous ganglion 763 Subchondral Resorption 713 Subchondral Sclerosis 923 Sublabral Foramen 933 Subligamentous extension 830 Subligamentous/Subtendinous Resorption 713 Subperiosteal (Osteoid Osteoma) 746 Subperiosteal abscess 822, 823 Subperiosteal Resorption 712 Subscapularis (Avulsion) 935 Subscapularis (Disruption) 935 Subscapularis Muscle 932 Subscapularis Tears 929 Subtrochanteric Fractures 1000 Subungual Exostosis 759 Sunburst 967 Super bone scan 718 Superior Glenohumeral Ligament 933 Superolateral migration (Osteoarthritis - Hip) 923 Supinator syndrome (Elbow) 956 Supracondylar fracture 944 Suprapatellar “bursa” 870 Suprascapular Nerve Entrapment 930 Supraspinatus Tendon (Tear) 935 Swan-Neck, Boutonniere deformities 914 Symmetric Polyarthritis 983 Symphysis pubis (Calcification) 951 Synchronous Osteosarcoma 750 Syndesmophyte 918 Synovial Chondroma 792 Synovial Chondromatosis 792, 799, 1006 Synovial Cyst 790, 792 Folds (Elbow) 956 Lipoma 792, 798 Osteochondromatosis 799 Plica 871 Sarcoma 781, 792, 793, 795, 802, 1012 Synovitis (Postoperative - Glenohumeral) 939 Syphilis 834, 953 Syringomyelia 848, 953 Systemic Lupus Erythematosis (SLE) 983, 1016 Tabetic arthropathy 953 Talar Neck Fractures 1002 Talus 995 Tarsal Coalition 880 Tarsal joints 1003 Tarsal Tunnel Syndrome 885 Telangiectasia 984 Telangiectatic Osteosarcoma 751 Tendinitis (Calcific) 952 Tendon Sheath 794 Tennis elbow 956 Tenosynovitis 876 Tenosynovitis (septic) 826 Tetracycline 748 TFCC 872 Thalassemia 968, 970 Thickened trabeculae 815

Thrombocytopenia 809 Thrombocytopenia with Absent Radii (TAR) 968, 972 Thumb Injury 947 Thyroid Cancer 963 Tibial collateral 870 Tibial Plateau Fractures 1001 Tile 995 Tile Classification 995 Tophi 950 Tori 856 Torus/ Buckle fracture 945 Transchondral fracture 879 Transient Osteoporosis 909 Transient Osteoporosis Hip 1015 Transient Regional Osteoporosis 977 Transverse Fractures (Pelvis) 999 Transverse ligament 863 Trap shooter’s shoulder 942 Trauma 839 Trauma (Dysbaric Disorders) 907 Trauma (Pelvis and Lower Extremity) 995 Trauma (Upper Extremity) 941 Traumatic bone “cyst” 850 Traumatic Spondylolisithes 845 Trevor Disease 759, 1020 Triangular Fibrocartilage (Calcification) 951 Triangular Fibrocartilage Complex 875 Triceps Injuries 958 Triquetrum Fracture 947 Triradiate pelvis 901 Trochlea 955 Trochlear sulcus 956 Trolley track sign 918 Tropical ulcer 834 Trough Sign 943 T-score 978 Tuberculosis 829, 973 Tuberculous Arthritis 748, 832 Osteomyelitis 830 Spondylodiscitis 830 Tubulation (Osteochondroma) 758 Tumoral Calcinosis 792, 1012 Turner syndrome 808 UCL and ulnocarpal ligaments 875 Ulcer (tropical) 834 Ulcerative Colitis 912 Ulcers 825 Ulnar fracture 945 nerve 872 Tunnel Syndrome 877 Unicameral bone cyst (UBC) 784, 944 Unilateral Facet Injury 841 Unusual infection 818 Upper Extremity Trauma 941 Van Neck 911 Vanishing Bone Disease 809 Vastus lateralis 869 Vastus medialis 869 Ventral (anterior) SIJ ligament 997 Vertebra Plana 891 I 11

Vertebral scalloping 981 Vertebroplasty 808 Vertical shear (Pelvic Trauma) 996 VISI deformity 875 Vitamin D: Prohormone 900 Volar Plate Avulsion 948 Voorhoeve Disease (Osteopathia Striata) 744 Wall Fracture (Pelvis) 999 West Point View 942 Whipple 912 Whiskering 914 Widened hip joint 826 Wimberger sign 834 Wormian Bones 1007 Wrisberg 859 Wrist 872 Wrist Subluxations 1019 Xenograft 709 X-linked hypophosphatasia 903 Yaws 834 Young-Burgess 995 Young-Burgess Classification 996 Young-Burgess vs Tile Classifications 995 Yttrium 90 795 Zonal pattern 801 Zonal phenomena 801 Z-score 978

I 12

Radiologic Pathology Fifth Edition VOLUME 3 Neuroradiology and Pediatric Radiologic Pathology Correlation

2006 2007

Editors Angela D. Levy, COL, MC, USA Chairman and Registrar Chief, Gastrointestinal Radiology

Ellen M. Chung, LTC, MC, USA Chief, Pediatric Radiology

Jeffrey R. Galvin, MD Chief, Chest Radiology

Kelly K. Koeller, MD Chief, Neuroradiology

Mark D. Murphey, MD Six Week Course Director Chief, Musculoskeletal Radiology

Paula J. Woodward, MD Chief, Genitourinary Radiology

Associate Editor Jean-Claude Kurdziel, MD

Illustrators Aletta A. Frazier, MD Dianne D. Engelby, MAMS, RDMS Heike Blum, MFA

Department of Radiologic Pathology Armed Forces Institute of Pathology Washington DC, USA

American Registry of Pathology Armed Forces Institute of Pathology Washington, DC 20306-6000 _____________________________________ © Copyright 2006 by the American Registry of Pathology. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means: electronic, mechanical, photocopy, recording, or any other information storage and retrieval system without written permission of the publisher. Made in the United States of America _____________________________________ Great care has been taken to guarantee the accuracy of the information contained in this volume. However, neither the American Registry of Pathology, Armed Forces Institute of Pathology, nor the editors and contributors can be held responsible for errors or for any consequences arising from the use of the information contained herein. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official nor as representing the views of the Departments of the Army, Air Force, Navy, or Defense. 987654321 Library of Congress Cataloging-in-publication Data [in process] ISBN 1-933477-00-8

Preface The Armed Forces Institute of Pathology’s Radiologic Pathologic Correlation course presented by the Department of Radiologic Pathology enters its 59th year of educating radiology residents worldwide. For the fifth year, our staff and visiting lecturers have contributed their lecture material and images to compile Radiologic Pathology 2006 – 2007, continuing the tradition of presenting richly illustrated material that teaches the pathologic basis of disease to improve our understanding of the imaging appearance of disease. We hope the efforts of our authors and editors have once again accomplished our goal of bringing the outstanding and unique Radiologic Pathologic Correlation course to your fingertips.

Acknowledgements The annual production of the Radiologic Pathologic Correlation course and syllabus is made possible through the tremendous support, dedication, and selfless service of countless individuals who work in the AFIP and the various institutions and organizations throughout the world that believe in the importance of teaching the principles of disease through radiologic pathologic correlation. The Department of Radiologic Pathology of the Armed Forces Institute of Pathology expresses our deepest appreciation and sincerest gratitude to: - All radiologists and radiology residents who have contributed case material to the Thompson Radiologic Pathologic Archive at the Armed Forces Institute of Pathology, - All pathologists in the AFIP who have donated their time and expertise to radiologic pathologic correlation, - All of our outstanding authors, illustrators, and department staff members who make the course and the syllabus happen effortlessly year after year, - And, to the extraordinary efforts of our production team, headed by JeanClaude Kurdziel, MD, who have tirelessly dedicated the spring and summer of the last five years to the production of this syllabus.

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Faculty – VOLUME 3 Neuroradiology

Pediatric Radiology

Kelly K. Koeller, MD

Ellen M. Chung, LTC, MC, USA

Kelly K. Koeller, MD, FACR Chief, Neuroradiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC and Associate Professor of Radiology Mayo Clinic Rochester, MN

Chief, Pediatric Radiology Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

Dorothy I. Bulas, MD Professor of Radiology and Pediatrics Children's National Medical Center The George Washington University School of Medicine and Health Sciences Washington, DC

Patricia A. Hudgins, MD Professor of Radiology Emory University Medical Center Atlanta, GA

Gael J. Lonergan, MD Chief of Radiology Children's Hospital of Austin Austin, TX

Mary E. (Lee) Jensen, MD Director of Interventional Neuroradiology Professor of Radiology and Neurosurgery University of Virginia Health System Charlottesville, VA

William E. Shiels II, D.O. Chairman, Department of Radiology Children 's Hospital Columbus, OH

Erin Simon Schwartz, MD Assistant Professor of Radiology University of Pennsylvania School of Medicine Pediatric Neuroradiologist The Children's Hospital of Philadelphia Philadelphia, PA

Marilyn J. Siegel, MD Professor of Radiology and Pediatrics Mallinckrodt Institute of Radiology Washington University Medical School St. Louis, MO and Former Distinguished Scientist Department of Radiologic Pathology Armed Forces Institute of Pathology Washington, DC

James G. Smirniotopoulos, MD Professor of Radiology, Neurology, and Biomedical Informatics Chair, Radiology and Radiological Sciences Uniformed Services University of the Health Sciences Bethesda, MD Wendy R. K. Smoker, MS, MD, FACR Professor of Radiology University of Iowa Medical Center Iowa City, IA

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Table of Contents – VOLUME 3 Neuroradiology Kelly K. Koeller, MD, FACR Imaging of Demyelinating Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1037 Lymphoma and Uncommon Neuroepithelial Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1045 Cerebral Intraventricular Neoplasms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1058 Imaging of the Temporal Bone: Anatomy and Congenital Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1068 Imaging of the Temporal Bone: Infectious and Neoplastic Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1076 Imaging of the Orbit: The Globe and Conal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1088 Imaging of the Orbit: Intraconal and Extraconal Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1097

James G. Smirniotopoulos, MD Patterns of Location: Infratentorial and Supratentorial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1106 Patterns of Enhancement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1126 The WHO 2000 Brain Tumor Classification . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1137 Non-Astrocytic Gliomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1149 Extraaxial Tumors: Other Non-Glial Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1158 Neoplasms of the Meninges . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1164 Pinealomas and, other Pineal Region Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1175 The Phakomatoses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1184

Mary E. Jensen, MD Subarachnoid Hemorrhage and Intracranial Aneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1210 Intracranial Vascular Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1220

Patricia A. Hudgins, MD Intracranial Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1231 Paranasal Sinuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1240 Sella and Parasellar Region . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1250

Erin Simon Schwartz, MD Congenital Spinal Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1260

Wendy R. K. Smoker, MS, MD, FACR Imaging of the Suprahyoid Neck: Superficial, Parapharyngeal and Carotid Spaces . . . . . . . . . . . . . . . . .1266 Imaging of the Suprahyoid Neck: Masticator and Parotid Spaces . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1274 Imaging of the Suprahyoid Neck: Pharyngeal Mucosal Space and Oral Cavity . . . . . . . . . . . . . . . . . . . .1282 Spine: Degenerative Disease and Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1290 Spinal Tumors, Cysts, and Mimics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1298 Congenital Abnormalities of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1307

Kelly K. Koeller, MD, FACR Neuroradiology Seminar 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1318 Neuroradiology Seminar 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1322

Pediatric Radiology Ellen M. Chung, LTC, MC, USA Childhood Urinary Tract Infection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1329 Neonatal GI Tract Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1341 Acute GI Disorders of Infants and Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1353 Diseases Affecting The Pediatric Airway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1363 Vascular Rings and Slings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1374 Cystic Renal Disease of Childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1381

Marylin J. Siegel, MD Pediatric Renal Tumors: Infancy and Young Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1390 Pediatric Adrenal Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1402 Pediatric Pelvic Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1414 Bone Marrow Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1425 Congenital Lung Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1435 Lung Diseases in Neonates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1444 Pediatric Cardiac Imaging Part I: Vascular Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1453 Pediatric Cardiac Imaging Part II: Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1463

v

Gael J. Lonergan, MD Congenital Heart Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1472 Forensic Radiology of Child Abuse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1491

Dorothy I. Bulas, MD Neonatal Brain: Radiologic Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1501

William E. Shiels II, DO Pediatric Liver Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1509 Pediatric Hip Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1518

Ellen M. Chung, LTC, MC, USA Pediatric Radiology Seminar I: Pulmonary Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1521 Pediatric Radiology Seminar II: Skeletal Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .1527 Pediatric Radiology Seminar III: Cystic Fibrosis & Pulmonary Infections of Immunocompromised Child .1535

vi

Neuroradiology

Imaging of Demyelinating Diseases Kelly K. Koeller, MD, FACR Imaging Hallmarks •

• • •

White matter location ➢ May involve basal ganglia Little or no mass effect Usually no calcification May or may not enhance

Figure 5-1-1

Normal Lesions [Figure 5-1-1] •

•

Virchow-Robin spaces ➢ Perivascular space of deep penetrating vessels ➢ Follows CSFsignal intensity Ependymitis granularis ➢ Frontal horn

Ependymitis granularis [Figure 5-1-2] • •

• •

Frontal horn “capping” “Swamp” of the brain Perivascular spaces follow CSF signal ➢ Axons with low myelin content on all MR sequences ➢ Interstitial CSF backed up ➢ Loss of ependyma Periventricular hyperintensity: increased in ischemic WM states No lymphatics in the brain

Demyelinating Disorders • • • • •

Multiple sclerosis Vascular Viral / post-viral demyelination Toxic / metabolic encephalopathies Iatrogenic white matter degeneration

Figure 5-1-2

Multiple Sclerosis •

• • •

Unknown etiology ➢ Viral: measles, Epstein-Barr virus (EBV) ➢ Genetic: chromosome 6, human lymphocyte antigens (HLA) loci ➢ Autoimmune: associations with Graves’, myasthenia gravis, ulcerative colitis, Crohn's, SLE Females (60%): especially with optic neuritis 95% cases: 18-50 years old Cooler climates: northern Europe, North America; southern South America

Multiple Sclerosis •

• •

Relapsing-remitting: 70% ➢ Numbness, dysesthesia, burning sensations ➢ 2 clinical attacks from 2 separate lesions ❖ At least 24 hours and at least 30 days apart Ependymitis granularis ➢ Partial or complete remission for months or years Progressive: 20% ➢ Primary progressive: slow onset without distinct attacks ➢ Secondary progressive: relapsing-remitting form with progressive disability “Monosymptomatic demyelinating”: 10%

McDonald et al, Ann Neurol 2001: 50-121-127

Neuroradiology

1037

Imaging of Demyelinating Disease

Multiple Sclerosis - Clinical • • •

Figure 5-1-3

Uthoff's phenomenon: symptoms worsened with exposure to heat Children: very rare especially before puberty Pregnancy ➢ Decreased risk during 2nd and third trimester ? ➢ ...but exacerbation rate of 75% in first 6 months post-partum

Multiple Sclerosis - Pathology • • •

Microglial infiltration Myelin disintegration and Perivascular cuffing of focal hypercellularity lymphocytes --> Predilection for periventricular zone

Active (enhancing) MS plaque compared to chronic (non-enhancing) MS plaque

Multiple Sclerosis - Imaging •

• • •

Periventricular distribution: classic ➢ 3 or more lesions during “event”: sensitive indicator for MS within 10 years Ovoid lesions often perpendicular to walls of ventricles (Dawson's fingers) Little mass effect for size of lesion Corpus callosum-septum pellucidum interface lesions: increase specificity and sensitivity of diagnosis

Figure 5-1-4

Radiology 1991;180:467-474; Radiology 1991; 180:215-221; Neurology 2003; 61:602-611

Multiple Sclerosis - Imaging [Figure 5-1-3] • •

Active plaques enhance (ring-like in some) Chronic lesions: do not enhance

Atrophy in chronic MS

McDonald Criteria - MR •

•

Dissemination in space (3 or more) ➢ 1 enhancing lesion or 9 T2 hyperintense lesions ➢ 1 or more infratentorial lesion ➢ 1 or more juxtacortical lesion ➢ 3 or more periventricular lesions Dissemination in time (at least 1) ➢ MRI more than 3 months after clinial event, enhancing lesion at different site ➢ No enhancing lesion: new T2 lesion or enhancing lesion on f/u study at least 3 months later ➢ MRI less than 3 months, new enhancing lesion on f/u study

Figure 5-1-5

Multiple Sclerosis – MR [Figure 5-1-4] • • •

Chronic cases: atrophy Fluid-attenuated inversion recovery (FLAIR): hyperintense MR Spectroscopy ➢ Decreased N-acetyl aspartate (NAA) in chronic plaques ➢ Increased choline, lipids and lactate


1038

Tumefactive MS plaque. Lack of mass effect and “non-neoplastic’ (NAA > choline) MR spectrogram are important clues to correct diagnosis

Neuroradiology

“Tumefactive” Multiple Sclerosis [Figure 5-1-5] • •

Tumor-like but… Lack of mass effect: most important clue for demyelination

Figure 5-1-6

Multiple Sclerosis – Variant Types [Figure 5-1-6] •

• •

Concentric sclerosis (Balo's) ➢ Alternating bands of myelination and demyelination, often in concentric fashion Acute: (Marburg) ➢ Rapid course ➢ Death in months ➢ Severe axonal loss Neuromyelitis optica (Devic syndrome) ➢ Both visual and spinal cord signs

Multiple Sclerosis vs. Transverse Myelitis • •

Balo concentric sclerosis

[Figure 5-1-7]

•

Spinal cord MS plaques: 7% Multiple sclerosis: peripheral, usually less than two segments, limited to one side ➢ Clinical cord syndrome: 60% had brain lesions Transverse myelitis: usually holocord, commonly involves gray matter

Figure 5-1-7

Tartaglino et al, Radiology. 1995;195:725-32; Tartaglino et al, Radiology. 1996;201:661-9

Vascular White Matter Disease • • • • • • •

Microangiopathy Arteriosclerosis / venous collagenosis Hypoxic-ischemic encephalopathy Posterior Reversible Encephalopathy Syndrome (PRES) Amyloid angiopathy Vasculitis Migraine

Transverse myelitis

Edwards, ed, Neuroimaging clinics 1993

Senescent White Matter Changes [Figure 5-1-8] •

•

•

“Microangiopathy”, “deep white matter ischemia, leukoariosis, etc.” ➢ Demyelination, axonal loss, gliosis, ischemic changes 30%-80% "normal" elderly patients ➢ More lesions, more likely to have neuropsychologic and cognitive problems ➢ ? correlation with dementia ➢ “Binswanger's”: clinical diagnosis, reserved only for dementia cases Do not involve corpus callosum

Figure 5-1-8

Arteriosclerosis •

• •

Long penetrating end arteries ➢ Few or no collateral vessels Pons, thalami, basal ganglia, deep white matter Prevalence increased with age and history of stroke

Venous Collagenosis • • •

Noninflammatory stenosis Occlusion of subependymal veins Associated with periventricular white matter changes

Senescent white matter changes. Note subcortical location

Moody et al, Radiology 1995; 194:469-476 Neuroradiology

1039


Posterior Reversible Encephalopathy Syndrome (PRES) •

Figure 5-1-9

[Figure 5-1-9]

• •

Hypertensive events: renal failure, pre/eclampsia, immunosuppressive drugs Loss of normal autoregulation: elevated hydrostatic pressure mediated by venous vasoconstriction? Posterior cerebral circulation: less sympathetic innervation, less ability to vasoconstrict ➢ Visual field deficits, headache, somnolence ➢ T2 hyperintensity ➢ Diffusion: usually increased (not restricted) ➢ Perfusion (CBV, CBF): decreased, normal, or increased

Casey et al, AJNR 2000; 21:1199-1206 Brubaker et al, AJNR 2005; 26:825-830; Schuuring et al AJNR 2003; 24:2085-2088

Senescent white matter changes. Note subcortical location

Viral and Postviral Demyelination • • • •

Encephalitis Acute disseminated encephalomyelitis Subacute sclerosing panencephalitis Human immunodeficiency virus infection and complications ➢ HIV encephalitis ➢ Progressive multifocal leukoencephalopathy

Acute Disseminated Encephalomyelitis (ADEM) • • • • • •

1-3 weeks post-infection or vaccination Monophasic: rubeola, vaccinia, varicella, mycoplasma, mumps, rubella ➢ No virus or bacteria isolated on autopsy Hemorrhagic type (Hurst variant): rapidly progressive onset Children > adults Good prognosis overall but 10%-20% significant neurological deficit or death Diagnosis of exclusion: long-term follow-up needed to rule out MS

Honkaniemi et al, AJRN 2001; 22:1117-1124; Rosman et al, J Child Neurol. 1997; 12:448-54

Figure 5-1-10

ADEM – Pathology and Imaging • • •

Autoimmune response —-> perivenous demyelination CT: normal or nonspecific hypoattenuation No mass effect

ADEM: MR Findings [Figure 5-1-10] •

• •

Asymmetric WM lesions ➢ Varying in size and number ➢ Little or no mass effect Variable enhancement Optic neuritis, myelitis

Kesselring et al, Brain 1990;113:291-302


1040

Neuroradiology

Progressive Multifocal Leukoencephalopathy (PML) • •

• • •

Figure 5-1-11

Defective cell-mediated immunity Marked decrease in prevalence with highly active anti-retroviral therapy (HAART) ➢ Pre-HAART: 1-7% of AIDS patients; 55-85% cases related to AIDS 5–84 y/o; peak: 6th decade JC virus (papovavirus) reactivation ➢ Affects oligodendrocytes: demyelination Extremely poor prognosis (death in 6 months) if untreated

Baqi et al, AIDS 1997; 11:1526-7

PML – Path and Imaging [Figure 5-1-11] •

• • •

Predominantly parieto-occipital and frontal ➢ Posterior fossa: 1/3 cases ➢ Subcortical white matter Typically no mass effect or enhancement ➢ Enhancement indicative of long-term survival? Characteristic scalloped lateral margin at gray matter-white matter junction May show hemorrhage

Whiteman et al, Radiology 1993; 187:233-240; Thurnher et al AJNR 2001; 22:977-984 PML with relative lack of mass effect and sparing of cortical gray matter

HIV Encephalitis [Figure 5-1-12] • • • • •

Much less common with anti-retroviral therapy Deep white matter and gray matter Psychomotor slowing, mental status changes, memory problems, apathy Direct or indirect infection of oligodendrocytes Demyelination and vacuolation ➢ Axonal loss and microglial nodules

Figure 5-1-12

Thurnher et al AJNR 2001; 22:977-984

HIV Encephalitis • •

•

Imaging often normal early in course Diffuse mild cerebral atrophy ➢ Cortical first, then central Ill-defined patchy areas ➢ Central white matter, basal ganglia, thalamus ➢ Bilaterally symmetric ➢ Usually no necrosis or edema ➢ No enhancement

Thurnher et al AJNR 2001; 22:977-984; Olson et al, Radiology 1988; 169:445-448

HIV encephalitis with characteristic cortical atrophy

Toxic Demyelination • •

• • •

Alcohol Ion balance disorders ➢ Osmotic myelinolysis ➢ Extrapontine myelinolysis Organic toxins (lipophilic solvents) Carbon monoxide poisoning (“interval” form) Drug abuse (poisoned heroin)

Neuroradiology

1041


Alcohol and the Brain [Figure 5-1-13 and 5-1-14] •

• •

Figure 5-1-13

Atrophy ➢ Cerebral hemisphere ➢ Superior vermis Marchiafava-Bignami disease ➢ Corpus callosum demyelination, necrosis ➢ Rare: cortical laminar necrosis Wernicke encephalopathy ➢ Thiamin deficiency ➢ Ophthalmoplegia, ataxia, confusion ➢ Medial thalamic nuclei ➢ Mamillary bodies: atrophy

Arbelaez et al, AJNR 2003; 24:1955-57; Johkura et al, AJNR 2005; 26:670-3; Donnal et al, AJNR 1990; 11:893-894 Marchiafava-Bignami disease with corpus callosal lesions. Note atrophy of cerebral hemisphere and superior cerebellar vermis

Osmotic Myelinolysis [Figure 5-1-15] • •

• • • •

“Central pontine myelinolysis” Variable clinical presentation ➢ Spastic quadraparesis, pseudobulbar palsy Incidence?: 0.16–3.7 % of autopsy cases Rapid osmotic change ➢ Vascular injury in gray matter - white matter apposition regions Demyelination: spares periphery of pons CT: Hypoattenuated

Figure 5-1-14

Ruzek et al, AJNR 2004; 25:210-213

Osmotic Myelinolysis [Figures 5-1-15 and 5-1-16] •

MR: ➢ T1WI: hypointense ➢ T2WI: hyperintense ➢ May return to normal in months to year ➢ Extrapontine (10%): basal ganglia, other sites

Iatrogenic Demyelinating Disorders: Chemotherapy •

Mineralizing microangiopathy ➢ Methotrexate ❖ Periventricular, centrum semiovale ❖ Patients < 5 y/o, meningeal leukemia, high-dose therapy: greatest risk

Wernicke encephalopathy with pathognomonic hyperintensity of both medial thalami

Figure 5-1-16

Davis et al, AJR 1986; 147:587-592; Cajade-Law et al in Neuroimag Clin, Edwards, ed. 1993;3:361-377

Figure 5-1-15

Osmotic (central pontine) myelinolysis on CT and T2 MR images Imaging of Demyelinating Disease

Extrapontine myelinolysis 1042

Neuroradiology

Radiation Injury [Figure 5-1-17] • •

•

Figure 5-1-17

Acute: no imaging findings Early delayed: >2 months after therapy ➢ White matter, basal ganglia, cerebral peduncles Late: ➢ Focal radiation necrosis: > 1 year ➢ Diffuse radiation injury: > 1 year ❖ Geographic pattern: conforms to radiation port ➢ Necrotizing leukoencephalopathy: as early as 3 months posttherapy

Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404

Radiation Necrosis [Figure 5-1-18] • •

• •

Tumor-like Metabolic imaging ➢ Increased activity: high-grade tumors ➢ Normal or decreased activity: radiation necrosis ➢ Less reliable for low-grade tumors MRS: increased lactate and choline in tumors vs. increased lactate in necrosis Diffusion-weighted imaging: ➢ Tumors: usually hypointense ➢ Necrosis: usually hyperintense

Radiation injury to white matter with typical "geographic" pattern of involvement

Rowley and Dillon in Neuroimag Clin, Edwards, ed, 1993;3:379-404

Demyelination Imaging Hallmarks •

• • •

Figure 5-1-18

White matter location ➢ May involve basal ganglia Little or no mass effect Usually no calcification May or may not enhance

Summary •

• •

• • •

Normal ➢ Virchow-Robin spaces: follow CSF ➢ Ependymitis granularis: frontal horn Multiple Sclerosis ➢ Periventricular ➢ Clinical diagnosis Vascular demyelination ➢ Senescent white matter changes ❖ Subcortical, do not involve corpus callosum ➢ Posterior Reversible Encephalopathy Syndrome (PRES) Viral / postviral demyelination ➢ ADEM ➢ PML ➢ HIV encephalitis Toxic / metabolic demyelination ➢ Alcohol ➢ Osmotic myelinolysis: centra pons Iatrogenic demyelination ➢ Chemotherapy and radiation injury

Radiation necrosis mimicking appearance of a glioblastoma multiforme

References 1. Arbelaez A, Pajon A, Castillo M. Acute Marchiafava-Bignami disease: MR findings in two patients. AJNR Am J Neuroradiol 2003; 24:1955-1957. 2. Baqi M, Kucharczyk W, Walmsley SL. Regression of progressive multifocal encephalopathy with highly active antiretroviral therapy. Aids 1997; 11:1526-1527. Neuroradiology

1043


3. Brubaker LM, Smith JK, Lee YZ, Lin W, Castillo M. Hemodynamic and permeability changes in posterior reversible encephalopathy syndrome measured by dynamic susceptibility perfusion-weighted MR imaging. AJNR Am J Neuroradiol 2005; 26:825-830. 4. Casey SO, Sampaio RC, Michel E, Truwit CL. Posterior reversible encephalopathy syndrome: utility of fluidattenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000; 21:1199-1206. 5. Davis PC, Hoffman JC, Jr., Pearl GS, Braun IF. CT evaluation of effects of cranial radiation therapy in children. AJR Am J Roentgenol 1986; 147:587-592. 6. Donnal JF, Heinz ER, Burger PC. MR of reversible thalamic lesions in Wernicke syndrome. AJNR Am J Neuroradiol 1990; 11:893-894; discussion 895-896. 7. Frohman EM, Goodin DS, Calabresi PA, et al. The utility of MRI in suspected MS: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2003; 61:602-611. 8. Gean-Marton AD, Vezina LG, Marton KI, et al. Abnormal corpus callosum: a sensitive and specific indicator of multiple sclerosis. Radiology 1991; 180:215-221. 9. Honkaniemi J, Dastidar P, Kahara V, Haapasalo H. Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol 2001; 22:1117-1124. 10. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. AJNR Am J Neuroradiol 2005; 26:670-673. 11. Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis. Brain 1990; 113 ( Pt 2):291-302. 12. McDonald WI, Compston A, Edan G, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001; 50:121-127. 13. Moody DM, Brown WR, Challa VR, Anderson RL. Periventricular venous collagenosis: association with leukoaraiosis. Radiology 1995; 194:469-476. 14. Nesbit GM, Forbes GS, Scheithauer BW, Okazaki H, Rodriguez M. Multiple sclerosis: histopathologic and MR and/or CT correlation in 37 cases at biopsy and three cases at autopsy. Radiology 1991; 180:467-474. 15. Olsen WL, Longo FM, Mills CM, Norman D. White matter disease in AIDS: findings at MR imaging. Radiology 1988; 169:445-448. 16. Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. J Child Neurol 1997; 12:448-454. 17. Rowley HA, Dillon WP: Iatrogenic white matter diseases. Neuroimaging Clin N Am 3:379–404, 1993 18. Ruzek KA, Campeau NG, Miller GM. Early diagnosis of central pontine myelinolysis with diffusion-weighted imaging. AJNR Am J Neuroradiol 2004; 25:210-213. 19. Schuuring J, Wesseling P, Verrips A. Severe tacrolimus leukoencephalopathy after liver transplantation. AJNR Am J Neuroradiol 2003; 24:2085-2088. 20. Tartaglino LM, Croul SE, Flanders AE, et al. Idiopathic acute transverse myelitis: MR imaging findings. Radiology 1996; 201:661-669. 21. Tartaglino LM, Friedman DP, Flanders AE, Lublin FD, Knobler RL, Liem M. Multiple sclerosis in the spinal cord: MR appearance and correlation with clinical parameters. Radiology 1995; 195:725-732. 22. Thurnher MM, Post MJ, Rieger A, Kleibl-Popov C, Loewe C, Schindler E. Initial and follow-up MR imaging findings in AIDS-related progressive multifocal leukoencephalopathy treated with highly active antiretroviral therapy. AJNR Am J Neuroradiol 2001; 22:977-984. 23. Whiteman ML, Post MJ, Berger JR, Tate LG, Bell MD, Limonte LP. Progressive multifocal leukoencephalopathy in 47 HIV-seropositive patients: neuroimaging with clinical and pathologic correlation. Radiology 1993; 187:233-240.


1044

Neuroradiology

Lymphoma and Uncommon Neuroepithelial Tumors Kelly K. Koeller, MD, FACR CNS Lymphoma •

• •

6.6%-15.4% of all primary brain tumors ➢ Only GBM, meningioma, and low-grade astrocytoma are more common Less than 1% of all body lymphomas Primary lymphoma much more common than secondary

Miller et al, Cancer 1994; 74:1383-1397; Henry et al, Cancer 1974; 34:1293-1302

Clinical •

•

Wide age range ➢ Peak: 4th to 5th decades ➢ Smaller peak: 1st decade (AIDS) Nonspecific clinical presentation ➢ Expanding mass lesion ➢ Encephalitis ➢ Stroke ➢ Cranial nerve palsies

Figure 5-2-1

Koeller et al, Radiographics 1997; 17:1497-1526

Immunocompromised Patients •

• • •

“Opportunistic neoplasm” ➢ Incidence much higher than in immunocompetent patients AIDS-defining diagnosis in HIV+ patients 2% of AIDS patients develop CNS lymphoma CNS mass lesion in AIDS patient ➢ Toxoplasmosis #1, lymphoma #2 ➢ Lymphoma: #1 in pediatric AIDS patient

Rosenblum et al, Ann Neurol 1988: 23:S13-S16; Koeller et al, Radiographics 1997; 17: 1497-1526 •

Two masses, both lymphoma, with one located around the ventricle while the other arises from the leptomeninges

Gross Pathology [Figure 5-2-1] • • •

Intra-axial nodule ➢ Grayish-pink, homogeneous, circumscribed ➢ Multifocal: 50% Leptomeningeal Uveal Intradural spinal

Figure 5-2-2


Histopathology [Figure 5-2-2] • • •

“Small blue cell tumor” Almost always B-cell type Perivascular space


Perivascular distribution of small blue lymphocytes (arrow, vessel) Neuroradiology

1045

Lymphoma & Uncommon Neuroepithelial Tumors

CT / MR Findings [Figures 5-2-3 to 5-2-5] •

• •

Figure 5-2-3

NCCT: Hyperattenuated mass ➢ Negative CT does not exclude diagnosis MR: ➢ T1WI: iso-to-hypointense to gray matter ➢ T2WI: hypointense ➢ Little mass effect for size of lesion Virtually all enhance ➢ Ring-like: necrosis; common in immunocompromised hosts

Lee et al, AJR 1986; 147:747-752; Schwaighofer et al, AJNR 1989; 10:725-729; Dina, Radiology 1991; 179:823-828

Figure 5-2-4 Lymphoma with characteristic CT hyperdensity and T2 hypointensity

Figure 5-2-5

CNS Lymphoma with true water restricted diffusion (DWI hyperintensity, left; ADC hypointensity, right)

General Neuroimaging Features [Figure 5-2-6] •

• •

Supratentorial location ➢ Deep gray matter: “classic”, 33% ➢ Cerebral white matter: 55% ➢ Cerebellar lesions: 10% Multiple lesions: 11%-47% ➢ More common in immunocompromised Recurrences: 50% at original site

Lymphoma in AIDS patient with ring-like enhancement secondary to necrosis


Figure 5-2-6

General Neuroimaging Features • • •

•

“Hugs “ ependyma or leptomeninges “Butterfly” pattern: corpus callosum “Ghost” tumor: vanishes with steroid or radiation therapy ➢ Avoid prior to biopsy Rare: calcification, hemorrhage

Jiddane et al, J Neurosurg 1986; 65:592-599; Vaquero et al, J Neurosurg 1984; 60:174-176

Lymphoma of right basal ganglia

Lymphoma & Uncommon Neuroepithelial Tumors

1046

Neuroradiology

PET / SPECT-Thallium [Figure 5-2-7] • • • •

Figure 5-2-7

Hypermetabolic Non-neoplastic lesions: hypometabolic High specificity and sensitivity False positives: interpretation errors, occasional hypermetabolic abscess

Hoffman et al, J Nucl Med 1993; 34:567575; Villringer et al, J Comput Assist Tomogr 1995; 19:532-536

Neuroepithelial Tumors •

• • • • • • • •

•

Astrocytic ➢ Pleomorphic xanthoastrocytoma Toxoplasmosis or lymphoma ? Oligodendroglial PET image shows hypermetabolic activity consistent with Mixed glial lymphoma Ependymal Choroid plexus Pineal parenchymal Neuroblastic Glial of uncertain origin Neuronal and mixed neuronal-glial ➢ Ganglioglioma / Gangliocytoma ➢ Desmoplastic Infantile Ganglioglioma ➢ Dysplastic cerebellar gangliocytoma ➢ Dysembryoplastic neuroepithelial tumor ➢ Cerebellar Liponeurocytoma Embryonal ➢ Supratentorial PNET ➢ Atypical teratoid / rhabdoid tumor

Ganglioglioma / Gangliocytoma • •

• •

About 1% of all brain tumors Children and young adults ➢ 80% > third or fourth ventricle Arise from arachnoidal cap cells within choroid plexus, tela choroidea, or velum interpositum 0.7% of all meningiomas Almost all are benign ➢ Predilection for sarcomatous change in children

Figure 5-3-15

Neurosurgery 1987; 20:465-468; AJNR 1995; 16:1378-1381; Radiology 1984; 153:435-442

Intraventricular Meningioma •

CT: well-defined globular mass ➢ Hyperattenuated compared to brain ➢ Calcification: 50%

Neurosurgery 1987: 20:465-468; AJNR 1995; 16:1378-1381; Surg Neurol 1994: 42:41-45

Intraventricular Meningioma [Figure 5-3-15] •

• •

MR: iso-to-hypointense compared to gray matter on T1WI ➢ Iso-to-hyperintense on T2WI Heterogeneous enhancement MRS: decreased NAA, creatine ➢ Increased choline

Intraventricular meningioma on contrast-enhanced axial T1weighted MR image

AJNR 1999; 20:882-885; AJNR 1994; 15:435-444

Choroid Plexus Metastasis • •

• •

Rare: 0.9-4.6% of all cerebral metastasis Renal cell carcinoma and lung carcinoma: most common in adults ➢ Children: neuroblastoma, Wilms’ tumor, retinoblastoma ➢ Others: melanoma, gastric carcinoma, colon carcinoma, lymphoma Lateral ventricle: most common Renal cell carcinoma metastasis may mimic meningioma

Figure 5-3-16

South Med J 1998; 91:1159-1162; Neurosurgery 1983; 13:430-434

Choroid Plexus Metastasis • • •

[Figure 5-3-16]

CT: iso- or hyperattenuated MR: hypointense on T1WI, hyperintense on T2WI Intense enhancement usually

Br J Radiol 1994; 67:223-243

Summary •

• •

Fourth Ventricle ➢ Ependymoma ➢ Subependymoma ➢ Choroid Plexus Papilloma Third ventricle ➢ Colloid Cyst ➢ All the others: less common Lateral Ventricle (anterior half) ➢ Subependymoma ➢ Central Neurocytoma ➢ Subependymal Giant Cell Astrocytoma ➢ Ependymoma ➢ Astrocytoma

Neuroradiology

Choroid plexus metastasis from renal cell carcinoma

1065

Cerebral Intraventricular Neoplasms

•

Lateral Ventricle (posterior half) ➢ Choroid Plexus Papilloma / Carcinoma ➢ Meningioma ➢ Metastasis

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20.

21.

22. 23. 24. 25. 26.

Baron Y, Barkovich AJ. MR imaging of tuberous sclerosis in neonates and young infants. AJNR Am J Neuroradiol 1999; 20:907-916. Bolen JW, Jr., Lipper MH, Caccamo D. Intraventricular central neurocytoma: CT and MR findings. J Comput Assist Tomogr 1989; 13:495-497. Chiechi MV, Smirniotopoulos JG, Jones RV. Intracranial subependymomas: CT and MR imaging features in 24 cases. AJR Am J Roentgenol 1995; 165:1245-1250. Coates TL, Hinshaw DB, Jr., Peckman N, et al. Pediatric choroid plexus neoplasms: MR, CT, and pathologic correlation. Radiology 1989; 173:81-88. Darling CF, Byrd SE, Reyes-Mugica M, et al. MR of pediatric intracranial meningiomas. AJNR Am J Neuroradiol 1994; 15:435-444. Ellenbogen RG, Winston KR, Kupsky WJ. Tumors of the choroid plexus in children. Neurosurgery 1989; 25:327335. Furie DM, Provenzale JM. Supratentorial ependymomas and subependymomas: CT and MR appearance. J Comput Assist Tomogr 1995; 19:518-526. Hassoun J, Gambarelli D, Grisoli F, et al. Central neurocytoma. An electron-microscopic study of two cases. Acta Neuropathol (Berl) 1982; 56:151-156. Hassoun J, Soylemezoglu F, Gambarelli D, Figarella-Branger D, von Ammon K, Kleihues P. Central neurocytoma: a synopsis of clinical and histological features. Brain Pathol 1993; 3:297-306. Healey EA, Barnes PD, Kupsky WJ, et al. The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery 1991; 28:666-671; discussion 671-672. Hoeffel C, Boukobza M, Polivka M, et al. MR manifestations of subependymomas. AJNR Am J Neuroradiol 1995; 16:2121-2129. Kahn EA, Luros JT. Hydrocephalus from overproduction of cerebrospinal fluid, and experiences with other parillomas of the choroid plexus. J Neurosurg 1952; 9:59-67. Killebrew K, Krigman M, Mahaley MS, Jr., Scatliff JH. Metastatic renal cell carcinoma mimicking a meningioma. Neurosurgery 1983; 13:430-434. Kleihues P, Cavenee WK eds. World Health organization Classification of Tumours, Pathology & Genetics: Tumours of the Nervous System. IARC, Lyon, France, 2000. Kudo H, Oi S, Tamaki N, Nishida Y, Matsumoto S. Ependymoma diagnosed in the first year of life in Japan in collaboration with the International Society for Pediatric Neurosurgery. Childs Nerv Syst 1990; 6:375-378. Lach B, Scheithauer BW, Gregor A, Wick MR. Colloid cyst of the third ventricle. A comparative immunohistochemical study of neuraxis cysts and choroid plexus epithelium. J Neurosurg 1993; 78:101-111. Lang I, Jackson A, Strang FA. Intraventricular hemorrhage caused by intraventricular meningioma: CT appearance. AJNR Am J Neuroradiol 1995; 16:1378-1381. Lobato RD, Sarabia M, Castro S, et al. Symptomatic subependymoma: report of four new cases studied with computed tomography and review of the literature. Neurosurgery 1986; 19:594-598. Majos C, Cucurella G, Aguilera C, Coll S, Pons LC. Intraventricular meningiomas: MR imaging and MR spectroscopic findings in two cases. AJNR Am J Neuroradiol 1999; 20:882-885. Matsumura A, Ahyai A, Hori A, Schaake T. Intracerebral subependymomas: clinical and neuropathological analyses with special reference to the possible existence of a less benign variant. Acta Neurochir Wien 1989; 96:15–25. McConachie NS, Worthington BS, Cornford EJ, Balsitis M, Kerslake RW, Jaspan T. Review article: computed tomography and magnetic resonance in the diagnosis of intraventricular cerebral masses. Br J Radiol 1994; 67:223-243. McGirr SJ, Ebersold MJ, Scheithauer BW, Quast LM, Shaw EG. Choroid plexus papillomas: long-term follow-up results in a surgically treated series. J Neurosurg 1988; 69:843-849. Menor F, Marti-Bonmati L, Mulas F, Poyatos C, Cortina H. Neuroimaging in tuberous sclerosis: a clinicoradiological evaluation in pediatric patients. Pediatr Radiol 1992; 22:485-489. Morantz RA, Kepes JJ, Batnitzky S, Masterson BJ. Extraspinal ependymomas. Report of three cases. J Neurosurg 1979; 51:383-391. Morrison G, Sobel DF, Kelley WM, Norman D. Intraventricular mass lesions. Radiology 1984; 153:435-442. Palma L, Celli P, Cantore G. Supratentorial ependymomas of the first two decades of life. Long-term follow-up of 20 cases (including two subependymomas). Neurosurgery 1993; 32:169-175.


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27. Pencalet P, Sainte-Rose C, Lellouch-Tubiana A, et al. Papillomas and carcinomas of the choroid plexus in children. J Neurosurg 1998; 88:521-528. 28. Pollack IF, Gerszten PC, Martinez AJ, et al. Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 1995; 37:655-666; discussion 666-657. 29. Raila FA, Bottoms WT, Jr., Fratkin JD. Solitary choroid plexus metastasis from a renal cell carcinoma. South Med J 1998; 91:1159-1162. 30. Rieger E, Binder B, Starz I, Oberbauer R, Ebner F, Urban C. Tuberous sclerosis complex: oligosymptomatic variant associated with subependymal giant-cell astrocytoma. Pediatr Radiol 1991; 21:432. 31. Scheinker IM: Subependymoma: A newly recognized tumor of subependymal derivation. J Neurosurg 1945, 2: 232-240 32. Scheithauer BW. Symptomatic subependymoma. Report of 21 cases with review of the literature. J Neurosurg 1978; 49:689-696. 33. Schiffer D, Chio A, Giordana MT, et al. Histologic prognostic factors in ependymoma. Childs Nerv Syst 1991; 7:177-182. 34. Sgouros S, Carey M, Aluwihare N, Barber P, Jackowski A. Central neurocytoma: a correlative clinicopathologic and radiologic analysis. Surg Neurol 1998; 49:197-204. 35. Sgouros S, Walsh AR, Barber P. Intraventricular malignant meningioma in a 6-year-old child. Surg Neurol 1994; 42:41-45. 36. Shepherd CW, Scheithauer BW, Gomez MR, Altermatt HJ, Katzmann JA. Subependymal giant cell astrocytoma: a clinical, pathological, and flow cytometric study. Neurosurgery 1991; 28:864-868. 37. Shuangshoti S, Roberts MP, Netsky MG. Neuroepithelial (colloid) cyst: pathogenesis and relation to choroid plexus and ependyma. Arch Pathol Lab Med 1965; 80:214-224, 38. Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Schut L. Subependymal giant cell astrocytomas in children. Pediatr Neurosurg 1994; 20:233-239. 39. Spoto GP, Press GA, Hesselink JR, Solomon M. Intracranial ependymoma and subependymoma: MR manifestations. AJNR Am J Neuroradiol 1990; 11:83-91. 40. Strenger SW, Huang YP, Sachdev VP. Malignant meningioma within the third ventricle: a case report. Neurosurgery 1987; 20:465-468. 41. Swartz JD, Zimmerman RA, Bilaniuk LT. Computed tomography of intracranial ependymomas. Radiology 1982; 143:97-101. 42. Waggenspack GA,Guinto FC Jr. MR and CT of masses of the anterosuperior third ventricle. AJNR, 1989; 10:105110 43. Wichmann W, Schubiger O, von Deimling A, Schenker C, Valavanis A. Neuroradiology of central neurocytoma. Neuroradiology 1991; 33:143-148. 44. Yamasaki T, Kikuchi H, Higashi T, Yamabe H, Moritake K. Two surgically cured cases of subependymoma with emphasis on magnetic resonance imaging. Surg Neurol 1990; 33:329-335.

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Imaging of the Temporal Bone: Anatomy and Congenital Lesions Kelly K. Koeller, MD, FACR Middle Ear •

•

Epitympanum ➢ Malleus head ➢ Short process of incus Mesotympanum ➢ Muscles: tensor tympani (V3), stapedius (VII) ➢ Ossicles: rest of malleus and incus, stapes ➢ Ligaments ➢ Nerves: chorda tympani (VII), Jacobson’s nerve (IX)

Inner Ear: Cochlea • • • • • •

Anterior to vestibule Promontory: bony ridge Modiolus: central axis, cochlear nerve Apex (cupola) Basal turn Cochlear aqueduct: perilymphatic duct

Inner Ear: Vestibule • • • •

Posterior to cochlea Oval window niche: partition from middle ear Lamina cribrosa: partition from internal auditory canal (IAC) Vestibular aqueduct: endolymphatic duct

Inner Ear: Semicircular Canals (SCC) [Figures 5-4-1 to 5-4-15] • • • • •

Lateral (horizontal) Superior: arcuate eminence Posterior Oriented ≈ 90° to each other Rotational acceleration

Figure 5-4-1

Axial CT at superior portion of temporal bone shows lumen of superior semicircular canal and upper portion of mastoid antrum and epitympanum

Figure 5-4-2

Subarcuate artery canal traversing through “hoop” of superior semicircular canal

Temporal Bone Anatomy and Congenital Lesions

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Figure 5-4-3 Superior portion of vestibule with malleus (anterior) and incus (posterior) within the epitympanum

Figure 5-4-4 Superior portion of internal auditory canal with labyrinthine canal containing the facial nerve on its way to the geniculate ganglion and tympanic facial segment along medial wall of middle ear

Figure 5-4-5 Superior portion of cochlea and internal auditory canal. Note vestibular aqueduct arising from posterior margin of temporal bone

Mid-portion of internal auditory canal and cochlea. Sinus tympani is located just lateral to vestibule. Small bony peak lateral to vestibule is pyramidal eminence. Facial nerve canal is located posterolateral to pyramidal eminence. Middle ear shows “parallel lines sign”: tensor tympani tendon anteriorly and incudostapedial junction with stapedial struts posteriorly. Struts mark the site of the oval window

Figure 5-4-6

Figure 5-4-7 Inferior portion of cochlea. Bony plate separating it from the middle ear is the cochlear promontory. Note jugular bulb in posterior temporal bone

Figure 5-4-8

Neuroradiology

Basilar turn of cochlea. Internal carotid artery is seen anterior to the cochlea and is delimited from the middle ear by a bony plate. Eustachian tube arises just lateral to the artery and heads along an anteromedial pathway towards the nasopharynx

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Coronal view shows internal carotid artery inferiorly separated from middle ear by bony plate. Cochlea is located immediately superior. Geniculate ganglion is located just superolateral to cochlea. Middle ear contains ossicles (malleus anterior to incus) within epitympanum. Tegmen tympani is bony plate separating middle ear from brain

Figure 5-4-9

Figure 5-4-10 Moving posteriorly, facial nerve segments are seen above and lateral to cochlea

Figure 5-4-11 Cochlea is separated by cochlear promontory from middle ear. Anterior portion of internal auditory canal is just coming into view. Scutum is well seen along superior margin of medial external auditory canal

Figure 5-4-12

Mid-portion of internal auditory canal with crista falciformis along its lateral margin. Vestibule now appears with lateral and superior semicircular canals. Facial nerve is located immediately inferior to lateral semicircular canal and above oval window

Figure 5-4-13 Posterior margin of vestibule with facial nerve as small soft tissue just prior to reaching posterior genu

Figure 5-4-14

Facial nerve at posterior genu


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Figure 5-4-15 Mastoid segment of facial nerve extending inferiorly to the stylomastoid foramen

Congenital Anomalies • • • •

• •

Outer ear Middle ear Inner ear Vascular ➢ Internal carotid artery ➢ Jugular vein Cholesteatoma Encephalocele

Congenital Malformations • • •

External and middle ear (1st and 2nd branchial arch) develop independent of inner ear (ectodermal) IAC development separate from inner ear development Anomalies of all 3 parts are rare ➢ Dysplasias and trisomies 13, 18, 21

Fisher and Curtin, Otolaryngol Clin North Am 1994; 27:511-531

Outer Ear Anomalies • • • •

Congenital aural dysplasia Bilateral: 33% Genetic disorder association External auditory canal (EAC) atresia: failure of recanalization (26th gestational week) ➢ Fibrous vs. bony plate ➢ CT: middle ear dysplasia or cholesteatoma

Figure 5-4-16

Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al, AJNR 1997; 18:53-65

Middle Ear Anomalies • • •

Temporomandibular joint (TMJ) anomalies TMJ higher and more posterior than normal Facial nerve displaced ➢ Vertical portion more anterior than normal ➢ Very important pre-operative finding

Robson et al, Neuroimag Clin North Am 1999; 9:133-135; Mayer et al, AJNR 1997; 18:53-65

Inner Ear Anomalies [Figure 5-4-16] •

Lateral semicircular canal anomaly ➢ Last semicircular canal to form ➢ Usually short and wide, less commonly narrow

Anomalous shortening and widening of lateral semicircular canal

Jackler and Luxford, Laryngoscope 1987; 97:2-14

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Complete Labrynthine Aplasia • • • •

Figure 5-4-17

Michel’s deformity 3rd gestational week Inner ear absent Small cystic cavity: single or multiple

Jackler and Luxford, Laryngoscope 1987; 97:2-14

Incomplete Partition / Dilatational Defects [Figure 5-4-17] • • • • • •

Mondini’s dysplasia (1791): cochlea with 1 and 1/2 turns Second most common form of congenital deafness (Schiebe’s deafness #1) 7th gestational week Small cochlea with incomplete or absent intrascalar septum Basilar turn present Common cavity in place of middle and apical turns

Paparella, Ann Otol Rhinol Laryngol Suppl 1980; 89(2 Pt 3):1-10

Other Cochlear Anomalies [Figure 5-4-18] •

• •

Common cavity ➢ 4th gestational week

Mondini dysplasia

➢ Cochlea and vestibule fused ➢ 25% of all cochlear anomalies Cochlear aplasia ➢ 5th gestational week ➢ Rest of inner ear normal or malformed Cochlear Hypoplasia ➢ 6th gestational week

Figure 5-4-18

➢ Small cochlear bud Jackler and Luxford, Laryngoscope 1987; 97:2-14

Large Endolymphatic Duct and Sac (LEDS) • •

Large vestibular aqueduct syndrome Dilated vestibular aqueduct ➢ Most common radiologic finding in early-onset SNHL ❖ > 1.5 mm diameter (lateral SCC) ❖ MR: look at T2W images ➢ Often associated with incomplete partition cochlear anomalies ➢ Progressive sensorineural hearing loss (SNHL) ➢ Etiology: hyperosmolar protein transmission?

Valvassori and Clemis, Laryngoscope 1978; 88:723-728; Mafee, AJNR 1992;13:805-819; Jackler and De la Cruz, Laryngoscope 1989; 99:1238-1243; Dahlen et al, AJNR 1997; 18:67-75; Davidson et al, AJNR 1999; 20:1435-1441

Inner Ear Anomalies: Associations •

• •

Otocraniofacial ➢ Crouzon’s, Apert’s, etc. Otocervical ➢ Klippel-Feil, Goldenhar’s, etc. Otoskeletal ➢ Osteogenesis imperfecta, osteopetrosis, etc.

Enlarged vestibular aqueduct. Note size in comparison to lateral semicircular canal

Romo, Casselman, and Robson in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003


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Aberrant Internal Carotid Artery [Figure 5-4-19] • • • •

Figure 5-4-19

90% females More common on right side Pulsatile tinnitus, conductive hearing loss (HL), otalgia Enhancing mass in hypotympanum (inferior tympanic canaliculus)

Sinnreich et al, Otolaryngol Head Neck Surg 1984; 92:194-206; Thiers et al, AJNR 2000; 21:1551-1554

Absent Internal Carotid Artery • • •

May be incidental discovery High association with intracranial aneurysms Nearly 30% present with subarachnoid hemorrhage

Aberrant internal carotid artery with soft tissue attenuation within middle ear and absent bony margin (Case courtesy of Wendy Smoker, MD, FACR)

Keen, Clin Proc 1946; 4:588-594; Martinez-Granero et al, Rev Neurol 1997; 25:1207-1209

Persistent Stapedial Artery • • • • • •

Figure 5-4-20

Rare: most seen at surgery Precursor for middle meningeal artery Small canal from carotid canal Crosses cochlear promontory Widened facial canal Absence of foramen spinosum

Thiers et al, AJNR 2000; 21:1551-1554

High Jugular Bulb (“Megabulb”) [Figure 5-4-20] • •

• • • •

Various definitions described Most common vascular anomaly of petrous temporal bone ➢ 3%–7% incidence More common on right side ➢ 75% jugular vein larger on right Usually poorly pneumatized mastoids No bony dehiscence Importance: surgical impact

Overton and Ritter, Laryngoscope 1973; 83: 1986-1991; Caldemyer et al, RadioGraphics 1997; 17:1123-1139 High jugular bulb with dehiscence along internal auditory canal

Other Jugular Vein Anomalies •

•

Dehiscent jugular bulb ➢ Direct communication with middle ear ➢ Lateral: pulsatile tinnitus, conductive hearing loss ➢ Medial: Meniere’s disease Jugular diverticulum ➢ Above, medial, posterior to petrous pyramid ➢ More common on left-side and in females

Figure 5-4-21

Couloigner et al, Eur Arch Otorhinolaryngol 1999; 256:224-229; Pappas et al, Otolaryngol Head Neck Surg, 1993; 109:847-852

Pial siderosis with thin bands of T2 hypointensity secondary to subarachnoid hemorrhage Neuroradiology

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Pulsatile Tinnitus Lesions •

Figure 5-4-22

[Figures 5-4-21 and 5-4-22]

• •

Congenital ➢ Aberrant internal carotid artery ➢ Dehiscent jugular bulb Tumor ➢ Paraganglioma ➢ Hemangioma Vascular ➢ Arteriovenous malformation / fistula ➢ Aneurysm ➢ Pial siderosis: VIII n. especially prone

Congenital Cholesteatoma (Epidermoid) • • •

Abnormal flow voids secondary to arteriovenous fistula

[Figures 5-4-23 and 5-4-24]

• • •

Child with conductive HL Aberrant epithelial rests Epitympanum, incudostapedial joint > petrous apex Globular mass +/– bone destruction Follows fluid signal intensity May have peripheral enhancement

Figure 5-4-23

Figure 5-4-24

Peron and Schuknecht, Arch Otolaryngol 1975: 101:498-505; Gao et al, AJNR 1992; 13:863-872

Congenital Dehiscence of Tegmen Tympani [Figure 5-4-25] •

Fusion of petrosquamosal suture by 1 year of age • Tiny openings: up to 34% of population ➢ Encephalocele, fistula: rare (requires dural weakening) Ossicular epidermoid with • Coronal plane best bone erosion Gavilan et al, Arch Otolaryngol 1984; 110-206-207; Gottlieb et al, Arch Otolaryngol 1998; 124:1274-1277

Contrast-enhanced coronal T1-weighted image shows rim enhancement of epidermoid involving right temporal bone

Figure 5-4-25

Axial T2-weighted image shows focal hyperintensity in region of epitympanum. Coronal CT images show soft-tissue density in epitympanum. Surgical exploration confirmed encephalocele Temporal Bone Anatomy and Congenital Lesions

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References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23.

Caldemeyer KS, Mathews VP, Azzarelli B, Smith RR. The jugular foramen: a review of anatomy, masses, and imaging characteristics. Radiographics 1997; 17:1123-1139. Couloigner V, Grayeli AB, Bouccara D, Julien N, Sterkers O. Surgical treatment of the high jugular bulb in patients with Meniere's disease and pulsatile tinnitus. Eur Arch Otorhinolaryngol 1999; 256:224-229. Dahlen RT, Harnsberger HR, Gray SD, et al. Overlapping thin-section fast spin-echo MR of the large vestibular aqueduct syndrome. AJNR Am J Neuroradiol 1997; 18:67-75. Davidson HC, Harnsberger HR, Lemmerling MM, et al. MR evaluation of vestibulocochlear anomalies associated with large endolymphatic duct and sac. AJNR Am J Neuroradiol 1999; 20:1435-1441. Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation. Epidermoid tumor of the cerebellopontine angle. AJNR Am J Neuroradiol 1992; 13:863-872. Gavilan J, Trujillo M, Gavilan C. Spontaneous encephalocele of the middle ear. Arch Otolaryngol 1984; 110:206207. Gottlieb MB, Blaugrund JE, Niparko JK. Imaging quiz case 1. Tegmental encephalocele. Arch Otolaryngol Head Neck Surg. 1998 Nov;124(11):1274, 1276-7. Jackler RK, De La Cruz A. The large vestibular aqueduct syndrome. Laryngoscope 1989; 99:1238-1242; discussion 1242-1233. Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987; 97:2-14. Keen JA. Absence of both internal carotid arteries. Clin Proc 1945-1946;4:588 Mafee MF, Charletta D, Kumar A, Belmont H. Large vestibular aqueduct and congenital sensorineural hearing loss. AJNR Am J Neuroradiol 1992; 13:805-819. Martinez-Granero MA, Martinez-Bermejo A, Arcas J, et al. [Unilateral agenesis of the internal carotid artery in childhood: description of a case]. Rev Neurol 1997; 25:1207-1209. Mayer TE, Brueckmann H, Siegert R, Witt A, Weerda H. High-resolution CT of the temporal bone in dysplasia of the auricle and external auditory canal. AJNR Am J Neuroradiol 1997; 18:53-65. Overton SB, Ritter FN. A high placed jugular bulb in the middle ear: a clinical and temporal bone study. Laryngoscope 1973; 83:1986-1991. Paparella MM. Mondini's deafness. A review of histopathology. Ann Otol Rhinol Laryngol Suppl 1980; 89:1-10. Pappas DG, Jr., Hoffman RA, Cohen NL, Holliday RA, Pappas DG, Sr. Petrous jugular malposition (diverticulum). Otolaryngol Head Neck Surg 1993; 109:847-852. Peron DL, Schuknecht HF. Congenital cholesteatomata with other anomalies. Arch Otolaryngol 1975; 101:498505. Robson CD, Robertson RL, Barnes PD. Imaging of pediatric temporal bone abnormalities. Neuroimaging Clin N Am 1999; 9:133-155. Romo LV, Casselman JW, Robson CD. Temporal Bone: Congenital Anomalies. In: Som P.M., Curtin H.D. (eds) Head and Neck Imaging, 4th edn. Mosby-Year Book Inc., St. Louis, 2003, pp: 1275-1360. Sinnreich AI, Parisier SC, Cohen NL, Berreby M. Arterial malformations of the middle ear. Otolaryngol Head Neck Surg 1984; 92:194-206. Smith ME, Fisher C, Weiss SW. Pleomorphic hyalinizing angiectatic tumor of soft parts. A low-grade neoplasm resembling neurilemoma. Am J Surg Pathol 1996; 20:21-29. Thiers FA, Sakai O, Poe DS, Curtin HD. Persistent stapedial artery: CT findings. AJNR Am J Neuroradiol 2000; 21:1551-1554. Valvassori GE, Clemis JD. The large vestibular aqueduct syndrome. Laryngoscope 1978; 88:723-728.

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Imaging of the Temporal Bone: Infectious and Neoplastic Lesions Kelly K. Koeller, MD, FACR Figure 5-5-1

Conductive Hearing Loss • •

Ossicular motion impeded Differential Diagnosis ➢ Cholesteatoma ➢ Hemangioma ➢ Glomus tympanicum ➢ Trauma: disruption ➢ Congenital ➢ Otosclerosis

Inflammatory Disease •

•

Mechanism ➢ Eustachian tube dysfunction ➢ Decreased intratympanic pressure ➢ Children: otitis media ➢ Adults: nasopharyngeal carcinoma Thin-section CT: soft tissue and fluid look alike ➢ Hounsfield units not helpful

Nemzek and Schwartz in Som and Curtin, Head and Neck Imaging, 4th ed, 2003, Mosby

Acquired Cholesteatoma • •

• •

[Figures 5-5-1 to 5-5-5]

Exfoliated keratin within sac of stratified squamous epithelium 98% of middle ear cholesteatomas ➢ Probably from retraction pocket in pars flaccida Prussak’s space: ossicles displaced medially Bone destruction ➢ Scutum and ossicles:coronal plane best ➢ Pars tensa: lateral semicircular canal, axial plane best

Artist rendition of retraction pocket at superior tympanic annulus caused by negative intratympanic pressure

Figure 5-5-2

Buckingham and Valvassori, Otolaryngol Clin North Am 1973; 6:363

Figure 5-5-3

Retraction pocket fills with epithelial debris from external auditory canal, creating a cholesteatoma Gross photograph of an acquired cholesteatoma, a sac of keratin lined with squamous epithelium

Temporal Bone Infectious and Neoplastic Lesions

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Acquired Cholesteatoma • • • •

Figure 5-5-4

May not be able to distinguish from simple debris early in course MR: T1 & T2 prolongation Does not enhance (granulation tissue does) Treatment: excision or exteriorization ➢ Open cavity (canal wall down) mastoidectomy ❖ Radical: stapes left ❖ Modified radical (Bondy): all ossicles left

Phelps and Lloyd, Radiology 1986; 37:359-364; O’Donoghue et al, Clin Otolaryngol 1987; 12:89; Ishii et al, JCAT 1991; 15:934-937; Nemzek and Schwartz in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003 Coronal CT image of acquired cholesteatoma with erosion of the scutum and ossicles

Acquired Cholesteatoma: Complications •

[Figure 5-5-6]

• •

Labyrinthine fistula: lateral SCC most common ➢ Labyrinthitis Facial nerve canal ∗Tegmen tympani: intracranial

Figure 5-5-5

• ∗Sigmoid sinus erosion / thrombosis • “Automastoidectomy”: into EAC ∗MR recommended

Silver et al, Radiology 1987; 164:47; Schwartz, Radiology 1984; 153:443-447; Nemzek and Schwartz in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003

Middle ear “soft tissue” without bone erosion (not cholesteatoma!). Left: granulation tissue. Right: middle ear fluid

Figure 5-5-6

Cholesteatoma with erosion of mastoid bone adjacent to sigmoid dural sinus

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Mastoiditis •

• •

Figure 5-5-7

[Figures 5-5-7 and 5-5-8]

Osteomyelitis: patchy opacification ➢ Loss of mastoid septations ➢ Demineralization Coalescent: single cystic cavity Complications ➢ Bezold abscess: zygomatic root, EAC ➢ Gradenigo syndrome: petrous apicitis ❖ VI palsy, V neuralgia, chronic otitis ➢ Sigmoid sinus thrombosis ➢ Meningitis, epidural abscess

Castillo et al, AJR 1998; 17:1491-1495; Mafee et al, Radiology 1985; 155:391

Necrotizing External Otitis

• • • • • •

Osteomyelitis: bone destruction Diabetics: Pseudomonas AIDS: Aspergillus Cartilage portion: fissures of Santorini ➢ Spreads rapidly into adjacent spaces ➢ Parotid, facial nerve, intracranial Goal: determine extent of disease by CT and MR In-111 WBC study: post-therapy

Coalescent mastoiditis. Only a single cavity remains within the mastoid bone as a result of osteomyelitis

Slattery and Brackmann, Otolaryngol Clin North Am 1996; 29:795-806; Ress et al, Laryngoscope 1997; 107:456-460; Grandis et al, Radiology 1995: 196:499-504

External Ear Masses • • • •

Figure 5-5-8

[Figure 5-5-9]

Exostosis: chronic cold water exposure Usually broad-based and bilateral Bony portion of EAC Not an osteoma ➢ Usually unilateral, pedunculated, and lateral to EAC

DiBartolomeo, Ann Otolaryngol 1979; 88(suppl 61):2-20; Turetsky et al, AJNR 1990; 11:1217-1218

External Ear Masses •

•

Keratosis obturans ➢ < 40 years old ➢ Sinusitis, bronchiectasis ➢ Hearing loss ➢ Smooth external auditory canal (EAC) widening ➢ Entire EAC often filled EAC cholesteatoma: 0.5% of all cholesteatomas ➢ Otorrhea ➢ Focal erosions

Mastoiditis with posterior fossa epidural abscess (Case courtesy of Vanessa Albernaz, MD)

Figure 5-5-9

Piepergerdes et al, Laryngoscope 1980; 90:383-391

Bilateral exostoses Temporal Bone Infectious and Neoplastic Lesions

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External Ear Neoplasms [Figure 5-5-10] •

• • •

Figure 5-5-10

Skin cancers ➢ Squamous cell carcinoma ❖ Most common malignant ear tumor ➢ Basal cell carcinoma ➢ Melanoma “Ceruminoma” Parotid tumors Metastasis

Schuknecht, Pathology of the Ear, Harvard, 1974; Maya et al in Som and Curtin, Head Neck Imaging, 4th ed, Mosby, 2003

Cerebellopontine Angle Masses: The “AMEN” Differential Diagnosis • • • • •

Acoustic schwannoma (60–91%) Meningioma (3%-7%) Epidermoid (2%-6%) Nonacoustic schwannoma (1%-5%): V, VII Others ➢ Ependymoma, medulloblastoma, pilocytic astrocytoma ➢ Paraganglioma ➢ Arachnoid cyst ➢ Lipoma, dermoid, teratoma

External auditory canal erosion secondary to neoplasm

Brackmann and Bartels, Otolaryngol Head Neck Surg 1980; 88:555-559; Valavanis et al, Clinical Imaging of the Cerebellopontine Angle, Springer-Verlag, 1980; Gonzalez-Revilla, Johns Hopkins Hosp Bull 1948(83):187-189

Figure 5-5-11

Acoustic Schwannoma [Figure 5-5-11] • • • •

8%-10% of intracranial tumors 60%-90% of CPA tumors Most: 30-70 years old Neurofibromatosis type 2 (NF2): children, bilateral in 96% ➢ Schwann cell tumors, multiple meningiomas, gliomas ➢ First-degree relative counseling +/- imaging screening

Kasantikul et al, J Neurosurg 1980; 52:28-35; Martuza and Eldridge, N Engl J Med 1988; 318:684-688; Kishore and O’Reilly, Clin Otolaryngol 2000; 25:561-565

Canalicular vestibular schwannoma with smooth remodeling of the canal wall and loss of crista falciformis

Acoustic (Vestibular) Schwannoma [Figure 5-5-12] •

• • •

Sensorineural hearing loss, vertigo, tinnitus ➢ Speech discrimination impaired: telephone use Arise from vestibular division CN VIII usually ➢ Direct pressure on cochlear division Benign neoplasm ➢ Slow growth (0.2 cm per year) ➢ Well circumscribed globular mass Histology: Antoni A and B fibers [Figure 5-12-8]

Figure 5-5-12

Komatsuzaki and Tsunoda, J Laryngol Otol 2001; 115:376-379; NIH Consensus Development Conference, Arch Neurol 1994; 51:201-207 ; Lanser et al, Otolaryngol Clin North Am 1992; 25:499-520

Antoni A (left) and Antoni B (right) cell populations of a schwannoma Neuroradiology

1079


Vestibular Schwannoma: Imaging •

Figure 5-5-13

[Figures 5-5-13 to 5-5-16]

• • • •

IAC widening with “mushroom expansion ➢ “Giant”: usually no IAC involvement CT: usually isodense to cerebellum ➢ Calcification and hemorrhage rare T1WI: iso- to hypointense T2WI: hyperintense Intense enhancement: into porus acousticus and no dural tail

Maya et al in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003; Moller et al, Neuroradiology 1978; 17:25-30; Tali et al, AJNR 1993; 14:1241-1247; Schmalbrock et al, AJNR 1999; 20:1207-1213 Vestibular schwannoma with classic mushroom morphology on pre-contrast and post-contrast axial T1-weighted images

Figure 5-5-14

Figure 5-5-15

Coronal T2-weighted FSE image of right-sided vestibular schwannoma

Figure 5-5-16 Focal enhancement of deep portion of internal auditory canal secondary to arteriovenous malformation (not vestibular schwannoma)

Cystic degeneration of large vestibular schwannoma


1080

Neuroradiology

Vestibular Schwannoma: Therapy •

• •

Figure 5-5-17

Surgical resection ➢ Larger masses ❖ Translabyrinthine: protect facial nerve ➢ Smaller masses ❖ Retrosigmoid: suboccipital approach ❖ Middle cranial fossa Stereotactic radiosurgery (gamma knife) up to 4 cm Poor surgical risk patients: serial MR

Jackler and Pitts, Otolaryngol Clin North Am 1992; 25:361-387; House and Shelton, Otolaryngol Clin North Am 1992; 25:347-359; Fucci et al, Am J Otol 1999; 20:497-508; Nakamura et al, AJNR 2000; 21:1540-1546

Meningioma •

• • • •

[Figures 5-5-17 and 5-5-18]

Usually eccentric to porus acousticus ➢ IAC involvement uncommon (16%) ➢ Frequently “trans-spatial” Broad dural base: hemispherical ➢ Obtuse bone-tumor angle ➢ Dural tail: 52%-72% ➢ Hyperostosis: highly characteristic NCCT:usually hyperdense (calcification: 25%) T1WI: isointense to gray matter T2WI: variable

Tentorial meningioma with extension into cerebellopontine angle

Figure 5-5-18

House and O’Conner, Handbook of Neurotological Diagnosis, Marcel-Dekker, 1987; Valavanis et al, Neuroradiology 1981; 22:111-121; Moller et al; Neuroradiology 1978; 17:25-30

Epidermoid • • • • • •

[Figure 5-5-19]

Soft, “pearly tumor” Irregular margins: “cauliflower” Follows CSF density and signal Usually no enhancement Diffusion-weighted imaging: hyperintense to CSF Differential Diagnosis: arachnoid cyst, cysticercosis, atypical dermoid, lipoma Cerebellopontine angle meningioma with numerous flow voids and fluid-fluid level. Note extension through foramina

Berger and Wilson. J Neurosurg 1985; 62:214-219; Gao et al, AJNR 1992; 13:863-872; Tampieri et al, AJNR 1989; 10:351-356; Tsuruda et al, AJR 1990; 155:1059-1065

Facial Nerve Palsy • •

•

Figure 5-5-19

MR: imaging study of choice Bell’s palsy: > 50%, nerve not enlarged ➢ Idiopathic, by definition (probably HSV) ➢ Imaging usually not performed Tumors: 6%, enlarged nerve ➢ Geniculate ganglion ❖ Schwannoma ❖ Hemangioma ➢ Epidermoid ➢ Parotid tumor spread

Tien et al, AJNR 1990; 11:735-741; Daniels et al, Radiology 1989; 17:807-809 Epidermoid of middle cranial fossa with extension into posterior fossa Neuroradiology

1081


Temporal Bone Paragangliomas •

• • •

Figure 5-5-20

Glomus jugulare: jugular foramen ➢ Jacobson’s (IX) and Arnold’s (X) nerve Glomus tympanicum: cochlear promontory ➢ Most common middle ear tumor ➢ Most common etiology of retrotympanic vascular mass Vagal paraganglioma: jugular ganglion Females 5:1; peak age: 40-60 years old

Rao et al, RadioGraphics 1999; 19:1605-1632

Jugulotympanic Paraganglioma [Figure 5-5-20] •

• •

• •

Neuroendocrine tumor ➢ Paraganglia: chemoreceptor function ➢ Functioning: 1%-3%, catecholamine secretion Early symptoms ➢ Conductive HL, pulsatile tinnitus Slow growth but locally invasive ➢ Mortality rate: 15% Metastasis very rare Path: chief cells (“Zellballen”), sustentacular cells “Zellballen” histologic appearance of paraganglioma


Figure 5-5-21

Jugulotympanic Paraganglioma: Imaging • • •

[Figures 5-5-21 to 5-5-23]

• • •

CT: irregular margins, “moth-eaten” erosion Glomus jugulare: may extend down carotid sheath MR: “Salt and pepper” appearance ➢ “Salt”: hyperintense foci (slow flow, hemorrhage) ➢ “Pepper”: serpentine flow voids Intense enhancement Angiography: ascending pharyngeal artery Radiologist’s goal: define extent


Figure 5-5-22

Glomus tympanicum (paraganglioma) (Case courtesy of William Kelly, MD)

Glomus jugulotympanicum (paraganglioma) Temporal Bone Infectious and Neoplastic Lesions

1082

Neuroradiology

Figure 5-5-23

“Salt-and-pepper” appearance of glomus jugulare on MR images

Jugular Foramen Masses • • • •

Paraganglioma: 90% Schwannoma: 9% Meningioma: 90% of cases calcification on CT ❖ Child < 3 y/o: highly suggestive for diagnosis Tri- / tetralateral retinoblastoma with pineal and/or suprasellar masses MR: not as specific as CT ➢ Hyperintense on T1WI and PD ➢ Hypointense on T2WI ➢ May miss lesions as large as 4mm ➢ Better for intracranial extension, extraocular spread

Char, Ophthalmology 1984; 91:1347-1350; Mafee, Radiol Clin North Am 1987; 25:667-681; Mafee et al, Ophthalmology 1989; 96:965-976

Persistent Hyperplastic Primary Vitreous (PHPV) [Figure 5-6-13] • •

• •

Failure of embryonic hyaloid system (primary vitreous) to regress normally and form the secondary vitreous by 5th-6th gestational month Isolated or part of more complex abnormality ➢ Bilateral: Norrie’s, Warburg’s Usually unilateral leukokoria and microphthalmos ➢ Lens opacity, RD, vitreous hemorrhage Persistent hyaloid (Cloquet’s) canal ➢ No calcification

Mafee and Goldberg, Radiol Clin North Am 1987; 25:683-692 Neuroradiology

1089 1091

Imaging of the Orbit: The Globe and Conal Lesions

Retinopathy of prematurity

Figure 5-6-13

Coats Disease [Figure 5-6-14] • • •

•

Juvenile males most common Usually unilateral (85%-90%) Peripheral telangiectasias ➢ Leak lipoproteinaceous exudate ➢ Retinal detachment May mimic retinoblastoma clinically

Coats, R Lond Ophthalmol Hosp Rep 1908; 17:440525; Reese, Am J Ophthalmol 1956; 42:1-8; Edward et al, Radiol Clin North Am 1988; 36: 1119-1131

Toxocariasis • • •

•

Chorioretinitis: Toxocara canis (nematode) Granuloma: eosinophilic abscess Persistent hyperplastic primary vitreous (PHPV) CT: homogeneous intravitreal density with hyaloid canal ➢ Retinal detachment, organized vitreous, inflammatory exudate ➢ Irregular thickening of uveoscleral coat MR: subretinal exudate, variably hyperintense on all sequences

Margo et al, Pediatr Ophthalmol Strabismus 1983; 20: 180-184; Wilder, Trans Am Acad Ophthalmol Otolaryngol 1950; 55:99-104

Figure 5-6-14

Uveal Melanoma •

• • •

Uvea ➢ Choroid, ciliary body, iris ➢ Derived from mesoderm & neuroectoderm ➢ Most highly vascular part of eyeball Melanoma: most common neoplasm of choroid Whites (15:1) ➢ Incidence increases with age Arises from choroid, elevates and may rupture Bruch’s membrane (mushroom shape)

Yanoff and Fine, Ocular Pathology, Harper and Row, 1975; Mafee, Radiol Clin North Am 1998; 36:1083-1099

Uveal Melanoma • • • •

•

Gross photograph of Coats Disease

[Figure 5-6-15]

Metastasis: liver > lung > bone > kidney > brain Diagnosis usually made by ophthalmoscopy or US CT: elevated, hyperdense, sharply marginated (usually) solid mass MR ➢ Hyperintense on T1WI and PDW ➢ Hypointense on T2WI Moderate enhancement

Duffin et al, Arch Ophthalmol 1981; 99:1827-1830; Enochs et al, Radiology 1997; 204:417-423; Mafee in Som and Curtin, Head and Neck Imaging, 4th ed., Mosby, 2003

Figure 5-6-15

Uveal melanoma Imaging of the Orbit: The Globe and Conal Lesions

1090 1092

Neuroradiology

Uveal Metastasis • • • • • •

Figure 5-6-16

[Figure 5-6-16]

Usually in the plane of the choroid with little increased thickness Mottled appearance, diffuse outline Breast and lung cancer most common Retina or choroid Bilateral 1/3 (melanoma rarely bilateral) Mucinous adenocarcinoma: mimic melanoma

Mafee, Radiol Clin North Am 1998; 36:1083-1099

Orbital Trauma • •

•

• • •

[Figures 5-6-17 and 5-6-18]

CT: imaging modality of choice Fractures ➢ Isolated: orbital rim (Waters view) ➢ Blowout: inferior wall; medial wall: 1/2 ➢ Nasoethmoidal (NOE) complex ❖ canthal injury common ➢ Zygomatic complex (ZC) ➢ Lefort types: I, II, III ➢ Orbital apex-optic canal Hemorrhage ➢ Retrobulbar: most common ➢ Extraconal ➢ Subperiosteal ➢ Sub-Tenon capsule ➢ Subdural (optic nerve sheath) Optic nerve injury Eyeball injury: phthisis bulbi Foreign Body

Bilateral uveal metastases

Figure 5-6-17

Figure 5-6-18

Blow-out fracture

Orbital fracture extending towards optic canal Neuroradiology

1091 1093


Conal Lesions • • •

Graves Pseudotumor Others ➢ Lymphoproliferative disease ➢ Metastasis: 7%, breast carcinoma, nodular ➢ Arteriovenous fistula or vascular congestion ➢ Acromegaly ➢ Amyloid ➢ Cysticercosis / Trichinosis

Thyroid Orbitopathy (Graves Disease) •

• • •

Autoimmune disorder: orbital soft tissues, thyroid, extremities ➢ Superior cervical lymph channel: drains both thyroid and orbit Incidence: 0.5% (U. S.) Most common orbital disorder Most common cause of exophthalmos in adults ➢ 15%–28% of unilateral exophthalmos ➢ 80% of bilateral exophthalmos

Figure 5-6-19

Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999; Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003

Graves Disease •

• • • •

Most (up to 80%) patients are or will be hyperthyroid ➢ Euthyroid (10%) Family history: 30% Range: 15–86 years old (peak: 30–50) Females much more common (4:1) Males, patients > 50y/o: more severe disease

Kendler et al, Arch Ophthalmol 1993; 111:197-201

Graves Disease •

•

Acute phase ➢ Inflammatory reaction: congestion, hypertrophy, fibrosis of orbital fat / muscles ➢ Mucopolysaccharides accumulate in EOMs Chronic phase: exophthalmos (34%–93%) ➢ Fibrosis ➢ Restrictive myopathy ➢ Diplopia

Graves Disease

Figure 5-6-20

Rubin and Sadun in Yanoff and Duker, Ophthalmology, Mosby, 1999

Graves Disease: Imaging •

• • •

[Figures 5-6-19 and 5-6-20]

Muscle Enlargement ➢ Inferior rectus ➢ Medial rectus ➢ Superior muscle complex ➢ Lateral rectus ➢ ? Related to innervation and fiber size Tendon spared “Dirty” retrobulbar fat: inflammation Apex: optic nerve compression

Mafee in Som and Curtin, Head and Neck Imaging, 4th ed, Mosby, 2003

Graves Disease with sparing of the tendinous insertions Imaging of the Orbit: The Globe and Conal Lesions

1092 1094

Neuroradiology

Pseudotumor • • • • •

Nongranulomatous inflammation No known cause Diagnosis of exclusion Second most common (~5%) orbital disease (after Graves disease) Children: 6%–16% of all cases, more frequently bilateral

Blodi and Gass, Br J Ophthalmol 1968; 52:79-93; Flanders et al, J Comput Assist Tomogr 1989; 13:40-47; Weber et al, Radiol Clin North Am 1999; 37:151-168

Figure 5-6-21

Pseudotumor •

• •

Acute form ➢ Abrupt onset of pain usually ➢ Lid swelling, redness, ptosis, proptosis Chronic form ➢ Fixation signs: diplopia, proptosis Sites ➢ Lacrimal gland (lacrimal adenitis) ➢ Extraocular muscles (myositic form) ➢ Cavernous sinus (Tolosa-Hunt)

Tolosa, J Neurol Neurosurg Psychiatry 1954; 17:300-302; Hunt, Neurology 1961; 11:56-62

Pseudotumor: EOM • • • • • •

Tendons involved (unlike Graves disease) Ragged “fluffy” muscle border Inward bowing of muscle contour at globe insertion “Dirty” retrobulbar fat May extend intracranially (apical orbital inflammation) or onto optic nerve (ON) sheath (perineuritis) Bone destruction rare

Trokel and Hilal, Am J Ophthalmol 1979; 87:503-512; Flanders et al, J Comput Assist Tomogr 1989; 13:40-47

Pseudotumor: Imaging •

•

[Figures 5-6-21 and 5-6-22]

CT ➢ Nonspecific ➢ Moderate enhancement MR ➢ Hypointense on T1WI and T2WI

Pseudotumor with tendinous involvement and rapid response to steroid therapy on follow-up

Figure 5-6-22

Pseudotumor with characteristic T1 and T2 hypointensity Neuroradiology

1093 1095


References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18.

19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32.

Abramson DH, Ellsworth RM, Kitchin FD, Tung G. Second nonocular tumors in retinoblastoma survivors. Are they radiation-induced? Ophthalmology 1984; 91:1351-1355. Abramson DH, Ellsworth RM, Tretter P, Javitt J, Kitchin FD. Treatment of bilateral groups I through III retinoblastoma with bilateral radiation. Arch Ophthalmol 1981; 99:1761-1762. Blodi FC, Gas JD. Inflammatory pseudotumour of the orbit. Br J Ophthalmol 1968; 52:79-93. Char DH, Hedges TR, 3rd, Norman D. Retinoblastoma. CT diagnosis. Ophthalmology 1984; 91:1347-1350. Coats G, Lond R. Forms of retinal diseases with massive exudation. Ophthalmol Hosp Rep 1908; 17:440-525. Duffin RM, Straatsma BR, Foos RY, Kerman BM. Small malignant melanoma of the choroid with extraocular extension. Arch Ophthalmol 1981; 99:1827-1830. Edward DP, Mafee MF, Garcia-Valenzuela E, Weiss RA. Coats' disease and persistent hyperplastic primary vitreous. Role of MR imaging and CT. Radiol Clin North Am 1998; 36:1119-1131, x. Eller AW, Jabbour NM, Hirose T, Schepens CL. Retinopathy of prematurity. The association of a persistent hyaloid artery. Ophthalmology 1987; 94:444-448. Ellsworth RM. The practical management of retinoblastoma. Trans Am Ophthalmol Soc 1969; 67:462-534. Enochs WS, Petherick P, Bogdanova A, Mohr U, Weissleder R. Paramagnetic metal scavenging by melanin: MR imaging. Radiology 1997; 204:417-423. Flanders AE, Mafee MF, Rao VM, Choi KH. CT characteristics of orbital pseudotumors and other orbital inflammatory processes. J Comput Assist Tomogr 1989; 13:40-47. Hunt WE, Meagher JN, Lefever HE, Zeman W. Painful opthalmoplegia. Its relation to indolent inflammation of the carvernous sinus. Neurology 1961; 11:56-62. Jakobiec FA, Tso MO, Zimmerman LE, Danis P. Retinoblastoma and intracranial malignancy. Cancer 1977; 39:2048-2058. Kaufman LM, Mafee MF, Song CD. Retinoblastoma and simulating lesions. Role of CT, MR imaging and use of Gd-DTPA contrast enhancement. Radiol Clin North Am 1998; 36:1101-1117. Kendler DL, Lippa J, Rootman J. The initial clinical characteristics of Graves' orbitopathy vary with age and sex. Arch Ophthalmol 1993; 111:197-201. Kodilinye HC. Retinoblastoma in Nigeria: problems of treatment. Am J Ophthalmol 1967; 63:469-481. Kyritsis AP, Tsokos M, Triche TJ, Chader GJ. Retinoblastoma--origin from a primitive neuroectodermal cell? Nature 1984; 307:471-473. Mafee MF, Goldberg MF, Cohen SB, et al. Magnetic resonance imaging versus computed tomography of leukocoric eyes and use of in vitro proton magnetic resonance spectroscopy of retinoblastoma. Ophthalmology 1989; 96:965-975; discussion 975-966. Mafee MF, Goldberg MF, Greenwald MJ, Schulman J, Malmed A, Flanders AE. Retinoblastoma and simulating lesions: role of CT and MR imaging. Radiol Clin North Am 1987; 25:667-682. Mafee MF, Goldberg MF. Persistent hyperplastic primary vitreous (PHPV): role of computed tomography and magnetic resonance. Radiol Clin North Am 1987; 25:683-692. Mafee MF, Peyman GA. Retinal and choroidal detachments: role of magnetic resonance imaging and computed tomography. Radiol Clin North Am 1987; 25:487-507. Mafee MF. The eye. In: Som PM, Curtin HD, eds. Head and neck imaging. 4th ed. St. Louis: Mosby–Elsevier Science; 2003 Mafee MF. Uveal melanoma, choroidal hemangioma, and simulating lesions. Role of MR imaging. Radiol Clin North Am 1998; 36:1083-1099 Margo CE, Katz NN, Wertz FD, Dorwart RH. Sclerosing endophthalmitis in children: computed tomography with histopathologic correlation. J Pediatr Ophthalmol Strabismus 1983;20:180-184 Pendergrass TW, Davis S. Incidence of retinoblastoma in the United States. Arch Ophthalmol 1980; 98:1204-1210. Reese AB. Telangiectasis of the retina and Coats' disease. Am J Ophthalmol 1956; 42:1-8. Rubin RM, Sadun AA. Ocular myopathies. In: Yanoff M, Duker JS, eds. Ophthalmology. St. Louis: Mosby; 1999. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954; 17:300-302. Trokel SL, Hilal SK. Recognition and differential diagnosis of enlarged extraocular muscles in computed tomography. Am J Ophthalmol 1979; 87:503-512. Weber AL, Romo LV, Sabates NR. Pseudotumor of the orbit. Clinical, pathologic, and radiologic evaluation. Radiol Clin North Am 1999; 37:151-168, xi. Wilder HC. Nematode endophthalmitis. Trans Am Acad Ophthalmol Otolaryngol 1950:99-109. Yanoff K, Fine BS, Ocular Pathology . Hagerstown: Harper & Row, 1975.


1094 1096

Neuroradiology

Imaging of the Orbit: Intraconal and Extraconal Lesions Kelly K. Koeller, MD, FACR Figure 5-7-1

Intraconal Lesions • • • • • • •

Optic nerve glioma Optic nerve sheath meningioma Cavernous hemangioma Schwannoma Lymphoma Fibrous histiocytoma Varix, arteriovenous malformation

Optic Nerve Glioma • • •

• • •

3% of all orbital tumors; 4% of gliomas Peak age: 2–8 years (range: birth to 60 years) 50% associated with neurofibromatosis type 1 (NF-1) and frequently bilateral ➢ 10%–15% of all NF-1 cases Optic atrophy on ophthalmoscopy Arise from glial cells of optic nerve Slow growth usually; may grow in spurts

Azar-Kia et al, Radiol Clini North Am 1987; 25:561-581

Optic Nerve Glioma • • • •

• •

Optic nerve glioma with characteristic "kinking" of the nerve

[Figure 5-7-1]

Juvenile: pilocytic astrocytoma Adult: glioblastoma multiforme (non-NF-1 cases) Fusiform enlargement: kinking, buckling CT: iso- to hypodense ➢ Calcification rare T1WI: hypointense, T2WI: hyperintense Heterogeneous enhancement

Azar-Kia et al, Radiol Clin North Am 1987; 25:561-581; Haik et al, Ophthalmology 1987; 94:709-717

Figure 5-7-2

Optic Nerve Sheath Meningioma • • • •

5% of all orbital tumors Less than 1% of all meningiomas “Extradural” meningiomas: associated with “blistering” of adjacent bone Slowly progressive loss of vision, proptosis ➢ Optociliary venous shunts, disk pallor, visual loss: highly suggestive

Sibony et al, Ophthalmology 1984; 11:1313-1326

Optic Nerve Sheath Meningioma • • • • •

•

[Figure 5-7-2]

Well-defined tubular thickening of ON CT: Calcification common T1WI: Isointense to ON T2WI: Iso- to hypoattenuated “Tram-track” enhancement ➢ Fat suppression essential May be eccentric, extend intracranially

Optic nerve sheath meningioma with tram-track enhancement (upper Daniels et al, AJNR 1982: 3:181-183; Azar-Kia et al, Radiol Clin North image) and calcification (lower image) Am 1987; 25:561-581 in 2 different patients Neuroradiology

1097

Imaging of the Orbit: Intraconal and Extraconal Lesions

Cavernous Hemangioma • • •

•

•

• •

[Figure 5-7-3]

Most common vascular orbital tumor in adults Peak age: 25-40 y/o (range 25-70) Well-circumscribed mass (pseudocapsule) ➢ Intraconal (83%) Benign non-infiltrative hamartoma ➢ Large dilated sinusoid-like spaces ➢ Slowly progressive enlargement ➢ Prominent arterial supply usually absent CT ➢ Hyperattenuated ➢ Phleboliths ➢ Bone remodeling ➢ Variable enhancement MR ➢ T1WI: mixed ➢ T2WI: iso-to-hyperintense Hemorrhage occasionally

Bilaniuk, Radiol Clin North Am 1999; 37:169-183; Mafee et al, Radiol Clin North Am 1987; 25:529-559

Figure 5-7-3

Cavernous hemangioma with pressure erosion of orbital roof

Schwannoma •

• • •

1% of all orbital tumors: usually intraconal ➢ Arise from cranial nerves, not optic nerve ➢ Isolated or neurofibromatosis association Benign with slow growth ➢ Well-encapsulated Painless proptosis Compresses or engulfs optic nerve

Carroll et al, Radiol Clin North Am 1999; 37:195-202

Schwannoma • •

• • •

[Figure 5-7-4]

Fusiform to oval-shaped mass CT: Isoattenuated to extraocular muscles ➢ More hyperdense areas = Antoni A cells T1WI: Iso- to hypodense T2WI: Hyperintense Marked enhancement

Figure 5-7-4

Orbital Lymphoma •

• • • •

Lymphoid tumors: 10%-15% of orbital masses ➢ Lymphoma, pseudolymphoma, lymphoid hyperplasia 10% of all lymphomas as primary site ➢ 75% have or will have systemic lymphoma ➢ Lacrimal gland: most common site ➢ EOMs rarely involved Non-Hodgkin’s (B-cell): majority Proptosis, ptosis, diplopia Rubbery firm masses

Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et al, Radiol Clin North Am 1997; 25:601-612;

Schwannoma Imaging of the Orbit: Intraconal and Extraconal Lesions

1096 1098

Neuroradiology

Orbital Lymphoma •

• • • • •

Figure 5-7-5

[Figure 5-7-5]

Molds itself along margins of normal structures ➢ Bone erosion: late finding Usually well-defined, round to oval CT: homogeneous, mildly hyperattenuated T1WI: hypointense T2WI: iso-to-hypointense Mild to marked enhancement

Valvassori et al, Radiol Clin North Am 1999; 37:135-150; Flanders et al, Radiol Clin North Am 1997; 25:601-612;

Fibrous Histiocytoma • • • •

• •

[Figure 5-7-6]

Most common primary orbital mesenchymal tumor in adults 1% of all primary orbital tumors Mean age: 42 y/o Usually benign (66%) ➢ Malignant: bone erosion, hemorrhage, post-radiation therapy for retinoblastoma Well-defined intra-or extraconal mass Moderate to marked enhancement

Font and Hidayat, Hum Pathol 1982; 13:199; Mafee in Valvassori, Mafee, and Carter, Imaging of the Head and Neck, Thieme, 1995

Orbital Varix • • •

• •

Orbital lymphoma with characteristic molding of the tumor against the orbital globe

[Figure 5-7-7]

Most common cause of spontaneous orbital hemorrhage Focal venous dilatation Valsalva: stress proptosis ➢ Lobulated mass Phleboliths Spontaneous thrombosis common

Figure 5-7-6

Bilaniuk, Radiol Clin North Am 1999; 37:169-183

Figure 5-7-7 Malignant fibrous histiocytoma

Orbital varix with enlargement upon Valsalva maneuver on post-contrast image (bottom) Neuroradiology

1097 1099


Arteriovenous Fistula •

• • • •

Figure 5-7-8

[Figure 5-7-8]

Usually post-trauma ➢ Spontaneous less common (Ehlers-Danlos, osteogenesis imperfecta, pseudoxanthoma elasticum) Orbital bruit, proptosis, chemosis CT/MR: dilated superior ophthalmic vein Angiography diagnostic Endovascular occlusion: treatment of choice

Tan et al, Radiol Clin North Am 1987; 25:849-861

Extraconal Lesions • • • • •

Lymphangioma* Metastasis* Rhabdomyosarcoma* Dermoid/epidermoid Paranasal sinus disease ➢ Infection ➢ Neoplasm

*commonly “intercompartmental”

Lymphangioma • • •

• • •

Carotid-cavernous fistula with enlarged superior ophthalmic vein. Lateral view from cerebral angiogram shows abnormal flow through cavernous sinus, petrosal sinuses, and superior ophthalmic vein

[Figure 5-7-9]

Children and young adults Exophthalmos with viral infection Lymphoid follicles, dilated spaces ➢ Infiltrative; do not respect fascial planes ➢ Hemorrhage common Extraconal space primarily CT/MR: Heterogeneous appearance ➢ Hemorrhage or cystic fluid Variable enhancement (venous channels)

Bilaniuk, Radiol Clin North Am 1999; 37:169-183; Mafee et al, Radiol Clin North Am 1987; 25:529-559


Lymphangioma with hemorrhage in 2 different patients

Metastatic Lesions •

• •

[Figure 5-7-10]

10% of orbital tumors ➢ 1/3- bony orbit, 1/3- globe, 1/3- scattered Increasing incidence (longer survival) Primary site ➢ Breast: 42% ➢ Lung: 11% ➢ Unknown primary: 11% ➢ Prostate: 8% ➢ Melanoma: 5% ➢ Children: neuroblastoma, leukemia, Ewing’s


Orbital metastasis from unknown primary neoplasm 1098 1100

Neuroradiology

Metastatic Lesions • • • • •

Figure 5-7-11

9 months average survival (lung carcinoma, melanoma worst) Hematogenous spread Diplopia, proptosis, pain, vision loss Enophthalmos: breast carcinoma Isolated lateral rectus enlargement metastasis or pseudotumor

Rhabdomyosarcoma [Figure 5-7-11] •

• •

Most common primary orbital malignancy tumor in children ➢ Most: 2-5y/o; 90% younger than 16 y/o ➢ Rapidly progressive but usually painless Arise from undifferentiated mesenchyme in orbital fat (not from extraocular muscles) ➢ Children: embryonal (70%) and alveolar types ➢ Adults: pleomorphic type 90% 5-year survival with complete resection ➢ 35% if significant residual disease

Rhabdomyosarcoma with characteristic bone destruction

Mafee et al, Radiol Clin North Am 1998; 36:1215-1227;

Rhabdomyosarcoma • • •

• • •

Figure 5-7-12

Superior orbit predilection Homogeneous mass CT: isoattenuated to muscle ➢ Bone destruction common in larger lesions ➢ Necrosis, calcification, hemorrhage uncommon T1WI: hypointense T2WI: hyperintense Moderate to marked enhancement

Mafee et al, Radiol Clin North Am 1998; 36:1215-1227

Dermoid / Epidermoid [Figure 5-7-12] •

• • • •

Most common congenital orbital lesion ➢ Many manifest in 2nd-3rd decades Superolateral: most comon location Arise at sutures or diplöe Well-defined mass with fat or fluid signal in upper corners of orbit Remodel bone without destruction

Kaufman et al, Radiol Clin North Am 1998; 36:1149-1163

Orbital Cellulitis [Figures 5-7-13 and 5-7-14] •

• •

Dermoid

Classification ➢ Pre-septal cellulitis: eyelid ➢ Post-septal cellulitis ➢ Subperiosteal phlegmon and abscess ➢ Cavernous sinus thrombosis Usually paranasal (ethmoid) sinusitis Usually does not extend into intraconal space

Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am 1998; 36:1165-1183

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1099 1101


Figure 5-7-14

Figure 5-7-13

Orbital cellulitis. Note involvement along lamina papyracea

Peri-orbital cellulitis

Fungal sinusitis [Figure 5-7-15] •

•

Immunocompromised patients ➢ Rhino-orbital mucormycosis ➢ Aspergillosis Reversal of typical findings in sinus disease ➢ Increasing protein, decreasing water content ➢ CT: hyperattenuated ➢ T1WI: hyperintense ➢ T2WI: hypointense (can mimic air)

Chandler et al, Laryngoscope 1970; 80:1414; Eustis et al, Radiol Clin North Am 1998; 36:1165-1183

Lacrimal Gland Lesions •

• •

50% inflammatory/lymphoproliferative ➢ Sarcoid ➢ Sjogren’s ➢ Lymphoma: frequent anterior/posterior extension ➢ Pseudotumor: 15% of all orbital pseudotumor 50%: epithelial tumors ➢ 50%: benign (pleomorphic adenoma, benign mixed cell tumor) ➢ 50%: malignant (adenoid cystic, malignant mixed, mucoepidermoid, adeno, squamous cell, anaplastic) Imaging: pre-op planning

Figure 5-7-15

Zimmerman et al, Int Ophthalmol Clin 1962; 2:337-367; Mafee et al, Radiol Clin North Am 1987; 25:767-779

Fungal sinusitis


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Neuroradiology

Lacrimal Gland Lesions [Figure 5-7-16 and 5-7-17] •

•

Figure 5-7-16

Inflammatory lesions ➢ Oblong mass ➢ Molded enlargment of lacrimal gland Pleomorphic adenoma ➢ Long duration ➢ Rounded mass ➢ Bone remodeling ➢ Bone destruction: malignant epithelial tumors

Jakobiec et al, Am J Ophthalmol Clin 1962; 2:337-367

Lacrimal Sac Lesions •

•

[Figure 5-7-18]

Malignant: 57% ➢ Epithelial: 75% ❖ Squamous cell ❖ Transitional cell ❖ Mucoepidermoid ➢ Mesenchymal: fibrous histiocytoma ➢ Lymphoid: lymphoma ➢ Neural ➢ Metastasis Benign: 43% ➢ Diverticulum ➢ Pneumatocele ➢ Mucocele ➢ Papilloma ➢ Polyp ➢ Fibroma ➢ Dermoid

Lacrimal lymphoma

Figure 5-7-17

Stefanyszyn et al, Ophthal Plast Reconstr Surg 1994; 10:169-184; Pe’er et al, Ophthalmology 1996; 103:1601-1605

Figure 5-7-18 Lacrimal pleomorphic adenoma

Lacrimal sac sarcoma with bone destruction

Neuroradiology

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Summary • • • •

•

•

•

•

Retinoblastoma: most common intraocular malignancy of childhood Uveal melanoma: most common malignancy of the globe in adults Uveal metastasis: frequently bilateral Most common diseases of the orbit ➢ 1. Graves: no tendon involvement ➢ 2. Lymphoma ➢ 3. Pseudotumor: involves tendon Intraconal lesions ➢ Optic nerve tumors ❖ Glioma ❖ Nerve sheath meningioma ➢ Cavernous hemangioma ➢ Nerve sheath tumors ➢ Lymphoma ➢ Fibrous histiocytoma ➢ Varix ➢ Carotid-cavernous fistula Extraconal lesions ➢ Lymphangioma ➢ Metastases: 10% of orbit masses ➢ Rhabdomyosarcoma ➢ Dermoid ➢ Sinus disease Lacrimal gland lesions ➢ “The 50% gland” ➢ 50% inflammatory/lymphoproliferative ➢ 50% neoplasms ❖ 50% benign, 50% malignant Lacrimal sac lesions: most are malignant

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

Azar-Kia B, Naheedy MH, Elias DA, Mafee MF, Fine M. Optic nerve tumors: role of magnetic resonance imaging and computed tomography. Radiol Clin North Am 1987; 25:561-581. Bilaniuk LT. Orbital vascular lesions. Role of imaging. Radiol Clin North Am 1999; 37:169-183, xi. Carroll GS, Haik BG, Fleming JC, Weiss RA, Mafee MF. Peripheral nerve tumors of the orbit. Radiol Clin North Am 1999; 37:195-202, xi-xii. Chandler JR, Langenbrunner DJ, Stevens ER. The pathogenesis of orbital complications in acute sinusitis. Laryngoscope 1970; 80:1414-1428. Daniels DL, Williams AL, Syvertsen A, Gager WE, Harris GJ. CT recognition of optic nerve sheath meningioma: abnormal sheath visualization. AJNR Am J Neuroradiol 1982; 3:181-183. Eustis HS, Mafee MF, Walton C, Mondonca J. MR imaging and CT of orbital infections and complications in acute rhinosinusitis. Radiol Clin North Am 1998; 36:1165-1183, xi. Flanders AE, Espinosa GA, Markiewicz DA, Howell DD. Orbital lymphoma. Role of CT and MRI. Radiol Clin North Am 1987; 25:601-613. Font RL, Hidayat AA. Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases. Hum Pathol 1982; 13:199-209. Haik BG, Saint Louis L, Bierly J, et al. Magnetic resonance imaging in the evaluation of optic nerve gliomas. Ophthalmology 1987; 94:709-717. Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal fossa lesions. Am J Ophthalmol 1982; 94:785-807. Kaufman LM, Villablanca JP, Mafee MF. Diagnostic imaging of cystic lesions in the child's orbit. Radiol Clin North Am 1998; 36:1149-1163, xi. Mafee MF, Haik BG. Lacrimal gland and fossa lesions: role of computed tomography. Radiol Clin North Am 1987; 25:767-779. Mafee MF, Pai E, Philip B. Rhabdomyosarcoma of the orbit. Evaluation with MR imaging and CT. Radiol Clin North Am 1998; 36:1215-1227, xii. Mafee MF, Putterman A, Valvassori GE, Campos M, Capek V. Orbital space-occupying lesions: role of computed tomography and magnetic resonance imaging. An analysis of 145 cases. Radiol Clin North Am 1987; 25:529-559.


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15. Mafee MF. Imaging of the orbit. In: Valvassori GE, Mafee MF, Carter BL, eds. Imaging of the head and neck. New York: Thieme, 1995; 302-328 16. Pe'er J, Hidayat AA, Ilsar M, Landau L, Stefanyszyn MA. Glandular tumors of the lacrimal sac. Their histopathologic patterns and possible origins. Ophthalmology 1996; 103:1601-1605. 17. Sibony PA, et al: Optic Nerve Sheath Meningiomas. Ophthalmology 1984, 91(11): 1313-1326. 18. Stefanyszyn MA, Hidayat AA, Pe'er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994; 10:169-184. 19. Tan WS, Wilbur AC, Mafee MF. The role of the neuroradiologist in vascular disorders involving the orbit. Radiol Clin North Am 1987; 25:849-861. 20. Valvassori GE, Sabnis SS, Mafee RF, Brown MS, Putterman A. Imaging of orbital lymphoproliferative disorders. Radiol Clin North Am 1999; 37:135-150, x-xi. 21. Zimmerman LE, Sanders TE, Ackerman LV. Epithelial tumors of the lacrimal gland: prognostic and therapeutic significance of histologic types. Int Ophthalmol Clin 1962; 2:337-367.

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Patterns of Location: Infratentorial and Supratentorial James G. Smirniotopoulos, MD PATTERN ANALYSIS •

Basic Approach ➢ Where is the lesion ? ❖ Intraaxial ❖ Extraaxial ❖ Intraventricular ➢ Where is the lesion ? ❖ Supratentorial ❖ Infratentorial ➢ How old is the patient ? ❖ Child ❖ Adult ➢ What about Sex ?

INTRA-AXIAL • • • • • • • • • • •

Cortex Gray-white Junction Deep White Matter Deep Gray Matter Glioma Medulloblastoma Hemangioblastoma Metastases Infarct/hematoma AVM/congenital Abscess/inflammation

EXTRA-AXIAL LESIONS • • • • • • • • • • • • •

Subarachnoid Subdural Epidural Calvarium (Skull Base) Subgaleal Scalp (Soft-tissues) Meningioma Pituitary adenoma Craniopharyngioma Schwannoma Chordoma Dermoid/epidermoid, cyst, lipoma Hematoma, metastasis, infection

BASIC APPROACH CLASSIC LOCATIONS • • • • • • • • •

Foramen magnum Cerebellopontine angle (CPA) Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Cranial Nerves • • • • • • • •

Figure 5-8-1

Olfactory (I) Optic (II) Oculomotor (III) Trochlear (IV) Trigeminal (V) Abducens (V) Facial (VII) Vestibulocochlear (VIII)

When looking into the IAC (internal auditory canal) notice that 7 is UP and Coke (cochlear) is DOWN

Internal Auditory Canal • • • •

S – Schwannoma (8th >> > 5th) A – aneurysm, arachnoid cyst M – meningioma, mets E – epidermoid, ependymoma, CPP

• • • •

S – Schwannoma (8th >> > 5th) A – aneurysm, arachnoid cyst M – meningioma, mets E – epidermoid, ependymoma, CPP

[Figures 5-8-1 and 5-8-2]

CPA MASSES Differential Figure 5-8-2

CPA MASSES Demographics • • •

7/9 (Schwannoma, 8th > > 5th) 1/9 Meningioma (tentorial/petrous) 1/9 “Other”: ➢ Epidermoid Cyst (1/18) ➢ Mets, aneurysm, etc. ➢ Glioma (ependymoma, CPP) ➢ Arachnoid cyst ➢ Cystadenoma of endolymph ➢ Glomus tumor Vestibular Schwannoma begins as an intracanalicular mass; then it grows out of the canal into the cerebellopontine angle cistern

Intracanalicular Schwannoma [Figure 5-8-3]

Vestibular Schwannoma Figure 5-8-3

[left] T2W image shows CSF, normal nerve, and round mass. [right] T1WGd image shows enhancement of mass. The normal 7th and 8th nerves do NOT enhance in this location

Neuroradiology

1105 1107


Young Schwannoma – Old Schwannoma [Figure 5-8-4] •

Figure 5-8-4

Benign Cystic Degeneration

Vestibular Schwannoma •

• •

•

IAC origin ➢ IAC involved ➢ IAC Enlarged (70%) Spherical Mass ➢ encapsulated Heterogeneous if large ➢ > 20 mm Enhance “always”

Trigeminal Schwannoma [Figure 5-8-5] Figure 5-8-5

The larger – and older – Schwannoma is heterogeneous due to benign cystic degeneration

Figure 5-8-6

Trigeminal Schwannoma may present as a “dumbbell” mass, bilobed, with one lobe in the cavernous sinus and one in the poster fossa – lateral pontine cistern

Bilateral Vestibular Schwannoma

This mass is hemispheric – but does not extend into the canal. [Courtesy of Bob Peyster, MD]

Meningioma Hyperostosis Meningioma

Figure 5-8-7 Tentorial Meningioma Meningioma [Figures 5-8-6 and 5-8-7] • • • • •

•

Tentorium or Dura IAC Normal Hemispherical Enhance Homogeneous Hyperostosis ➢ 15%-40% Dural Tail ➢ 70%-90%

[left] T1W image – no enhancement, undulating (wavy) margin. Note the wispy internal structure. [right] T2W image – isointense to CSF Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Epidermoid vs. Arachnoid Cyst •

•

CLASSIC LOCATIONS • • • • • • • • •

Figure 5-8-8

Epidermoid Inclusion Cyst [Figure 5-8-8] ➢ CPA most common ➢ Extraaxial CPA Lesion ➢ IAC Normal ➢ Undulating Margin ➢ CSF - ‘like’ ❖ Not identical ➢ NO Enhancement ➢ Wispy internal structures Arachnoid Cyst ➢ Middle fossa common ➢ Extraaxial CPA Lesion ➢ IAC Normal ➢ Rounded Mass ➢ Identical to H2O on CT and all MR sequences ❖ T1, PD, T2, FLAIR, DWI, ADC ➢ NO Enhancement ➢ NO ‘structure’

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

This epidermoid inclusion cyst is only isointense to CSF on the T2W image

Figure 5-8-9

Central Posterior Fossa Lesion • • • •

Could be Intraaxial Could be Intraventricular Could be extending from vermis into ventricle Could be extending from ventricle into vermis

Fourth Ventricle - Schematic [Figure 5-8-9] Central Masses [Figure 5-8-10]

Schematic of central posterior fossa mass: Did it begin in the 4th ventricle, in the medullary velum, or in the cerebellum?

CHILD - CEREBELLAR/IVth • • • •

Medulloblastoma (PNET) Astrocytoma (usu. Pilocytic) Ependymoma Post fossa cysts

Figure 5-8-10

ADULT - CEREBELLAR/IVth • • • •

•

Metastasis Hemangioblastoma Hemorrhage, infarct Glioma ➢ Ependymoma ➢ Astrocytoma Abscess

Schematic diagram of medulloblastoma and ependymoma. Copyright 2005 Neuroradiology

1107 1109


Medulloblastoma [Figures 5-8-11 and 5-8-12] •

• • • •

Figure 5-8-11

‘Homogeneous’ ➢ finely irregular Cyst and Hemorrhage are uncommon 10 mm ➢ balloon sella ➢ Visual Sx ❖ if >6 mm above sella ❖ bitemporal hemianopsia

Lhermitte-Duclos. T1 and T2 weighted images show a striated or “courduroy” appearance, classic for dysplastic gangliocyoma

Figure 5-8-27

Elevated Prolactin •

• • •

• •

Microadenoma ➢ < 10mm diameter ➢ Entirely intrasellar Macroadenoma ➢ > 10 mm Stalk Effect ➢ Blocks Prolactin Inhibitory Factor ➢ 40-150 ng PRL vs. 28 for nl. Hypothyroidism ➢ “Cross Reaction” from TSH Exogenous Pharmacologic Pituitary macroadenoma with hyperintensity from old hemorrhage

Neuroradiology

1111 1113


Macroadenoma [Figure 5-8-28]

Figure 5-8-28

Sella and Suprasellar [Figure 5-8-29] Craniopharyngioma – 2 Types •

•

Child ➢ Adam Ant – inomatous ❖ enamel organ of tooth ➢ Commonly Cystic ➢ ‘Machine Oil’ ➢ Commonly Calcified ➢ Adherent to brain ❖ pilocytic astrogliosis Adult ➢ Squamous and Papillary ➢ Commonly Solid ➢ Calcification less common ➢ Easier to resect

Pituitary macroadenoma with hyperintensity from old hemorrhage

Craniopharyngioma [Figure 5-8-30] Figure 5-8-29 Figure 5-8-30

Craniopharyngioma, you barely see how the hypothalamus is draped over the top of the mass

Craniopharyngioma, expansile remodeling of sella turcica

Craniopharyngioma “Machine Oil”

Figure 5-8-31

Craniopharyngioma [Figure 5-8-31]

Craniopharyngioma


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Neuroradiology

Where is the Hypothalamus ? •

Figure 5-8-32

[Figure 5-8-32]

Hypothalamic Glioma – Pilocytic astrocytoma

Where is the Clivus? • • • •

[Figure 5-8-33]

Chordoma: Notochord rests Midline Bone destruction

Figure 5-8-33

Hypothalamic Glioma – Pilocytic astrocytoma

Figure 5-8-34

Chordoma of the clivus Schematic of location of remnant notochord tissue. Copyright 2003

Notochord: Chordoma & Thornwaldt [Figure 5-8-34]

SELLA/PARASELLAR •

Figure 5-8-35

Differential Features: ➢ ADULT – Pituitary adenoma ➢ CHILD – Craniopharyngioma or Glioma (hypothalamus or optic ) > EG, etc ➢ SELLA NORMAL – NOT pituitary ➢ Ca++ – Craniopharyngioma, but... ➢ HYPEROSTOSIS – Meningioma ( exp. “blistering” ) ➢ CLIVUS – Chordoma, mets, NP Ca ➢ Remember – rule out vascular lesions (aneurysms)

Pulsation Artifact: Phaseencoding direction [Figure 5-8-35]

Giant (> 2.5 cm) cerebral aneurysm

CLASSIC LOCATIONS • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle

Neuroradiology

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• • • •

Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Figure 5-8-36

Colloid Cyst [Figure 5-8-36] •

Hydrocephalus: Vents > Sulci

THIRD VENTRICLE [Figure 5-8-37] •

Differential: ➢ Colloid cyst ➢ Cysticercosis ➢ Craniopharyngioma ➢ Hypothalamic and thalamic glioma ➢ CPP, ependymoma ➢ Neurocytoma ➢ Basilar tip aneurysm

Colloid cyst

Figure 5-8-37

Colloid Cyst [Figure 5-8-38 to 5-8-40] Figure 5-8-38

Colloid cyst

Differential Diagnosis – 3rd ventricle. Copyright 2005


Colloid cyst. T1-weighted image shows slight hyperintensity before gadolinium and no definite enhancement


Colloid Cyst. Marked hypointensity on T2W image

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Neuroradiology

HYDROCEPHALUS •

• • •

Figure 5-22-41

Differential Diagnosis: ➢ Over production of CSF (CPP) ➢ Obstruction of CSF flow: ❖ Obstructive/internal hydrocephalus ❖ Communicating/external hydrocephalus Under reabsorption of CSF: SAH Compensatory: Ex vacuo/enlargement

CSF Homeostasis [Figure 5-8-41] Normal Ventricular System [Figure 5-8-42] Schematic of CSF Homeostasis Copyright 2004

Figure 5-22-42

Schematic of Normal Ventricular System Copyright 2004

Foramen of Monro Obstruction Aqueduct Obstruction Non-traumatic hemorrhage [Figures 5-8-43 and 5-8-44]

Figure 5-22-44

Figure 5-22-43

Non-traumatic hemorrhage in the right thalamus. Copyright 2006

Non-traumatic hemorrhage in the right thalamus

Neuroradiology

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Hypertensive Hemorrhage

Figure 5-8-45

Hemorrhage into a mass •

NOTE: Vasogenic Edema

ARTERIOLOSCLEROSIS

What do they have in Common? [Figure 5-8-45] • • • • • •

Multiple Bilateral Symmetric Anatomic Basal ganglia Toxic and/or Metabolic: ➢ Acquired ➢ Congenital

CT – medial lenticular lesion – Globus Pallidus. MR – lateral lenticular lesion – Putamen

CO Poisoning [Figure 5-8-46]

Figure 5-8-46

MetOH Intoxication Tx for MetOH - Fomepazole • • •

Fomepazole (Antizole, 4-methylperazole) is a synthetic alcohol dehydrogenase inhibitor for IV administration Clear yellow liquid, mw 82.1, mp 25º C (77º F) INDICATIONS: Antidote for ethylene glycol, or methanol poisoning of suspected EG ingestion ➢ PRECAUTIONS: Dilute in > 100 mL NS, follow hepatic enzymes & WBC (eos) during Rx, interaction with ethanol (compete for ADH) ➢ DOSE: 15 mg/kg load, 10 mg/kg Q 12 h x 4 doses, then 15 mg/kg Q 12 h till EG < 20 mg/dL

Deep Lesions •

•

White Matter: ➢ Leukoencephalopathy ➢ “Bad White Matter Disease” ➢ Small vessel disease ➢ Hypertension ➢ Glial Neoplasm ➢ Astrocytoma (incl. GBM) ➢ Oligodendroglioma Deep White and Gray Matter ➢ Lymphoma ➢ Toxoplasmosis Both occur in HIV/AIDS, multiple lesions

Carbon monoxide toxicity. Notice there are bilateral medial lenticular (globus pallidus) signal abnormalities – hypointense on T1 and hyperintense on T2

Figure 5-8-47

Solitary Deep Lesion - Thalamus [Figure 5-8-47] Glioblastoma – WHO Grade 4 •

A solitary, deep, irregular, heterogeneous, ring-enhancing mass with vasogenic edema

Glioblastoma multiforme of the thalamus and temporal lobe Patterns of Location: Infratentorial and Supratentorial

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Neuroradiology

Glioblastoma Multiforme [Figure 5-8-48]

Figure 5-8-48

Toxoplasmosis [Figure 5-8-49] •

2 patients with typical lesions

BASAL GANGLIA THALAMUS: •

• •

BILATERAL SYMMETRIC (toxic/metabolic): ➢ PUTAMEN – Methanol ➢ GLOBUS PALLIDUS – CO Poisoning BILATERAL ASYMMETRIC (hematogenous): ➢ INFECTION (TOXO, etc.) UNILATERAL (acquired/neoplastic): ➢ THALAMIC GLIOMA (astrocytoma) ➢ HYPERTENSIVE HEMATOMA (exclusion)

T2 - T1gad Glioblastoma multiforme with extensive vasogenic edema

CLASSIC LOCATIONS • • • • • • • • • •

Figure 5-8-49

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle Pineal Region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Patterns in Neuroradiology • • • • • • • • •

Cerebello-Pontine Angle Fourth Ventricle/Cerebellum Sella/Parasellar Basal Ganglia/Third Ventricle Lateral Ventricle Pineal Region Deep Hemispheric/Periventricular Cortical/Subcortical Convexity Extraaxial

Intraventricular Neoplasms • • • • • • • • •

Ependymoma (and subependymoma) Choroid plexus papilloma Subependymal giant cell astro. Meningioma Colloid cyst (3rd) Medulloblastoma (4th) Dermoid/epidermoid Central neurocytoma Mets, lymphoma, Germ Cell

Toxoplasmosis

Subependymal Giant Cell Astro

Neuroradiology

(From Vince Mathews, M.D. IU)

1117 1119


Lateral Ventricle @ f. Monro •

Figure 5-8-50

Lateral Ventricle/caudate ➢ Subependymal Giant Cell Astro. ❖ TUBEROUS SCLEROSIS, Enhances & Ca++ ➢ Subependymoma ❖ Variant of Ependymoma ❖ No Ca++, no enhancement ➢ Central Neurocytoma ❖ Septum pellucidum ➢ Cyst/Cavum septum pellucidum ➢ Huntington Chorea ❖ Atrophy

Lateral ventricular masses [Figure 5-8-50] Lateral Ventricle - Trigone • • • • • •

Schematic of lateral ventricular masses. Copyright 2005

Meningioma Choroid Plexus Papilloma Xanthogranuloma Metastasis Lipoma Choroid Cyst

Trigone or Atrium Trapped Temporal Horn Choroid Plexus Papilloma • •

• •

• • •

Attached to normal Choroid Plexus Lobulated ➢ Fronds ➢ Papillae Trigone of lateral vent ➢ Children Fourth ventricle ➢ Adults Third ventricle CPA cistern Hydrocephalus ➢ Obstruction ➢ Production =/= Resorption

CSF Overproduction? Central Neurocytoma •

Central ➢ Often centered on septum pellucidum ➢ Extension into both lateral ventricles ➢ Hyperdense on CT ➢ Gray matter on MR ➢ Spontaneous Bleed ➢ Calcifications

CLASSIC LOCATIONS • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle


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Neuroradiology

• • • •

Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial

Figure 5-8-51

Pineal/Quadrigeminal Cistern Region •

• •

•

“Pinealomas” ➢ Germ cell tumors ❖ Seminoma ❖ Teratoma Pineal cell tumors ➢ Pineoblastoma ➢ Pineocytoma Gliomas (regional) ➢ Brainstem, callosum, thalamus Other ➢ Dermoid, lipoma, arachnoid cyst ➢ Meningioma ➢ Vein of Galen malformations

Pineal region mass. Germinoma. Copyright 2005

Figure 5-8-52

Pineal Region Mass [Figure 5-8-51] Germinoma [Figure 5-8-52] •

• • • • • •

Central ➢ Pineal Region ➢ Suprasellar Cistern Homogeneous Hyperdense to GM Isointense to GM Uniform Enhancement CSF Seeding ? May ENGULF Pineal Ca++ Pineal region Germinoma

Pineal Cyst-Asymptomatic • • • • •

[Figure 5-8-53]

Figure 5-8-53

T1W sagittal and T1W-Gd axial Cyst is ovoid and hypointense Enlargement of pineal Cyst wall enhances minimally Quadrigeminal plate not compressed

Pineal Cyst CLASSIC LOCATIONS • • • • • • • • •


Pineal cyst

DEEP AND PERIVENTRICULAR • • • • • • •

Glioma (astrocytoma, oligodendro.) Lymphoma (usually primary in CNS) Toxoplasmosis, CMV (ependymitis) Leukoencephalopathy (WM) Arteriolar sclerosis (HT) Infarcts (lacunar, tri-watershed) Hemorrhage

Neuroradiology

1119 1121


Glioblastoma Multiforme [Figures 5-8-54 and 5-8-55]


Expansile lesion of the corpus callosum

Lymphoma PCNSL [Figure 5-8-56]

Two “butterfly” lesions. One with peripheral dense enhancement is a GBM; and, the other with softer more uniform enhancement is primary CNS lymphoma

Figure 5-8-56

Figure 5-8-57

FLAIR T2W T1W Gd+ Primary CNS Lymphoma – an expansile enhancing lesion of the corpus callosum

PCNSL: Immunocompetent Cytomegalovirus – note the thin rim of abnormal enhancement cause by ependymitis (Courtesy Vince Mathews, M.D.)

RIM PHOMA CMV [Figure 5-8-57] Multiple Sclerosis [Figure 5-8-58] CLASSIC LOCATIONS

Figure 5-8-58

Cortical and subcortical • • • • • • • • •

Foramen magnum Cerebellopontine angle Fourth ventricle/Cerebellum Sella/parasellar/suprasellar Basal ganglia/Third ventricle Lateral ventricle/Pineal region Deep hemispheric/periventricular Cortical and subcortical Convexity Extraaxial Multiple Sclerosis. Classic Dawson fingers – ovoid lesions perpendicular to the ventricle from perivenous inflammation


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Neuroradiology

CONVEXITY INTRAAXIAL •

• •

Figure 5-8-59

Gray-white junction ➢ Hematogenous neoplasm ➢ Hematogenous infection ➢ Hematogenous thrombi (multiple infarcts) Infarction/ischemia Vasculitis (infectious,autoimmune)

Hematogenous dissemination [Figure 5-8-59] • • •

Multiple Cortical/subcortical Ring Lesions ➢ smooth ➢ round ➢ uniform thickness

CEREBRAL INFARCTION [Figure 5-8-60] • • • •

Abrupt Onset Gray Matter Involved Little Mass Effect Vascular Territory & Wedge Shape

Hematogenous dissemination. Multiple cortical ring-enhancing lesions – necrotic metastases from breast carcinoma on chemotherapy

Figure 5-8-60

Cerebral infarction. MCA territory, with matching lesions on DWI and ADC map

Figure 5-8-61

Time is Brain ! • • • •

Therapeutic Windows: 3 hours for IV tPA 6 hours for IA thrombolysis 9 hours for IV ‘Bat Spit’? ➢ an enzyme known as desmoteplase or DSPA ➢ isolated from the saliva of Desmodus rotundus ➢ vampire bat, Central and South America, 1oz NOTE: Clock starts with last time patient was observed ‘normal’. If you wake with a stroke, that might be bedtime … unless you get up at night

PCA Infarct

Lights up like a lightbulb on MRI DWI

PCA Infarct [Figure 5-8-61] Neuroradiology

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CVA : Progression of CT findings

Figure 5-8-62

Old Infarct •

Wallerian Degeneration

Dysembryoplastic Neuroepithelial Tumor (DNET) [Figures 5-8-62 and 5-8-63] •

Imaging Features ➢ Cortical, most in temporal lobe ➢ Well-circumscribed mass ➢ Hypointense T1W, Hyperintense T2W ➢ Nodular Cortical Mass ❖ Multicystic ❖ Megagyric - Assoc. calvarial erosion ➢ No Edema ➢ No or Minimal Mass Effect ➢ +/- Calvarial Erosion ➢ Occasional Enhancement

DNET – Usually a cortical lesion, often wedgeshaped

Figure 5-8-63

DDX: TUBEROUS SCLEROSIS •

• • •

Cortical Hamartomas (“Tubers): ➢ Appear to Spare Superficial Cortex ➢ Tend to be Multiple Accompanying Subependymal Nodules + Family History Generalized Seizures

CLASSIC LOCATIONS • • • • • • • • •


DNET – Usually a cortical lesion, often wedgeshaped

CONVEXITY EXTRAAXIAL Differential [Figure 5-8-64] •

• •

EPIDURAL (sub-periosteal) ➢ (Hematoma, empyema, mets) ➢ (biconvex, acute, limited by sutures) SUBDURAL (epi-arachnoid) ➢ (Hematoma, empyema, mets) ➢ (Crescentic, subacute, crosses sutures) MENINGIOMA ➢ (hyperdense, hemispheric,hyperostosis, homogeneous enhancement)

Figure 5-8-64

Epidural = Subperiosteal [Figure 5-8-65] Epidural Metastasis

Schematic of epidural (left) and subdural (right) localization. Copyright 2006

Subdural = Epi-arachnoid [Figure 5-8-66] Subdural Metastasis Meningioma – Pre and Post Gd


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Meningioma - Dural Tail

Figure 5-8-65

MENINGIOMA [Figure 5-8-67] •

The 4H+ Tumor ➢ homogeneous ➢ hyperdense ➢ homogeneous enhancement ➢ hemispheric ➢ hyperostosis ➢ hormonally modulated

Figure 5-8-66

Epidural hematoma

Bilateral chronic subdural hematomas

Figure 5-8-67

Meningioma. Hyperdense on plain CT, hemispheric, homogeneous enhancement, hyperostosis – the 4H+ tumor

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Patterns of Enhancement James G. Smirniotopoulos, MD Figure 5-9-1

Why Give Contrast? Contrast Enhancement •

Vascularity ➢ Blood Volume (rCBV) Perfusion MTT

•

➢ Blood Flow (rCBF) ➢ Arteries & veins > capillary Permeability ➢ Capillary (leakage)

Blood Brain Barrier Breakdown

Mechanisms of Enhancement [Figure 5-9-1] • •

Vascularity Permeability

Contrast Enhancement •

•

VASCULAR (intravascular) PHASE ➢ Inc. Blood Flow/Hypervascular ➢ AVM, Meningioma, GBM ➢ TRUE "Luxury" Perfusion ➢ Hyperemic Swelling (“malignant brain edema”) INTERSTITIAL (extravascular) PHASE ➢ Blood-brain-barrier breakdown) ➢ Acute inflammation (MS) ➢ Neoplasm, Abscess, “granulation” tissue ➢ Ischemia, “luxury” perfusion, contusion

Two mechanisms of Contrast Enhancement: Increased vascularity (rCBV and rCBF); and, increased permeability from breakdown of the blood-brain-barrier (BBBB)

Figure 5-9-2

Types of Radiology Contrast •

• •

Barium (BaSO4) for ingestion and enema ➢ Insoluble suspension Iodine for ingestion and enema ➢ Gastrograffin I+ and Gd+ Intravascular Contrast Agents ➢ Ionic Contrast ❖ High Osmolarity - Magnevist® – a chelate of Gd - di-N-methylglucamine salt of gadopentetate (Gd-DTPA) ❖ Iso and Low Osmolar - Iodixanol ➢ Non-Ionic Contrast ❖ ProHance® – Gadoteridol ❖ Omniscan® - Gadoimide

Time Density Curves [Figure 5-9-2] Variability in BBB permeability and perfusion [Figure 5-9-3]

Patterns of Enhancement

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The bolus creates a high intravascular concentration gradient that pushes contrast across a permeable membrane into the tissue interstitial space

Figure 5-9-3

Variable degrees of permeability alteration may create variable time-density curves for interstitial (extravascular) enhancement

Neuroradiology

Double Dose of Contrast

Figure 5-9-4

Steroid Suppression of Enhancement [Figure 5-9-4]

MR vs. CT [Figures 5-9-5 and 5-9-6] Figure 5-9-5

Steroids may reduce or completely suppress visible enhancement

Enhancement on CT and MR are similar – except for intraarterial and pachymeningeal (dural) enhancement

What makes the BBB?

Figure 5-9-6

Semi-permeable Capillaries •

• •

Brain ➢ Blood-brain-barrier Testicle ➢ Blood-Testicle barrier Ovary ➢ Blood-Ovary barrier

Ultrastructure of BBB [Figure 5-9-7] •

•

Neural capillary ➢ astrocytic feet ➢ continuous BM ➢ tight junctions ➢ no pinocytosis Non-neural or ABBB ➢ no astrocytic feet ➢ fenestrated BM ➢ intercellular gaps ➢ pinocytosis

Figure 5-9-7 Enhancement on CT and MR are similar – except for intraarterial and dural enhancement

Schematic of Ultrastructure of blood-brain-barrier

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The Berlin Wall and the BBB [Figure 5-9-8] •

• • •

•

Figure 5-9-8

Who built the Berlin Wall? ➢ The East Germans Why? ➢ To keep out the West Germans But … the barrier works in BOTH directions Some things are kept out ➢ Drugs, Contrast material Some things are kept in: ➢ Hemosiderin ➢ Vasogenic Edema

CNS: Normal Tissues w/o BBB • • • • • •

DURA (falx and tentorium) ARACHNOID ? (it’s avascular) CHOROID PLEXUS PINEAL GLAND (epiphysis) PITUITARY GLAND (hypophysis) CTZ (area postrema of medulla oblongata) ➢ one of the "Circumventricular Organs"

Hemosiderin cannot be cleared from the brain and spinal cord because of the blood-brain-barrier

Physiologic Why? •

•

Why do we have a BBB? ➢ To protect the brain ➢ To create the ionic environment for nerve conduction Why do we have tastebuds? ➢ So that we eat things good for us ❖ Salt ❖ Sweet ❖ Sour ❖ Umami (MSG) ❖ Bitter

Normal Enhancement • • • •

• • • •

Choroid Plexus Pineal Pituitary Stalk Pituitary Gland (anterior and posterior) ➢ Hypophyseal Portal System Cavernous sinus and dural reflections Nasal turbinates Sinonasal mucosa Extracranial muscles and mucosa

Plain vs. Enhanced Fat -Suppressed T1W - Gd Nasal Cycle • • • • • • • •

Vasocongestion ~/~ vasoconstriction 6 – 8 hour cycle alternation Humidify and warm the air Secrete mucus (1 – 2 liters/day) Chronic vasocongestion would cause submucosal edema Breathe mostly through the vasoconstricted side (~ 75%-85%) Yogi’s can control which nostril So can Tom Cruise (Minority Report)


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Fat -Suppressed T1W – Gd

Figure 5-9-9

Musk Ox – Nasal Turbinates Fat -Suppressed T1W - Gd [Figure 5-9-9] Cranial Nerve Enhancement • •

•

Optic Nerve – Never normal * Seventh Nerve: ➢ Inside facial canal – Yes, asymmetric ~70% ➢ Geniculate ganglion – 98% ➢ Tympanic > labyrinthine > mastoid ➢ May represent perineural vessels Eighth Nerve – Never normal

Abnormal optic nerve enhancement – optic neuritis

* Neuroradiology 1997 Mar;39(3):207-12.

Figure 5-9-10

Plain vs. Enhanced [Figure 5-9-10] Contrast Enhancement – Phases ANGIO(I–) R-N (Tc+) C.T. (I–) MRI (Gd+)

VASCULAR ++++ + (flow) + +/–

BBB – + (static) +++ +++

Enhancement vs. Edema

Normal pineal enhancement. Visualization depends on the timing of injection and the molecular weight of the contrast


Morphologic Patterns ➢ Homogeneous (solid) ➢ Heterogeneous (non-uniform) ➢ Ring (unilocular/multilocular) ➢ Serpentine ("Gyriform“) ❖ Serpiginous?

Serpiginous •

“A creeping skin eruption.”


Location ➢ SUPERFICIAL (CORTICAL/GYRAL) ➢ GREY-WHITE JUNCTION ➢ DEEP WHITE MATTER ➢ PERIVENTRICULAR, EPENDYMAL

Neuroradiology

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Figure 5-9-11

Cortical/Gyriform ➢ Cerebral Ischemia / Infarction ➢ CSF or sub-pial spread ➢ Meningo-encephalitis ➢ S.A.H. ➢ Leptomeningeal Malformation (SW) ➢ Meningioangiomatosis (NF2)

Listeria Monocytogenes [Figure 5-9-11] CNS Bacterial Infections •

•

•

Meningitis - Listeria Monocytogenes

Birth to Four Weeks ➢ 2-10 cases / 10k births ➢ Group B streptococcus ➢ E. coli ➢ Listeria monocytogenes 3 mo. to 3 yrs ➢ Haemophilus influenzae (Type B) ➢ Strep pneumoniae ➢ Meningococcus (Neisseria meningitidis) Over 3 yrs to Adult ➢ Strep pneumoniae ➢ N. meningitidis

CSF Signal ? [Figure 5-9-12]

Figure 5-9-12

Flair shows “dirty CSF” from protein and pus in the SAS (Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)

Figure 5-9-13

Enhancement? [Figure 5-9-13] •

Leptomeningeal Enhancement Pneumococcal Meningitis

Pachymeningeal Enhancement •

[Figure 5-9-14]

Intracranial Hypotension

Figure 5-9-14 Multiple symmetric areas of abnormal leptomeningeal enhancement from meningitis Notice the abnormal ehancement of the entire suprasellar cistern (Zulmarie Roig, MD, Gil Gonzalez, MD, MGH)

Pachymeningeal enhancement (Courtesy Laszlo Mechtler, DNI)


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Hemorrhagic Infarction [Figure 5-9-15]

Figure 5-9-15

Ischemic Enhancement •

•

•

Acute and/or Reperfusion enhancement ➢ True “luxury perfusion” 2° to acidosis ➢ BBBB after 4-6 hours of ischemia Subacute to Chronic enhancement ➢ Ingrowth of capillaries from surface ➢ Primarily in GM (cortex and deep) ➢ Peak intensity at 2-3 weeks ➢ Fades away over weeks to months Atrophy replaces Enhancement

Blood Brain Barrier [Figure 5-9-16] • • • • • •

Hemorrhagic infarction shows early and dense enhancement due to reperfusion

Cirrhosis Hyperbilirubinemia Bilirubin bound to Albumin Albumin can’t cross the Blood-brain-barrier BBB is abnormal in infarct Mostly gray-matter

Figure 5-9-16

H&P •

Pt is a 25 yo woman, PMHx of BCP, presenting w/ acute mental status changes, afebrile Actually… • Pt is a 34 yo marine stationed at Guantanamo Bay Cuba, presenting w/ acute mental status changes, febrile

HSV Encephalitis [Figure 5-9-17] Figure 5-9-17

Gyral enhancement in Sturge-Weber disease

Figure 5-9-18

Herpes encephalitis

Meningioma – Dural Tail [Figure 5-9-18] Dural Tail • • • • •

Meningioma - Dural Tail

Curvilinear enhancement AKA “dural flair” First reported w/meningioma First reported to be neoplastic invasion What is it REALLY? ➢ Thickening of the dura ➢ Vasocongestion of the dura ➢ Edema of the dura

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Contrast Enhancement [Figure 5-9-19] •

Figure 5-9-19

Ring Lesion ➢ Circumferential or peripheral/marginal enhancement, surrounding a central nonenhancing region. ➢ In turn, this is often surrounded by a large area of “edema”. ➢ May be unilocular or multilocular.

Rules for Ring Enhancing Masses [Figure 5-9-20]

Abscess

Figure 5-9-20

Differential appearance of ring enhancing lesions

Contrast Enhancement • • • •

Contrast leaks into interstitium from vessels without BBB Remains localized within millimeters of where it leaks out Not “simple diffusion” but rather “BULK FLOW” at a very slow rate (Glacier Not River)


Ring Lesions Differential ➢ M – Metastasis, MS ➢ A – Abscess (Also Cerebritis) ➢ G – Glioblastoma, Granuloma ➢ I – Infarct (Esp. Basal Ganglia) ➢ C – Contusion (Rare) ➢ A – AIDS (Toxo, Etc.) ➢ L – Lymphoma (in Aids) ➢ D – Demyelination (Active) ➢ R – Resolving Hematoma Radiation Change (Necrosis)


Ring Lesion Features For Infection ➢ ORGANIZED ABSCESS ➢ thin and uniform wall (3-7mm.) ➢ smooth inner margin does not “fill in” on CT, MR, even after time delay imaging ➢ CEREBRITIS (infection w/o organization): ➢ variable wall (may be smooth) smooth/variable inner margin ➢ often has “fill-in” on DDD ➢ (w/o fluid level)


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Contrast Enhancement- Abscess • •

• • •

Figure 5-9-21

2 – 4 wks. for ORGANIZED WALL 2 LAYERS ➢ inner MESENCHYMAL (capillaries,fibroblasts, collagen) ➢ outer ASTROGLIAL (reactive astrocytes) WALL facing GM is well formed 3-5 mm WALL FACING WM IS THINNER/WEAKER (Daughter Abscess) Ventricular Spill (“pyocephalus”)

Abscess [Figures 5-9-21 to 5-9-23] • • • • • • •

Round Smooth Regular Convex all around Rim of Edema Restricted Diffusion MRS shows ➢ AA peaks ➢ Acetate ➢ Succinate

Figure 5-9-22

Cerebral abscess in thalamus

Figure 5-9-23

Abscess. Viscous Pus and Coagulation Necrosis cause restricted diffusion

Figure 5-9-24 Abscess

Contrast Enhancement [Figure 5-9-24] • •

•

Ring Lesion Features For Neoplasm NECROTIC NEOPLASM: ➢ thick and irregular wall ➢ shaggy inner margin (usually) ➢ may “fill in” heterogeneously on DDD CYSTIC NEOPLASM: ➢ thin wall +/– MURAL NODULE ➢ PART OF WALL MAY NOT ENHANCE ❖ smooth inner margin ❖ uniform fluid enhancement ❖ or FLUID LEVEL

Neuroradiology

Ring enhancing lesion: Glioblastoma

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Glioblastoma Multiforme

Figure 5-9-25

Pilocytic Astrocytoma [Figure 5-9-25] •

Cyst with mural nodule?

Tumefactive Demyelination [Figure 5-9-26]

Open (Incomplete) Ring Sign • •

Demyelinating Disease Fluid-secreting (“Cystic”) Neoplasms

Pilocytic Astrocytoma

Masdeau JC, Moreira J, Trasi S, Visintainer P, Cavaliere R, Grundman M: The open ring. A new imaging sign in demyelinating disease. J Neuroimaging 1996; 6(2):104-107.

Figure 5-9-26

Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P: Open-ring imaging sign: highly specific for atypical brain demyelination. Neurology 2000; 54(7):1427-1433

Contrast Enhancement: Hematoma • •

EARLY: Hyperdense, round/oval homogeneous mass of RBC’s with proportional mass effect for volume Edema “Halo”, not spreading LATER: Iso-/Hypodense, smaller Reactive capillaries form outside Uniform rim of enhancement May see “vasogenic” edema spreading

Hematoma – Halo of serum [Figure 5-9-27]

Tumefactive Demyelination

Figure 5-9-28

Figure 5-9-27

Acute Hematoma - halo of edema Subacute to chronic may have vasogenic edema

Acute Hematoma - halo of edema Subacute to chronic may have vasogenic edema

Figure 5-9-29

Reactive Ring Enhancement [Figure 5-9-28] MCA infarct [Figure 5-9-29] Ring Enhancing Mass •

•

Benign ➢ Round ➢ Smooth ➢ Thin wall Malignant ➢ Undulating ➢ Irregular ➢ Thick wall


MCA infarct involving cortex and basal ganglia

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Neuroradiology

Post-Operative Enhancement •

• • •

•

Figure 5-9-30

RESIDUAL TUMOR ➢ Left behind RECURRENT TUMOR ➢ It grew back Infection Normal Postoperative Change ➢ surgical “trauma”, healing, gliosis Radiation Necrosis

Contrast Enhancement – Surgical Change and/or Residual Neoplasm? [Figure 5-9-30] •

• •

•

Surgical Enhancement typically after 24-48 hrs ➢ Scan early (24 hours) or scan late (4-6 wks) ➢ May fade after a few weeks but may last for months ➢ Gd+ enhancement may begin in 4-6 hours In the Operative Bed ➢ Mixed w/ residual tumor? ➢ Along the Margins of Resection ➢ Thin and uniform in brain (CT/MR) LINEAR meningeal/dural enhancement on MR ➢ Not lumpy-bumpy Small amts of air, blood are normal ➢ No instruments or sponges, etc.!!

Normal enhancement after ventricular shunt catheter insertion

References 1. 2. 3. 4. 5.

6. 7. 8. 9.

10. 11.

12. 13. 14.

Ahmadi J, Hinton DR, Segall HD, Couldwell WT. Surgical implications of magnetic resonance-enhanced dura. Neurosurgery. 1994 Sep;35(3):370-7;discussion 377. Aoki S, Sasaki Y, Machida T, Tanioka H. Contrast-enhanced MR images in patients with meningioma: importance of enhancement of the dura adjacent to the tumor. AJNR Am J Neuroradiol. 1990 Sep-Oct;11(5):935-8. Asari S, Yabuno N, Ohmoto T. Magnetic resonance characteristics of meningiomas arising from the falcotentorial junction. Comput Med Imaging Graph. 1994 May-Jun;18(3):181-5. Ekinci G, Akpinar IN, Baltacioglu F, et al. Early-postoperative magnetic resonance imaging in glial tumors: prediction of tumor regrowth and recurrence. Eur J Radiol 2003; 45:99-107. Goldsher D, Litt AW, Pinto RS, Bannon KR, Kricheff II. Dural "tail" associated with meningiomas on Gd-DTPAenhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment. Radiology. 1990 Aug;176(2):447-50. . Helie O, Soulie D, Sarrazin JL, Derosier C, Cordoliani YS, Cosnard G. [Magnetic resonance imaging and meningiomas of the posterior cerebral fossa. 31 cases] J Neuroradiol. 1995 Dec;22(4):252-70. French. Henegar MM, Moran CJ, Silbergeld DL. Early postoperative magnetic resonance imaging following nonneoplastic cortical resection. J Neurosurg 1996; 84:174-179. Hutzelmann A, Palmie S, Buhl R, Freund M, Heller M. Dural invasion of meningiomas adjacent to the tumor margin on Gd-DTPA-enhanced MR images: histopathologic correlation. Eur Radiol. 1998;8(5):746-8. Hutzelmann A, Palmie S, Freund M, Buhl R, Heller M. [Dura thickening adjacent to intracranial, para-dural spaceoccupying lesions in MRI. Histologic correlation] Aktuelle Radiol. 1997 Nov;7(6):305-8. German. PMID: 9467021 Hutzelmann A, Palmie S, Zimmer C, Benz T, Leweke F, Freund M. [The meningeal sign: a new appraisal] Rofo. 1996 Apr;164(4):314-7. German. Ildan F, Tuna M, Gocer AP, Boyar B, Bagdatoglu H, Sen O, Haciyakupoglu S, Burgut HR. Correlation of the relationships of brain-tumor interfaces, magnetic resonance imaging, and angiographic findings to predict cleavage of meningiomas. J Neurosurg. 1999 Sep;91(3):384-90. Kaufman BA, Moran CJ, Park TS. Computer tomographic scanning within 24 hours of craniotomy for a tumor in children. Pediatr Neurosurg 1995; 22:74-80. Kawahara Y, Niiro M, Yokoyama S, Kuratsu J. Dural congestion accompanying meningioma invasion into vessels: the dural tail sign. Neuroradiology. 2001 Jun;43(6):462-5. Lai PH, Ho JT, Chen WL, et al. Brain abscess and necrotic brain tumor: discrimination with proton MR spectroscopy and diffusion-weighted imaging. AJNR Am J Neuroradiol 2002; 23:1369-1377.

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15. Martin-Duverneuil N, Sola-Martinez MT, Miaux Y, et al. Contrast enhancement of the facial nerve on MRI: normal or pathological? Neuroradiology 1997; 39:207-212. 16. Masdeau JC, Moreira J, Trasi S, Visintainer P, Cavaliere R, Grundman M: The open ring. A new imaging sign in demyelinating disease. J.Neuroimaging 1996; 6(2):104-107. 17. Masdeu JC, Quinto C, Olivera C, Tenner M, Leslie D, Visintainer P: Open-ring imaging sign: highly specific for atypical brain demyelination. Neurology 2000; 54(7):1427-1433. 18. Nagele T, Petersen D, Klose U, Grodd W, Opitz H, Voigt K. The "dural tail" adjacent to meningiomas studied by dynamic contrast-enhanced MRI: a comparison with histopathology. Neuroradiology. 1994 May;36(4):303-7. 19. Nakasu S, Nakasu Y, Matsumura K, Matsuda M, Handa J. Interface between the meningioma and the brain on magnetic resonance imaging. Surg Neurol. 1990 Feb;33(2):105-16. 20. Nakau H, Miyazawa T, Tamai S, Tsuchiya K, Shima K, Shirotani T, Chigasaki H. Pathologic significance of meningeal enhancement ("flare sign") of meningiomas on MRI. Surg Neurol. 1997 Dec;48(6):584-90; discussion 590-1. 21. Quekel LG, Versteege CW. The "dural tail sign" in MRI of spinal meningiomas. J Comput Assist Tomogr. 1995 Nov-Dec;19(6):890-2. 22. Sakai K, Tada T, Fukasaku K, Kyoshima K, Kobayashi S. Histological examination of the gadolinium-enhanced dura mater around meningiomas on magnetic resonance imaging. Neurol Med Chir (Tokyo). 1993 Jul;33(7):42933. 23. Sato M, Matsumoto M, Kodama N. Meningeal enhancement surrounding meningiomas on Gd-DTPA MRI. Fukushima J Med Sci. 1998 Jun;44(1):1-11. 24. Sato N, Bronen RA, Sze G, et al. Postoperative changes in the brain: MR imaging findings in patients without neoplasms. Radiology 1997; 204:839-846. 25. Sekiya T, Manabe H, Iwabuchi T, Narita T. [The dura mater adjacent to the attachment of meningiomas: its enhanced MR imaging and histological findings] No Shinkei Geka. 1992 Oct;20(10):1063-8. Japanese. 26. Takeguchi T, Miki H, Shimizu T, Kikuchi K, Mochizuki T, Ohue S, Ohnishi T. The dural tail of intracranial meningiomas on fluid-attenuated inversion-recovery images. Neuroradiology. 2004 Feb;46(2):130-5. Epub 2004 Jan 28. 27. Wilms G, Lammens M, Marchal G, Van Calenbergh F, Plets C, Van Fraeyenhoven L, Baert AL. Thickening of dura surrounding meningiomas: MR features. J Comput Assist Tomogr. 1989 Sep-Oct;13(5):763-8. 28. Yamaguchi N, Kawase T, Sagoh M, Ohira T, Shiga H, Toya S. Prediction of consistency of meningiomas with preoperative magnetic resonance imaging. Surg Neurol. 1997 Dec;48(6):579-83.


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Radiologic Grading of Astrocytoma and The WHO 2000 Brain Tumor Classification James G. Smirniotopoulos, MD Brain Neoplasia: Frequency [Figure 5-10-1]

Figure 5-10-1

Childhood CNS Tumor Demographics •

367 Syrian children, collected from 1993-2002 ➢ Supratentorial - 47% ➢ Infratentorial - 53% ➢ Male 52%:48% Female ➢ Overall Incidence: ❖ Medulloblastoma 27% ❖ Astrocytoma 26% ❖ Craniopharyngioma 14% ➢ Posterior Fossa Only: ❖ Medullo (PNET) 54% ❖ Astrocytoma 23% ❖ Ependymoma 17%

The frequency of the various primary central Kadri H,Mawla AA, Murad L: Incidence of childhood brain nervous system tumors ranges from 2% for tumors in Syria (1993-2002) Pediatric Neurosurgery meningioma and mixed oligoastrocytoma to 40% 2005; 41:173-177 for glioblastoma multiforme (GBM) and 42% for infiltrative astrocytoma Pediatric Posterior Fossa • • •

454 posterior fossa patients All under the age of 18 402 tumors: ➢ 37.1% Cerebellar astrocytoma (149) ➢ 34.6% Medulloblastoma (PNET) (139) ➢ 11.4% Brain stem astrocytoma (46) ➢ 7% Ependymoma (28) ➢ 9.9% "other" (40)

Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439.

Traditional Tumor Grading •

• •

PATHOLOGIST ➢ LOW GRADE ➢ HIGH GRADE RADIOLOGIST ➢ NON-ENHANCING ➢ ENHANCING NEUROSURGEON ➢ “SUCKABLE” ➢ “NON-SUCKABLE”

Kernohan-Sayre (AFIP) Grading System: • • •

• •

GRADE I – “BENIGN” or “Low-Grade” GRADE II – “BENIGN” or “Low-Grade” GRADE III – ANAPLASTIC ➢ atypia, pleomorphism, mitoses, etc. GRADE IV- MALIGNANT ➢ Mitoses, Vascularity, Endothelial changes ➢ Necrosis ➢ Glioblastoma Multiforme NOTE: Numerous modifications exist, most into three grades, e.g..: Low Grade (Benign), Anaplastic, and GBM (w/ NECROSIS).

Neuroradiology

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The WHO 2000 Brain Tumor Classification

Pathologic – Radiologic Correlation Pathology

Radiology

Cellularity

T2 SI, DWI & ADC

Endothelial proliferation and Vascularity Necrosis

Enhancement, PWI, and Permeability Imaging Ring Lesion, MRS, DWI & ADC

Hemorrhage

T1 and T2 SI

Labeling Indices

MRS, Th 201 and FDG

Infiltration

T1 and T2 SI, DTI

ASTROCYTOMA: Five Year Survival Grading Systems: Sem Rad Onc (1991); 1: 2-9 Kernohan

Berger

Benign (1)

Astrocytoma

WHO 2000 1 Pilocytic,SEGA 2 Astrocytoma

Benign (2) Anaplastic

3 Anaplastic

Glioblastoma

4 Glioblastoma

Anaplastic (3) Glioblastoma (4)

Define the Problem • • • •

Some Low Grade Enhance Some Low Grade Do Not Some Low Grade => GBM Some Low Grade Do Not

WHO Classification • • • •

Defines Histologic Subtypes Grades Biologic Potential Allows International Cooperation Ascending scale of Aggression from 1-4

WHO Correlation •

Low Grade ➢ Long-Term Survival: Possible Cure ➢ Stable History (No Progression): Possible Cure

WHO Grading CNS Tumors GRADE 1 GRADE 2 GRADE 3 GRADE 4

JPA PXA PXA GBM

SGCA

GANG

MENING HPC HPC

ANAPLASTIC

CNS NEOPLASM-GLIAL: Prognostic Factors • • •

Location Age Histology


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“BENIGN” ASTROCYTOMA •

Figure 5-10-2

Two types: ➢ Low grade (“benign”) ❖ Diffuse (Adults) ➢ Low grade “special” ❖ Circumscribed (Children)

Normal Diffuse Astrocytoma [Figure 5-10-2] WHO Gr1 - Pilocytic Astrocytoma [Figure 5-10-3] Circumscribed vs Diffuse Circumscribed Astrocytoma

Diffuse astrocytoma: Individual neoplastic cells spread out through the white matter. In WHO Gr 2, this is only noted as “increased cellularity”

Astrocytoma: Circumscribed •

• • •

“Special” astrocytomas ➢ Astrocytoma of young ➢ Various locations ➢ Well circumscribed (yet, no capsule) Do NOT spread along WM Do NOT change grade (except PXA) Constellation of findings correlates w/ Histology

Figure 5-10-3

Pilocytic Astrocytoma •

Cystic Cerebellar Astrocytoma Juvenile Pilocytic Astrocytoma (“PA” or “JPA”)

Pilocytic Astrocytoma • • • • • • • •

Synonyms: Polar Spongioblastoma, Cystic Cerebellar Astrocytoma Cell of Origin: Astrocyte (bi-polar, hairlike) Associations: in ON w/ NF-1 Incidence: 3%–6% of ALL Cranial, 32% of Child Age: 5–15 (Zulch 3–7) Sex: Slight F (11/9) Location: Cerebellum, Chiasm/Hypothal, Optic Treatment: Surgery, patience Prognosis: 77% at 5 yrs, 75% at 10 yrs, 75% at 15 yrs

Circumscribed astrocytoma, like pilocytic astrocytoma, have “pushing margins” and are often fluid-secreting

Pilocytic Astrocytoma: Radiology •

[Figures 5-10-4 to 5-10-6]

•

• •

Cerebellum, Diencephalon ➢ rare in BS or Cerebrum Majority have significant “cyst” ➢ “Cyst and Mural Nodule” ❖ part of lining does NOT enhance ➢ Nodule may be heterogeneous ➢ Exceptional purely solid Nodule NOT hyperdense Calcification in 5%–25%

Figure 5-10-4

Pilocytic astrocytoma with classic “cyst and nodule” morphology Neuroradiology

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Figure 5-10-5

Figure 5-10-6

Gross picture of pilocytic astrocytoma Pilocytic astrocytoma with classic “cyst and nodule” morphology

Figure 5-10-7

WHO Gr1 - Pilocytic Astrocytoma Pathology •

• • •

Biphasic pattern ➢ dense pilocytic glia ➢ Rosenthal fibers ➢ loose microcystic areas No necrosis Low grade Abnormal capillaries ➢ allow enhancement ➢ fluid production

Pilocytic Astrocytoma

Figure 5-10-8

Grading Problems in Gliomas 51 Pilocytic (WHO Gr. 1) KERNOHAN MAYO-ST.ANNE 1 26% 1 2% 2 69% 2 55% 3 6% 3 35% 4 0% 4 8% • By conventional “feature counting” most pilocytic astrocytomas were overgraded.

Pilocytic Astrocytoma • •

Pilocytic Astrocytoma (Courtesy of Paul Sherman)

Variant Appearance Variant Location

Pilocytic Astrocytoma [Figure 5-10-7] • •

A Cyst with mural nodule? Not Always !!!

Figure 5-10-9

Pilocytic Astrocytoma [Figure 5-10-8] Pilocytic Astrocytoma: Locations [Figure 5-10-9] • • • • •

CEREBELLUM Chiasm And Optic Nerve Hypothalamus/thalamus Cerebral Hemisphere Spinal Cord (Intramedullary)

Pilocytic astrocytoma of hypothalamus The WHO 2000 Brain Tumor Classification

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•

Pilocytic Astrocytoma ➢ Enhance ➢ Cyst w/ Nodule Cystic ➢ Hypodense nodule ➢ Calcification ➢ NOT vascular ➢ Nodule location varies

Hemangioblastoma Enhance Solid Hyperdense nodule Never Ca++ Hypervascular, Flow Voids Nodule is “Subpial”

Figure 5-10-10

Pilocytic Astrocytoma (Juvenile Pilocytic) • • • • •

Childhood, Young Adults Benign, no mitosis/necrosis Circumscribed – Enhancing Cyst Formation, Mural Nodule Cerebellum and Diencephalon ➢ (Optic tracts, Hypothalamus)

WHO Grade I •

• •

Circumscribed Astrocytoma ➢ JPA (Pilocytic) ➢ SGCA (Subependymal Giant Cell) Ganglioglioma Meningioma

Subependymal Giant Cell Astro [Figure 5-10-10]

Subependymal Giant Cell Astrocytoma

Astrocytomas •

“SPECIAL” ASTROCYTOMAS ➢ Circumscribed Growth: ❖ Pilocytic ❖ Subependymal Giant Cell ❖ Pleomorphic Xantho-Astrocytoma

Circumscribed Astrocytoma Pleomorphic Xanthoastrocytoma • • • • • • •

Rare Variant of Astrocytoma Arises from Subpial Astrocytes Affects Superficial Cerebral Cortex and Meninges Skull erosion (scalloped excavation) Temporal > Frontal > Parietal WHO Grade 2,3 50% progress over time

Pleomorphic Xanthoastrocytoma •

IMAGING: ➢ CT APPEARANCE: ❖ Well-Circumscribed Hypodense or Cystic Mass ❖ Often Isodense Solid Nodule that Intensely Enhances ❖ May Mimic Juvenile Pilocytic Astrocytoma ❖ Calcifications Rare

Pleomorphic Xanthoastrocytoma •

MR APPEARANCE: ➢ Well-Circumscribed Mass of Variable Size ➢ Superficial Cortical Location ➢ T1: Low/Mixed Signal, ➢ T2: High/Mixed Signal ➢ Often with Cystic Component ➢ Solid Portion Intensely Enhances ➢ Adjacent Meninges May Enhance (Tail) ➢ Little or No Mass Effect

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Astrocytomas •

“Ordinary” Astrocytoma ➢ Diffuse Infiltration of WM by: ❖ Fibrillary Astrocytes ❖ Protoplasmic Astrocytes ❖ Gemistocytic Astrocytes ❖ WHO 2,3,4 (NOT 1) ❖ KS & Mayo Grades 1–4

Where do Glioblastomas come from?

• Progressive Transformation from lower grade diffuse astrocytoma - OR • Arise de novo

Diffuse Astrocytoma … too many cells ! •

Mild cellular atypia

KERNOHAN (KS) ANAPLASIA

1

2

3

4

0

Min

>1/2

Marked

CELLULARITY

Mild

Mild

Inc

Marked

MITOSIS

0

0

Plus

Marked

Min

Min

Marked

ENDOTHELIAL 0 Proliferation NECROSIS TRANSITION ZONE

Marked

Figure 5-10-11

Astrocytoma: Diffuse (Fibrillary, protoplasmic, etc.) • • • • • •

“Adult type” or “Hemispheric” Astrocytoma Diffusely infiltrate brain, along WM tracts Continuum, from low-grade to high-grade Genetic Alterations 17 => 9 => 10 Many Progress in Histology over time, changing from WHO Gr. 2 => Gr. 3 => Gr. 4 (GBM) Imaging tends to correlate with histology, especially at the ends of spectrum

Astrocyte Mutation [Figure 5-10-11] Successive mutations in Astrocytoma

Diffuse Astrocytoma [Figures 5-10-12 and 5-10-13] Figure 5-10-12

All three grades of astrocytoma in one patient The WHO 2000 Brain Tumor Classification

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Diffuse Astrocytoma • • • •

Figure 5-10-13

‘Astrocytoma’ Anaplastic Astrocytoma Glioblastoma Multiforme The Eastwood Method: ➢ The Good ➢ The Bad ➢ The Ugly

Astrocytoma: Radiologic Grading •

• •

TYPE 1 – WHO 2, KS Grade 1–2, “Benign” ➢ Homogeneous ➢ No Enhancement, No Vasogenic Edema TYPE 2 – WHO Grade 3, Anaplastic ➢ Variable Enhancement, Edema ➢ 50% enhance – 50% don’t TYPE 3 – WHO Grade 4 Glioblastoma ➢ Heterogeneous (Necrosis, Blood) ➢ Ring Enhancement, Edema

Diffuse astrocytoma is a spectrum of tumors, pathologically and by imaging. The classification into three grades (WHO 2,3, or 4) may be an artificial segmentation along a continuum

“Benign” Astrocytoma: WHO 2, KS 1–2, Mayo 1 •

•

YOUNGER PATIENT ➢ CHILDHOOD ➢ Young Adults (20’s – 40’s) NL VESSELS (NO NEOVASCULARITY) ➢ BBB INTACT ➢ NO EDEMA ➢ NO ENHANCEMENT ➢ NO TUMOR VESSELS

Figure 5-10-14

Benign – Diffuse •

HOMOGENEOUS ➢ NO NECROSIS ➢ NO HEMORRHAGE ➢ INCREASED WATER ❖ DARK and Poorly Demarcated on CT ❖ Dark and Sharp on T1W ❖ BRIGHT and Sharp on T2W ➢ MICROCYST >>> MACROCYST (macrocysts occur in JPA, etc.)

PD

T2 Gr 2 Fibrillary Astrocytoma

Gr 2 Fibrillary Astrocytoma [Figures 5-10-14 and 5-10-15]

Figure 5-10-15

T1T1gad non Gr 2 Fibrillary Astrocytoma – no enhancement after gadolinium Neuroradiology

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Gr 2 Astrocytoma: PWI [Figure 5-10-16] •

Figure 5-10-16

Reduced perfusion

Gliomatosis Cerebri [Figure 5-10-17] Figure 5-10-17 T1-gad

T2

Female, acute stroke (3 days), MCA occlusion

Figure 5-10-18 Gliomatosis Cerebri – a diffuse astrocytoma infiltrating two or more lobes of the brain

Gliomatosis Cerebri: Diffuse Astrocytoma – 2 lobes [Figure 5-10-18]

Spread along White Matter Tracts [Figure 5-10-19]

Figure 5-10-19 Gliomatosis Cerebri – a diffuse astrocytoma infiltrating two or more lobes of the brain

Figure 5-10-20

Diffuse Grade 2 astrocytoma spreading through white matter, including corpus callosum

Astrocytoma: Microcystic change

Gliomatosis Cerebri Astrocytoma: Microcystic Change [Figure 5-10-20]

Figure 5-10-21

Astrocytoma [Figure 5-10-21]

Pontine astrocytoma – WHO Gr 2 The WHO 2000 Brain Tumor Classification

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Modes of Spread • • • •

1. 2. 3. 4.

Figure 5-10-22

Natural passages Along surfaces Along tracts Across the meninges

Spread Along Tracts: • • • • •

Corona Radiata Peduncles Corpus Callosum Anterior Commisure Arcuate Fibres

Astrocytes Track Along WM

Progressive transformation of WHO Grade 2 astrocytoma to Glioblastoma multiforme

Pontine Astrocytoma Figure 5-10-23

Pontine Astrocytoma: WHO 2 WHO 2 … GBM [Figure 5-10-22] Expanded Brain Anaplastic Astrocytoma: Overall Characteristics • • • • • •

Grade III malignant glioma Anaplastic astrocytoma – no enhancement in this example – Less aggressive than GBM, malignant about 50% will enhance with somewhat better prognosis Frequency: highest in young adults (30 – 40 years) Recurrence: often as a higher-grade glioma Challenge: difficult to remove completely with surgery Median survival: 3 – 4 years

Anaplastic Astrocytoma [Figure 5-10-23] Anaplastic Astrocytoma ( WHO 3 ) •

Increased Cellularity, +/- minimal vascular changes, no necrosis , no hemorrhage

GBM - Glioblastoma “Malignant” Astrocytoma: •

•

•

Older patient ➢ 40’s and up ➢ exceptions (PNET) ➢ ~ 1/2 arise from previous low grade (2–3) Abnormal Vessels (neovascularity) ➢ BBB abnormality ➢ vasogenic edema ➢ contrast enhancement ➢ irregular vessels, shunting, etc. HETEROGENEOUS ➢ hemorrhage (old/new) ➢ tumor necrosis ➢ tumor itself

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Astrocytoma Gr4: Angiogenesis

Figure 5-10-24

Glioblastoma Multiforme [Figures 5-10-24 and 5-10-25] Figure 5-10-25 T2

T1-gad

Glioblastoma Multiforme. Photomicrograph at high power shows both angiogenesis and pseuopalisading necrosis

Glioblastoma Multiforme

Figure 5-10-26 (Gr 4) Glioblastoma: PWI-CBV [Figure 5-10-26] Glioblastoma – WHO Grade 4 Mechanisms of Enhancement Ultrastructure of BBB •

Neural capillary ➢ astrocytic feet ➢ continuous BM ➢ tight junctions ➢ no pinocytosis

ABBB or Non-neural no astrocytic feet fenestrated BM intercellular gaps pinocytosis Increased perfusion due to angiogenesis in a glioblastoma multiforme

GBM •

• •

Center of Abnl Density/Intensity ➢ variegated necrosis ENHANCING RIM ➢ hypercellular, fleshy neoplasm ➢ greatest neovascularity Corona of Abnl Density/Intensity ➢ “edematous” white matter ➢ areas of microscopic neoplastic infiltration

Figure 5-10-27

GBM - Glioblastoma Glioblastoma multiforme with pseudopalisading necrosis

Pseudopalisading Necrosis [Figure 5-10-27] Ring Lesion and Infiltration [Figure 5-10-28]

Figure 5-10-28

Schematic of Glioblastoma multiforme – there are neoplastic cells infiltrating into the surrounding white matter The WHO 2000 Brain Tumor Classification

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Glioblastoma Multiforme (Surrounding Zone of Infiltration) •

•

Figure 5-10-29

GBM arose from a preexisting low grade ➢ surrounding lower grade neoplasm ➢ may also transform over time GBM arose de novo ➢ sends cells to invade the brain

Terrorist Cells Infiltrate Brain Glioblastoma Multiforme GBM - Multifocal [Figure 5-10-29] DWI of Glioblastoma (Gr 4) Multifocal glioblastoma multiforme. Microscopic infiltration does not always produce macrosopic changes that are visible on CT, MR, or even at gross pathology

Ring Enhancing Mass Ring Lesion Differences [Figure 5-10-30]

Figure 5-10-31

Figure 5-10-30

Glioblastoma Multiforme vs. Abscess (toxoplasmosis). The abscess rim is thinner, without a shaggy inner margin

Glioblastoma Multiforme vs. Abscess (toxoplasmosis). The viscous pus and white cell infiltrate in the abscess causes restricted diffusion – bright on DWI

DWI: Necrosis vs. PUS [Figure 5-10-31] Glioblastomas: Growth/Spread

Figure 5-10-32 Glioblastoma: Ependymal spread GBM [Figure 5-10-32] GBM – Thicker on Surface

Two different patients with GBM – the tumor flourishes when it reaches the rich vascularity of the cerebral cortex

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X-Ray Perfusion Imaging [Figure 5-10-33]

Figure 5-10-33

MR Perfusion Imaging [Figure 5-10-34] GBM with increased rCBV New Tools for Grading and Staging •

•

Radiology ➢ Perfusion Imaging rCBV and rCBF ➢ Diffusion Imaging, ADC and DTI ➢ Spectroscopy ➢ PET/SPECT ➢ Monoclonal Ab. Pathology ➢ Labeling Index ➢ Chromosome Analysis ➢ Histochemical ➢ Electron Microscopy

Glioblastoma multiforme. The early draining veins reflect increased perfusion and a shortened mean transit time (MTT)

Figure 5-10-34

DTI and Tumor Imaging Astrocytes Track Along WM Glioblastoma Multiforme Two Port Radiotherapy •

•

Bad News ➢ Can’t define full extent of tumor by any current test Good News ➢ 90% of tumor recurrence within 2cm of enhancing rim

Glioblastoma multiforme

Figure 5-10-35

Glioblastoma Multiforme [Figure 5-10-35] Define the Problem • • • • • •

Some Low Grade Enhance* Some Low Grade Do Not Some Low Grade => GBM Some Low Grade Do Not* *These are the Circumscribed Astrocytomas The others are the Diffuse Astrocytomas

WHO Astrocytoma Summary Butterfly glioma – Glioblastoma multiforme References 1. 2.

3. 4.

Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (1993-2002) Pediatric Neurosurgery 2005; 41:173-177 Levin VA, Leibel SA, Gutin PH. Neoplasms of the central nervous system. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds. Cancer: Principles & Practice of Oncology. Vol 2. 5th ed. Philadelphia, Pa: Lippincott-Raven Publishers; 1997:2022-2082. Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439. Pobereskin LH, Chadduck JB: Incidence of brain tumours in two English counties: a population based study. J Neurol Neurosurg Psychiatry 2000; 69: 464-471.


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Non-Astrocytic Gliomas James G. Smirniotopoulos, MD PRIMARY NEOPLASMS – Neuroectodermal •

•

Figure 5-11-1

Neuroectoderm – ➢ Embryologic Neural Tube ➢ “Neuroepithelial” Broad Categories ➢ Glial Tumors (GLIOMAS) ➢ Embryonal/Immature (P.N.E.T.’s) ➢ Neuronal (Neurocytoma) ➢ Mixed (Ganglioglioma)

Neuroectodermal Tumors • • • • • • • • • • • •

Astrocytoma Circumscribed Diffuse Ependymoma Choroid Plexus Tumors Oligodendroglioma Medulloblastoma (PNET) Atypical Rhabdoid Tumor Ganglioglioma Central Neurocytoma Lhermitte-Duclos Dysembryoplastic Neuroepithelial Tumor

Ependymoma – Gross Axial section

Figure 5-11-2

EPENDYMOMAS – Brain and Cord •

•

Cell of Origin: Ependyma ➢ Lining of ventricles ➢ Central canal/filum terminale ➢ “rests” in parenchyma Subtypes: ➢ Anaplastic/Malignant ➢ Immature (Ependymoblastoma) ➢ Myxopapillary (cauda equina)

EPENDYMOMA •

WHO Classification ➢ Ependymoma (WHO grade II) ❖ Variants: cellular, papillary, clear cell, tanycytic, mixed ➢ Anaplastic ependymoma (WHO gr III) ➢ Myxopapillary ependymoma ➢ Subependymoma

Ependymoma – heterogeneous central mass

Figure 5-11-3

EPENDYMOMAS – Demographics • • • • • •

5%–6% of All Intracranial 70% occur in Males 70% arise in the Fourth Ventricle 70% present in Childhood 70% of All Intramedullary 5 year survival – 50%

EPENDYMOMAS – Gross Pathology •

[Figures 5-11-1 to 5-11-5]

Soft Intracavitary Mass ➢ “cast of ventricle” ➢ extrude out foramina ➢ invade floor of 4th (pons/medulla)

Neuroradiology

Ependymoma – Enhancing and heterogeneous, small “cysts” and calcified chunks 1147 1149

Non-Astrocytic Gliomas

• •

Heterogeneous ➢ small/large cystic areas ➢ calcification (often chunks) Spinal Cord – sharply circumscribed

Figure 5-11-4

Figure 5-11-5

Ependymoma – Tumor extends into lateral recess of 4th ventricle

Figure 5-11-6

Ependymoma – Tumor extends into vallecula of cisterna magna

Schematic Ependymoma [Figure 5-11-6] Ependymoma [Figure 5-11-7] Ependymoma CPA Cistern [Figure 5-11-8] Figure 5-11-7

Schematic of Ependymoma filling lumen of fourth ventricle. Copyright 2004

Figure 5-11-8

Ependymoma in the cerebellopontine angle cistern Ependymoma filling lumen of fourth ventricle Non-Astrocytic Gliomas

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Extra-ventricular Ependymoma • • • •

Figure 5-11-9

[Figures 5-11-9 and 5-11-10]

•

More common in children More common in cerebral hemisphere Arise from *rests* of Ependymal Cells Better prognosis ➢ Not infiltrating ➢ Not communicating with CSF Often partially cystic

CHOROID PLEXUS NEOPLASMS – Introduction •

•

Normal Choroid Plexus (CP) ➢ Makes majority of CSF ➢ Forms papillary fronds: ❖ Vascular core ❖ Ependyma is modified into CHOROID EPITHELIUM Neoplasms: ➢ CP Papilloma (benign) WHO Grade 1 ➢ CP Carcinoma (malignant) WHO Grade 3

Extra-ventricular Ependymoma – Note fluid-fluid level formed by contrast layering within the cyst

Figure 5-11-10

CHOROID PLEXUS TUMORS • •

Choroid plexus papilloma (WHO grade I) Choroid plexus carcinoma (WHO grade III)

CHOROID PLEXUS NEOPLASMS – Demographics •

•

Tumor of Childhood: ➢ Uncommon ( Children (8:1) Age peak 35 – 45 yrs) Supratentorial – 85% Slow growth, Long Hx (10 years) ➢ Prognosis better with 1p and 19q mutations

Choroid plexus papilloma – Note pattern of bilateral hydrocephalus Non-Astrocytic Gliomas

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OLIGODENDROGLIOMA • • •

•

Figure 5-11-17

Gr 1 - Rare Gr 2 - Conventional oligodendroglioma Gr 3 - Anaplastic oligo: ➢ Hypercellularity, atypia, mitoses, endothelial proliferation, necrosis Gr 4 - GBM-like (rare) ➢ Not biologically equivalent to Gr 4 fibrillary astrocytoma

OLIGODENDROGLIOMA – Gross Pathology • • • • •

•

Arises in White Matter Grow toward cortex! Unencapsulated Not as infiltrating as astro. Heterogeneous ➢ myxoid areas (“cystic”) ➢ hemorrhage C A L C I F I C A T I O. N !

OLIGODENDROGLIOMA – Radiology •

• •

• •

Heterogeneous Hemispheric Mass ➢ Ca++, Cysts – “myxoid change”, Blood products Oligodendroglioma. CT shows very dense Extend to Cortex and infiltrate GM calcifications, highly suggestive and characteristic ➢ Gyriform or dot-dash Ca++ of oligodendroglioma ➢ Scalloped erosion inner table Figure 5-11-18 2/3 will enhance ➢ +/– anaplasia MR +/- special pulse sequences for Ca++ detection MR Spectroscopy? ➢ Potential for tumor grade, but not subtype or genetics

MR - What is it? Oligodendroglioma [Figure 5-11-17] •

CT Shows DENSE Ca++

Oligo-astrocytoma •

Nothing Specific … Looks like Diffuse Astrocytoma Oligodendroglioma – Heterogeneous peripheral mass that involves the cortex with thick curvilinear calcifications

Oligodendroglioma [Figure 5-11-18] •

CT Shows DENSE Ca++

Chickenwire Vascularity [Figure 5-11-19]

Figure 5-11-19

Fried Egg: round dark nucleus surrounded by a clear halo – this is an artifact of fixation Chicken-wire: The capillary vessels form a "chicken wire“ pattern around nests of cells Neuroradiology

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Oligodendroglioma •

Figure 5-11-20

Combined 1p/19q loss ➢ Associated with prolonged survival ➢ Response to PVC (procarbazine, vincristine CCNU® [Lomustine]) chemotherapy ➢ 50% volume decrease in 100% ➢ Median survival 10 yrs vs 2 yrs ❖ 95% 5 yr survival ➢ Most powerful predictor on multivariate analysis

Cairncross et al. J NCI 1998;90:1473 External granular cell layer – normal in fetus and infants (up to 12 months old)

Old Elephants Age Gracefully Oligo Epend Astro GBM •

90% 50% 25% 15% (Incidence of Ca++)

Oligodendroglioma MEDULLOBLASTOMA [Figure 5-11-20] •

•

•

Cell of origin: ➢ “medulloblast” – NOT! Bi-potential embryologic cells: ➢ Migrate from 4th to form CRBLL ➢ Glial and neuronal differentiation ➢ External Granular Cells (fetus) ➢ Internal Granular Layer (mature) Primitive Neuroectodermal Tumor - PNET

MEDULLOBLASTOMA – Demographics •

• • •

1st or 2nd most common cerebellar neoplasm in children ➢ 1/5 – 1/3 of ALL pediatric CNS ➢ M:F 1.1 – 2:1 May be congenital (present at birth up to 60 days) ➢ most (1/2) < 15 yrs. ➢ however, 1/3 present from 15–35 yrs. 5 year survival >> 50% => approached 75%-85% Primitive Neuroectodermal Tumor

Pediatric Posterior Fossa • • •

454 posterior fossa patients All under the age of 18 402 tumors: ➢ 37.1% Cerebellar astrocytoma (149) ➢ 34.6% Medulloblastoma (PNET) (139) ➢ 11.4% Brain stem astrocytoma (46) ➢ 7% Ependymoma (28) ➢ 9.9% "other" (40)

Parizek J, et al: Posterior cranial fossa surgery in 454 children. Childs' Nerv Syst 1998; 14:426-439.

Childhood CNS Tumor Demographics •

367 Syrian children, collected from 1993-2002 ➢ Supratentorial - 47% ➢ Infratentorial - 53% ➢ Male 52%:48% Female ➢ Overall Incidence: ❖ Medulloblastoma 27% ❖ Astrocytoma 26% ❖ Craniopharyngioma 14% ➢ Posterior Fossa Only ❖ Medullo (PNET) 54%


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❖ ❖

Astrocytoma 23% Ependymoma 17%

Kadri H,Mawla AA, Murad L: Incidence of childhood brain tumors in Syria (19932002) Pediatric Neurosurgery 2005; 41:173-177

Figure 5-11-21

MEDULLOBLASTOMA – Gross Pathology •

[Figures 5-11-21 and 5-11-22]

•

Arise from: ➢ post. (inf.) Medullary Vellum ➢ Vermis (midline cerebellum) Morphology: ➢ expansile, spherical, Unencapsulated ➢ post. 4th ventricle ➢ residual ANT. Crescent of CSF ➢ “HOMOGENEOUS” ❖ (Ca++. Cyst. Heme are UN-common) Medulloblastoma (PNET) – Rounded mass in the central posterior fossa

Figure 5-11-22

Figure 5-11-23

Medulloblastoma – a small, round, blue-cell tumor

RADIATION CHEMOTHERAPY • • •

Dividing Cells Neovascularity Pharmaceuticals ➢ Tested against murine leukemia ➢ Small round blue cell tumor ➢ Cis-platinum ❖ Dividing cells ❖ Electrical field ❖ Platinum electrodes

MEDULLOBLASTOMA – Micro Pathology •

Small Round “BLUE CELL” Tumor ➢ Immature, high Nuclear:Cytoplasm ➢ Both Neuronal and Glial features (occasional astrocytic differentiation) ➢ Form “Rosettes” (Homer-Wright) cells arranged in a circle surround core with linear fibrils ➢ Densely cellular ➢ Necrosis/Hemorrhage are not rare

Medulloblastoma (PNET) – Rounded mass in the central posterior fossa

MEDULLOBLASTOMA – Radiology [Figure 5-11-23] • •

•

Post. Fossa, Behind/in 4th vent. “HOMOGENEOUS” (he’s lying!) ➢ grossly uniform, ❖ but, finely heterogeneous ➢ hyperdense on CT (w/o Ca++) ➢ hypo-/isointense to GM on MR (cellularity and high N:C ratio) ➢ (Ca++ and “cysts” < 15%) Enhance with contrast

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Fourth Ventricle - Schematic [Figure 5-11-24]

Figure 5-11-24

Medulloblastoma (PNET) [Figures 5-11-25 and 5-11-26] Figure 5-11-25

Schematic of posterior fossa masses. Can you really determine where the mass originated when it is more than three centimeters in diameter? Copyright 2004

Medulloblastoma (PNET) – Rounded mass arising in the cerebellum – not from the 4th ventricle roof

Figure 5-11-26

Medulloblastoma •

[Figure 5-11-27]

• • • •

‘Homogeneous’ ➢ finely irregular Cyst and Hemorrhage are uncommon common and proximal internal carotid arteries Arch 4 ➢ Right --> regresses (portion of right subclavian) ➢ Left --> persists as LEFT AORTIC ARCH Arch 5 – rudimentary in humans Arch 6 – pulmonary arteries and ducti arteriosi ➢ Right regresses ➢ Left becomes ligamentum arteriosum Dorsal aortae ➢ Right regresses – part of RSCA ➢ Left becomes descending aorta

Figure 6-5-2

In the development of the normal left aortic arch, the gap or complete regression occurs in the 8th segment of the right dorsal aorta. The normal arch has 3 branches – the brachiocephalic (innominate), the left common carotid, and the left subclavian arteries

Vascular Rings and Slings

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Figure 6-5-3 Edwards postulated the existence of a double arch with bilateral ducti arteriosi in the embryo. This double arch can be represented by this ring. The T in the center represents the pulmonary arteries. The black lines connecting the main pulmonary arteries to the aorta represent the bilateral ducti arteriosi. The anterior center of the ring (dotted circle) represents the ventral or ascending aorta branching into bilateral arches which join to form the descending aorta in the posterior center of the ring (dotted circle). Each arch gives rise to its own carotid and subclavian artery. The anterior solid circles represent the bilateral carotid arteries, and the posterior solid circles are the bilateral subclavian arteries. The portion of the ring between the carotid and subclavian arteries is formed from the 4th pharyngeal arch. The portion of the ring dorsal to the subclavian artery represents the contribution of the 8th segment of the dorsal aorta. These are the most common sites of gaps or complete regression of a portion of the ring that give rise to the different types of aortic arches

Figure 6-5-4

Figure 6-5-5

Diagram representing the normal regression of the 8th segment of the right dorsal aorta and right ductus arteriosus in development of the normal left arch. The right subclavian artery is separated from the descending aorta and arises in common with the right common carotid artery from the ascending aorta

In the development of the aberrant right subclavian artery, there is early obliteration of the right 4th arch, and the 8th segment of the right dorsal aorta persists, so the right subclavian artery maintains its connection to the descending aorta, becoming the last branch from the aortic arch (arrow). Thus, there are four, rather than the normal three, branches of the aortic arch and there is no brachiocephalic (innominate) artery. The descending aorta is on the left, so the aberrant RSCA then crosses behind the esophagus to get to the right side

Aberrant RSCA [Figures 6-5-5 to 6-5-8] • • • • •

Asymptomatic in children – normal variant Due to early complete obliteration of right 4th arch and persistence of 8th segment of the right dorsal aorta Left arch RSCA originates distal to LSCA Posterior impression on the esophagus

Figure 6-5-6

The gap or obliteration occurs in the right 4th arch. The aortic arch is on the left

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Figure 6-5-7

Figure 6-5-8

When the RSCA crosses from left to right a posterior impression is created on the esophagus, as seen on the lateral view (left image). In the AP view (right image), a normal left aortic arch is seen as well as an oblique impression on the contrast-filled esophagus extending from the left arch to the right shoulder

The oblique course of the aberrant right subclavian artery creates a sloped contour of the aortic knob (arrow) that may be visible in older children and adults

Determining Side of Arch [Figures 6-5-9 and 6-5-10] • • •

Figure 6-5-10

Tracheal deviation, buckling or impression – the most reliable sign indicating the side of the aortic arch Asymmetric density of pedicles Descending aorta can be on right or left with right arch

Figure 6-5-9

PA chest radiograph showing rightsided impression on the trachea due to right aortic arch

Normal tracheal buckling away from the left arch on an expiratory chest radiograph (arrow)

Right Arch •

•

Branching patterns ➢ Aberrant LSCA ➢ Mirror image ➢ Isolated LSCA – congenital subclavian steal High association with congenital heart disease

Mirror Image Right Arch [Figures 6-5-11 and 6-5-12] • • • •

Association with CHD 98% 25% of patients with tetralogy of Fallot have mirror image right arch 35% of patients with truncus arteriosus have mirror image right arch 90% of patients with mirror image right arch have tet


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Figure 6-5-11

Figure 6-5-12

Patient with right-sided arch and descending aorta and enlarged heart with upturned apex, a classic plain film diagnosis of Tetralogy of Fallot

Figure 6-5-13 Diagram showing the mirror-image branching right arch from the front. The ductus is on the left (arrow) so this is not a ring. A rare variant with ductus arteriosus from proximal descending aorta coursing behind esophagus to left pulmonary artery, the so-called retro esophageal ductus, is a true ring

Aberrant LSCA [Figure 6-5-13] • • •

• • • • • •

Mirror image of aberrant RSCA Left ductus arteriosus completes the ring Symptomatic patients typically have a a tight ductus or large diverticulum of Kommerell (dilation of origin of aberrant artery) This type needs to be distinguished from double arch with MRI or angiography Association with congenital heart disease 5-12% Dysphagia lusoria Posterior impression on the esophagus Right-sided impression on the trachea Ddx: double arch

Diagram showing development of right arch aberrant left subclavian artery which is the mirror image of left arch aberrant right subclavian artery (shown on the left). The right image shows early obliteration of the left 4th pharyngeal arch, separating the left subclavian artery from the left common carotid artery. Persistence of the 8th segment of the left dorsal aorta maintains continuity of the left subclavian artery with the descending aorta. The left ductus completes the ring

Right arch with aberrant LSCA and left ductus [Figure 6-5-14]

Figure 6-5-14

This diagram shows the right arch with aberrant left subclavian artery viewed from anterior. Note the four branches of the aortic arch, the last of which is the left subclavian artery. The left ductus (arrow) completes the ring, so that the trachea and esophagus are completely surrounded by aorta and pulmonary arteries

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Right arch aberrant LSCA with posterior impression on esophagus [Figures 6-5-15 to 6-5-18]

Figure 6-5-16

Figure 6-5-15

CT of same patient showing right arch and posterior course of the aberrant left subclavian artery (arrow) Esophagram shows posterior impression on the esophagus at the level of the aortic arch. When the patient is in the AP projection the right arch is identified. If there were a left arch, this patient would have an asymptomatic normal variant. With the right arch, the diagnosis is a complete vascular ring

Cervical aortic arch • • • • •

Figure 6-5-18

Arch above the clavicle – not a specific arch anomaly May have pulsatile mass in supraclavicular fossa 80% are right arches Half are symptomatic rings Most common variant - right arch that descends on the right then crosses to left behind esophagus and gives off left subclavian artery and left ductus

Double Aortic Arch [Figures 6-5-19 to 6-5-21]

• • • • • •

Figure 6-5-17

Persistence of both left and right fourth arches Arteriogram showing a Most common symptomatic vascular right arch with aberrant ring LSCA and a Right is usually larger, higher and diverticulum of posterior Kommerell (arrow), or enlargement of the Anterior and bilateral lateral origin of the aberrant impressions on the trachea subclavian artery Posterior and bilateral lateral impressions on esophagus Treatment is to ligate the nondominant arch

This sagittal MR image in a 4 month old with recurrent respiratory infections, shows the aberrant subclavian artery in cross section posterior to the trachea (arrow). Normally there is no large artery behind the trachea

Figure 6-5-19

Figure 6-5-20

Esophagram in same patient. The double arch causes bilateral lateral impressions on the esophagus as seen on the AP view (left image). On the lateral view (right image), there is a prominent posterior impression caused by the joining arches. Thus, there are 3 impressions on the esophagus


PA chest radiograph in a patient with double aortic arch, showing a higher larger right sided aortic impression on the trachea and a smaller, lower left sided impression (arrow). The left impression is often difficult to discern, so double aortic arch is in the differential of right aortic arch seen on plain film

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Pulmonary Sling [Figures 6-5-22 and 6-5-23] • • • • • • •

•

Figure 6-5-21

AKA anomalous pulmonary artery Left PA originates from right Anterior impression on esophagus at level of carina Posterior impression on trachea Ductus passes from origin of RPA to aorta forming a complete ring around the trachea only Compression of bronchus intermedius by anomalous artery Associated tracheal abnormalities ➢ Tracheomalacia ➢ Complete tracheal rings ➢ T-shaped trachea Other associated anomalies – abnormal pulmonary lobation, bronchus suis, CHD

Figure 6-5-22 3-D MRA of a double aortic arch, viewed from posterior

Figure 6-5-23

Left image is a lateral view from an esophagram showing posterior impression on the trachea and anterior impression on the esophagus at the level of the hila. Black blood MRI image shows the left pulmonary artery originating from the right and coursing behind the trachea to get to the left lung

Illustration of pulmonary sling viewed from anterior with ascending aorta cut away to show the left pulmonary artery (arrow) originating from the right, then passing between the trachea and esophagus to get to the left side

Innominate Artery Compression Syndrome • • • •

Normally the innominate artery passes in front of the trachea just below the thoracic inlet In infants it arises more to the left than in adults and there is also thymus in this region, so it may cause symptomatic compression of the trachea Increased incidence of symptomatic compression in patients with dilated esophagus Compression decreases with advancing age

Midline Aorta Carina Compression Syndrome •

Midline course of descending aorta or abnormal position of the carina allows aorta to compress carina or mainstem bronchus

Other CV Abnormalities That Can Compress the Airway Congenital Heart Disease • • •

Tetralogy of Fallot with absent pulmonary valve Large left to right shunts Massive cardiomegaly

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Absent Pulmonary Valve Syndrome [Figure 6-5-24] • • • •

Figure 6-5-24

Variant of Tetralogy of Fallot Severe pulmonic regurgitation Aneurysmal dilatation of proximal left and right pulmonary arteries Compression of adjacent bronchi

PA and lateral chest radiographs in patient Tetralogy of Fallot with absent pulmonary valves. Note the massive enlargement of the pulmonary arteries, hyperinflation of the lungs due to bilateral bronchial compression and associated right aortic arch References Texts 1. Donnelly LF. Fundamentals of Pediatric Radiology. Philadelphia: W.B. Saunders Company, 2001. 2. Kirks DR, ed. Practical Pediatric Imaging. 3rd ed. Philadelphia: Lippincott-Williams & Wilkins, 1998. 3. Swischuk LE. Imaging of the Newborn, Infant, and Young Child, 5th ed. Philadelphia: Lippincott-Williams & Wilkins, 2004. Journal Articles 1. Berdon WE and Baker DH. Vascular anomalies and the infant lung: rings, slings and other things. Semin Roentgenol 1972;7:39-63. 2. Berdon WE. Rings, slings and other things: vascular compression of the infant trachea updated from the midcentury to the millennium—the legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD. Radiology 2000;216:624-632. 3. Bisset GS III et. Al. Vascular rings: MR imaging. AJR Am J Roentgenol 1987;149:251 4. Donnelly LF, Bisset GS 3rd , McDermott B. Anomalous midline location of the descending aorta: a cause of compression of the carina and left mainstem bronchus in infants. AJR AM J Roentgenology 1995; 164:705-707. 5. Donnelly LF, Strife JL, Bisset GS III. The spectrum of extrinsic lower airway compression in children: MR imaging. AJR Am J Roentgenol 1997;168:59-62 6. Kussman BD, Geva T, McGowan FX. Cardiovascular causes of airway compression. Paediatr Anaesth 2004;14:6072. 7. Newman R, Meza MP, Tobin RB, et al. Left pulmonary artery sling: diagnosis and delineation of associated tracheobronchial anomalies with MR. Pediatr Radiol 1996;26:661-668 8. Pickhardt PJ, Siegel MJ, Gutierrez FR. Vascular rings in symptomatic children: frequency of chest radiographic findings. Radiology 1997;205:581-582 9. Shuford WH, Sybers RG, Edwards FK. The three types of right aortic arch. AJR 1970;109:67-74


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Cystic Renal Disease of Childhood Ellen Chung, LTC, MC Terminology • • •

Cyst Polycystic kidney disease – ARPKD and ADPKD only Multicystic kidney

Old Classification1 • • • •

Type I, infantile polycystic kidney disease Type II, multicystic dysplastic kidney Type III, adult polycystic kidney Type IV, cortical cysts associated with massive hydronephrosis

1 Osthanondh V, Potter EL. Pathogenesis of polycystic kidneys: historical survey.

Arch Pathol 1964;77:459

Genetically-based Classification2 •

•

Nongenetic ➢ Multicystic dysplasia ➢ Multilocular cyst (tumor) ➢ Simple cyst/calyceal diverticulum ➢ Medullary sponge kidney ➢ Acquired cystic disease Genetic ➢ Autosomal recessive polycystic kidneys ➢ Autosomal dominant polycystic kidneys ➢ Juvenile nephronophthisis (AR)-medullary cystic disease (AD) complex ➢ Cysts associated with multiple malformation syndromes

2Glassberg KI, Stephens FC, Lebowitz RL, et al. Renal dysgenesis and cystic

disease of the kidney: a report of the Committee on Terminology, Nomenclature and Classification, Section on Urology, American Academy of Pediatrics. J Urol 1987, Oct; 138:1085

Simple Renal Cyst • • • • •

Uncommon in children Usually solitary Found with increasing frequency due to US screening in patients with UTI Arise in renal cortex Do not communicate with collecting system

Simple Renal Cyst • • • •

Observed on PNUS ◗ screen for malformation syndrome US criteria for simple cyst ◗ no further imaging unless recurrent symptoms of infection Otherwise ◗ CT to exclude tumor No treatment unless symptomatic or obstructing collecting system

Calyceal Diverticulum • • • • • •

Cyst that communicates with collecting system Need contrast study to distinguish from cyst Urine stasis leads to infection and stone formation Look for stone in tic on US, KUB, or non-con CT Delayed images show contrast-filling of the cyst and a neck Treatment – surgical ablation if symptomatic

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Cystic Renal Disease of Childhood

Medullary Sponge Kidney • • • • • •

Figure 6-6-1

Congenital focal dilation of collecting tubules usually presenting in adulthood Associated with stones or infection but may be found incidentally Usually bilateral Medullary nephrocalcinosis – radially aligned Streaky linear densities in involved pyramids US may show calcifications before plain film

Acquired Renal Cysts • • •

AIDS Hemo- and peritoneal dialysis ➢ Increase in number and size with length time on dialysis Complications – intracyst or subcapsular or perinephric hemorrhage

Multicystic Dysplastic Kidney • • • • •

Most common form of cystic renal disease in infants and children One of the most common causes of renal mass in first week of life Rarely bilateral Due to early severe in utero obstruction Extreme end of spectrum of UPJO

Classic MCKD gross specimen showing nonreniform shape, cysts of multiple sizes and no identifiable renal parenchyma

Multicystic Dysplastic Kidney • • •

Risk of abnormality of contralateral kidney 20-50% - UPJO, VUR Negligible renal function Nodular blastemal elements in 3-5% - risk of Wilms tumor

MCDK - Presentation • • •

Figure 6-6-2

PNUS Neonate with abdominal mass Incidentally in older child – mimics agenesis

MCDK – Gross Pathology [Figures 6-6-1 to 6-6-3] • • • • • • •

Macrocysts of variable size Randomly distributed Cysts do not communicate Hydronephrotic variant – large central cyst Rarely segmental – upper pole of duplex or lower crossed fused ectopic No identifiable normal parenchyma Associated atresia of ureter or infundibulopelvis

Hydronephrotic variant of MCDK with reniform shape and patent central pelvis

Figure 6-6-3

Stillborn baby with bilateral MCDK. Plain radiograph shows small, bellshaped thorax with airless lungs and bulging flanks. Autopsy specimen (viewed from posterior) reveals bilateral enlarged kidneys which are much larger than the hypoplastic lung Cystic Renal Disease of Childhood

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MCDK - Histopathology [Figure 6-6-4] • • • •

Figure 6-6-4

Histologic hallmark – presence of immature dysplastic-appearing tubules surrounded by collarettes of PAS-staining condensed mesenchyme Cysts of varying size formed by dilated, dysplastic tubules Cysts can occur in any part of the nephron High nuclear to cytoplasmic ratio ◗ dysplasia

MCDK - Imaging [Figures 6-6-5 and 6-6-6] • • • • • • •

Large mass with cysts of varying sizes scattered throughout Cysts do not communicate Hydronephrotic type – large central cyst but no identifiable parenchyma No identifiable cortex or medulla Nonreniform shape Nuclear renogram – no significant excretion VCUG – 25% VUR

Figure 6-6-5 Photomicrograph of dysplastic kidney showing primitive ducts surrounded by mesenchymal collarettes

Figure 6-6-6

Prenatal ultrasound showing right MCDK

MCDK - DDx •

•

Multilocular cystic kidney ➢ A tumor ➢ Cysts within the intervening septa Severe hydronephrosis ➢ UPJO ➢ Nuclear renogram ➢ Contralateral kidney also affected

Renal ultrasound showing normal right kidney and left MCDK. Note noncommunicating cysts and lack of visible normal renal parenchyma

MCDK – Course and Prognosis [Figures 6-6-7 and 6-6-8]

• • • •

Natural history of a true MCDK is to resolve Formerly these were all removed due to rare reports of nephroblastoma1 Now followed to resolution If they do not resolve, surgical removal is indicated to prevent complications of infection and neoplasm

Figure 6-6-7

1Strife JL, Souza AS, Kirks DR, Strife CF, Gelfand MJ, Wacksman J.

Multicystic dysplastic kidney in children: US follow-up. Radiology. 1993 Mar;186(3):785-8.

Autosomal Recessive Polycystic Kidney Disease (ARPKD) • • • • •

Kidneys and liver – ectasia and fibrosis Kidneys – ectasia of the collecting tubules Delayed CT diagnosis of left MCDK Liver – biliary duct ectasia and periportal fibrosis which has regressed to a partially calcified nubbin Latter develops in early childhood Degrees of renal and liver involvement are inversely proportional and determine age of presentation and prognosis

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ARPKD – Spectrum of Presentation Blyth and Ockenden Clinical Classification1

Figure 6-6-8

[Figures 6-6-9 and 6-6-10]

• • • •

•

“Perinatal” – 90% of tubules involved, bilateral nephromegaly, Potter syndrome, death in first week “Neonatal” – 60%, present in first month, death by 1 year “Infantile” – 25%, present at 3-6 mo “Juvenile” – 10%, presentation in first decade ➢ Portal hypertension ➢ Incidental finding on US Presentation in second decade with renal failure

1Blyth H, Ockenden BG. Polycystic disease of kidney and liver

CT showing Wilms tumor which arose in a left MCDK

presenting in childhood. J Med Genet. 1971 Sep;8(3):257-84.

Figure 6-6-9

Figure 6-6-10

ARPKD Autopsy

ARPKD – Pathology - Kidneys [Figure 6-6-11] • • • • • • •

Large kidneys “Cysts” are dilated collecting tubules predominantly in the medulla Dilated (1-2mm) tubules arranged in a fan-shape Cortex relatively spared No dysplasia On cut section, cortex and medulla unrecognizable Few coalescent macrocysts

ARPKD – Pathology- Liver [Figures 6-6-12 and 6-6-13] • • • •

All associated with congenital hepatic fibrosis = ductal plate Gross image showing Potter facies malformation Dilation of interlobular bile ducts associated with a variable amount of portal fibrosis (Caroli syndrome) All portal areas are expanded and contain dilated ducts at the periphery with blood vessels in the middle Sinusoidal portal hypertension

ARPKD – Plain Film • • • •

Bilateral flank masses in newborns Small, bell-shaped thorax Pneumothorax Older children may have slightly enlarged kidneys, hepatosplenomegaly, and/or ascites

Figure 6-6-11

ARPKD - Ultrasound [Figure 6-6-14] •

• • •

Large kidneys with increased echogenicity in the medulla due to multiple accoustic interfaces of dilated, ectatic ducts May mimic nephrocalcinosis Compressed, spared cortex may form relatively dark rim Poor delineation of cortex, medulla, sinus


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ARPKD. From left to right cut gross specimen, photomicrograph and diagram show dilated collecting tubules in the medulla Pediatric Radiology

Figure 6-6-12 Formation of the intrahepatic biliary radicals begins with a single layer of primitive ductal plate surrounding the portal vein. Some insult can cause congenital hepatic fibrosis by promoting fibroblast proliferation.

Figure 6-6-13

Figure 6-6-14

Gross specimen showing ectatic biliary ducts of Caroli disease

ARPKD - Ultrasound [Figure 6-6-15] • • •

High frequency linear transducer may resolve tubular structures in fan-like array or tiny cysts Occasional macrocysts Those who present as children have milder renal findings – normal or mild nephromegaly, +/- increased echogenicity of medulla, loss of corticomedullary differentiation

Ultrasound - Liver [Figure 6-6-16] • • •

ARPKD. Coronal ultrasound shows both kidneys to be markedly enlarged and echogenic centrally with a relatively sonolucent rim of compressed cortex

Increased echogenicity +/- ductal ectasia in older children Portal radicals surrounded by bile ducts Splenomegaly, varices, and ascites may also be seen in older children

Figure 6-6-16

Figure 6-6-15

12 yo diagnosed in infancy with ARPKD, now preop for liver transplant. Ultrasound on left shows markedly dilated biliary ducts with hepatic artery and portal vein branches in the center, creating a target appearance. CT shows same target appearance of biliary ducts as well as cysts in the renal medulla and splenomegaly due to portal hypertension

ARPKD. High frequency linear transducer ultrasound images show echogenic, markedly enlarged kidneys in which tiny cysts can be resolved

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ARPKD - Other Imaging [Figures 6-6-17 to 6-6-19] • • • • • •

Imaging beyond US is rarely necessary Spoke-wheel or striated appearance of nephrogram Prolonged nephrographic phase Calyces compressed, separated and distorted In older children, mild tubular ectasia similar to medullary sponge kidney MR - few cortical cysts in about half of patients


ARPKD. CT of infant showing markedly enlarged kidneys with striated delayed nephrograms due to compression of parenchyma by radially oriented dilated fluid-filled collecting ducts. The kidneys are of density similar to water due to the unopacified urine in the dilated ducts. Note rim of enhancing cortex (arrow). Also note left kidney hypodense macrocyst (block arrow)

Excretory urogram of ARPKD showing bilateral massively enlarged kidneys with striated nephrograms and distorted collecting systems

ARPKD - Prognosis • • •

Infantile – poor prognosis due to renal insufficiency and pulmonary hypoplasia Outcome in childhood is better than previously thought Early recognition and management are important

Figure 6-6-19

Differential Diagnosis Large Echogenic Kidneys in Neonate • •

Glomerulocystic disease ADPKD

Glomerulocystic Disease • • •

Rare, sporadic or heritable (AD) May be found in some patients with malformative syndromes Occasionally found in children with family history of ADPKD

Glomerulocystic Disease Clinical Features • •

Present early with renal failure and palpable abdominal masses Renal function normal but deteriorates with age

ARPKD with biliary duct ectasia. CT of the kidneys shows delay of transit of contrast into dilated, urine-filled collecting tubule Cystic Renal Disease of Childhood

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Glomerulocystic Disease Pathology [Figure 6-6-20] • •

Figure 6-6-20

Cystic dilation of Bowman capsule and proximal convoluted tubule Periportal fibrosis, bile duct hyperplasia and hepatic cysts may also be found

Glomerulocystic Disease Ultrasound • • •

Echogenic normal-sized to enlarged kidneys Poor corticomedullary differentiation Tiny cysts may be seen in cortex (vs. ARPKD)

Autosomal Dominant Polycystic Kidney Disease (ADPKD) • • • • •

Much more common than AR Three genetic loci – PKD1-3 Family screening routine Spontaneous mutations frequent Cysts become larger and more numerous with age

Autosomal Dominant Polycystic Kidney Disease (ADPKD) • • •

Glomerulocystic disease in a patient with Zellweger or cerebrohepatorenal syndrome. Gross and low magnification images show small cortical cysts

Cysts not usually seen in 1st and 2nd decades but can be seen in neonates screened for positive family history Affects multiple organs – kidneys, liver, pancreas, spleen, seminal vesicles, ovaries Association with occult intracranial aneurysms – screen in adulthood

ADPKD - Presentation • • •

Present in 4th-5th decade with hypertension and renal failure Does not present in childhood but may be found incidentally or secondary to flank pain due to bleeding into cyst Rarely presents in infancy – minimal cysts in enlarged, echogenic kidneys

ADPKD - Pathology • • • • •

Enlarged but reniform kidneys Cysts of varying size scattered throughout the kidney (cortex and medulla) Usually bilateral but may be asymmetric or even unilateral Abnormality of the ampullary and interstitial portions of the collecting tubules and nephrons Hepatic fibrosis is rare

Figure 6-6-21

ADPKD - Ultrasound [Figure 6-6-21] • • • • •

Infantile presentation – small, spherical cysts on high resolution US (vs. fan-like, tubular appearance in AR) Older children - cysts of varying size in cortex and medulla Can be unilateral at presentation Normal size or slightly enlarged Look for cysts in liver and pancreas

ADPKD - Prognosis • •

Most develop renal failure in 4th-5th decade Presentation in infancy – more severe renal cystic disease, more hypertension, more rapid progression to renal failure than adult relatives

Medullary Cystic Disease Complex • • • • •

Juvenile nephronophthisis – AR, presents in first decade Medullary cystic disease – AD, presents in 3rd decade Polydypsia, polyuria, salt wasting, severe anemia Progressive renal failure Growth retardation

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Neonatal ADPKD. Kidneys are echogenic but high resolution ultrasound helps to distinguish small cysts (arrows) (vs. radially aligned, tubular cysts in ARPKD) Cystic Renal Disease of Childhood

Medullary Cystic Disease Complex Pathology

Figure 6-6-22

[Figure 6-6-22]

• • • •

1.0.

Transmastoid view demonstrates lateral and fourth ventriculomegaly

Figure 6-17-3

Normal Anatomy: Premature Brain •

•

Preterm cerebral vessels penetrate from meninges to periventricular walls ➢ single vessel walls Term cerebral vessels-watershed at cortex ➢ smooth muscle AA, collagen VV

Nelson et al AJNR 1991:215 Longitudinal ultrasound via the anterior fontanelle demonstrates irregular flow of the sagittal sinus consistent with sagittal sinus thrombosis Pediatric Radiology

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Neonatal Brain

Neonatal Hypoxic-Ischemic Injury • • •

Figure 6-17-4

Injuries are the result of partial or global asphyxia due to various causes: intrauterine, sepsis, apneic episodes Partial asphyxia: IVH, PVL Global asphyxia: multifocal ischemic necrosis

Injury patterns: Term infant [Figure 6-17-4] •

•

Prolonged Partial Asphyxia ➢ Watershed infarction (ACA-MCA-PCA; “parasagittal”) - cortex / white matter Profound asphyxia: ➢ Basal ganglia, ➢ Rolandic cortex

Germinal Matrix • • • • •

Origin of neuronal/glial development. Rich arterial supply perforators of ACA, MCA, PCA. Drain to deep venous system. Regression posterior to anterior b/w 24-28 wks Involutes by 32 wks

Axial CT demonstrates diffuse cortical infarction in a term infant with severe hypoxic ischemic injury

Germinal Matrix Hemorrhage • • • •

Most common origin of preterm hemorrhages 20% of preterms injury to endothelium of germinal matrix vessels. Hypoxia- decrease myocardial energy reserve > circulatory failure > hypotension > cerebral ischemia > venous hypertension

Funato M, Munksgard 1994:456; Milligan D Lancet 1980:26:896

Germinal Matrix Hemorrhage •

•

O2 delivery reduced w/ hemorrhage. ➢ Switch from aerobic to anaerobic metabolism ➢ Increase lactate formation. Blood ruptures into ventricles ➢ Dilatation, ➢ Disrupt ependymal lining

Figure 6-17-5

Modified Papile Classification • • • •

Grade I Subependymal hemorrhage Grade II Intraventricular blood w/ no/min ventricular dilatation Grade III Intraventricular blood w/ prominent ventricular dilatation Grade IV/PHI Parenchymal hemorrhage associated with IVH

Sagittal US demonstrates a Grade 1 subependymal hemorrhage

Grade I IVH [Figure 6-17-5]

Neonatal Brain

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Grade II IVH w/out dilatation [Figure 6-17-6]

Figure 6-17-6

Grade II IVH w/ distended ventricle [Figure 6-17-7] Grade III - IVH w/ distended ventricle [Figure 6-17-8] Periventricular Hemorrhagic Infarction [Figure 6-17-9] Figure 6-17-9 Coronal and sagittal US images demonstrate Grade II intraventricular hemorrhage with blood extending into the ventricle

Figure 6-17-7

Coronal sonogram demonstrates a heterogeneous region in the right parietal periventricular white matter consistent with a periventricular hemorrhagic infarction and ventriculomegaly

Coronal US and axial CT images of Grade III intraventricular hemorrhage with blood filling and dilated the ventricles

Periventricular Hemorrhagic Infarction (PHI/ Grade IV IVH) • • •

Figure 6-17-8

15% with IVH dev PHI Same side as IVH, after IVH has occurred Usually unilateral or asymmetric

Periventricular Hemorrhagic Infarction (PHI/ Grade IV IVH) •

Hemorrhagic necrosis in PVWM dorsal/lat of lateral ventricle where medullary veins confluent.

Periventricular Hemorrhagic Infarction • •

PET scans demonstrate parenchymal ischemia beyond boundaries of hemorrhage Further injury may be due to accumulation of metabolic toxins due to impaired blood flow

Volpe JJ. Neurology of the Newborn 1987

Periventricular Hemorrhagic Infarction Sonography Time

Pathology

Early

hemorrhagic infarcts PVWM surrounding ischemia Subacute cystic cavities diminished myelin Chronic

porencephalic cyst ventricular dilatation gliosis

Pediatric Radiology

Coronal sonogram demonstrate dilated ventricles filled with hemorrhage consistent with an evolving grade III intraventricular hemorrhage

US findings echogenic areas mass effect retraction of clot, porencephalic cyst ventricular debri/dilat porencephalic cyst ventricular dilatation atrophy 1501 1503

Neonatal Brain

IVH: Sonography • • •

•

Figure 6-17-10

US very useful -lesions well visualized deep in the brain Difficult to make diagnosis clinically Screening recommendations ➢ Preterms than border zones ➢ ?Intrinsic vulnerability of periventricular glial cells and intrinsic metabolic properties. ➢ O2 reduced >lactic acid accumulation

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•

Glial cells are differentiating to astrocytes & oligodendroglia with active myelination. Intense metabolic activity w/ high O2 demandsvulnerable to hypoxia [Figure 6-17-11]

Figure 6-17-11

Periventricular Leukomalacia:Sonography • • •

Infarction occurs in deep cerebral WM Bilateral symmetric US not sensitive in detecting PVL ➢ Difficult to distinguish from anisotropic effect of periventricular halo ➢ Echodensities resolve and can be missed

Periventricular Leukomalacia:Sonography Time

Pathology

US Findings

Acute 1-3 days

focal necrosis petechial hemor

patchy areas of inc echogenicity PVWM

Subacute 1-2 wks 2-3 wks

diminished myelin cystic cavities

decreasing echogenicity may be normal small cysts “Swiss cheese”

Chronic

cysts disappear gliosis

cysts disappear vent dilatation

Coronal sonogram demonstrates multiple cysts in the periventricular white matter consistent with periventricular leukomalacia

Periventricular Leukomalacia • • •

Less severe PVL-diminished myelin result in dilated ventricles. Severe PVL will cavitate Cysts eventually resolve -gliotic scarring

Figure 6-17-12

Periventricular Leukomalacia:CT [Figures 6-17-12 and 6-17-13]

• • •

Difficult transporting premature infants Due to high water content, difficult differentiating acute PVL from nl preterm brain Chronic: CT useful extent of lesions, atrophy ➢ Irregular lateral ventricles ➢ Prominent sulci ➢ Subcortical gray matter abuts ventricles ➢ Small cysts missed

Figure 6-17-13

Axial CT image demonstrates diffuse periventricular and cortical edema with intraventricular hemorrhage

Axial image demonstrates ventricular dilatation with irregular walls and marked loss of periventricular white matter Pediatric Radiology

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Neonatal Brain

Periventricular Leukomalacia: MRI

Figure 6-17-14

[Figure 6-17-14]

• •

•

Acute/subacute: dec. sig T1, inc. sig T2 in PVWM High water content makes edema difficult to detect on T1, due to delayed myelination, high signal on T2 apparent. Periatrial gliosis; involves the subependymal periatrial white matter (tapedum) Distal corpus callosum (pre-splenium) thinned

Periventricular Leukomalacia:Outcome • •

•

Outcome correlates with cystic cavitation PVWM traversed by fibers of motor cortex results in spastic diplegia ➢ Lower> Upper extremities Infants with larger lesions often delayed

Nonhemorrhagic Infarction • • •

Rare, term infants 90% major handicaps Perinatal asphyxia ➢ Emboli ➢ Congenital Heart disease ➢ Meningitis ➢ Polycythemis

Axial T2w image demonstrates high signal in the periventricular white matter

Profound Asphyxia • •

Energy requirements related to state of myelination. Most metabolically active and mature regions with most advanced Figure 6-17-15 ➢ myelination, ➢ perfusion ➢ glucose uptake are regions that suffer the most damage • Complete arrest - injury determined by ➢ metabolic maturity of brain ➢ myelination ➢ autoregulation ➢ watershed pattern ➢ excitatory neurotransmitter release Coronal US demonstrates diffuse increased ➢ severity and duration of event echogenicity of the cerebral cortex with slit ventricles Profound Asphyxia: Sonography Anterior cerebral artery Resistive Index < .5 • Diffuse hypoxic ischemic encephalopathy often consistent with arterial vasodilatation due to loss superimposed on IVH/PVL of sutoregulation

Profound Asphyxia in Preterms • • • •

Preterm have low O2 demand and immature cardiopulmonary control Neuronal injury can occur in ventral pons, inferior olivary nuclei, subiculum of hippocampus Unlike term infant, less involvement of basal ganglia, thalami, brainstem PVWM injury dominating

Profound Asphyxia in Term Infants •

Involvement of basal ganglia, brainstem ➢ hippocampus ➢ posterior and medial lentiform nuclei ➢ lateral thalami ➢ cortical gyri

Profound asphyxia Term: Sonography [Figure 6-17-15] •

Acute - increased echogenicity ➢ small vents/sulci ➢ Doppler - Loss of autoregulation RI < .6

Neonatal Brain

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Pediatric Radiology

Figure 6-17-16

Profound asphyxia: CT [Figure 6-17-16] • •

Acute-low attenuation cortex, basal ganglia, periventricular white matter, laminar necrosis Chronic-diffuse atrophy prominent extraaxial fluid spaces and sulci

Profound asphyxia: MRI findings in Preterm Infants •

•

PVWM changes dominate ➢ T1,T2 shortening in peritrigonal WM ➢ T1,T2 prolongation in cerebral WM Chronic - atrophy ➢ Relative sparing of cerebral cortex ➢ Hippocampal and brainstem atrophy ➢ Thin corpus collosum

Profound asphyxia: MRI findings in term infants •

Short T, at times short T2 relaxation times ➢ Basal ganglia ➢ Hippocampus ➢ Posterior and medial lentiform nuclei ➢ Lateral thalami ➢ Cortical gyri

Axial CT images demonstrate low attenuation of the cortex, basal ganglia as well as periventricular white matter.in this term infant following profound asphyxia

Figure 6-17-17

Cerebellar Infarction [Figure 6-17-17] • • • • • • •

Cerebellum felt to be less vulnerable to anoxic damage - sparing during hypoxic ischemic episodes May not be rare in preterms Due to echogenicity of CBL, infarction and hemorrhage easily missed US Most common watershed distribution - between the superior cerebellar artery and the PICA Can be lobar /holohemispheric; +/- hemorrhagic Most in early premature (28-32) with hx of hypotension

Mercuri Ped Rad 1997;27:139 Tsuru Acta Neuropath 1995:90;400

US- Posterior Fossa Hemorrhages [Figures 6-17-18 and 6-17-19] • • •

Coronal MRI demonstrates right cerebellar infarction in a child with a history of prematurity

Easy to miss Close evaluation of sagittal view Additional views ➢ transmastoid view ➢ posterior fontanelle

Figure 6-17-19

Figure 6-17-18

Lateral image of the skull demonstrates where the transducer is placed for imaging via the mastoid .B. Axial sonogram demonstrates a heterogenous lesion in the posterior fossa consistent with a posterior fossa hemorrhage

Sagittal midline image of a normal brain clearly demonstrates the vermis (arrow). B. Sagittal midline image demonstrates a heterogeneous region in the posterior fossa with no visualization of the vermis in an infant with a posterior fossa hemorrhage

Pediatric Radiology

1505 1507

Neonatal Brain

Conclusions • • • •

Neurosonology is an integral part of care in the neonate. Type of hypoxic injuries vary with gestational age US sensitive for IVH screening, less sensitive for identifying PVL, infarcts and posterior fossa hemorrhages Flexibility in technique – Doppler, transmastoid view- important in identifying subtle anomalies

References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

Barkovich AJ, Sargent SK: Profound asphyxia in the premature infant: imaging findings AJNR 1995;16;1837. Benson JE et al. Intracranial Neonatal Neurosonography: An Update. Ultrasound Quarterly 2002: 18;89 Boo NY et al. Early cranial US changes as predictors of outcome during first yr of life. J Ped Child Healthy 2000;36:363 Bulas DI, Taylor GA, Fitz CR, Revenis ME, Glass P, Ingram JD. Posterior fossa intracranial hemorrhage in infants treated with extracorporeal membrane oxygenation: Sonographic findings. AJR 1991; 156:571. Bulas DI. Vezina G: Anoxic injury in the Preterm infant Radiologic evaluation Radiologic Clinics of North America, Vol 37, Nov 1999:1147. Bulas DI. TCD: Practical Applications in Pediatrics. Applied Radiology 1999, April 7-15. Chadduck WM, Duong DH Kast JM et al: Pediatric cerebellar hemorrhage. Child Nerv Syst 1995;110:579. Perlman JM, Rollins N: Surveillance protocol for the detection of intracranial abnormalities in premature neonates. Arch Pediatr Adol Med 2000;154:822. Rumack CM et al. Timing and course of neonatal intracranial hemorrhage using US. Radiology 1985:154:101 Rumack C, Drose J. Neonatal and Infant Brain Imaging ed Rumack et al. Diagnostic Ultrasound. Elsevier Mosby 2005 Taylor GA. Recent advances in neonatal cranial ultrasound and Doppler techniques. Clin Perinataol 1997;24:677 Seibert JJ et al. Use of power Doppler in pediatric neurosonography: a pictorial essay. Radiographics 1998;18:879 Volpe JJ: Neurobiology of periventricular leukomalacia in the premature infant Pediatr Res 2001;50:553-562 Vohr B, Allan WC, Scott DT et al: Early onset IVH in preterm neonates: Incidence of neurodevelopmental handicap. Semin Perinatol 1999;23:212.

Neonatal Brain

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Pediatric Radiology

Pediatric Liver Tumors William E. Shiels II, DO • • • • •

Pediatric Liver Tumors: GOALS

• • •

Types and presentations Pathologic features Clinical features Imaging Therapeutic implications/correlation

Pediatric Liver Tumors: ABC’s of Liver Tumors Age of patient Biologic imaging features Chemistry-blood ➢ Alpha-fetoprotein ➢ Endothelial growth factor

• • • • •

Benign Epithelial Tumors Benign Mesenchymal Tumors Malignant Epithelial Tumors Malignant Mesenchymal Tumors Metastases

• • •

Focal Nodular Hyperplasia Hepatocellular Adenoma Hepatic cysts

• • •

Mesenchymal Hamartoma Hemangioendothelioma Hemangioma

• • •

Hepatoblastoma Hepatocellular Carcinoma Fibrolamellar Carcinoma

• • •

Undifferentiated embryonal sarcoma Embyronal Rhabdomyosarcoma Angiosarcoma

• • • • •

Neuroblastoma Burkitt’s Lymphoma Sarcomas Wilms’ tumor Other

Categorization

Benign Epithelial Tumors

Benign Mesenchymal Tumors

Malignant Epithelial Tumors

Malignant Mesenchymal Tumors

Metastases

• •

Mesenchymal Hamartoma Mesenchymal tissue, disorganized bile ducts, hepatocytes, fluid filled spaces Developmental disturbance: ➢ Portal/biliary obstruction, lymphangiomatous tissue

Pediatric Radiology

1509

Pediatric Liver Tumors

• • • • •

Mesenchymal Hamartoma: Pathology [Figures 6-16-1 and 6-16-2]

Figure 6-18-1

Multicystic, variable size: 2–25cm 15–30% pedunculated 80% right lobe, slow growing- fluid accumulation Sharply demarcated, lobulated Mesenchymal stroma, hepatocytes, cysts: ➢ Biliary/lymphangiomatous origin (Portal tract)

Figure 6-18-2

Hepatic mesenchymal hamartoma with smoothly marginated nonaggressive cysts within a fibrous stroma

Figure 6-18-3 Photomicrograph with fibrous stroma and cysts predominantly lined by flat vascular endothelium from the lympatic component of the hamartoma • •

Mesenchymal Hamartoma: Clinical Enlarging abdominal mass Child < 2 yrs old ➢ Can Dx in utero • Otherwise asymptomatic • Negative alpha-fetoprotein

Sonography with smooth walled cysts with thin septations, similar to those seen in lymphatic malformations

Mesenchymal Hamartoma: Imaging • • •

[Figures 6-18-3 to 6-18-6]

• •

Figure 6-18-5

Hepatomegaly, abdominal mass US: Multilocular cysts, hypoechoic Less frequent-solid mass with few cysts Rare to find hemorrhage, Ca +2 MR: Cysts-low T1, high T2 signal

Figure 6-18-4

CT with few macrocysts in the mesenchymal hamartoma

Figure 6-18-6

Sonography demonstrating microcystic component in the hamartoma

Cystic mesenchymal hamartoma with single macrocyst with normal adjacent liver


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Pediatric Radiology

• • • •

SURGICAL Enucleation, partial hepatectomy Incision/drainage of cysts Marsupialize large cyst(s)

• • •

Age: Young (less than 3y/o) Biologic imaging: Cystic Chemistry: Normal alpha-fetoprotein

• • • • • •

Most common hepatic tumor- first year of life Usually Dx in first few months of life Female predominance 1.5:1 Growing mass, clinically symptomatic Vascular, high flow, mass-stroma History of involution in first year, if survivor

Mesenchymal Hamartoma: Treatment

Figure 6-18-7

Mesenchymal Hamartoma

Infantile Hemangioendothelioma Large vascular spaces in hemangioendothelioma with normal adjacent liver

Figure 6-18-8

Infantile Hemangioendothelioma: Pathology • • • • •

[Figures 6-18-7 and 6-18-8]

• • • • • •

Solitary, may be multicentric 0.2–15 cm diameter May be well demarcated, no capsule Dilated vascular spaces- anastomosing,+ stroma Central necrosis/fibrosis, hemorrhage, Ca2+ ➢ Evidence of regression

Infantile Hemangioendothelioma: Clinical Infant, usually less than 2 months Abdominal mass, CHF, Kasabach/Merritt, DIC Occasionally asymptomatic hepatomegaly Massive hemoperitoneum Multiple cutaneous angiomas-40% Normal alpha-fetoprotein (positive EGF)

Infantile Hemangioendothelioma: Imaging

Top micrograph with small “tight” vascular spaces in a hemamgioendothelioma from a child with no heart failure. Bottom specimen from a child with high output failure from left-to-right shunting in the hemangioendothelioma; note the large vascular spaces associated with arteriovenous anastomoses

• •

[Figures 6-18-9 to 6-18-15]

Hepatomegaly, abdominal mass US: Heterogeneous, speckled Ca2+ ➢ High flow, venous/arterial: A-V shunts ➢ Aortic caliber decrease-after celiac artery • CT: Hypodense, 40% Ca2+, periph. enhancement • MR: Low T1, High T2 signal, +/– hemorrhage • Angio: Vascular, A-V shunting, large celiac A.

Figure 6-18-9

Flow-failure chest radiographic image from left-to-right shunt at the level of the liver in patient with hepatic hemangioendothelioma

Pediatric Radiology

Figure 6-18-10

Large vascular spaces in hemangioendothelioma prior to color flow and duplex Doppler interrogation

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Figure 6-18-11

Figure 6-18-12

Decrease in abdominal aortic caliber from left-to-right shunting of blood at the level of the celiac artery in patient with hemanioendothelioma

Figure 6-18-13 Diffuse hepatic involvement of hemangioendothelioma

Figure 6-18-14 Multiple discrete liver hemangiomas in patient who is clinically asymptomatic

Figure 6-18-15

Draping peripheral feeding vessels in liver hemangioma

Dynamic CT with centripetal contrast enhancement of liver multiple hemangiomas Pediatric Liver Tumors

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Pediatric Radiology

• •

Infantile Hemangioendothelioma: Treatment

Figure 6-18-16

Spontaneous involution in 12–18 months CHF- embolotherapy with interferon ➢ Interferon-alpha 1, 2a, 2b response in weeks • Embolize hepatic artery- coils, balloons PRN ➢ A-V shunts ➢ Particles (large) where possible • May couple with surgery PRN • •

Hemangioendothelioma Age: Young (Infants) Biologic imaging: ➢ Vascular-High flow state ➢ Solid stroma • Chemistry: Normal alpha-fetoprotein ➢ Positive endothelial growth factor

Photomicrograph of hepatoblastoma demonstrating cords and nests of malignant cells

Figure 6-18-17

• •

Hepatoblastoma Most common primary liver tumor in childhood Third most common abdominal malignancy ➢ After neuroblastoma, Wilms’ tumor • May be familial • Associations: Trisomy 18, Beckwith-Wiedemann, hemihypertropy, familial polyposis, exposure- metals, petroleum products, paints, oral contraceptives, Fetal Alcohol Syndrome • • • • • •

Hepatoblastoma: Pathology- Gross Solitary 80%, right lobe predominance May be lobulated 5–20 cm Nodular with fibrous bands throughout Fleshy; +/– hemorrhage, necrosis, Ca2+ Rare diffuse infiltration, adjacent liver-normal

Slide left with right hepatic lobe nodular solid hepatoblastoma in an otherwise normal liver. Slide right with same tumor demonstrating the typical nodular appearance in the gross specimen

•

Hepatoblastoma: Pathology-Histologic [Figure 6-18-16]

Figure 6-18-18

Epithelial ➢ Fetal 30%, Pink-cytoplasm, sparse mitoses ➢ Embryonal 20%- blastemic appearance (blue-H&E) ➢ Macrotrabecular- cords of tumor cells- 3% ➢ Small cell- 3% • Mixed epithelial-mesenchymal- 45%; Teratoid ➢ osteoid differentiation, also muscle, cartilage • AFP stain positive • • • • • •

Hepatoblastoma: Clinical Abdominal mass, hepatomegaly Anemia Child 90% < 5 yrs old, 65–70% under 2 yrs Males> females 2:1 Positive alpha-fetoprotein No prior history of liver disease

Hepatoblastoma: Imaging • • • • •

[Figures 6-18-17 to 6-18-19]

Hepatomegaly, abdominal mass, 10–15% Ca2+ US: Heterogeneous, predominantly solid CT: Heterogeneous, lobulated MR: Low T1, High T2 Angio: Tumor neovascularity

Pediatric Radiology

Unenhanced CT with focus of calcification in a teratoid variety of hepatoblastoma. Heterogeneous enhancement of same tumor involving left and right hepatic lobes

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• •

Hepatoblastoma: Treatment [Figure 6-18-20]

Figure 6-18-19

Pre-op chemotherapy ( 50%…90%) Surgical: Resectable 90%; 35% mortality ➢ Fetal best prognosis • Vascular definition- multisegmentectomy • Mets: Pulmonary, periaortic nodes, brain • Chemoembolization/RF after surgery PRN

Hepatoblastoma • • •

Heptoblastoma with solid nature in CT and gross specimen. Cystic change is irregular due to central necrosis of the solid tumor

Age: Young (infants, young children) Biologic imaging features: Solid Chemistry: Positive alpha-fetoprotein

Figure 6-18-20

• •

Hepatocellular Carcinoma #2 primary liver malignancy in childhood Often underlying liver disease/cirrhosis ➢ Tyrosinemia, alpha-1-antitrypsin deficiency, glycogen storage dz, biliary atresia, chronic hepatitis (HBsAg +), Fanconi anemia, methotrexate induced hepatic fibrosis • May be primary • • • • • • •

Multinodular, diffuse; less common solitary 2–25 cm Hemorrhage, cysts scattered with nodules Fibrous/cirrhotic background Adjacent liver abnormal Cords and nest- malignant hepatocytes Fibrolamellar variant: favorable prognosis

• • • • • •

Abdominal mass, hepatomegaly Abdominal pain Child > 4 yrs old, 12–14 yrs mean age Male predominance > 2:1 Positive alpha-fetoprotein + prior history of liver disease

Hepatocellular Carcinoma: Pathology [Figure 6-18-21]

Chemoembolization of residual tumor focus following surgery in patient who is not a liver transplantation candidate

Hepatocellular Carcinoma: Clinical

Figure 6-18-21

Hepatocellular Carcinoma: Imaging • • •

[Figures 6-18-22 and 6-18-23]

Hepatomegaly, abdominal mass US: Heterogeneous, mostly solid, hypoechoic CT: Hypodense/isodense ➢ Heterogeneous enhancement • MR: Low T1, High T2 (esp Fat Sat FSE), ➢ PV invasion • Similar appearance to aggressive hepatoblastoma

Nodular nature(cords and nests of tumor cells) of solid hepatocellular carcinoma

Figure 6-18-23

Figure 6-18-22

Nodular appearance of solid epithelial tumor (hepatocellular carcinoma) in 14 year old patient, with positive AFP


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Nests of inhomogeneously enhancing HCC Pediatric Radiology

• •

Hepatocellular Carcinoma: Treatment [Figures 6-18-24 to 6-18-26]

Figure 6-18-24

Pre-op chemotherapy Surgical: Resectable 20%, >75% mortality ➢ Fibrolamellar- best prognosis • Vascular definition- multisegmentectomy • Chemoembolization/RF after surgery PRN • • •

Hepatocellular Carcinoma

• • • • • •

Age of patient: Teens Biologic imaging: Solid, multifocal Chemistry-blood ➢ Positive alpha-fetoprotein

Undifferentiated Embryonal Sarcoma

RF ablation of focal hepatoma (left image) with needle seen during US guided RF ablation (right image).

#4 liver malignancy in childhood AKA malignant mesenchymoma Highly aggressive neoplasm Most children 6–10 yrs old, M=F Abdominal mass, fever, wt loss Normal alpha-fetoprotein

Figure 6-18-25

Undifferentiated Embryonal Sarcoma: Pathology • • • • • • •

Solid, globular, areas of necrosis/hemorrhage 2–30 cm Right lobe dominance (75%) Fibrous pseudocapsule, occasional-pedunculated Spindle/stellate shaped sarcomatous cells Myxoid background Local recurrence and metastasis Left slide with small echogenic focus of microbubbles form at the tip of needle (arrow) in the earliest phase of coagulation necrosis. The small focus of microbubbles grows to become a large area of echogenic necrotic tumor (right)

Undifferentiated Embryonal Sarcoma: Imaging [Figures 6-18-27 to 6-18-30] • • • • •

Hepatomegaly, abdominal mass US: Complex mass CT: Hypodense-heterogeneous enhancement MR: Low T1, High T2 Angiography: Hypovascular

Figure 6-18-26

Figure 6-18-27

Figure 6-18-28

Focal scar 1 year following RF tumor ablation

Complex US appearance of embryonal sarcoma in 11 year female

Demonstrable pseudocapsule anteriorly surrounding mesenchymal sarcoma Pediatric Radiology

1513 1515


Figure 6-18-29

Figure 6-18-30

MR with heterogeneous cystic and solid foci in embryonal sarcoma Multiple areas of cystic change in embryonal sarcoma in 10 y/o male • • • • •

Undifferentiated Embryonal Sarcoma: Treatment

• • •

Surgical: Resection Chemotherapy, XRT Poor prognosis Mean survival = 12 months Recurrence Dx 12–16 months after surgery

Figure 6-18-31

Undifferentiated Embryonal Sarcoma

• • • • • •

Age of patient: Adolescents/teens Biologic imaging: Mixed solid/cystic Chemistry-blood ➢ Negative alpha-fetoprotein

Liver Metastases [Figures 6-18-31 and 6-18-32]

• • •

Neuroblastoma- Stage 4 and 4s Burkitt’s lymphoma Wilms’ tumor Leukemia (AML) Sarcomas Other malignancies

Studded appearance of stage 4S neuroblastoma diffusely involving the liver

ABC’s of Pediatric Liver Tumors

Figure 6-18-32

Age of patient Biologic imaging features Chemistry-blood ➢ Alpha fetoprotein ➢ Endothelial growth factor

Pediatric Liver Tumors: Summary Summary chart of classic features of common pediatric liver tumors Tumor

Age

Characteristics

Inf. Hemangio

< 1 yr

Mes Hamartoma

< 2 yr

Hepatoblastoma HCC Emb Rhabdo UES Metastases

< 3 yr > 4 yr < 5 yr > 6 yr any

Solid, Ca+2, vasc, AFP (-), involutes with interferon Cystic > solid, AFP (-) Ca+2, solid, vasc, AFP(+) solitary


Solid, vasc, AFP (+), multifocal Solid > cystic, mild vasc, AFP(-) Cystic > solid, AFP (-) Solid or cystic

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Typical appearance of Burkitt Lymphoma involving both the liver and the posterior gastric wall in patient with fatty liver

Pediatric Radiology

References 1. 2. 3. 4.

Von Schweinitz, D. Management of liver tumors in childhood. Semin Ped Surg 2006; 15(1):17-24 Stocker JT. Hepatic tumors in children. Clin Liver Dis. 2001 Feb;5(1):259-81, viii-ix. von Schweinitz D. Neonatal liver tumours. Semin Neonatol. 2003 Oct;8(5):403-10. Tiao GM, Bobey N, Allen S, Nieves N, Alonso M, Bucuvalas J, Wells R, Ryckman F. The current management of hepatoblastoma: a combination of chemotherapy, conventional resection, and liver transplantation. J Pediatr. 2005 Feb;146(2):204-11. 5. Burrows PE, Dubois J, Kassarjian A. Pediatric hepatic vascular anomalies. Pediatr Radiol. 2001 Aug;31(8):533-45. 6. Dubois J, Hershon L, Carmant L, Belanger S, Leclerc JM, David M. Toxicity profile of interferon alfa-2b in children: A prospective evaluation. J Pediatr. 1999 Dec;135(6):782-5. 7. Dachman AH, Pakter RL, Ros PR, Fishman EK, Goodman ZD, Lichtenstein JE. Hepatoblastoma: radiologic-pathologic correlation in 50 cases. Radiology. 1987 Jul;164(1):15-9. 8. Gerber DA, Arcement C, Carr B, Towbin R, Mazariegos G, Reyes J. Use of intrahepatic chemotherapy to treat advanced pediatric hepatic malignancies. J Pediatr Gastroenterol Nutr. 2000 Feb;30(2):137-44. 9. Sun XY, Wu ZD, Liao XF, Yuan JY. Tumor angiogenesis and its clinical significance in pediatric malignant liver tumor. World J Gastroenterol. 2005 Feb 7;11(5):741-3. 10. Rhim H, Dodd GD 3rd, Chintapalli KN, Wood BJ, Dupuy DE, Hvizda JL, Sewell PE, Goldberg SN. Radiofrequency thermal ablation of abdominal tumors: lessons learned from complications. Radiographics. 2004 Jan-Feb;24(1):4152. 11. Iannitti DA, Dupuy DE, Mayo-Smith WW, Murphy B. Hepatic radiofrequency ablation. Arch Surg. 2002 Apr;137(4):422-6.

Pediatric Radiology

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Pediatric Hip Sonography: Practical Radiologic Pathology William E. Shiels II, DO Figure 6-19-1

• •

Ultrasound of the Hip Developmental dysplasia The irritable hip ➢ Septic arthritis, toxic synovitis, ➢ Arthritis, LCP, hemophilia • US guided intervention • Practical points • • •

DDH - Risk Factors Family History: 12-36% Breech: up to 23% (esp female) Torticollis, metatarsus adductus, oligohydramnios

• •

DDH - Ultrasound Technique High frequency linear Static views (Graf) ➢ Coronal • Dynamic views (Harcke) ➢ Transverse and coronal ➢ Barlow maneuver • Standard minimum exam • • •

Coronal left hip sonography: Transducer in right hand, left hand pistons the left leg/hip

Figure 6-19-2

Dynamic Standard Minimum Exam [Figures 6-19-1 and 6-19-2] Static coronal image Static transverse image Transverse image with stress

Coronal Image [Figures 6-19-3 and 6-19-4] Transverse right hip sonography: Transducer in left hand, right hand pistons the right leg/hip

Figure 6-19-3

Figure 6-19-4

Coronal hip anatomy illustrates hyaline cartilage of the acetabular roof, femoral head, and triradiate cartilage; fibrocartilaginous acetabular labral tip Essential static coronal hip sonogram with Graf alpha angle. Note the critical horizontal orientation of the iliac body for accurate depiction of normal and pathologic anatomy

Pediatric Hip Sonography

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Pediatric Radiology

Static Transverse Image [Figure 6-19-5]

Figure 6-19-5

•

DDH - Coronal Rigidly standardized protocol ➢ Exam technique, interpretation ➢ Alpha, beta angles • Reproducible • Large European experience • • •

Normal - Graf I Physiologically Immature - Graf IIa Dysplasia - Graf IIb and above

• • • • •

Toxic synovitis Septic arthritis Arthritis - JRA, post-infectious Legg-Calve-Perthes Hemophilia

• • • •

Pain Limitation of motion Fever, WBC, ESR, CRP 50% with effusion

• • •

Direct visualization More sensitive than plain film Guided aspiration

DDH - Practical Grading

The Irritable Hip

Note femoral metaphysis in view in the transverse image obtained with the hip flexed. Femoral head deeply seated in the hip socket

The Irritable Hip

Hip Effusion - Ultrasound

Pediatric Radiology

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• • • •

High frequency linear Supine, hip neutral Anterior, parallel femoral neck Comparison views

• • • •

Fluid distends joint capsule >2mm difference abnormal Fluid echogenicity unreliable Doppler flow unreliable

• • • • •

Fever Elevated WBC, ESR, CRP Symptoms more severe Staph, strep, H. flu Must obtain fluid for Dx


Figure 6-19-6


Septic Arthritis [Figures 6-19-6 and 6-19-7]

Essential Principles of US Guidance

Cursors delineate the effusion in the anterior hip joint space [Figure 6-19-8]

Figure 6-19-7

Figure 6-19-8

Small hip effusion in left image; right image with needle aspiration of the effusion

Critical principles of sonographic guidance; needle aligned in the center of the sound beam, straight longitudinal alignment of needle along the transducer face/sound beam short axis

References 1. Harcke HT, Grissom LE. Pediatric hip sonography. Diagnosis and differential diagnosis. Radiol Clin North Am. 1999 Jul;37(4):787-96. 2. Grissom LE, Harke HT. Developmental Dysplasia of the Pediatric Hip with Emphasis on Sonographic Evaluation. Semin Musculoskelet Radiol. 1999;3(4):359-370 3. ACR Practice Guideline for the Performance of the Ultrasound Examination for Detection of Developmental Dysplasia of the Hip. American College of Radiology Practice Standards/Guidelines 2004. American College of Radiology, Reston, VA.Headquarters Office: 1891 Preston White Dr, Reston, VA 20191, (703) 648-8900© 2004 American College of2004 American College of Radiology 4. Eich GF, Superti-Furga A, Umbricht FS, Willi UV. The painful hip: evaluation of criteria for clinical decisionmaking. Eur J Pediatr. 1999 Nov;158(11):923-8. 5. Buchmann RF, Jaramillo D. Imaging of articular disorders in children. Radiol Clin North Am. 2004 Jan;42(1):15168, vii. 6. Givon U, Liberman B, Schindler A, Blankstein A, Ganel A. Treatment of septic arthritis of the hip joint by repeated ultrasound-guided aspirations. J Pediatr Orthop. 2004 May-Jun;24(3):266-70.


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Pediatric Radiology

Pediatric Seminar 1: Pulmonary Infections Ellen Chung, LTC, MC CASE 1: 5 month with fever and cough

Normal thymus mimicking RUL pneumonia

Follow-up

Follow-up radiograph in one month shows expected decrease in size of the thymus and the thymic sail sign

Normal Thymus • • • • • •

Sail sign, wave sign, notch or sulcus No mass effect on trachea or vessels Homogeneous in density Commonly seen on CXR up to age 5 years then regresses in latter half of first decade Thymic rebound Masses can arise in thymus – leukemia/lymphoma, teratoma, thymolipoma, lymphatic malformation

Pediatric Radiology

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Pediatric Seminar 1: Pulmonary Infections

CASE 2: 12 yo with fever and cough Round Pneumonia • • • •

Unique to children – usually < 8 yo Usually posterior lower lobes Almost always bacterial – pneumococcus Exclude bone erosion – ddx: neoplasm

CT reveals a complex mass in the right posterior mediastinum with calcifications and extension through the chest wall and into the spinal canal. This is neuroblastoma

Tumors that look like pneumonia • • •

Neuroblastoma Chest wall masses Parenchymal mass ➢ Pleuropulmonary blastoma ➢ Plasma cell granuloma ➢ Cyst

In the differential of round pneumonia is mediastinal or chest wall mass. PA and lateral chest radiographs show a round mass in the region of the medial right lower lobe. Note the splaying of the ribs on the right adjacent to the mass. This is therefore a mass and not a pneumonia

Pulmonary Blastoma • • • • •

Arises from primitive mesenchymal blastema Histologically reminiscent of Wilms tumor Unlike other embryonal tumors, it is more commonly found in adults May present as solitary nodule or huge mass Heterogeneous, cystic areas

Plasma Cell Granuloma • • • • • • •

AKA inflammatory pseudotumor Localized proliferation of a variety of cells, mostly plasma cells Reactive, organizing pneumonia Patient often asx Chunky calcifications May be cystic May have spiculated margins or air bronchograms


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Pediatric Radiology

CASE 3: 17 mo with fever and cough without improvement after 1 week of oral abx

PA and lateral chest radiographs show dense opacification of the right lower lobe with silhouetting of the right hemidiaphram. Right lateral pleural thickening is also seen

Complicated Pneumonia • • • • •

Suspect in cases of near white out H. flu less than 2 y.o. Pneumococcus S. aureus CT vs. US ➢ Ultrasound may be better determining which fluid collections need drainage

Ultrasound of the right chest shows pleural fluid with septations and consolidated, airless lung that transmits sound. The hyperechoic foci with ringdown artifact represent round air collections

Multiple axial CT images show the pleural fluid adjacent to the right lower lobe consolidation. Note that the lung enhances and is not necrotic. The fluid-filled cavities with non-nondependent air collections are therefore most consistent with small abscesses rather than necrotic cavities

CASE 4: 17 yo with fever and cough, seizure disorder and DM

Round cavity in superior segment of right upper lobe. Clinical history suggests mild immunocompromise and possibility of aspiration

Pediatric Radiology

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Differential Diagnosis • • •

Pulmonary abscess Cavitary pulmonary necrosis Pneumatocele

Pulmonary Abscess • •

CT – fluid or air-filled cyst with enhancing, thick, irregular wall Both necrosis and small abscesses in children have good outcomes with antibiotics only

Pneumatoceles • • • • •

Thin walled cavity seen in the recovery phase usually of infection Staphylococcus, pneumococcus, tuberculosis Blunt chest trauma, hydrocarbon pneumonitis, Langerhans cell histiocytosis Bronchial obstruction leading to air trapping and alveolar rupture Pulmonary necrosis. Contrast this appearance to Pneumothorax or mediastinum case #3 with empyema and abscesses. Note decreased enhancement of surrounding parenchyma compared to more anterior parenchyma with preservation of enhancement of the visceral pleura (arrow)

CASE 5: Adult with history of pulmonary infection as a child

Left PA chest radiograph shows asymmetric density of lungs and smaller left PA compared to right. Right expiratory PA chest radiograph shows air trapping in left lung

Swyer-James Syndrome •

• •

Bronchiolitis obliterans ➢ Idiopathic, viral, toxic inhalation,drug reaction, collagen vascular dz, transplant, chronic aspiration. ➢ Adenovirus as child – unilateral hyperlucent lung Small hyperlucent lung, hypoplastic ipsilateral artery Reticular nodular pattern with hyperinflation, central bronchiectasis


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Pediatric Radiology

CASE 6: Adult with right chest pain

A round mass is seen in the right phrenicovertebral angle posterior to the right atrium

Differential Diagnosis • • • •

Bronchopulmonary foregut malformation CPAM Neurogenic tumor Sequestration

Sequestration •

• • • •

Area of pulmonary tissue that does not have a normal connection to the bronchial tree Systemic arterial supply Can present as an infant or young child or as a young adult Often a history of recurrent infections in the same lobe May contain air

Sequestration • •

LLL most common Multiloculated cystic or solid mass • Intralobar vs. extralobar CT reveals a predominantly cystic mass with focal nodular thickening ➢ Pleural investment along the posterior wall in the right posterior mediastinum. The lower ➢ Extalobar – own pleural right image reveals an additional simple cyst in the left superior investment mediastinum ➢ Extralobar – systemic venous drainage ➢ Extralobar – infants, associated anomalies ➢ Extralobar-congenital/intralobar-acquired • CT vs. MRI vs. US – define vascular supply and drainage

Arteriogram shows the blood supply to the right lower lobe sequestration is coming from the celiac axis below the diaphragm. Abdominal blood supply frequent, and alters the surgical approach. The superior mediastinal lesion was a foregut duplication cyst

Pediatric Radiology

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CASE 7: 9 yo with SOB

PA and lateral chest radiographs show bilateral lower lobe consolidation with silhouetting of both hemidiaphragms. Also note the large cardiac silhouette, absence of the splenic shadow, and cholecystectomy clips (unusual in children)

Acute Chest Syndrome • • • •

Fever, chest pain, shortness of breath Rib infarction with splinting and atelectasis versus infection Treated with oxygen, antibiotics and pain medication +/- plasma exchange Look for associated findings of SS ➢ CM ➢ Absent splenic shadow ➢ Evidence of gallbladder disease ➢ Skeletal findings

CASE 8: 5 yo with fever and SOB Pediatric TB – Clinical Findings • • • • • •

Asymptomatic Cough Fever Malaise / FTT Respiratory distress Lethargy

Pediatric TB – Radiologic Findings • • • • • •

Normal Focal infiltrate – Ghon lesion Unilateral hilar adenopathy – Ranke complex Paratracheal adenopathy Subcarinal adenopathy Calcified granulomatous nodes


Left image shows unilateral hilar and right paratracheal adenopathy. Right image also shows secondary RML atelectasis

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Pediatric Radiology

Pediatric Seminar 2: Skeletal Dysplasia Ellen Chung, LTC, MC Radiologic Approach •

•

Assess Proportion ➢ Rhizo-, meso-, or acromelia ➢ +/- platyspondyly Assess Components of Bone ➢ Epiphyses small or irregular ◗ epiphyseal dysplasia ➢ Metaphyses widened, flared, or irregular ◗ metaphyseal dysplasia ➢ Diaphyses widened or thickened ◗ diaphyseal dysplasia

Achondroplasia Group •

All have abnormalities of the same chromosomal locus and gene product, fibroblast growth factor receptor 3 (FGFR3) ➢ Thanatophoric dysplasia ➢ Achondroplasia ➢ Hypochondroplasia

Thanatophoric Dysplasia • • • • •

AD Probably the most common lethal bone dysplasia Skull - kleeblatschadel in type II Thorax - very short ribs and handlebar clavicles Spine – small flat vertebral bodies with round anterior ends, U or H-shaped on AP

Thanatophoric Dysplasia •

• •

Pelvis ➢ Small, flared iliac bones ➢ Very narrow sacrosciatic notches, flat dysplastic acetabula Extremities – telephone receiver Femora

Thanatophoric dysplasia. Note small flat vertebral bodies, very short ribs, and “telephone receiver” femora

Achondroplasia • • •

Most common nonlethal skeletal dysplasia AD, spontaneous mutation rate 8-% Skull ➢ Large with midface hypoplasia ➢ Small skull base and foramen magnum

Achondroplasia •

•

Spine ➢ Very short pedicles – risk of spinal canal stenosis ➢ Decrease in interpediculate distance – lumbar spine Pelvis ➢ Elephant-ear iliac wings ➢ Flat acetabular roofs ➢ Narrow sacrosciatic notches

Achondroplasia •

Extremities ➢ Rhizo- > meso- and acromelia ➢ Hands – brachydactyly with metaphyseal cupping of MC’s ➢ Knees – chevron and inverted chevron deformities ➢ Hips proximal femoral fade out and hemispheric capital femoral epiphyses

Pediatric Radiology

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Pediatric Seminar 2: Skeletal Dysplasia

Case 1 – 5 yo with short stature

Achondroplasia. AP view of the extremities show short widened bones with flared metaphyses and chevron deformities of the distal femora and proximal tibiae

Achondroplasia. AP view of the pelvis shows flared “elephant ear” iliac wings, shallow acetabular roof, narrowed sacrosciatic notches conferring a champagne-glass configuration to the pelvis, and narrowing of the interpediculate distance in the lower lumbar spine

Short Rib-Polydactyly Group •

•

Includes ➢ SRP I-IV - some with, some without polydactyly ➢ Asphyxiating thoracic dysplasia ➢ Chondroectodermal dysplasia Shortest ribs of all dysplasias

Short Rib-Polydactyly • • •

Thorax – shortest ribs, horizontal ribs Pelvis – small ilia, notched acetabula Extremities ➢ Micromelia ➢ Rolling pin-shaped or round-ended or spiked femora ➢ Ovoid tibiae ➢ Polydactyly in some types

Asphyxiating Thoracic Dysplasia (Jeune Syndrome) •

•

Mixed prognosis ➢ Some succumb early from respiratory compromise ➢ Others die later from progressive nephropathy Thorax ➢ Long and barrel-shaped ➢ Handlebar clavicles ➢ Short horizontal ribs with flared ant ends

Achondroplasia. AP views of the hands show brachydactyly with cupping of the metacarpal metaphyses

Asphyxiating Thoracic Dysplasia (Jeune Syndrome) • •

•

Spine – normal Pelvis ➢ Trident acetabular roof ➢ Flared iliac wings ➢ Narrowed SS notches Extremities – cone-shaped epiphyses in hands


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Pediatric Radiology

Case 2 – Newborn with severe respiratory distress

Asphyxiating thoracic dystrophy. Gross photo shows bilateral polydactyly of the hands and very small chest

Asphyxiating thoracic dystrophy

Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) •

• •

Nonskeletal findings important in diagnosis ➢ Hair, nail and teeth abnormalities ➢ Congenital heart disease Thorax – small with short ribs Pelvis ➢ Trident acetabula ➢ Small, flared iliac wings ➢ Narrowed SS notches

Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) •

Extremities ➢ Generalized shortening ➢ Exostosis of proximal medial tibia ➢ Post-axial polydactyly ➢ Capitate-hamate fusion ➢ Extra carpal bone ➢ Cone-shaped epiphyses

Case 3 – 6 year old with history of congenital heart disease

Chondroectodermal dysplasia. AP views of the hands show bilateral postaxial polydactyly and cone shaped epiphyses (arrows)

Pediatric Radiology

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Chondrodysplasia Punctata Group • •

•

All have epiphyseal stippling Rhizomelic ➢ AR, death in first year ➢ Spine – coronal clefts ➢ Symmetric bilateral shortening of femora Conradi-Hunermann ➢ X-linked dominant ➢ Asymmetric shortening of limbs ➢ Diffuse stippling of the spine

Case 4 – newborn boy with respiratory distress

Chondroectodermal dysplasia. AP view of pelvis and LE’s shows short long bones and short, flared iliac wings, with trident-shaped acetabular roofs

Chondrodysplasia punctata. Note diffuse stippled epiphyses and coronal clefts in the thoracic spine

Metaphyseal Chondrodysplasia Group • •

All have normal spine and wide irregular metaphyses Jansen-type ➢ Most severe ➢ Infantile presentation ➢ AD ➢ Extremities – extensive irregular, expanded metaphyses ➢ Hyperparathryroidism

Metaphyseal Chondrodysplasias • •

Schmid-type – mildest, metaphyseal flaring, especially around knees Shwachman-Diamond – AR ➢ Pancreatic insufficiency – malabsorption and lipomatosis of pancreas ➢ Cyclic neutropenia – recurrent infections

Metaphyseal Chondrodysplasias •

McKusick-type ➢ Cartilage-hair hypoplasia ➢ High frequency in the Amish and Finnish populations ➢ Hirschprung disease ➢ Immune deficiency and increased risk of malignancy, especially leukemia and lymphoma


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Pediatric Radiology

Case 5 – 12 yo boy with short stature and unusual hair McKusick-Type • • •

Spine – square vertebral bodies Extremities – flaring, cupping and fragmentation of metaphyses, especially at the knees Hands – shortening with metacarpal and phalangeal cupping and coning

Dysplasias with Prominent Membranous Bone Involvement •

Cleidocranial dysplasia ➢ AD, marked variability in expression Metaphyseal chondrodysplasia, McKusick type. ➢ Drooping narrow chest, hypermobile shoulders, Note widening and irregularity of metaphyses and dental anomalies about the knees ➢ Mild short stature ➢ Skull – wormian bones and wide, open anterior fontanelle

Dysplasias with Prominent Membranous Bone Involvement •

Cleidocranial dysplasia ➢ Thorax – hypoplasia or absence of clavicles, downward sloping ribs ➢ Spine – posterior wedging of vertebral bodies ➢ Pelvis - high, narrow iliac wings, absence or hypoplasia of pubic bones ➢ Extremities – tapered distal phalanges

Case 6 – Fretful 8 mo whose pediatrician thinks he has bilateral clavicular fractures

Cleidocranial dysplasia with wormian bones

Cleidocranial dysplasia. With hypoplastic clavicles with pseudarthroses

Cleidocranial dysplasia. Note absence of ossified pubic bones, narrowed sacrosciatic notches and narrow ilia

Pediatric Radiology

Cleidocranial dysplasia. Tapered distal phalanges

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Dysostosis Multiplex Group • • •

Mucopolysaccharidoses and mucolipidoses All AR All produce similar radiographic complex of findings

Hurler Syndrome • • •

•

Present in infancy or early childhood Skull – J-shaped sella Thorax ➢ Short thick clavicles ➢ Oar-shaped ribs Spine ➢ Gibbus deformity ➢ Inferior beaked T-L vertebral bodies

Hurler Syndrome • •

Pelvis – small flared iliac wings with inferior tapering and steep acetabular roofs Extremities ➢ Wide diaphyses of long bones and metacarpals ➢ Pointed proximal metacarpal poles

Case 7 – short 3 yo with unusual facial appearance

Dysostosis multiplex due to Hurler syndrome. AP chest shows thick clavicles and paddleshaped ribs. Lateral spine shows gibbus deformity at thoracolumbar junction and inferior beaking of vertebral bodies Hurler syndrome. AP pelvis shows flared iliac wings with inferior tapering and steep acetabular roofs

Hurler syndrome. Another patient with a J-shaped sella. Dental abnormalities are related to enlargement of the tongue Another patient with Hurler syndrome showing the pointed proximal poles of the metacarpals


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Pediatric Radiology

Morquio Syndrome • • • •

No J-shaped sella Vertebral beak is in the middle Ribs are widened but not oar-shaped Proximal metacarpal poles are rounded

Dysplasias with Decreased Density • •

Very large group of conditions that share an abnormality of type I collagen Osteogenesis Imperfecta Type II ➢ Invariably lethal Hurler syndrome. Second patient with coarsening of the soft tissues of the ➢ Skull – poor or absent ossification face including the tongue ➢ Thorax – small chest with beaded ribs

Osteogenesis Imperfecta •

•

Type II ➢ Spine – very poor ossification with collapse of vertebral bodies ➢ Extremities – accordion femora Other types ➢ Skull – more than 8-10 wormian bones, variable ossification ➢ Extremities – variable osteoporosis and fractures

Case 8 – 31-week fetus

Osteogenesis imperfecta. Prenatal ultrasound shows no shadowing by the skull. The near side of the brain is much too well visualized. Also the transducer is indenting the skull

Increased Bone Density •

Osteopetrosis (Albers-Schonberg Disease) ➢ Failure to resorb primary spongiosa ➢ Severe precocious type – AR ➢ Delayed type – AD ➢ Reduced bone marrow space ◗ anemia and extramedullary hematopoesis

Osteopetrosis – Radiographic Findings • • • •

Generalized increased bone density Skull – thick and dense especially at the base with foraminal narrowing Spine – “sandwich” or “picture-frame” vertebral bodies Extremities ➢ Widened metaphyses with dense bands ➢ Bone-within-bone appearance

Pediatric Radiology

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OI. No ossification of the membranous portions of the skull. Small chest with beaded ribs and accordioned long bones due to multiple in utero fractures


Osteopetrosis with diffusely dense bones, bone-within-bone appearance, and deformity due to pathologic femoral neck fractures Osteopetrosis. Acute femoral neck fracture on earlier film

Osteopetrosis with sandwich vertebrae

Pyknodysostosis • • • •

• •

AR, presents in infancy Micrognathia, short fingertips, fractures Generalized osteosclerosis Skull ➢ Wormian bones ➢ Marked delay in closure of sutures and fontanelles ➢ Obtuse mandibular angle Thorax – resorption of acromial ends of clavicles Extremities – resorption of phalangeal tufts

Case 9 – 17 year old with short stature

Pyknodysostosis. Skull shows persistent unfused sutures and very obtuse mandibular angle

Pyknodysostosis. Diffuse increased bone density and resorption of phalangeal tufts


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Pediatric Radiology

Pediatric Seminar 3: Cystic Fibrosis and Pulmonary Infections of the Immunocompromised Child Ellen Chung, LTC, MC Case 1 – 15 yo with fever and respiratory distress Cystic Fibrosis Epidemiology1 •

• • •

Most common lethal autosomal recessive disease in white populations (1:2500 live births) Rare in blacks (4% of cases) Very rare in Asians (0.2% of cases) Exceedingly rare in Native Americans (0.02% of cases)

1Cystic Fibrosis Foundation Registry 1990

Annual Data Report.

Cystic Fibrosis Epidemiology1 • Mean age at diagnosis 6 mo • 80% diagnosed by age 3 years • 10% of newly diagnosed are 18 years or older • 52.8% male • 11% of new diagnoses due to family history of CF

PA chest radiograph shows bilateral hyperinflation and right much greater than left linear and nodular opacity predominantly in the upper lobes. Waters view of the paranasal sinuses demonstrates complete opacification of the paranasal sinuses

1Cystic Fibrosis Foundation Registry. 2004 Annual Data Report.

Cystic Fibrosis CF Gene • • • • •

Long arm of chromosome 7 Encodes a large single chain protein, CF transmembrane conductance regulator (CFTR) CFTR forms cell membrane chloride channel 3 base pair deletion (delta F508 mutation) accounts for 70% CF cases Remaining cases due to over 1100 different mutations

Cystic Fibrosis Cellular Physiology • • • •

Normal CFTR: Epithelial chloride channel supplies luminal water by osmosis Abnormal CFTR: Decreased water flow produces viscous inspissated luminal secretions Exocrine duct obstruction Enhanced bacterial colonization

Cystic Fibrosis Exocrine Sites • • • • • • •

Bronchioles and small bronchi Pancreas Intestinal crypts Biliary ducts Vas deferens Cervix Sweat and salivary glands

Pediatric Radiology

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Pediatric Seminar 3: Cystic Fibrosis

Cystic Fibrosis Clinical Presentation • • • • • •

Neonatal intestinal obstruction in 16-20% Respiratory manifestations & pansinusitis Failure to thrive Malabsorption Unexplained hypochloremic acidosis Positive family history

Cystic Fibrosis Protean Expressions •

•

Pulmonary Manifestations ➢ Recurrent infection ➢ Pulmonary insufficiency Gastrointestinal Manifestations ➢ Pancreatic abnormalities ➢ Intestinal obstruction ➢ Nonobstructive bowel manifestations ➢ Hepatobiliary disease ➢ Gastrointestinal malignancy

Cystic Fibrosis Lung Disease • • • • • • •

Principal cause of morbidity and mortality Abnormal mucus obstructs terminal airways Decreased mucociliary transport Air trapping & increased dead space Colonization by S. Aureus, H. flu, Pseudomonas sp., atypical mycobacteria, Burkholderia cepacia complex Bronchiectasis Alveoli usually spared

Cystic Fibrosis Pathogenesis of Lung Disease • • • • •

Airway macrophages promote neutrophil (PMN) influx Elastase from autolyzed PMNs digests elastin, causing bronchiectasis and fibrosis Elastase is powerful mucus secretagogue Neutrophil death release high molecular weight DNA (Pulmozyme) Abnormal CFTR protein may bind pathogens (mucoid strain of Pseudomonas)

Cystic Fibrosis Pulmonary Complications • • • • • •

Pneumothorax Allergic bronchopulmonary aspergillosis Acute and chronic respiratory failure Hemoptysis from dilated bronchial arteries Pulmonary hypertension Cor pulmonale

Lung Disease in CF Pathologic Features • • • •

Bronchi filled with mucoid exudate laden with degenerating neutrophils Bronchial mucosa features increased goblet cells & focal metaplastic squamous epithelium Ciliary changes Bronchiectasis


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Pediatric Radiology

Case 2 – 18 yo with respiratory distress

PA chest radiograph shows tunneled central venous catheter, bilateral linear and confluent opacities, and bilateral hilar enlargement

Lung Disease in CF Radiographic Features • • • • •

Increased lung volumes Bronchial impaction (“finger in glove” appearance) Hilar adenopathy Saccular bronchiectasis with upper lobe preponderance Thin-walled subpleural cysts

Case 3 - 14 yo with fever and cough Lung Disease in CF Cysts • • • • •

Cyst seen on CXR - saccular bronchiectasis vs abscess Less likely pneumatocele AFL may be seen in bronchiectasis or abscess Abscess rare in older children except with CF Subpleural blebs may be seen on HRCT

Case 4 – 13 yo with recurrent respiratory tract infections

PA radiograph of the chest shows a round mass with air fluid level in the right middle lobe

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High resolution chest CT image on the left is in expiratory phase of respiration and shows mosaic pattern typical of small airways disease. Image on right shows dilated bronchi with thickened walls due to bronchiectasis

Lung Disease in CF - HRCT • • • • •

Currently CT is not part of the routine follow-up of CF patients. CXR and PFT are. HRCT is much more sensitive than CXR for bronchiectasis – 90% HRCT is much more sensitive for early and reversible changes of CF than CXR or PFT HRCT is becoming an outcome surrogate for CF Objective evaluation of HRCT is prerequisite

Case 5 – 14 yo with malabsorption

Axial IV contrast enhanced CT image shows complete replacement of the pancreas with fat

Exocrine Pancreatic Insufficiency • • • •

> 80% have clinical pancreatic insufficiency Insufficient lipolytic & proteolytic enzymes for normal digestion & absorption of nutrients Steatorrhea correlates with enzyme output < 10% of normal Delta 508 mutation – higher incidence of pancreatic insufficiency and earlier onset of lung disease and colonization with pseudomonas

“Pancreatic Sufficiency” • • • • • • •

10-15% of CF patients Do not require enzyme supplements Better nutritional status Older at diagnosis – later onset of lung disease Lower Pseudomonas colonization rates Better prognosis May convert to pancreatic insufficiency with age (genetically determined)

Cystic Fibrosis Endocrine Dysfunction • • • •

Glucose intolerance in 30-50% Diabetes mellitus develops in 1% of children & 13% of adults Screened annual starting at age 14 DM due to pancreatic fibrosis & other unknown factors


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Pediatric Radiology

Cystic Fibrosis of the Pancreas Pathologic Features • • • • • • •

Proximal duct obstruction from inspissated pancreatic juice Acinar atrophy & inflammation Progressive interstitial fibrosis Fatty replacement Duct ectasia Micro- & macrocysts Calcification – punctate and diffuse or chunky

Cystic Fibrosis of the Pancreas Imaging Findings • • • •

Radiographs: Punctate pancreatic calcifications US: Small echogenic pancreas CT: Fat attenuation, +/- calcifications, small cysts, complete pancreatic replacement by macrocysts (rare) MRI: Variable signal intensity depending on amount of fat & fibrosis

Case 6 - 12 yo with failure to thrive

Sonogram on the left shows simple cyst anterior to the SMV and posterior to the left lobe of the liver. CT shows two simple cysts in the pancreas

Case 6 – Newborn with emesis and abdominal distension

KUB of infant shows many loops of dilated, unfolded bowel with soap bubble lucencies in the right lower quadrant

Pediatric Radiology

Contrast enema in the same patient showing a microcolon. The ileum is of larger caliber than the colon and shows multiple filling defects (arrow)

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GI Manifestations of CF Intestinal Obstruction • • • • •

Meconium ileus Meconium plug syndrome Distal intestinal obstruction syndrome (“meconium ileus equivalent”) Intussusception Fibrosing colonopathy

GI Manifestations of CF Meconium Ileus • • • • •

Earliest clinical manifestation of CF 10-15% of CF patients present with meconium ileus All pts. with meconium ileus have CF Dysfunction of secretory intestinal epithelium plus panc enzyme insufficiency Distal small bowel obstruction from dessicated meconium pellets

Meconium Plug Syndrome • • • • •

Colonic obstruction in neonates Not a syndrome but a symptom 25% have CF The rest have functional immaturity of the colon or Hirshprung disease Contrast enema may relieve obstruction

Distal Intestinal Obstruction Syndrome (DIOS) • • • • • • • •

Formerly termed “meconium ileus equivalent” Reserve dx for patients with obstruction 10-15 % of CF pts (usually adolescents & adults) Results from fluid loss and poor compliance with pancreatic enzyme replacement May mimic appendicitis (appy uncommon in CF) Distal obstruction pattern on plain films Fecal mass in RLQ Enema may be therapeutic but usually treated medically

GI Manifestations of CF Rectal Prolapse • • • • •

Occurs in 20% of CF patients Presents in first years of life Resolves spontaneously by approx. 5 years Associated with bulky stools, diarrhea, or constipation Improved by pancreatic enzyme supplementation

GI Manifestations of CF Intussusception • • • •

Occurs in approx. 1% of pts. with CF Mean age of presentation = 10 years Usually ileocolic Lead points: adherent fecal residue, enlarged lymphoid follicles, chronically distended appendix, or DIOS

GI Manifestations of CF Fibrosing Colonopathy • • •

Contrast enema shows shortening and loss of haustration in ascending colon with short focal narrowing. Also there is a large round filling defect proximal to the narrowing. Colonscopy revealed the narrowing was a stricture and the filling defect was an inflammatory pseudopolyp

Usually right colon High-strength pancreatic enzyme supplementation compounded by high protease intake strongly implicated Submucosal fibrosis, fatty infiltration, mural thickening, haustral loss, shortening, stricture formation


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Pediatric Radiology

Case 7 – 10 yo with abdominal pain

Air lucency is seen in the wall of the colon in the left upper quadrant on this CT image representing pneumatosis

Cystic Fibrosis Nonobstructive Bowel Manifestations • • • • • •

Thickened nodular mucosal folds in duodenum & small bowel “Jejunization” of colon Duodenal ulcer seen at autopsy in 10% Gastroesophageal reflux Barrett esophagus Pneumatosis intestinalis

Cystic Fibrosis Hepatobiliary Disease • • • • • •

Cholelithiasis (cholesterol stones) in 12-24% Microgallbladder at autopsy in 25% Atrophy or obstruction of cystic duct Distal CBD stricture Fatty liver Focal biliary cirrhosis and portal hypertension

Case 8 – 16 year old with hematemesis

Left images shows longitudinal linear filling defects in the esophagus indicating varices. Also note bronchial artery embolization coils. Axial image from a contrast enhanced CT shows fat density in the liver, cholecystectomy clips, and a large coronary vein in the left upper quadrant. (Patient underwent splenectomy as an infant.)

Focal Biliary Cirrhosis • • • • • •

Pathognomonic for CF Up to 40% of CF pt Attributed to thickened intrahepatic bile duct secretions Periductal inflammation, focal biliary fibrosis, & ductular proliferation Multinodular cirrhosis in 5 - 12% Portal hypertension and end-stage liver disease 1%

Pediatric Radiology

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Radiologic Pathology 2006-2007 - Volume III - INDEX

1st Branchial Cleft Cysts 1278 Aberrant Internal Carotid Artery 1073 Aberrant LSCA 1377, 1454 Aberrant RSCA (Right Subclavian Artery) 1375 Abnormal Ureteral Insertion 1332 Abnormal Axis of Intrarenal Collecting System 1333 Abscess 1133 Abscess (Intracranial) 1234 Absent Internal Carotid Artery 1073 Absent Pulmonary Artery 1455 Absent Pulmonary Valve Syndrome 1380 Abuse injuries (Child) - Differential Diagnosis 1499 Accessory Parotid Tissue 1275 Achondroplasia 1527 Acinic Cell Carcinoma 1280 Acoustic Schwannoma 1079 Acquired Cholesteatoma 1076 Acquired Renal Cysts 1382 Active plaques (MS) 1038 Acute Chest Syndrome 1526 Acute disseminated encephalomyelitis 1040 Acute Disseminated Encephalomyelitis (ADEM) 1040 Acute GI Disorders (Infants and Children) 1353 Acute Meningitis 1232 Acute Pulmonary Interstitial Emphysema 1446 Acute Pyeloneprhitis 1335 Acyanotic CHD 1472 Acyanotic CHD with Increased PBF 1473 ADC (Aids Dementia Complex) 1237 Adenoid Cystic Carcinoma 1280 Adenoid Enlargement 1368 Adenoma 1251 Adenoma (Pleomorphic) 1279 Adenoma Sebaceum 1198 Admixture Lesions 1479 Adrenal Hematoma 1410 Hemorrhage 1410 Masses (Pediatric) 1402 Medullary Tumors 1402 Metastases 1410 Adrenocortical Cancer 1409 Adrenocortical Tumors 1409 Aggressive Fibromatosis 1276 AIDS Dementia Complex (ADC) 1237 AIDS related infections (Intracranial) 1231 Air Leak 1445 “Pseudocysts” 1447 Pneumomediastinum 1447 Pneumothorax 1447 Airway (Pediatric) 1363 Albers-Schonberg Disease 1533 Alcohol 1041 Allergic fungal sinusitis 1244 with polyps 1244 Alobar Holoprosencephaly 1310 Alveolar Proteinosis 1451 AMEN Differential Diagnosis 1079 Amyloid angiopathy 1039 Anencephaly 1307 Aneurysm (suprasellar) 1255 Aneurysms (Intracranial) 1210 Deconstructive Therapy 1218 Dissecting 1212

Saccular 1212 Angiomyolipoma 1201, 1399 ANGIOMYOMATOSIS vs. LYMPHANGIOMYOMATOSIS 1202 Annular Tear/Fissure 1290 Annulus of Zinn 1089 Anomalous innominate artery 1454 Antenatal Pelvicaliectasis 1336 Anterior chamber: aqueous humor 1088 Antoni A and B fibers 1079 Antral Web 1342 Antrochoanal polyp 1245, 1368 Anus (Imperforate) 1351 Aortic Arch 1378 Double 1378 Embryology 1374 Anomalies 1454 Aortic Coarctation 1466 Aortic Stenosis 1470 Aorticopulmonary Window 1477 Appendicitis 1360 Aqueous humor (Globe) 1088 Aqueous Protein Solution 1163 Arachnoid Cyst 1109 Arnold Chiari Malformation 1308 Arrested Pulmonary Development 1439 Arteriosclerosis 1039 Arteriosclerosis / venous collagenosis 1039 Arteriovenous Fistula (Orbit) 1100 Arteriovenous Malformation (Lung) 1442 Ash-Leaf Spots 1199 Asphyxia 1506 Asphyxiating Thoracic Dysplasia (Jeune Syndrome) 1528 Aspirated Foreign Body 1365 Aspiration syndromes 1448 Astrocyte Mutation 1142 Astrocytoma 1058, 1141, 1109, 1145 Circumscribed 1139 Diffuse 1142 Pilocytic 1139 Atresia Colonic 1349 Duodenal 1345 Esophageal 1341 Ileal 1347 Jejunal 1347 Tricuspid 1468 Atrial Septal Defect 1463, 1474 Atrial Switch 1469 Atrioventricular Canal 1477 Atrioventricular Septal Defect 1464 Atypical Teratoid / Rhabdoid Tumor 1053 Autosomal Dominant Polycystic Kidney Disease (ADPKD) 1387 Autosomal Recessive Polycystic Kidney Disease (ARPKD) 1383 AVM (Brain) 1222 AVM Grading (Intracranial) 1222 Azygos Continuation 1460 Azygous Vein 1459 Basal Ganglia Thalamus 1119 Beckwith-Wiedemann syndrome 1388 Benign Astrocytoma 1139, 1143 Benign Lymphoepithelial Lesions 1278 I1

Benign Sacrococcygeal Teratoma 1422 Benign sinus lesions 1247 Berdon Syndrome 1349 Bezoar 1356 Bezold abscess 1078 Biliary Cirrhosis (Cystic Fibrosis) 1541 Binswanger's 1039 Bladder Diverticula 1332 Blood Brain Barrier 1131 Blowout Orbital Trauma 1093 Blunt trauma 1498 Blyth and Ockenden Clinical Classification (ARPKD) 1384 Bone Marrow Components 1425 Bone Marrow Imaging (Pediatric) 1425 Bony Orbit 1088 Borden Classification (Dural AVF) 1225 Borrelia burgdorferi 1237 Bourneville Disease 1197 Brachial Plexus Traction Injury 1296 Brain (Congenital Abnormalities) 1307 Brain Tumor 1137 Branchial Cleft Cyst 1269, 1278 Bright cerebellum sign 1496 Bronchial Atresia 1372 Bronchogenic Cyst 1437 Bronchopulmonary Dysplasia 1447 Butterfly pattern 1046 Callosal Dysgenesis 1316 Calyceal Diverticulum 1381 Capillary Telangiectasia (Brain) 1229 Carbon monoxide poisoning 1041 Carcinoma (Choroid Plexus) 1151 Cardiac Imaging (Pediatric) 1453 Cardiomediastinal Silhouette Size 1473 Carotid Artery 1073 Carotid Body Paragangliomas 1272 Carotid Space 1269 Catecholamine Production 1402 Caudal Regression Syndrome 1265 Cavernous Angioma (Brain) 1228 Cavernous Hemangioma 1098 Cavernous Malformation (Brain) 1229 Cavernous Sinus Invasion (Pituitary Macroadenoma) 1254 Cellulitis (Orbit) 1101 Central Neurocytoma 1058, 1060, 1061 Central Posterior Fossa Lesion 1109 Cephaloceles 1307 Cerebellar Infarction 1507 Cerebellar Liponeurocytoma 1050 Cerebellopontine Angle Masses 1079 Cerebral edema 1496 Cerebral Hemiatrophy (Dyke-Davidoff-Masson Syndrome) 1312 Cerebral Infarction 1123 Cerebral Intraventricular Neoplasms 1058 Cerebral Neuroblastoma 1157 Cerebritis 1231 Ceruminoma 1079 Cervical aortic arch 1378 Cervical Fascia 1266 Chamber Assessment 1472 CHARGE Syndrome 1366 Chemotherapy 1042, 1155 Chiari I Malformation 1308 Chiari II Malformation - (Arnold Chiari Malformation) 1308 Chiari III Malformation 1309 Chiari IV Malformation 1309 Chiasmatic-hypothalamic glioma 1256

Child Abuse 1491 Differential Diagnosis 1499 Childhood Neck Neoplasms 1370 Choanal Atresia 1366 Cholesteatoma 1074, 1076 Cholesterol Granuloma (Cyst) 1083 Chondrodysplasia Punctata 1530 Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) 1529 Chondrosarcoma 1084 Chordoma 1158 Choriocarcinoma 1178 Chorioretinitis 1092 Choroid Plexus Carcinoma 1063, 1064 Metastasis 1065 Neoplasms 1151 Papilloma 1063 Tumors 1063 Choroidal Detachment 1090 Chylothorax 1448, 1451 Clear Cell Sarcoma (Kidney) 1396 Cleft Brain 1314 Clivus 1115 Cloquet’s canal 1091 Closed-Lip (Fused) 1314 CNS Bacterial Infections 1130 CNS infections (acquired) 1232 CNS Lyme Disease 1237 CNS Lymphoma 1045 CNS Neoplasms – Chromosome Loss of Heterozygosity 1192 CNS Neoplasms: Chromosomes 1184 CNS Tuberculosis 1235 Coats’ Disease 1092 Cochlea 1068 Colloid Cyst 1058, 1062, 1116, 1163 Colonic Aganglionosis (Total) 1351 Colonic Atresia 1349 Common Atrium 1485 Complete Atrioventricular Septal Defect 1464 Complete Labrynthine Aplasia 1072 Complete Transposition of Great Vessels 1479 Complicated Pneumonia 1523 Compression Fractures 1430 Conal Lesions 1094 Congenital Abnormalities of the Brain 1307 Adrenal Hyperplasia 1411 Anomalies (UTI) 1329 Cholesteatoma (Epidermoid) 1074 Dehiscence of Tegmen Tympani 1074 Heart Disease 1379, 1463, 1472 hyperplasia (Adrenal) 1410 Lobar Emphysema 1435 Lung Malformations 1435 Megacalyces 1339 Megacystis-Megaureter 1339 Midline Nasal Mass 1367 Spinal Anomalies 1260 Congenitally Corrected TGV 1480 Contrast Enema (Malrotation) 1344 Contrast Enhancement 1126 Contrast Enhancement- Abscess 1133 Contrast Enhancement: Hematoma 1134 Contusion (CNS) 1324 Convexity Extraaxial Differential 1124 Convexity Intraaxial 1123 I2

Cord Herniation (Idiopathic Transdural) 1296 Cord injury 1497 Coronary Artery Aneurysms-Kawasaki 1460 Cortical Tubers 1200 Cranial injury 1494 Cranial Nerve Enhancement 1129 Nerves 1107 Nerves III, IV, VI 1090 Vascular Development 1194 Craniopharyngioma 1114, 1253, 1255, 1319 Creutzfeldt-Jakob Disease 1235 Crohn Disease 1360 Crohn's 1037 Cryptococcus meningitis 1238 Cryptococcus neoformans 1238 CSF Dissemination 1156 Homeostasis 1152 Spread - Zuckerguss (Sugar Icing) 1157 CT Angiography: Basic Protocol 1453 Cyanotic CHD 1467, 1472, 1478 Cyanotic CHD with Decreased PBF 1487 Cyst (Neurenteric) 1264 Cystadenoma (Ovary) 1418 Cystic Adenomatoid Malformation 1436 Fibrosis 1535 CF Gene 1535 of the Pancreas 1539 Masses (Pediatric Renal Tumors) 1398 neoplasms (Ovary) 1415 Neoplasms: Ovarian Teratoma 1417 nephroma 1398 Partially Differentiated Nephroblastoma 1398 Renal Disease of Childhood 1381 Renal Tumor (Multilocular) 1398 Cysticercosis (Intracranial) 1236 Cysts of the CNS 1160 Dandy-Walker Malformation 1312 Darling’s Classification 1481 Dating of intracranial blood 1496 DAVF (Dural AVF) 1225 Dawson's fingers 1038 DBO’s MR Signal Abnormalities 1191 Deep and Periventricular 1121 Degenerative Disc Disease 1290 Degenerative Disease (Spine) 1290 Dehiscence of Tegmen Tympani 1074 Dehiscent jugular bulb 1073 DeMorsier’s Syndrome (Septo-Optic Dysplasia) 1310 Demyelinating Diseases 1037 Demyelination 1039, 1040, 1041 Imaging 1043 Dermal Sinus (Dorsal) 1264 DERMOID 1162 Dermoid / Epidermoid (Orbit) 1101 Dermoid Cysts 1287 Desmoplastic Infantile Ganglioglioma / Astrocytoma 1048 Differential diagnosis of abuse injuries 1499 Dilated Azygous Vein 1460 Dirty retrobulbar fat (Grave's Disease) 1094 Disc Disease 1290 Extrusion with Migration 1291 herniations and types 1291 Disorders of Neuronal Proliferation 1313, 1314

Distal Intestinal Obstruction Syndrome (DIOS) 1540 Dorsal Induction (Disorders of) 1307 Double Aortic Arch 1378 Double Arch 1454 Double Outlet Right Ventricle 1485 Double Ring sign 1085 Drug abuse 1041 Ductus Arteriosus 1456 Duodenal Atresia/Stenosis/Web 1345 Hematoma 1356 Duplication Cyst (GI tract location) 1360 Dural Tail 1131, 1169 Dyke-Davidoff-Masson Syndrome 1196, 1312 Dysembryoplastic Neuroepithelial Tumor 1051 Dysgenesis (Callosal) 1316 Dysgerminoma 1419 Dysostosis Multiplex 1532 Dysphagia 1374 Dysplasias with Prominent Membranous Bone Involvement 1531 Dysplastic Cerebellar Gangliocytoma (Lhermitte-Duclos Disease) 1049 EAC cholesteatoma 1078 Ear 1068 Ebstein Malformation 1488 Echogenic Kidneys in Neonate 1386 Ectopic Ureter 1332 Eisenmenger Physiology 1478 Elevated Prolactin 1113 Ellis-van Creveld Syndrome 1529 Embryonal Sarcoma 1515 small cell tumor 1053 Emphysema (Pulmonary Interstitial) 1446 Encephalitis 1040, 1231 Encephalocele 1074 Endocardial Cushion Defect 1477 Endolymphatic Sac Tumor 1205 Enteric Duplication Cyst 1360 Enteric Fistula (Dorsal) 1263 Enterocolitis (Necrotizing) 1353 Enterocolitis (Neutropenic) 1361 Ependymitis granularis 1037 Ependymoma 1058, 1109, 1112, 1149 Epidermoid 1081, 1161 Epidermoid Inclusion Cyst 1109 Epidermoid vs. Arachnoid Cyst 1109 Epidural abscess 1231 Epidural Phlegmon / Abscess 1295 Epiglottitis 1364 Epstein-Barr virus (EBV) 1037 Esophageal Atresia 1341 Esthesioneuroblastoma 1248, 1368 Ethmoid sinus 1240 Exocrine Pancreatic Insufficiency 1538 Exostoses 1288 Exostosis (External Ear) 1078 External auditory canal (EAC) atresia 1071 External Ear Masses 1078 External Ear Neoplasms 1079 External Otitis 1078 Extraaxial Lesions 1106 Extraaxial Tumors 1158 Extraconal Lesions 1100 Extralobar Sequestration 1440 Extraocular muscles (EOM) 1089 Extrapontine myelinolysis 1041

I3

Facet Joint Synovial Cysts 1292 Facial Nerve Palsy 1081 Failed Back Surgery Syndrome (see also FBBS) 1292 Fallot 1487 FBSS (Failed Back Surgery Syndrome) 1293 Cervical Spinal Canal Stenosis 1293 Ossification (Posterior Longitudinal Ligament) 1294 Posterior Longitudinal Ligament (Ossification) 1294 Type I Arachnoiditis 1293 Type II Arachnoiditis 1293 Fibromatosis (Aggressive - Masticator Space) 1276 Fibromatosis Colli 1267, 1371 Fibrosing Colonopathy (Cystic Fibrosis) 1540 Fibrous Dysplasia (Paranasal Sinuses) 1246 Fibrous Histiocytoma (Orbit) 1099 Filum Terminale (Congenital Anomalies) 1263 Fissures of Santorini 1078 Focal Biliary Cirrhosis 1541 Follicular cysts 1415 Foreign Body 1365, 1366 Forensic Radiology of Child Abuse 1491 Fourth Ventricle 1109 Fractures (Healing) 1493 Fried egg appearance 1061 Frontal horn “capping” 1037 Frontal Lipoencephalocystocele 1307 Frontal sinus 1241 Functional Ovarian Cyst 1416 Fungal sinusitis 1102, 1245 Ganglioglioma / Gangliocytoma 1047 Ganglioneuroblastoma 1402, 1408 Ganglioneuroma 1402 Gastric Atresia 1342 Gastrostomy Tubes 1356 Gastrulation 1263 Gaucher Disease 1431 Germ Cell Tumors 1176, 1418 Germ Cell Tumors-AFIP Series 1177 Germinal Matrix 1502 Germinoma 1121, 1177 Germinoma (Infundibular) 1257 Ghost tumor 1046 GI Disorders (Acute - Infants and Chidren) 1353 GI Tract Obstruction 1341 Glioblastoma Multiforme 1119, 1147, 1322 Glioma 1109 Glioma (Chiasmatic-hypothalamic) 1256 Globe 1088 Globus pallidus 1191 Glomerulocystic Disease 1386 Glomus jugulare 1082 Glomus tympanicum 1082 Gradenigo’s syndrome 1078 Grading Problems in Gliomas 1140 Graf System (Pediatric Hip) 1519 Granulocyte Colony Stimulating Factor 1429 Granulosa-theca Cell Tumor 1419 Graves’ 1037 Graves’ Disease 1094 Great Vessels (Transposition) 1468 Grey Matter Heterotopias 1315 HAART 1239 Hamartoma 1510 Hamartoma (hypothalamic) 1257 Healing of fractures 1493 Healing of metaphyseal fractures 1492 Hemangioblastoma 1109, 1111, 1203, 1320 Hemangioblastomatosis 1202

Hemangioendothelioma 1511 Hemangioma 1370 Hemangiomas (suprahyoid Neck) 1266 Hemangiopericytoma 1173 Hemangiopericytoma vs. Meningioma 1173 Hematoma (Duodenal) 1356 Hemimegalencephaly 1313 Hemorrhage 1502 Adrenal 1410 Child Abuse 1495 Intracranial 1221 Hemorrhagic Cysts (Ovary) 1416 Hemorrhagic Infarction 1131 Hemorrhagic Ovarian Cysts 1416 Henoch-Schönlein Purpura 1356 Hepatobiliary Disease (Cystic Fibrosis) 1541 Hepatoblastoma 1513 Hepatocellular Carcinoma 1514 Hernia (Inguinal) 1357 heroin 1041 Herpes encephalitis 1234 Heterotopias (Gray Matter) 1315 High Jugular Bulb (“Megabulb”) 1073 Highly Active Antiretroviral TX (HAART) 1239 Hip Effusion 1519, 1520 Hip Sonography 1518 Hirschprung Disease 1350 HIV encephalitis 1040, 1041 HIV Leukoencephalopathy 1238 Holoprosencephaly (Alobar) 1310 Holoprosencephaly (Semilobar) 1310 Horseshoe kidney 1330 HSV Encephalitis 1131 Hurler Syndrome 1532 Hyaline Membranes 1444 Hydranencephaly 1310 Hydrocarbon aspiration 1366 Hydrocephalus 1181 Hydrocolpos 1420 Hydronephrosis 1338 Hyperostosis in Meningiomas 1171 Hypertrophic Pyloric Stenosis 1354 Hypogenetic Lung Syndrome 1439 Hypoperfusion complex (Child Abuse) 1499 Hypopharyngeal cyst 1369 Hypoplastic left heart syndrome 1466, 1485 Hypothalamic Hamartoma 1257 Hypothalamus 1115 Hypoxic-ischemic encephalopathy 1039 Iatrogenic Demyelinating Disorders: Chemotherapy 1042 Iatrogenic white matter degeneration 1037 Idiopathic Transdural Cord Herniation 1296 Ileal Atresia 1347 Ileus (Meconium) 1348 Imaging Studies (UTI) 1331 Immaturity of the Colon (Functional) 1349 Immune reconstitution syndrome 1239 Immunocompromised Child 1535 Immunocompromised Patient 1045 Imperforate Anus 1351 Incudostapedial disruption 1085 Infantile Hemangioendothelioma 1511 Infarction 1506, 1507 Infections (Intracranial) 1231 Infections (Spine) 1290, 1295 Inferior orbital fissure 1088 Inflammatory disease of the salivary glands 1369 Infratentorial 1106

I4

Inguinal Hernia 1357 Inner Ear Anomalies 1071 Innominate Artery Compression 1455 Innominate Artery Compression Syndrome 1379 Interhemispheric extra-axial hemorrhage 1495 Internal Auditory Canal 1107 Internal Carotid Artery 1073 Interrupted Pulmonary Artery 1455 Intestinal Obstruction (Neonatal) 1341 Intraaxial Lesions 1106 Intraconal Lesions 1097 Intracranial Aneurysms 1210 blood (Dating) 1496 Germ Cell Tumors 1176 Germinoma 1177 Infections 1231 Lipoma 1182 Vascular Malformations 1220 Intralobar Sequestration 1440 Intramural Hemorrhage (GI - Differential) 1356 Intrarenal Collecting System (Abnormal Axis) 1333 Intrarenal Reflux 1334 Intrasellar Pathology 1251 Intraventricular Lesions 1106 Intraventricular Meningioma 1065 Intussusception 1357 Cystic Fibrosis 1540 Reduction 1358 Inverted Papilloma (Paranasal Sinuses) 1247 Irritable Hip 1519 Ischemic Enhancement 1131 Jantene Procedure 1469 Jejunal Atresia 1347 Jeune Syndrome 1528 JNA (Juvenile Nasopharyngeal Angiofibroma) 1246 Joubert’s Syndrome 1313 Jugular bulb 1073 Jugular Diverticulum 1073 Foramen Masses 1083 Paragangliomas 1270 Jugulotympanic Paraganglioma 1082 Juvenile Angiofibroma 1367 Juvenile Nasopharyngeal Angiofibroma (JNA) 1246 Kawasaki disease 1460 Keratosis obturans 1078 Kernohan-Sayre (AFIP) Grading System 1137 Kidney (Medullary Sponge) 1382 Kidney (Multicystic Dysplastic) 1399 Kidney (Rhabdoid Tumor of) 1395 Labrynthine Aplasia 1072 Lacrimal Gland Lesions 1102 Lacrimal Sac Lesions 1103 Langerhans Cell Histiocytosis (Sella) 1258 Large Endolymphatic Duct and Sac (LEDS) 1072 Laryngeal- Tracheopapillomatosis 1372 Laryngomalacia 1371 Laryngotracheal cleft 1371 Left (Double) Superior Vena Cava 1458 Left paramediastinal structures (Differential Diagnosis) 1458 Left Superior Vena Cava 1458 Leukocoria 1090 Leukoencephalopathy (HIV) 1238 Lhermitte-Duclos Disease 1049 Lingual Thyroid 1370 Lingual Thyroid Gland 1285 Lipoencephalocystocele (Frontal) 1307

Lipoma (Intraspinal) 1262 Liposarcoma (Suprahyoid Neck) 1267 Lissencephaly 1314 Listeria Monocytogenes 1130 Liver Metastases 1516 Liver Tumors (Pediatric) 1509 Lobar emphysema 1435 Long bone shaft fracture 1491 Long Parotid Tails 1275 Low Back Pain 1290 Low Intestinal Obstruction 1347 Lumbar 1292 Facet Arthropathy 1292 Spinal Canal and Foraminal Stenosis 1292 Lung Agenesis 1439 Lung Disease(Cystic Fibrosis) 1537 Lung Diseases in Neonates 1444 Lyme disease 1231 Lymphangioma (Orbit) 1100 Lymphatic Malformations (SupraHyoid Neck) 1267 Lymphocytic hypophysitis 1252 Lymphoepithelial Lesions (Benign - Suprahyoid Neck) 1278 Lymphoma 1324, 1371 CNS 1045 Orbit) 1098 Pediatric Renal Tumors) 1400 Sella) 1258 in AIDS 1324 vs. Toxoplasmosis 1324 Macroglossia 1369 Madelung’s Disease 1267 Malfixation (Duodenum) 1343 Malignant Astrocytoma 1145 Compression Fracture 1430 Germ Cell Tumors 1418 Meningioma 1172 Sinus Lesions 1247 Malrotation – UGI 1344 Malrotation (Duodenum) 1343 Mandibular Hypoplasia 1369 Marburg 1039 Marchiafava-Bignami disease 1042 Marfan syndrome 1460 Marrow Components 1425 Conversion 1427 Depletion Fatty Replacement 1432 Distribution 1427 Reconversion 1428 Replacement or Infiltration 1429 Masses - Ring Enhancing (CNS) 1322 Masticator Space 1274 Mastoiditis 1078 Mature Teratoma (Ovary) 1417 Maxillary sinus 1241 McKusick 1531 Measles 1037 Meckel Diverticulum 1359 Meckel-Gruber Syndrome 1388 Meconium Aspiration Syndrome 1449 Meconium Ileus 1348 Cystic Fibrosis 1540 Meconium Peritonitis 1348 Meconium Plug Syndrome 1540 Mediastinal Bronchogenic Cysts 1438 Medullary Cystic Disease Complex 1387

I5

Medullary Sponge Kidney 1382 Medullary Tumors (Adrenal) 1402 Medulloblastoma 1109, 1154, 1318 Medulloblastoma - Desmoplastic 1157 Megabulb 1073 Megacalyces 1339 Megalencephaly (Unilateral) 1313 Megaureter 1339 Melanoma (Uveal) 1092 Membranous Tracheitis 1365 Meninges (Neoplasms) 1164 Meningioma 1065, 1081, 1108, 1125, 1164 MENINGIOMA *Imaging Features: CT vs. MR 1169 Angiography Transit Time 1170 Atypical Imaging 1172 Dural Tail 1131 Hyperostosis 1171 Vasogenic Edema 1167 Suprahyoid Neck 1270 Suprasellar 1254 Tentorial 1320 MR Imaging 1167 Meningitis 1231 Meningocele 1262 Mesenchymal Hamartoma 1509 Mesoblastic Nephroma 1396 Metabolic imaging 1043 Metaphyseal Chondrodysplasia 1530 Metaphyseal fracture 1491-1492 Metastasis (Pituitary and Infundibulum) 1259 Metastatic Lesions (Orbit) 1100 Methotrexate 1042 Michel’s deformity 1072 Microangiopathy 1039 Microgastria 1342 Microgyria 1314 Middle Ear 1068 Midgut Loop (Normal Rotation) 1343 Midgut Volvulus 1343 Migraine 1039 Mirror Image Right Arch 1376 Modic Changes 1291 Modified Papile Classification 1502 Brain (Neonatal) 1501 Mondini’s dysplasia 1072 Monosymptomatic demyelinating 1037 Morquio Syndrome 1533 MR Angiography: Basic Technique 1453 Mucocele 1246 Mucoepidermoid Carcinoma 1281 Mucopolysaccharides 1094 Multicystic Dysplastic Kidney 1382, 1399 Multilocular Cystic Renal Tumor 1398 Multiple sclerosis 1037, 1039, 1325 MR 1038 Mustard Procedure 1469 myasthenia gravis, 1037 myelinolysis 1041 myelitis 1040 Myelocystocele (Terminal) 1262 Myelofibrosis 1431 Myeloid Depletion: MRI 1432 Myelomeningocele 1260 Nasal Cycle 1128 Dermoid 1367

polyps 1368 Nasopharyngeal Carcinoma (NPSCCa) 1283 Neck Neoplasms (Child) 1370 Necrotizing Enterocolitis 1353 Necrotizing External Otitis 1078 Neonatal Brain 1501 GI Tract Obstruction 1341 Hypoxic-Ischemic Injury 1502 Low Intestinal Obstruction (Differential Diagnosis) 1351 Pneumonia 1450 Respiratory Distress 1444 Lung Diseases 1444 Neoplasms (Benign - Masticator Space) 1276 Neoplasms (Malignant - Masticator Space) 1277 Neoplasms of the Meninges 1164 Nephroblastoma (Cystic Partially Differentiated) 1398 Nephroblastomatosis 1394 Cortical Nodule 1395 Diffuse 1394 Nephrogenic Rests: Location 1394 Nephroma (mesoblastic) 1396 Nerve Sheath Tumors 1187 Neuroblastic Tumors 1402 Neuroblastoma 1371, 1402 Stage Distribution 1408 Neuroectodermal Tumor 1053 Neuroepithelial Tumors 1047 Neurofibroma vs. Schwannoma 1188 Neurofibromas (Pelvis) 1423 Neurofibromatosis 1185 Type 2 1191 Type 1 or von Recklinghausen Disease 1185 Neuromyelitis optica (Devic syndrome) 1039 Neuronal Proliferation (Disorders of) 1313, 1314 Neutropenic Enterocolitis 1361 Non-Astrocytic Gliomas 1149 Non-Glial Lesions 1158 Nonhemorrhagic Infarction 1506 Non-Hodgkin Lymphoma (NHL) -Pharyngeal Mucosal Space 1283 Non-Lissencephalic Cortical Dysplasias Microgyria/Polymicrogyria 1314 Normal Cranial Nerve Enhancement 1129 Enhancement 1128 Marrow (MR Features) 1426 Pineal Calcification 1175 Thymus 1521 Vertebral Marrow: MRI 1428 Norrie’s 1091 Obstruction (GI Tract - Neonatal) 1341 Olfactory Neuroblastoma 1248 Olidodendroglioma 1152 Oncocytoma (Suprahyoid Neck) 1280 Ophthalmic veins 1088 Opportunistic neoplasm 1045 Optic Nerve Glioma 1097 Sheath Meningioma 1097 Optic neuritis 1040 Oral Cavity Normal Anatomy 1286 Orbit 1088 Orbital Cellulitis 1101 fissures 1088 Lymphoma 1098

I6

septal system 1088 Trauma 1093 Varix 1099 Organic toxins 1041 Oropharynx 1284 Osmotic myelinolysis 1041, 1042 Ossicular Derangement 1085 Ossifying Renal Tumor of Infancy 1397 Osteogenesis Imperfecta 1533 Osteoma (Paranasal Sinuses) 1246 Osteomyelitis 1431 Osteomyelitis (Spine) 1295 Pyogenic 1295 Tuberculous 1295 Osteopetrosis (Albers-Schonberg Disease) 1533 Osteoporotic Fracture 1430 Ostiomeatal complex (Paranasal Sinuses) 1241 Ostium Primum ASD 1464 Otitis 1078 Otosclerosis 1085 Otospongiosis 1085 Outer Ear Anomalies 1071 Ovarian Cancer 1420 Cyst 1416 Cystadenoma 1418 Maturation 1414 Tumors 1415 Ovary (Prepubertal vs Postpubertal) 1414 Palatine Tonsil Enlargement 1368 Pancreas (Child Abuse) 1498 Pancreatic injury 1498 Papillary Cystadenoma Lymphomatosum 1280 Papillary Endolymphatic Sac Tumor 1083 Papilloma 1151 Papovavirus 1238 Paradoxical Embolus 1475 Paragangliomas 1082, 1270 Paranasal Sinuses 1240 Paraovarian cysts 1415, 1417 Parapharyngeal Abscess 1365 Parapharyngeal Space 1267, 1268 Parasellar Region 1250 Parinaud Syndrome 1175 Parotid Space 1278 Parotid Tail 1275 Partial Anomalous Venous Return (Pulmonary) 1456 Patent Ductus Arteriosus 1456, 1476 Patterns of Enhancement 1126 Patterns of Location 1106 Pediatric Adrenal Masses 1402 Airway 1363 Hip Sonography 1518 Liver Tumors 1509 Pelvic Masses 1414 Posterior Fossa Tumors 1318 Renal Tumors 1390 Tuberculosis 1526 Pelvic Masses (Pediatric) 1414 Pelvicaliectasis (Antenatal) 1336 Peritonitis (Meconium) 1348 Periventricular Hemorrhagic Infarction 1503 Sonography 1503 Periventricular Leukomalacia 1504 periventricular white matter 1039 Persistent hyaloid (Cloquet’s) canal 1091 Persistent Hyperplastic Primary Vitreous (PHPV) 1091

Persistent Interstitial Pulmonary Emphysema 1446 Persistent Stapedial Artery 1073 Petrous Apex (Differential Diagnosis) 1083 Phakomatoses 1184 Pharyngeal Mucosal Space 1282 Pharyngeal Perforation 1342 Pheochromocytoma 1408 Phlegmon / Abscess (Epidural) 1295 Pial A-V Fistula 1225 Pilocytic Astrocytoma 1110, 1134, 1139 Pilocytic Astrocytoma (Juvenile Pilocytic) 1141 Pineal Calcification 1175 Pineal Cyst 1121, 1181 Pineal Neoplasms Laboratory Tests 1179 Parenchyma 1180 Region Masses 1175, 1320 Region Neoplasms 1178 Pineal/Quadrigeminal Cistern Region 1121 Pinealomas 1121, 1175 Pineoblastoma 1121, 1180 Pineocytoma 1121, 1180 Pituitary 1250 Pituitary Adenoma 1113, 1253 Apoplexy 1254 Macroadenoma 1253 Neoplasms 1251 Plasma Cell Granuloma 1522 Pleomorphic Adenoma 1279, 1288 Pleomorphic Xanthoastrocytoma 1051, 1141 Plexiform Neurofibromatosis 1423 Pneumatoceles 1524 Pneumonia 1523 Pneumonia (term & premature neonates) 1448 Polycystic Kidney Disease (autosomal Recessive) 1383 Polymicrogyria 1314 Port Wine Stain 1193 Post fossa cysts 1109 Posterior chamber 1088 Posterior Fossa Malformations 1312 Tumors (Pediatric) 1318 Posterior Hyaloid Detachment 1090 Posterior Reversible Encephalopathy Syndrome (PRES) 1039, 1040 Posterior rib fractures (visualization) 1493 Posterior Urethral Valves 1337 Precocious Puberty 1175 Premature Births 1444 Premature Brain 1501 Prepubertal ovary 1414 Primary Megaureter 1339 Pringle’s Disease 1199 Profound Asphyxia 1506 Progressive multifocal leukoencephalopathy 1040 Progressive Multifocal Leukoencephalopathy (PML) 1041 Prolactin 1113 Prolactinoma 1251, 1253 Proteinosis (Alveolar) 1451 Proximal Neonatal Intestinal Obstruction 1347 Pseudotumor (Orbit) 1095 Pubertal ovary 1414 Pulmonary Abscess 1524 Arterial Anomalies 1455 Artery Stenosis 1470

I7

Atresia with Intact Ventricular Septum 1489 AVM 1442 Blastoma 1522 Blood Flow 1472 Bronchogenic Cyst 1438 Hypoplasia 1439 Infections 1521 Infections (Immunocompromised Child) 1535 Interstitial Emphysema 1446 Sequestration 1440 Sling 1379, 1455 Underdevelopment 1439 Venous Anomalies 1456 Pulsatile Tinnitus Lesions 1074 Pyeloneprhitis 1335 Pyknodysostosis 1534 Pyloric Stenosis 1354 Pyogenic Abscess (Intracranial) 1234 Pyogenic Osteomyelitis 1295 Pyriform Aperture stenosis 1367 Radiation 1155 Injury (Brain) 1043 Necrosis vs. Tumor (CNS) 1323 Ranulas 1287 Rathke Cleft Cyst 1252 Reactive Airways Disease 1365 Rebleeding 1497 Rectal Prolapse (Cystic Fibrosis) 1540 Rectus: medial, lateral, superior, inferior 1089 Red Marrow Signal 1427 Reflux Nephropathy 1335 Renal Agenesis 1329 Renal Cell Cancer 1399 Renal Cyst 1381 Renal Ectopia 1330 Renal Ectopia and Fusion 1330 Renal Tumors (Infancy and Young Children) 1390 Respiratory Distress (Neonatal) 1444 Respiratory Distress Syndrome (RDS) 1444 Retained fetal lung fluid 1448, 1449 Retina 1089 Retinal Detachment (RD) 1090 Retinoblastoma 1091 Gene 1091 Retinopathy of prematurity 1092 Retrobulbar (“Postseptal”) Space 1089 Retropharyngeal Cellulitis 1364 Reversal sign 1496 Rhabdoid Tumor 1054 Rhabdoid Tumor of Kidney 1395 Rhabdomyosarcoma 1368, 1420 Orbit 1101 Male Bladder & Prostate 1421 Rhabdomyosarcomatoid variant of Wilms tumor 1053 Rhombencephalosynapsis 1313 Rib fracture 1492 Rib Notching 1189 Right aortic arch 1454 Right Arch 1376 Right paramediastinal structures (Differential Diagnosis) 1459 Right to Left Shunts 1487 Ring Enhancing Mass 1132 Ring Lesion Features For Infection 1132 Ring Lesions Differential 1132 Ring-enhancing Masses (CNS) 1322 Risk Factors (subarachnoid Hemorrhage) 1211

Rotation of Midgut Loop 1343 Round Pneumonia 1522 Rules for Ring Enhancing Mass 1132 Sacrococcygeal Teratoma 1421 SAH (Subarachnoid Hemorrhage) 1210 Aneurysms (Intracranial) - Infectious 1218 Aneurysms (Intracranial) - Treatment Options 1218 Clinical Grading Scale 1213 CT 1214 CTA 1215 DSA 1215 induced Vasospasm 1214 Infectious Intracranial Aneurysms 1218 Lumbar puncture 1214 MRA 1215 MRI 1215 Outcomes 1213 Patterns 1213 Radiologic Grading Scale 1213 Risk Factors 1210 Screening 1217 Salivary glands (Inflammatory disease) 1369 Salt and pepper appearance 1082 Sarcoidosis (CNS) 1321 Sarcoidosis (Sella) 1258 SATCHMO 1319 SCCa (Squamous Cell Carcinoma) 1248 Schizencephaly 1314 Schwannoma 1107, 1188, 1192 Acoustic - Vestibular 1079 Orbit 1098 Scimitar Syndrome 1457 Sclera 1089 Scutum 1076 Second Branchial Cleft Cyst 1269 Secundum ASD 1464 Segmental Spinal Dysgenesis 1265 Sella 1250 Sella/Parasellar Region - Differential 1113 Sellar Masses: “SATCHMO” 1319 Semicircular Canals (SCC) 1068 Semilobar Holoprosencephaly 1310 Senescent White Matter Changes 1039 Senile Macular Degeneration 1090 Septic Arthritis (Hip) 1520 Septo-Optic Dysplasia (DeMorsier’s Syndrome) 1310 Sequestration 1525 Sertoli-Leydig cell tumor 1419 Sex Cord-Stromal Tumors 1419 Shaking mechanism 1492 Short Rib-Polydactyly 1528 Shunt Lesions 1463, 1487 Sickle Cell Anemia 1429 Simple Renal Cyst 1381 Single Ventricle 1484 Physiology 1489 Sinus Mass Differential 1368 Sinus Venosus ASD 1464 Sinuses (Paranasal) 1240 Sinusitis 1243 Sinusitis (Fungal) 1102 Sjogren’s Syndrome 1279 Skeletal Dysplasia 1527 Skeletal injury (evaluation) 1494 Skull fracture 1497 SLE 1037 Soap-bubble appearance 1051 I8

Spetzler-Martin Grading System (Intracranial Vascular Malformations) 1222 Sphenoid sinus 1242 Spina Bifida 1262 Spinal Anomalies (Congenital) 1260 Caudal Regression Syndrome 1265 Chiari II Malformation 1261 Complex Dysraphic States 1263 Dorsal Dermal Sinus 1264 Dorsal Enteric Fistula 1263 Fibrolipomatous Infiltration of Filum 1263 Gastrulation 1263 HemiMMC/Hemimyelocele 1261 Intraspinal Lipoma 1262 Lipoma with Dorsal Defect 1261 Lipomyelomeningocele 1261 Meningocele 1262 Myelocele (Myeloschisis) 1261 Myelomeningocele 1260 Neurenteric Cyst 1264 Persistent Terminal Ventricle 1263 Posterior Spina Bifida 1262 Segmental Spinal Dysgenesis 1265 Spinal Dysraphism 1260 Split Cord Malformation 1264 Terminal Myelocystocele 1262 Tight Filum Terminale 1263 Spinal Dysraphism 1260 Spine 1290 Spine injury 1493 Split Cord Malformation 1264 Squamous cell carcinoma 1248 Stapedial Artery 1073 Stridor 1363, 1374 Sturge-Weber Syndrome 1193 Subacute sclerosing panencephalitis 1040 Subarachnoid Hemorrhage 1210 Subdural empyema 1231 Subependymal Giant Cell Astrocytoma 1058, 1061 Subependymal Nodules 1200 subependymal veins 1039 Subependymoma 1058, 1059 Subepidermal Fibrosis 1199 Subglottic edema 1363 Subglottic Hemangioma 1370 Sugar Icing 1156 Superior and inferior ophthalmic veins 1088 Superior Left Intercostal Vein 1458 Superior orbital fissure 1088 Suprahyoid Neck 1266 Suprasellar Masses 1253 Supratentorial 1106 Supratentorial Primitive Neuroectodermal Tumor 1052 Surfactant 1445 Surfactant B protein deficiency 1448 Swyer-James Syndrome 1436, 1524 Systemic Gas Embolism 1447 Systemic Venous Anomalies 1458 Taenia solium 1236 Tegmen Tympani 1074 Temporal Bone Fracture 1084 Temporal Bone: Anatomy 1068 Congenital Lesions 1068 Infectious Lesions 1076 Neoplastic Lesions 1076

Temporomandibular joint (TMJ) anomalies 1071 Tenon’s capsule 1089 Tentorial Meningioma 1320 Teratoid Tumor 1053 Teratoma 1178, 1371 Ovary 1417 Sacrococcygeal 1421 vs. Dermoid (Pineal Region) 1178 Tetralogy of Fallot 1455, 1467, 1487 Thanatophoric Dysplasia 1527 Thiamin deficiency 1042 THIRD VENTRICLE 1116 Thoracic MRA & CTA 1453 Thymus (Pediatric - Normal) 1521 Thyroglossal Duct Cysts 1284 Thyroid Gland (Lingual) 1285 Thyroid Orbitopathy (Graves’ Disease) 1094 Time Density Curves 1126 Tinnitus 1074 Tongue Base Mass 1369 Top 10 Pelvic Lesions 1423 Total Anomalous Pulmonary Venous Return 1480 Total Anomalous PV Return 1469 Total Colonic Aganglionosis 1351 Toxic Demyelination 1041 Toxocara canis 1092 Toxocariasis 1092 Toxoplasmosis 1119 Intracranial 1238 Tracheal bronchus 1372 Tracheal Stenosis 1372 Tracheomalacia 1371 Transient Tachypnea of Newborn 1449 Transposition of Great Vessels 1468 Transverse Myelitis 1039 Tricuspid Atresia 1468, 1484, 1489 Trilateral Retinoblastoma 1180 Truncus Arteriosus 1469, 1483 Tuberculosis (Intracranial) 1231 Tuberculosis Pediatric 1526 Tuberous Sclerosis or Bourneville Disease 1197 Tumefactive Demyelination 1134 Tumor Blush 1171 UGI (Upper GI Tract - Malrotation) 1344 ulcerative colitis 1037 Uncommon Neuroepithelial Tumors 1045 Undifferentiated Embryonal Sarcoma 1515 Unilateral Megalencephaly (Hemimegalencephaly) 1313 Upper esophageal foreign body 1366 Ureterocele 1333 Ureteropelvic duplication 1333 Ureteropelvic Junction Obstruction 1338 Urethral Valves (Posterior) 1337 Urinary Tract Infection (Child) 1329 US Guidance 1520 Uterine Morphology: Maturation 1415 Uvea: choroid 1089 Uveal Melanoma 1092 Uveal Metastasis 1093 VACTERL 1342 Vagal paraganglioma 1082 Vagale Paragangliomas 1271 Vaginal Rhabdomyosarcoma 1421 Vallecular Cyst 1369 Valvular Pulmonic Stenosis 1470 Varix (Orbit) 1099 Vascular Anomalies (Pediatric Cardiac Imaging) 1453 Vascular Malformations (Intracranial) 1220

I9

Vascular Mediated Disorders (Bone Marrow) 1433 Edema 1433 Ischemia 1433 Ischemia & Edema: Causes (Bone Marrow) 1433 Vascular Rings and Slings 1374 Vascular White Matter Disease 1039 Vasculitis 1039 Vein Of Galen Malformation 1181 Venous Anomaly (Intracranial - Developmental) 1226 Venous Collagenosis 1039 Ventral Induction (Disorders of) 1310 Ventricular Septal Defect 1474 Ventricular Septal Defects 1465 Vesicoureteric Reflux 1332 Vestibular Schwannoma 1079, 1108 Vestibule 1068 Viral and Postviral Demyelination 1040 Viral Croup 1363 Virchow-Robin spaces 1037 Visceral injury 1498 Vitreous body 1088 Volvulus (Midgut) 1343 von Hippel-Linmdau Syndrome: NIH Classification 1202 von Recklinghausen Disease 1185 Warburg’s 1091 Warthin’s Tumor 1280 Wernicke encephalopathy 1042 White Matter Changes (Senescent) 1039 WHO 2000 Brain Tumor Classification 1137 Whole-body MRI 1426 Wilms Tumor 1390 Wishart Disease 1191 Wolman Disease 1411 Xanthoastrocytoma 1052 Xanthoastrocytoma (Pleomorphic) 1141 Yellow Marrow Signal 1427 Zellweger Syndrome 1388

I 10

Radiologic Pathology: 2006-2007

Essentials of Radiologic Science

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Lung Pathology (Current Clinical Pathology)

Essentials of Radiologic Science Workbook

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Forensic Pathology,

Bone Pathology

Head and Neck Pathology (Consultant Pathology)

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Knight's Forensic Pathology (Saukko, Knight's Forensic Pathology)

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Molecular Pathology Protocols

Pathology of Hepatocellular Carcinoma

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Ocular Pathology, 6Th Edition

Essentials of Rubin's Pathology

forensic pathology reviews

Fetal and Neonatal Pathology

Fundamentals of Toxicologic Pathology,

Color Atlas of Pathology

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Paul and Juhl's Essentials of Radiologic Imaging

Advances in Plant Pathology, Volume 11 (Advances in Plant Pathology)

Molecular Pathology of Hematolymphoid Diseases (Molecular Pathology Library)

Bone Marrow Pathology

Radiologic Pathology: 2006-2007

Essentials of Radiologic Science

Pathology

Lung Pathology (Current Clinical Pathology)

Essentials of Radiologic Science Workbook

Concise Pathology

Forensic Pathology,

Bone Pathology

Head and Neck Pathology (Consultant Pathology)

Liver Pathology

Oral Pathology

Ocular pathology

Knight's Forensic Pathology (Saukko, Knight's Forensic Pathology)

Pathology Illustrated

Molecular Pathology Protocols

Pathology of Hepatocellular Carcinoma

Brain Tumors (Consultant Pathology)

Ocular Pathology, 6Th Edition

Essentials of Rubin's Pathology

forensic pathology reviews

Fetal and Neonatal Pathology

Fundamentals of Toxicologic Pathology,

Color Atlas of Pathology

Case Files Pathology

Fundamentals of renal pathology

Atlas of Genitourinary Pathology

Pathology Of Lying

Paul and Juhl's Essentials of Radiologic Imaging

Advances in Plant Pathology, Volume 11 (Advances in Plant Pathology)

Molecular Pathology of Hematolymphoid Diseases (Molecular Pathology Library)

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