Radiology Review Manual

Help Introduction Breast DDx, Anatomy, Disorders Musculoskeletal System DDx, Anatomy, Disorders Heart and Great Vesse...

Author: Wolfgang F. Dähnert

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Help Introduction

Breast DDx, Anatomy, Disorders

Musculoskeletal System DDx, Anatomy, Disorders

Heart and Great Vessels DDx, Anatomy, Disorders

Central Nervous System - Skull and Spine DDx, Anatomy, Disorders

Liver, Bile Ducts, Pancreas, and Spleen DDx, Anatomy, Disorders

Central Nervous System - Brain DDx, Anatomy, Disorders

Gastrointestinal Tract DDx, Anatomy, Disorders

Orbit DDx, Anatomy, Disorders

Urogenital Tract DDx, Anatomy, Disorders

Ear, Nose, And Throat DDx, Anatomy, Disorders

Obstetrics and Gynecology DDx, Anatomy, Disorders

Chest DDx, Anatomy, Disorders

Nuclear Medicine, Statistics, Contrast

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INTRODUCTION About the Author Preface Abbreviations

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MUSCULOSKELETAL SYSTEM Differential diagnosis of musculoskeletal system Anatomy and metabolism of bone Bone and soft-tissue disorders

Home : MUSCULOSKELETAL SYSTEM

Differential diagnosis of musculoskeletal system DIFFERENTIAL-DIAGNOSTIC GAMUT OF BONE DISORDERS DELAYED BONE AGE BONE SCLEROSIS Diffuse Osteosclerosis Constitutional Sclerosing Bone Disease Solitary Osteosclerotic Lesion Multiple Osteosclerotic Lesions Dense Metaphyseal Bands Bone-within-bone Appearance OSTEOPENIA Osteoporosis Osteomalacia Localized Osteopenia Bone Marrow Edema Transverse Lucent Metaphyseal Lines Frayed Metaphyses PERIOSTEAL REACTION Solid Periosteal Reaction Interrupted Periosteal Reaction Symmetric Periosteal Reaction In Adulthood Periosteal Reaction In Childhood BONE TUMOR Age Incidence of Malignant Bone Tumors Tumor Matrix of Bone Tumors Pattern of Bone Destruction Tumor Position in Transverse Plane Tumor Position in Longitudinal Plane Tumorlike Conditions INTRAOSSEOUS LESION Bubbly Bone Lesion Nonexpansile Unilocular Well-demarcated Bone Defect Nonexpansile Multilocular Well-demarcated Bone Defect Expansile Unilocular Well-demarcated Osteolysis Poorly Demarcated Osteolytic Lesion Without Periosteal Reaction Poorly Demarcated Osteolytic Lesion With Periosteal Reaction Mixed Sclerotic And Lytic Lesion Trabeculated Bone Lesion Lytic Bone Lesion Surrounded By Marked Sclerosis mnemonic:"BOOST" Multiple Lytic Lesions Lytic Bone Lesion In Patient granulocytic3.Metastasis (bronchus, lung, upper GI, prostate, kidney)4.Angioimmunoblastic lymphadenopathy(b)Inflammatory adenopathy1.Tuberculosis / histoplasmosis (may lead to fibrosing mediastinitis)2.Blastomycosis (rare) / coccidioidomycosis3.Sarcoidosis (predominant involvement of paratracheal nodes)4.Viral pneumonia (particularly measles + cat-scratch fever)5.Infectious mononucleosis / pertussis pneumonia6.Amyloidosis7.Plague / tularemia8.Drug reaction9.Giant lymph node hyperplasia= Castleman disease10.Connective tissue disease (rheumatoid, SLE)11.Bacterial lung abscess(c)Inhalational disease adenopathy1.Silicosis (eggshell calcification also in sarcoidosis + tuberculosis)2.Coal workers pneumoconiosis3.BerylliosisB.FOREGUT CYST1.Bronchogenic / respiratory cyst: cartilage, respiratory epithelium2.Enteric cyst = esophageal duplication cyst3.Extralobar sequestration (anomalous feeding vessel)4.Hiatal hernia5.Esophageal diverticula: Zenker, traction, epiphrenicC.PRIMARY TUMORS (infrequent)1.Carcinoma of trachea2.Bronchogenic carcinoma3.Esophageal tumor:leiomyoma, carcinoma, leiomyosarcoma 4.Mesothelioma5.Granular cell myoblastoma of trachea (rare)D.VASCULAR LESIONS1.Aneurysm of transverse aorta2.Distended veins (SVC, azygos vein)3.Hematoma Posterior Mediastinal Mass A.NEOPLASMNeurogenic tumor (largest group): 30% malignant (a)Tumor of peripheral nerve origin more common in adulthood 80% appear as round masses with sulcus lower attenuation than muscle (in 73%)1.Schwannoma = neurilemoma (32%): derived from sheath of Schwann without nerve cells2.Neurofibroma (10%): contains Schwann cells + nerve cells, 3rd + 4th decade3.Malignant schwannoma(b)Tumor of sympathetic ganglia origin more common in childhood 80% are elongated with tapered borders1.Ganglioneuroma (23-38%): second most common tumor of posterior mediastinum after neurofibroma2.Neuroblastoma (15%): highly malignant undifferentiated small round cell tumor originating in sympathetic ganglia, > L(g)Marfan syndrome, Ehlers-Danlos syndrome(h)Pulmonary infarction(i)Lymphangiomyomatosis + tuberous sclerosismnemonic:"THE CHEST SET"Trauma Honeycomb lung, Hamman-Rich syndrome Emphysema, Esophageal rupture Chronic obstructive pulmonary disease Hyaline membrane disease Endometriosis Spontaneous, Scleroderma Tuberous sclerosis Sarcoma (osteo-), Sarcoidosis Eosinophilic granuloma Tuberculosis + fungus Types: 1.Closed pneumothorax = intact thoracic cage2.Open pneumothorax = "sucking" chest wound3.Tension pneumothorax = accumulation of air within pleural space due to free ingress + limited egress of air Pathophysiology: intrapleural pressure exceeds atmospheric pressure in lung during expiration (check-valve mechanism) Frequency:in 3-5% of patients with spontaneous pneumothorax, higher in barotrauma displacement of mediastinum / anterior junction line deep sulcus sign = on frontal view larger lateral costodiaphragmatic recess than on opposite side diaphragmatic inversion total / subtotal lung collapse collapse of SVC / IVC / right heart border (decreased systemic venous return + decreased cardiac output)N.B.:Medical emergency!4.Tension hydropneumothorax sharp delineation of visceral pleural by dense pleural space mediastinal shift to opposite side air-fluid level in pleural space on erect CXR PNEUMOTHORAX SIZE Average Interpleural Distance (AID) = (A + B + C) ÷ 3 [in cm] converts to percentage of pneumothorax

Radiographic signs in upright position: white margin of visceral pleura separated from parietal pleuraDDx: skin fold, air trapped between chest wall soft tissues, hair braid) absence of vascular markings beyond visceral pleural margin Radiographic signs in supine position: 1.Anteromedial pneumothorax (earliest location) outline of medial diaphragm under cardiac silhouette sharp delineation of mediastinal contours (SVC, azygos vein, left subclavian artery, anterior junction line, superior pulmonary vein, heart border, IVC, deep anterior cardiophrenic sulcus, pericardial fat-pad)2.Subpulmonic pneumothorax (second most common location) hyperlucent upper abdominal quadrant deep lateral costophrenic sulcus sharply outlined diaphragm in spite of parenchymal disease visualization of anterior costophrenic sulcus visualization of inferior surface of lung3.Apicolateral pneumothorax (least common location) visualization of visceral pleural line4.Posteromedial pneumothorax (in presence of lower lobe collapse) lucent triangle with vertex at hilum V-shaped base delineating costovertebral sulcus5.Pneumothorax outlines pulmonary ligament Prognosis:resorption of pneumothorax occurs at a rate of 1.25% per day (accelerated by increasing inspired oxygen concentrations) Notes:

Home : CHEST : Differential diagnosis of chest disorders : PLEURA

Pleural Effusion A.TRANSUDATE (protein level of 1.5-2.5 g/dL)Pathophysiology:result of systemic abnormalities causing an outpouring of low-protein fluid(a)Increased hydrostatic pressure1.Congestive heart failure (in 65%)bilateral (88%); right-sided (8%); left-sided (4%); least amount on left side due to cardiac movement, which stimulates lymphatic resorption 2.Constrictive pericarditis (in 60%)(b)Decreased colloid-oncotic pressure-decreased protein production1.Cirrhosis with ascites (in 6%):right-sided (67%)-protein loss / hypervolemia1.Nephrotic syndrome (21%), overhydration, glomerulonephritis (55%), peritoneal dialysis2.Hypothyroidism(c)Chylous effusion Most frequent cause of isolated pleural effusion in newborn with 15-25% mortality! chylomicrons + lymphocytes in fluid B.EXUDATEPathophysiology:increased permeability of abnormal pleural capillaries with release of high-protein fluid into pleural spaceCriteria: pleural fluid total protein / serum total protein ratio of >0.5 pleural fluid LDH / serum LDH ratio of >0.6 pleural fluid LDH >2/3 of upper limit of normal for serum LDH (upper limit for LDH ~200 IU) pleural fluid specific gravity >1.016 protein level >3 g/dL effusion with septation / low-level echoes "split pleura" sign on CECT = thickened enhancing visceral + parietal pleura separated by fluid extrapleural fat thickening of >2 mm + increased attenuation (edema / inflammation) (a)Infection1.Empyema =parapneumonic effusion characterized by presence of pus ± positive culture-exudative phase = inflammation of visceral pleura results in increased capillary permeability with weeping of high-protein fluid into pleural space-fibrinopurulent phase = inflammatory cells + neutrophils pour into pleural space + fibrin deposition on pleural surfaces-organizing phase = recruitment of fibroblasts + capillaries results in deposition of collagen + granulation tissue on pleural surfaces = pleural fibrosisRx:decortication if active infection persists Organism:S. aureus, gram-negative + anaerobic bacteria positive Gram stain positive culture (anaerobic bacteria most frequent) gross pus (WBC >15, 000/cm3 ) pH 1000 IU/L glucose 70%, positive culture (only in 20-25%) 4.Fungi: Actinomyces, Nocardia5.Parasites: amebiasis (secondary to liver abscess in 15-20%), Echinococcus6.Mycoplasma, rickettsia (in 20%) empyema necessitatis = chronic empyema attempting to decompress through chest wall (in TB, actinomycosis, aspergillosis, blastomycosis, nocardiosis) (b)Malignant disease (in 60%) positive cytologic resultsCause:lung cancer (26-49%), breast cancer (8-24%), lymphoma (10-28%, in 2/3 chylothorax), ovarian cancer (10%), malignant mesothelioma containing hyaluronic acid (5%)Pathogenesis: -pleural metastases (increase pleural permeability)-lymphatic obstruction (pleural vessels, mediastinal nodes, thoracic duct disruption)-bronchial obstruction (loss of volume + resorptive surface)-hypoproteinemia (secondary to tumor cachexia)Rx:sclerosing agents: doxycycline, bleomycin, talc (c)VascularPulmonary emboli (in 15-30% of all embolic events): often serosanguinous (d)Abdominal disease1.Pancreatitis / pancreatic pseudocyst / pancreaticopleural fistula (in 2/3): usually left-sided pleural effusion high amylase levels2.Boerhaave syndrome:left-sided esophageal perforation 3.Subphrenic abscess pleural effusion (79%) elevation + restriction of diaphragmatic motion (95%) basilar platelike atelectasis / pneumonitis (79%)4.Abdominal tumor with ascites5.Meigs-Salmon syndrome =primary pelvic neoplasms (ovarian fibroma, thecoma, granulosa cell tumor, Brenner tumor, cystadenoma, adenocarcinoma, fibromyoma of uterus) cause pleural effusion in 2-3%; ascites + hydrothorax resolve with tumor removal6.Endometriosis7.Bile fistula (e)Collagen-vascular disease1.Rheumatoid arthritis (in 3%):unilateral; R > L (in 75%), recurrent alternating sides; pleural effusion relatively unchanged in size for months; predominantly in men; LOW GLUCOSE content of 20-50 mg/dL (in 70-80%) without increase following IV infusion of glucose (DDx: TB, metastatic disease, parapneumonic effusion) 2.SLE (in 15-74%)most common collagenosis to give pleural effusion, bilateral in 50%; L > R enlargement of cardiovascular silhouette (in 35-50%)3.Wegener granulomatosis (in 50%)4.Sjögren syndrome5.Mixed connective tissue disease6.Periarteritis nodosa7.Postmyocardial infarct syndrome (f)Traumatichemorrhagic, chylous, esophageal rupture, thoracic / abdominal surgery, intrapleural infusion = "infusothorax" (0.5%), radiation pneumonitis (g)Miscellaneous1.Sarcoidosis2.Uremic pleuritis (in 20% of uremic patients)3.Drug-induced effusion CXR: first 300 ml not visualized on PA view (collect in subpulmonic region first, then spill into posterior costophrenic sinus) lateral decubitus views may detect as little as 25 ml hemidiaphragm + costophrenic sinuses obscured extension upward around posterior > lateral > anterior thoracic wall (mediastinal portion fixed by pulmonary ligament + hilum) meniscus-shaped semicircular upper surface with lowest point in midaxillary line associated collapse of ipsilateral lung Massive pleural effusion: enlargement of ipsilateral hemithorax displacement of mediastinum to contralateral side severe depression / flattening / inversion of ipsilateral hemidiaphragm visible air bronchogramSubpulmonic effusionSubpulmonic / subdiaphragmatic / infrapulmonary pleural effusion: peak of dome of pseudodiaphragm laterally positioned acutely angulated costophrenic angle increased distance between stomach bubble and lung blunted posterior costophrenic sulcus thin triangular paramediastinal opacity (mediastinal extension of pleural effusion) flattened pseudodiaphragmatic contour anterior to major fissure (on lateral CXR)CT: fluid outside diaphragm fluid elevating crus of diaphragm indistinct fluid-liver interface fluid posteromedial to liver (= bare area of liver)CAVE:"central oval" sign of ascites may be seen in subpulmonic effusion with inverted diaphragm Unilateral Pleural Effusion The majority of massive unilateral pleural effusions are malignant (lymphoma, metastatic disease, primary lung cancer)!1.Neoplasm2.Infection: TB3.Collagen vascular disease4.Subdiaphragmatic disease5.Pulmonary emboli6.Trauma: fractured rib7.Chylothorax Left-sided Pleural Effusion 1.Spontaneous rupture of the esophagus2.Dissecting aneurysm of the aorta3.Traumatic rupture of aorta distal to left subclavian artery4.Transection of distal thoracic duct5.Pancreatitis: left-sided (68%), right-sided (10%), bilateral (22%)6.Pancreatic + gastric neoplasmRight-sided Pleural Effusion 1.Congestive heart failure2.Transection of proximal thoracic duct3.PancreatitisPleural Effusion & Large Cardiac Silhouette 1.Congestive heart failure (most common) cardiomegaly prominence of upper lobe vessels + constriction of lower lobe vessels prominent hilar vessels interstitial edema (fine reticular pattern, Kerley lines, perihilar haze, peribronchial thickening) alveolar edema (perihilar confluent ill-defined densities, air bronchogram) "phantom tumor" = fluid localized to interlobar pleural fissure (in 78% in right horizontal fissure)2.Pulmonary embolus with right-sided heart enlargement3.Myocarditis / pericarditis with pleuritis(a)viral infection(b)tuberculosis(c)rheumatic fever (poststreptococcal infection)4.Tumor: metastatic, mesothelioma5.Collagen-vascular disease(a)SLE (pleural + pericardial effusion)(b)rheumatoid arthritis Pleural Effusion & Subsegmental Atelectasis 1.Postoperative (thoracotomy, splenectomy, renal surgery) secondary to thoracic splinting + small airway mucous plugging2.Pulmonary embolus3.Abdominal mass4.Ascites5.Rib fractures Pleural Effusion & Lobar Densities 1.Pneumonia with empyema2.Pulmonary embolism3.Neoplasm(a)bronchogenic carcinoma (common)(b)lymphoma4.Tuberculosis Pleural Effusion & Hilar Enlargement 1.Pulmonary embolus2.Tumor(a)bronchogenic carcinoma(b)lymphoma(c)metastasis3.Tuberculosis4.Fungal infection (rare)5.Sarcoidosis (very rare)

Notes:


Hemothorax A.TRAUMA1.Closed / penetrating injury2.Surgery3.Interventional procedures: thoracentesis, pleural biopsy, catheter placementB.BLEEDING DIATHESIS1.Anticoagulant therapy2.Thrombocytopenia3.Factor deficiencyC.VASCULAR1.Pulmonary infarct2.Arteriovenous malformation3.Aortic dissection4.Leaking atherosclerotic aneurysmD.MALIGNANCY1.Mesothelioma2.Lung cancer3.Metastasis4.LeukemiaE.OTHER1.Catamenial hemorrhage2.Extramedullary hematopoiesis rapidly enlarging high-attenuation pleural effusion on CT heterogeneous attenuation hyperattenuating areas of debris fluid-hematocrit level

Notes:


Solitary Pleural Mass =density with incomplete border and tapered superior + inferior borders, difficult to distinguish from chest wall mass (rib destruction reliable sign of chest wall mass) 1.Loculated pleural effusion ("vanishing tumor")2.Organized empyema3.Metastasis4.Local benign mesothelioma5.Subpleural lipoma: may erode adjacent rib6.Hematoma7.Mesothelial cyst8.Neural tumor: schwannoma, neurofibroma9.Localized fibrous tumor of pleura 10. Fibrin bodies = 3-4 cm large tumorlike concentrations of fibrin forming in serofibrinous pleural effusions; usually near lung base

Notes:


Multiple Pleural Densities diffuse pleural thickening with lobulated borders 1.Loculated pleural effusion:infectious, hemorrhagic, neoplastic 2.Pleural plaques3.Metastasis (most common cause)Origin:lung (40%), breast (20%), lymphoma (10%), melanoma, ovary, uterus, GI tract, pancreas, sarcoma Metastatic adenocarcinoma histologically similar to malignant mesothelioma!4.Diffuse malignant mesotheliomaalmost always unilateral, associated with asbestos exposure 5.Invasive thymoma (rare) contiguous spread, invasion of pleura, spreads around lung NO pleural effusion6.Thoracic splenosis =autotransplantation of splenic tissue to pleural space following thoracoabdominal trauma; discovered 10-30 years later asymptomatic / recurrent hemoptysis one or several nodules in left pleura / fissures measuring several mm to 6 cm positive Tc-99m-sulfur colloid scan, indium-111-labeled platelets, Tc-99m-labeled heat-damaged RBCs mnemonic:"Mary Tyler Moore Likes Lemon"Metastases (especially adenocarcinoma) Thymoma (malignant) Malignant mesothelioma Loculated pleural effusion Lymphoma

Notes:


Pleural Thickening A.TRAUMA1.Fibrothorax (most common cause)=organizing effusion / hemothorax / pyothorax dense fibrous layer of approx. 2 cm thickness; almost always on visceral pleura frequent calcification on inner aspect of pleural peelB.INFECTION1.Chronic empyema: over bases; history of pneumonia; parenchymal scars2.Tuberculosis / histoplasmosis: lung apex; associated with apical cavity3.Aspergilloma: in preexisting cavity concomitant with pleural thickeningC.COLLAGEN-VASCULAR DISEASE1.Rheumatoid arthritis: pleural effusion fails to resolveD.INHALATIONAL DISORDER1.Asbestos exposure: lower lateral chest wall; basilar interstitial disease (14 days)(4)Pleuroperitoneal shunt; tetracycline pleurodesis; mediastinal radiation; intrapleural fibrin glue; pleurectomy Notes:

Home : CHEST : Chest disorders

COAL WORKERS PNEUMOCONIOSIS =CWP = ANTHRACOSIS = ANTHRACOSILICOSIS=coal dust inhalation taken up by alveolar macrophages, in part cleared by mucociliary action (particle size >5 µ), in part deposited around bronchioles + alveoli, coal dust in itself is inert, but admixed silica is fibrogenic Simple CWP =aggregates of coal dust = coal macules(usually 10 years) nodularity correlates with amount of collagen (NOT amount of coal dust)Cx :(1)Chronic obstructive bronchitis(2)Focal emphysema(3)Cor pulmonale

Notes:


COCCIDIOIDOMYCOSIS Organism:dimorphic soil fungus Coccidioides immitis; arthrospores in desert soil spread by wind aerosolized in dry dust; highly infectiousGeographic distribution: endemic in southwest desert of USA (San Joaquin Valley, central southern Arizona, western Texas, southern New Mexico) + northern Mexico + in parts of Central + South America; similar to histoplasmosis Mode of infection:deposited in alveoli after inhalation + maturation into large thick-walled spherules with release of hundreds of endosporesDx:(1)culture of organism(2)spherules in pathologic material (demonstrated with Gomori-methenamine silver stain)(3)positive skin test(4)complement fixation titer Primary Coccidioidomycosis Chronic Respiratory Coccidioidomycosis Disseminated Coccidioidomycosis (in 1%) Notes:

Home : CHEST : Chest disorders : COCCIDIOIDOMYCOSIS

Primary Coccidioidomycosis =ACUTE RESPIRATORY COCCIDIOIDOMYCOSIS

60-80% asymptomatic

"valley fever" = influenza-like symptoms

desert

rheumatism (33%) = immune-complex-mediated arthritis (most commonly in ankle) rash, erythema nodosum / multiforme (5-20%) segmental / lobar consolidation patchy infiltrates mainly in lower lobes (46-80%) frequently subpleural + abutting fissures peribronchial thickening hilar adenopathy (20%) pleural effusion (10%) Notes:


Chronic Respiratory Coccidioidomycosis Prevalence:5% of infected patients

symptoms of postprimary tuberculosis

hemoptysis in 50% one / several

well-defined nodules (= coccidioidomycoma) of 5-30 mm in size (in 5%) persistent / progressive consolidation "grape skin" thin-walled cavities (in 10-15%), in 90% solitary, 70% in anterior segment of upper lobes (DDx: TB), 3% rupture into pleural space due to subpleural location (pneumothorax / empyema / persistent bronchopleural fistula) bronchiectasis mediastinal adenopathy (10-20%) Notes:


Disseminated Coccidioidomycosis (in 1%) =secondary phase of hematogenous spread to meninges, bones, skin, lymph nodes, subcutaneous tissue, joints (except GI tract) skin granulomas / abscesses micronodular "miliary" lung pattern pericardial effusion Notes:


CONGENITAL LOBAR EMPHYSEMA =progressive overdistension of one / multiple lobesM:F = 3 :1 Etiology: (a)deficiency / dysplasia / immaturity of bronchial cartilage(b)endobronchial obstruction (mucosal fold / web, prolonged endotracheal intubation, inflammatory exudate, inspissated mucus)(c)bronchial compression (PDA, aberrant left pulmonary artery, pulmonary artery dilatation)(d)polyalveolar / macroalveolar hyperplasiaAssociated with:CHD in 15% (PDA, VSD) respiratory distress (90%) + progressive cyanosis within first 6 months of lifeLocation:LUL (42-43%), RML (32-35%), RUL (20%), two lobes (5%) hazy masslike opacity immediately following birth (delayed clearance of lung fluid in emphysematous lobe over 1-14 days) air trapping hyperlucent expanded lobe (after clearing of fluid) compression collapse of adjacent lobes contralateral mediastinal shift widely separated vascular markingsMortality:10%Rx:surgical resection Notes:


CONGENITAL LYMPHANGIECTASIA 1.PRIMARY PULMONARY LYMPHANGIECTASIA (2/3)=abnormal development of lungs between 14-20th week of GA characterized by anomalous dilatation of pulmonary lymph vesselsPath:subpleural cysts, ectatic tortuous lymph channels in pleura, interlobular septa + along bronchoarterial bundles; NO obstructionAge:usually manifest at birth; 50% stillborn; M = FMay be associated with:total anomalous pulmonary venous return, hypoplastic left heart, Noonan syndrome respiratory distress within few hours of birthSite:diffuse involvement of both lungs, occasionally only in one / two lobes (with good prognosis) marked prominence of coarse interstitial markings (simulating interstitial edema) hyperinflation scattered radiolucent areas (dilated airways) patchy areas of pneumonia + atelectasis pneumothoraxPrognosis:in diffuse form invariably fatal at 20 mm lined by ciliated pseudostratified columnar epithelium, mucus-producing cells in 1/3Prognosis:excellent following resectionTYPE II(40%):Histo:multiple cysts 5-12 mm lined by ciliated cuboidal / columnar epitheliumPrognosis:poor secondary to associated abnormalitiesTYPE III(10%):Histo:solitary large bulky firm mass of bronchuslike structures lined by ciliated cuboidal epithelium with 3-5 mm small microcystsPrognosis:poor secondary to pulmonary hypoplasia / hydrops In 25% associated with: cardiac malformation, pectus excavatum, renal agenesis, prune-belly syndrome, jejunal atresia, chromosomal anomaly, bronchopulmonary sequestration Age of detection:children, neonates, fetus; M:F = 1:1 respiratory distress + severe cyanosis in first week of life (66%) / within first year of life (90%) due to compression of normal lung + airways superimposed chronic recurrent infection (10%) after first year of lifeLocation:equal frequency in all lobes (middle lobe rarely affected); more than one lobe involved in 20%; mostly unilateral without side preferenceCXR: almost always unilateral expansile mass with well-defined margins (80%) multiple air- / occasionally fluid-filled cysts sometimes solid appearance (retained fetal lung fluid / type III lesion) compression of adjacent lung contralateral shift of mediastinum (87%) hypoplastic ipsilateral lung proper position of abdominal viscera spontaneous pneumothorax (late sign)CT: Postnatally becoming obstructed and filled with air solitary / multiple fluid or air-fluid filled cysts with thin walls surrounding focal emphysematous changesOB-US: single large cyst / multiple large cysts of 2-10 cm in diameter (Type I) multiple small cysts of 5-12 mm in diameter (Type II) large homogeneously hyperechoic mass compared to liver (Type III) contralateral mediastinal shift (89%) polyhydramnios (25-75%, ? from compression of esophagus or increased fluid production by abnormal lung) / normal fluid (28%) / oligohydramnios (6%) fetal ascites (62-71%) fetal hydrops in 33-81% (decreased venous return from compression of heart / vena cava)Risk of recurrence:noneCx:ipsi- / bilateral pulmonary hypoplasiaPrognosis:50% premature, 25% stillborn Polyhydramnios, ascites, hydrops indicate a poor outcome! CAM becomes smaller in fetuses in many cases + occasionally almost disappears by birth!DDx:(1)Congenital lobar emphysema(2)Diaphragmatic hernia(3)Bronchogenic cyst (small solitary cyst near midline)(4)Neurenteric cyst(5)Bronchial atresia(6)Bronchopulmonary sequestration (less frequently associated with polyhydramnios / hydrops)(7)Mediastinal / pericardial teratoma Notes:


CYSTIC FIBROSIS =MUCOVISCIDOSIS = FIBROCYSTIC DISEASE=autosomal recessive multisystem disease characterized by mucous plugging of exocrine glands secondary to(a)dysfunction of exocrine glands forming a thick tenacious material obstructing conducting system(b)reduced mucociliary transportIncidence:1:2,000-1:2,500 livebirths; almost exclusively in Caucasians (5% carry a CF mutant gene allele); unusual in Blacks (1:17,000), Orientals, Polynesians The most common inherited disease among Caucasian Americans!Cause:cystic fibrosis gene (= transmembrane conductance regulator gene) on long arm of chromosome 7 creates a defective transmembrane ion transport protein through deletion of an amino-acid; the normal product represents an epithelial chloride channel that supplies luminal water by osmosis; >230 different gene mutations (in 70% F508)Screening (for 6 most common mutations of CF gene): carrier detection rate of 85% of Northern Europeans, 90% of Ashkenazi Jews, 50% of American Blacks Age at diagnosis:1st year of life (70%), by age 4 years (80%), by age 12 years (90%); mean age of 2.9 years; M:F = 1:1 elevated concentrations of sodium + chloride (>40 mmol/L for infants) in sweat decreased urinary PABA excretion infertility in males increased susceptibility to infection by Staphylococcus aureus + Pseudomonas aeruginosaPrognosis:median survival of 28 years; pulmonary complications are the most predominant cause of morbidity and death (90%) @Lung chronic cough recurrent pulmonary infections (reduced mucociliary clearance encourages Pseudomonas colonization) progressive respiratory insufficiency due to obstructive lung disease Location:predilection for apical + posterior segments of upper lobes "fingerlike" mucus plugging (mucoid impaction in dilated bronchi) within 1st month of life subsegmental / segmental / lobar atelectasis with right upper lobe predominance (10%) progressive cylindrical / cystic bronchiectasis (in 100% at >6 months of age) ± air-fluid levels due to prolonged mucus plugging preponderant in upper lobes parahilar linear densities + peribronchial cuffing focal peripheral / generalized hyperinflation secondary to collateral air drift into blocked airways) hilar adenopathy large pulmonary arteries (pulmonary arterial hypertension) recurrent local pneumonitis (initiated by staphylococcus / Haemophilus influenza, succeeded by Pseudomonas) allergic bronchopulmonary aspergillosis (with bronchial dilatation + mucoid impaction)CT: cylindrical (varicose / cystic) bronchiectasis peribrochial thickening bronchiectatic cyst (= bronchus directly leading into sacculation) in 56% interstitial cysts in 32% emphysematous bulla (= peripheral air space with long pleural attachment + without communication to bronchus) in 12% periseptal emphysema mucus plugs = tubular structures ± branching pattern subsegmental / segmental collapse / consolidationsNUC: matched patchy areas of decreased ventilation + perfusion Cx:(1)Pneumothorax (rupture of bulla / bleb), common + recurrent(2)Hemoptysis(3)Cor pulmonale(4)Hypertrophic pulmonary osteoarthropathy (rare) Cause of death:massive mucus plugging (95%) @GI tract (85-90%) chronic obstipation failure to thrive gastroesophageal reflux (21-27%) due to transient inappropriate lower esophageal sphincter relaxation meconium plug syndrome (25%, most common cause of colonic obstruction in the infant) distal intestinal obstruction syndrome (10-15-47%)= meconium ileus equivalent syndrome (in older child / young adult) meconium ileus (10-16% at birth) Earliest clinical manifestation of cystic fibrosis! fibrosing colonopathy = stricture of right colon with longitudinal shortening secondary to high-dose lipase supplementation thickened nodular duodenal mucosal folds (due to unbuffered gastric acid, production of abnormal mucus, Brunner gland hypertrophy) mild generalized small bowel dilatation with diffuse distortion + thickening of mucosal folds (at times involving colon + rectum) large distended colon with mottled appearance (retained bulky dry stool) pneumatosis intestinalis of colon (5%) from air block phenomena of obstructive pulmonary disease "microcolon" = colon of normal length but diminished caliber "jejunization of colon" = coarse redundant + hyperplastic colonic mucosa (distended crypt goblet cells) Crohn disease appendicitis rectal prolapse between 6 months and 3 years in untreated patients (18-23%)Cx:gastrointestinal perforation with meconium peritonitis (50%), volvulus of dilated segments, bowel atresia, intussusception at an average age of 10 years (1%) @Liver steatosis (30%) due to untreated malabsorption, dietary deficiencies, hepatic dysfunction, medications focal / multilobular biliary cirrhosis from inspissated bile signs of portal hypertension (clinically in 4-6%, autoptic in up to 50%) portal hypertension (in 1% of biliary cirrhosis) + hepatosplenomegaly + hypersplenism @Biliary treeHisto:mucus-containing cysts in gallbladder wall cholestasis (secondary to CBD obstruction) symptoms of gallbladder disease (3.6%) sludge (33%) cholelithiasis (12-24%): mostly cholesterol stones due to (1) interrupted enterohepatic circulation after ileal resection / (2) ileal dysfunction in distal intestinal obstruction syndrome gallbladder atony microgallbladder (25% at autopsy) thickened trabeculated gallbladder wall subepithelial cysts of gallbladder wall atresia / stenosis of cystic duct @PancreasHisto:dilatation of acini + cyst formation due to obstruction from protein plugs as a result of precipitation of relatively insoluble proteinsPath:progressive ductectasia, pancreatic atrophy, increased pancreatic lobulation, fibrosis due to recurrent acute pancreatitis, replacement by fat steatorrhea + malabsorption + fat intolerance due to exocrine pancreatic insufficiency in 80-90% without affecting endocrine function (once 98% of entire pancreas is damaged) Cystic fibrosis is the most common cause of exocrine pancreatic insufficiency in patients LIncidence:1:100,000Herniated organs:omental fat, transverse colon, liverOften associated with: chromosomal abnormality, mental retardation, heart defects, pericardial deficiency (a)abdominal viscera / fat may herniate into pericardial sac(b)heart may herniate into upper abdomenmnemonic:"4 Ms"Morgagni Middle (anterior + central location) Mature (present in older children) Minuscule (usually small) (3)Septum transversum defect = defect in central tendon(4)Hiatal hernia = congenitally large esophageal orifice(5)Eventration (5%) = upward displacement of abdominal contents secondary to a congenitally thin hypoplastic diaphragmUnilateral eventration may be associated with: Beckwith-Wiedemann syndrome, trisomy 13, trisomy 15, trisomy 18 Bilateral eventration may be associated with: toxoplasmosis, CMV, arthrogryposis Location:anteromedial on right, total involvement on left side; R:L = 5:1 small diaphragmatic excursions often lobulated diaphragmatic contour respiratory distress in neonatal period (life-threatening deficiency of small airways + alveoli) scaphoid abdomenHerniated organs: small bowel (90%), stomach (60%), large bowel (56%), spleen (54%), pancreas (24%), kidney (12%), adrenal gland, liver, gallbladder bowel loops in chest contralateral shift of mediastinum + heart complete (1-2%) / partial absence of diaphragm absence of stomach, small bowel in abdomen passage of nasogastric tube under fluoroscopic control entering intrathoracic stomach incomplete rotation + anomalous mesenteric attachment of bowel OB-US (diagnosis possible by 18 weeks GA): solid / multicystic / complex chest mass mediastinal shift nonvisualization of fetal stomach below diaphragm fetal stomach at level of fetal heart peristalsis of bowel within fetal chest (inconsistent) paradoxical motion of diaphragm with fetal breathing (defect in diaphragm sonographically not visible) scaphoid fetal abdomen with reduced abdominal circumference herniated liver frequently surrounded by ascites polyhydramnios (common, due to partial esophageal obstruction or heart failure) / normal fluid volume / oligohydramnios swallowed fetal intestinal contrast appears in chest (CT amniography confirms diagnosis)Cx:(1)Bilateral pulmonary hypoplasia(2)Persistent fetal circulation (postsurgical pulmonary hypertension)Prognosis:(1)Stillbirth (35-50%)(2)Neonatal death (35%) Survival is determined by size of defect + time of entry + associated anomalies (34% survival rate if isolated, 7% with associated anomalies)Indicators for poor prognosis: large intrathoracic mass with marked mediastinal shift, IUGR, polyhydramnios, hydrops fetalis, detection 40 mg/dL-late stage II empyema frank pus pH FPath:filling of alveolar spaces with foamy histiocytes + relative preservation of lung architecture + mild fibrosis (temporally homogeneous)Histo:alveoli lined by large cuboidal cells + filled with heavy accumulation of mononuclear cells (macrophages, NOT desquamated alveolar cells); relative preservation of alveolar anatomy; histologic uniformity from field to fieldPredisposed:smokers (history in up to 90%) asymptomatic weight loss dyspnea + nonproductive cough (for 6-12 months) clubbing of fingers mild pulmonary function abnormalities normal chest x-ray (3-22%) "ground-glass" alveolar pattern sparing costophrenic angles (25-33%), diffuse ground-glass opacities (15%) linear irregular opacities (60%), bilateral + basilar (46-73%) lung nodules (15%) honeycombing (13%) preserved lung volumeHRCT: Location:mainly middle + lower lung zones (73%); bilateral + symmetric (86%)Site:predominantly subpleural distribution (59%) patchy ground-glass attenuation irregular linear opacities (= fibrosis) + architectural distortion (50%) honeycombing + traction bronchiectasis (32%) fibrosis of lower lung zones in late stagePrognosis:better response to corticosteroid Rx than UIP (in 60-80%); median survival of 12 years; 5% 5-year mortality rate (overall 16-27%) Notes:


IDIOPATHIC PULMONARY HEMOSIDEROSIS =IPH = probable autoimmune process with clinical + radiologic remissions + exacerbations characterized by eosinophilia + mastocytosis, immunoallergic reaction, pulmonary hemorrhage, iron deficiency anemiaAge:(a)Chronic form: most commonly > Blacks @Pulmonary Langerhans cell histiocytosisPathogenesis: heavy cigarette smoking in young men with accumulation + activation of Langerhans cells (90% smokers) as a result of excess neuroendocrine cell hyperplasia + secretion of bombazine-like peptides Path: multifocal granulomatous infiltration centered on walls of bronchioles (= bronchiolitis) often extending into surrounding alveolar interstitium with subsequent bronchiolar destruction leading to thick-walled cysts presumably caused by check-valve bronchial obstruction + pneumothorax (no necrosis); in end-stage disease foci of LCG are replaced by fibroblasts forming CHARACTERISTIC stellate "starfish" scars with central remnants of persisting inflammatory cells CXR abnormalities more severe than clinical symptoms + pulmonary function tests! asymptomatic (up to 25%) nonproductive cough (75%) combination of obstructive + restrictive pulmonary function: presenting with pneumothorax in 15% fatigue, weight loss, fever (15-30%) dyspnea (40%) chest pain (25%) from pneumothorax / eosinophilic granuloma in rib diabetes insipidus (10-25%) lymphocytosis with predominance of T-suppressor cells on bronchoalveolar lavage (DDx: excess of T-helper cells in sarcoidosis)Location:usually bilaterally symmetric, upper lobe predominance, sparing of costophrenic anglesEvolutionary sequence: nodule - cavitated nodule thick-walled cyst - thin-walled cyst ill-defined / stellate nodules 3-10 mm (granuloma stage) diffuse fine reticular / reticulonodular pattern (cellular infiltrate) "honeycomb lung" = multiple 1-5 cm cysts + subpleural blebs (fibrotic stage) increased lung volumes in 1/3 (most other fibrotic lung diseases have decreased lung volumes!) pleural effusion (8%), hilar adenopathy (unusual) cavitation of large nodules (rare) thymic enlargementHRCT (combination virtually diagnostic): complex / branching thin-walled cysts CMV > Aspergillus > Pneumocystis(1)within 1st month:gram-negative bacteria, fungi (candidiasis, aspergillosis)(2)after 1st month:CMV, Pneumocystis carinii, bacteria, fungi fever, leukocytosis lobar / multilobar consolidation (due to bacterial > fungal pathogens) diffuse heterogeneous / ground-glass opacities (due to viral / disseminated fungal pathogens) nodular opacities (due to fungal / unusual bacterial pathogens / CMV / septic emboli)Cx:may progress rapidly to respiratory failure + deathDx:transbronchial / open biopsy (80% accurate)B.EXTRAPULMONARY INFECTIONthoracotomy wound infection, bacteremia, sepsis, empyema, central venous line infection

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Home : CHEST : Chest disorders : LUNG TRANSPLANT

Posttransplantation Lymphoproliferative Disease Incidence:4%Histo:spectrum from benign polyclonal proliferation of lymphoid tissue to non-Hodgkin lymphomaAssociated with:Epstein-Barr virusTime of onset:1 month to several years; related to immunosuppressive regimen solitary / multiple discrete nodules mediastinal / hilar lymphadenopathy

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Home : CHEST : Chest disorders : LUNG TRANSPLANT

Reperfusion Edema =REIMPLANTATION RESPONSE=infiltrate appearing within 48 hours after transplantation unrelated to fluid overload, LV failure, infection, atelectasis, or rejection; diagnosed by exclusionPathogenesis:permeability edema due to lymphatic disruption, pulmonary denervation, organ ischemia, traumaHisto:fluid accumulation in interstitium consistent with noncardiogenic pulmonary edemaTime course:manifests within 24 hours, peaks at 2nd-4th postoperative day, resolves at variable rate ranging from days to 1-2 weeks to months increasing hypoxia before extubation; poor correlation between radiographic severity + physiologic parametersLocation:perihilar areas + basal regions perihilar haze / rapid uni- or bilateral heterogeneously dense interstitial and/or air-space diseaseDx:per exclusion (radiographic changes not due to LV failure, hyperacute rejection, fluid overload, infection, atelectasis)

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LYMPHANGIOMYOMATOSIS =LYMPHANGIOLEIOMYOMATOSIS=? forme fruste of tuberous sclerosis=rare disorder characterized by (1) gradually progressive diffuse interstitial lung disease (2) recurrent chylous pleural effusions (3) recurrent pneumothoracesEtiology:unknownAge:17-50 years, exclusively in women of childbearing ageHisto:proliferation of atypical smooth muscle in pulmonary lymphatic vessels, blood vessels, and airwaysPathogenesis: proliferated smooth muscle obstructs (a) bronchioles (trapping of air, overinflation, formation of cysts, pneumothorax), (b) venules (pulmonary edema, hemorrhage, hemosiderosis), (c) lymphatics (thickening of lymphatics, chylothorax) May be associated with: Tuberous sclerosis (lung involvement in 1%) progressive exertional dyspnea + cough disease aggravated by pregnancy + oral contraceptives hemoptysis (30-40%), chyloptysis radiologic-physiologic discrepancy = severe airflow obstruction (reduced FEV1 , reduced ratio of FEV1 to forced vital capacity) despite relatively normal findings on CXR combination of restrictive + obstructive ventilatory defects: hypoxia, markedly impaired diffusing capacity positive immunohistochemical staining of LAM cells with HMB-45 (monoclonal antibody for melanocytic lesion) classic signs: coarse reticular interstitial pattern (caused by summation of multiple cyst walls) recurrent large chylothorax (20-50-75%) recurrent pneumothorax (40-50% at presentation; in 80% during course of disease) normal / increased lung volume The only interstitial lung disease to develop increase in lung volume! Kerley-B lines pulmonary cysts + honeycombing occasionally chylous ascites mediastinal + retroperitoneal adenopathy (from smooth muscle proliferation)HRCT: numerous randomly scattered thin-walled ( chrysotile > actinolite, anthophyllite, tremolite Occupational exposure of asbestos found in only 40-80% of all cases! 5-10% of asbestos workers will develop mesothelioma (risk factor of 30 compared with general population) No relation to duration / degree of exposure or smoking historyLatency period: 20-35-45 years (earlier than asbestosis; later than asbestos-related lung cancer)Peak age:50-70 years (66%); M:F = 2-4-6:1Path:multiple tumor masses involving predominantly the parietal pleura + to a lesser degree the visceral pleura; progression to thick sheetlike / confluent masses resulting in lung encasementHisto: (a) epithelioid (60%) (b) sarcomatoid (15%)(c) biphasic (25%); intracellular asbestos fibers in 25% Associated with:peritoneal mesothelioma; hypertrophic osteoarthropathy (10%)Staging (Boutin modification of Butchart staging) IAconfined to ipsilateral parietal / diaphragmatic pleuraIB+ visceral pleura, lung , pericardiumIIinvasion of chest wall / mediastinum (esophagus, heart, contralateral pleura) or metastases to thoracic lymph nodesIIIpenetration of diaphragm with peritoneal involvement or metastases to extrathoracic lymph nodesIVdistant hematogenous metastasesStage at presentation:II in 50%, III in 28%, I in 18%, IV in 4% nonpleuritic (56%) / pleuritic chest pain (6%) dyspnea (53%) fever + chills + sweats (30%) weakness, fatigue, malaise (30%) cough (24%), weight loss (22%), anorexia (10%) expectoration of asbestos bodies (= fusiform segmented rodlike structures = iron-protein deposition on asbestos fibers [a subset of ferruginous bodies])Spread: (a)contiguous: chest wall, mediastinum, contralateral chest, pericardium, diaphragm, peritoneal cavity; lymphatics, blood(b)lymphatic: hilar + mediastinal (40%), celiac (8%), axillary + supraclavicular (1%), cervical nodes(c)hematogenous: lung, liver, kidney, adrenal gland extensive irregular lobulated bulky pleural-based masses typically >5 cm / pleural thickening (60%) exudative / hemorrhagic unilateral pleural effusion (30-60-80%) without mediastinal shift ("frozen hemithorax" = fixation by pleural rind of neoplastic tissue); effusion contains hyaluronic acid in 80-100%; bilateral effusions (in 10%) distinct pleural mass without effusion (50 years (in 87%)FREQUENCY: Origin of pulmonary metsProbability of pulmonary mets 1.Breast22%Kidneyin 75%2.Kidney11%Osteosarcomain 75%3.Head and neck10%Choriocarcinomain 75%4.Colorectal 9%Thyroidin 65%5.Uterus 6%Melanomain 60%6.Pancreas 5%Breastin 55%7.Ovary 5%Prostatein 40%8.Prostate 4%Head and neckin 30%9.Stomach 4%Esophagusin 20% Incidence of pulmonary metastases: mnemonic: "CHEST" Choriocarcinoma60% Hypernephroma / Wilms tumor30 / 20%Ewing sarcoma18%Sarcoma (rhabdomyo- / osteosarcoma)21 / 15%Testicular tumor12% multiple nodules (in 75%) of varying sizes (most typical), 82% subpleural fine micronodular pattern: highly vascular tumor (renal cell, breast, thyroid, prostate carcinoma, bone sarcoma, choriocarcinoma) pneumothorax (2%): especially in children with bone tumorsCT: noncalcified multiple (>10) round lesions >2.5 cm likely to be metastatic connection to pulmonary arterial branches (75%) Solitary Metastatic Lung Nodule Calcifying Lung Metastases (FPredisposing factors: COPD (25-72%), previous TB (20-24%), interstitial lung disease (6%), smoking >30 pack-years (46%), alcohol abuse (40%), cardiovascular disease (36%), chronic liver disease (32%), previous gastrectomy (18%) Location:apical + anterior segments of upper lobes chronic fibronodular / fibroproductive apical opacities (indistinguishable from reactivation TB) cavitation in 80-95% apical pleural thickening in 37-56% additional patchy nodular alveolar opacities (due to bronchogenic spread) in ipsi- / contralateral lung in 40-70% adenopathy (0-4%) pleural effusion (5-20%) typically NO hilar elevationB.NONCLASSICAL FORM (20-30%)Age:7th-8th decade, 86% in Whites; M:F = 1:4Predisposing factors:NONELocation:predominantly in middle lobe + lingula multiple bilateral nodular opacities throughout both lungs in random distribution irregular curvilinear interstitial opacities (resembling bronchiectasis)C.ASYMPTOMATIC GRANULOMAS cluster of similar-sized nodulesD.ACHALASIA-RELATED INFECTIONwith M. fortuitum-chelonei E.DISSEMINATED DISEASEin immunocompromised patients: AIDS, transplant patients, lymphoproliferative disorders (esp., hairy cell leukemia), steroid + immunosuppressive therapy CT: multifocal bronchiectasis (79-94%), esp. middle lobe + lingula centrilobular nodules of varying sizes, usually 1 cm opacities initially in middle + upper lung zones at periphery of lung discoid contour (44%) = mass flat from front to back (thin opacity on lateral view, large opacity on PA view), medial border often ill-defined, lateral borders sharp + parallel to rib cage migration toward hila starting at lung periphery; bilateral symmetry apparent decrease in nodularity (incorporation of nodules from surroundings) cavitation (occasionally) due to ischemic necrosis / superimposed TB infection bullous scar emphysema pulmonary hypertension

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PSEUDOLYMPHOMA =reactive benign lesion = localized form of lymphocytic interstitial pneumonitis (LIP); no progression to lymphomaHisto:aggregates of plasma cells, reticulin cells, large + small lymphocytes with preserved lymphoid architecture resembling lymphoma histologically without lymph node involvementAssociated with:Sjögren syndrome mostly asymptomatic well-demarcated dense infiltrate infiltrate typically in central location extending to visceral pleura prominent air bronchogram NO lymphadenopathyPrognosis:occasionally progression to non-Hodgkin lymphomaRx:most patients respond well to steroids initially

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PSEUDOMONAS PNEUMONIA =most dreaded nosocomial infection because of resistance to antibiotics in patients with debilitating diseases on multiple antibiotics + corticosteroids; rare in communityOrganism:Pseudomonas aeruginosa, Gram-negative bradycardia temperature with morning peaks widespread patchy bronchopneumonia (secondary to bacteremia; unlike other Gram-negative pneumonias) predilection for lower lobes extensive bilateral consolidation "spongelike pattern" with multiple nodules >2 cm(= extensive necrosis with formation of multiple abscesses) small pleural effusions Notes:


PULMONARY ARTERIAL MALFORMATION =PAVM = PULMONARY ARTERIOVENOUS ANEURYSM = PULMONARY ARTERIOVENOUS FISTULA= PULMONARY ANGIOMA = PULMONARY TELANGIECTASIA=abnormal vascular communication between pulmonary artery and vein (95%) or systemic artery and pulmonary vein (5%)Etiology: (a)congenital defect of capillary structure (common)(b)acquired in cirrhosis (hepatogenic pulmonary angiodysplasia), cancer, trauma, surgery, actinomycosis, schistosomiasisPath:hemangioma of cavernous typePathophysiology: low-resistance extracardiac R-to-L shunt (which may result in paradoxical embolism); quantification with Tc-99m-labeled albumin microspheres by measuring fraction of dose reaching kidneys Age:3rd-4th decade; manifest in adult life, 10% in childhoodOccurrence: (a)isolated abnormality (40%)(b)multiple (in 1/3)associated withRendu-Osler-Weber syndrome (in 30-60-88%) = hereditary hemorrhagic telangiectasia Only 5-15% of patients with Rendu-Osler-Weber disease have pulmonary AVMs!Types: 1.Simple type (79%)=single feeding artery empties into a bulbous nonseptated aneurysmal segment with a single draining vein2.Complex type (21%)=more than one feeding artery empties into septated aneurysmal segment with more than one draining vein asymptomatic in 56% (until 3rd-4th decade) if AVM single and upper lobes; bilateral (8-20%); medial third of lung sharply defined, lobulated oval / round mass (90%) of 1 to several cm in size ("coin lesion") cordlike bands from mass to hilum (feeding artery + draining veins) in 2/3 single lesion, in 1/3 multiple lesions enlargement with advancing age change in size with Valsalva / Mueller maneuver / erect vs. recumbent position (decrease with Valsalva maneuver) phleboliths (occasionally) increased pulsations of hilar vesselsCT (98% detection rate): homogeneous circumscribed noncalcified nodule / serpiginous mass up to several cm in diameter vascular connection of mass with enlarged feeding artery + draining vein sequential enhancement of feeding artery + aneurysmal part + efferent vein on dynamic CTMR: (if contraindication to contrast / slow flow due to partial thrombosis / follow-up) signal void on standard spin echo / high signal intensity on GRASS imagesAngio (mostly obviated by MR / CT unless surgery or embolization contemplated) Cx:CNS symptoms are commonly the initial manifestation(1)Cerebrovascular accident: stroke (18%), transient ischemic attack (37%) secondary to paradoxical bland emboli(2)Brain abscess (5-9%) secondary to loss of pulmonary filter function for septic emboli(3)Hemoptysis (13%) secondary to rupture of PAVM into bronchus, most common presenting symptom(4)Hemothorax (9%) secondary to rupture of subpleural PAVM(5)PolycythemiaPrognosis:26% morbidity, 11% mortalityDDx: solitary / multiple pulmonary nodulesRx:embolization with coils / detachable balloons Notes:


PULMONARY CAPILLARY HEMANGIOMATOSIS =bilateral pulmonary disease behaving like a low-grade nonmetastatic vascular neoplasm with slowly progressive pulmonary hypertensionHisto:sheets of thin-walled capillary blood vessels infiltrating pulmonary interstitium + invading pulmonary vessels, bronchioles, and pleuraPathomechanism of pulmonary hypertension: (a)veno-occlusive phenomenon secondary to invasion of small pulmonary veins(b)progressive vascular obliteration secondary to in situ thrombosis + infarction(c)pulmonary scar formation secondary to recurrent pulmonary hemorrhageAge:20-40 years dyspnea on exertion cor pulmonale: jugular venous distension, pedal edema, ECG-signs of RV failure (DDx: pulmonary veno-occlusive disease) elevated PA pressures + normal pulmonary wedge pressure hemoptysis + pleuritic chest pain in 1/3(DDx: pulmonary thromboembolic disease)CXR: diffuse reticulonodular pattern focal areas of interstitial fibrosis (recurrent episodes of pulmonary hemorrhage + thrombotic infarction)CT: thickening + nodularity of inter- and intralobular septa + walls of pulmonary veins areas of ground-glass attenuation (= increased perfusion to extensive proliferating hemangiomatous tissue)Angio: combination of increased flow (to hemangiomatous areas) + decreased flow (to regions of thrombosis, infarction, and scarring)Prognosis:death after 2- to 12-year interval from onset of symptomsRx:bilateral lung transplantationDDx:(1)Pulmonary veno-occlusive disease(2)Idiopathic interstitial fibrosis(3)Primary pulmonary hypertension (no increase in lung markings)(4)Pulmonary hemangiomatosis (only in children, cavernous hemangiomas involving several organs) Notes:


PULMONARY CONTUSION =most common manifestation of blunt chest trauma, esp. deceleration traumaPath:exudation of edema + blood into air space + interstitiumTime of onset:apparent within 6 hours after trauma clinically inapparent hemoptysis (50%)Location:posterior (in 60%)Site:directly deep to site of impact / contrecoup irregular patchy / diffuse homogeneous extensive consolidation (CT is more sensitive) opacity may enlarge for 48-72 hours rapid resolution beginning 24-48 hours, complete within 2-10 days overlying rib fractures (frequent)CT: nonsegmental coarse ill-defined crescentic (50%) / amorphous (45%) opacification of lung parenchyma without cavitation "subpleural sparing" = 1-2 mm rim of uniformly nonopacified subpleural portion of lungCx:pneumothoraxDDx:fat embolism (1-2 days after injury) Notes:


PULMONARY INTERSTITIAL EMPHYSEMA =PIE = complication of respirator therapy with PEEPPathogenesis: gas escapes from overdistended alveolus, dissects into perivascular sheath surrounding arteries, veins, and lymphatics, tracks into mediastinum forming clusters of blebs; air-block = compression + obstruction of pulmonary veins + mediastinal structures by interstitial pulmonary emphysema / pneumomediastinum / pneumothorax (obstruction esp. during expiration) sudden deterioration in patients condition during respiratory therapy elongated lucencies following distribution of bronchovascular tree circular densities bilateral, symmetrical distribution lobar overdistension (occasionally)Cx:pneumomediastinum, pneumothorax, subcutaneous emphysema, pneumopericardium, intracardiac air, pneumoperitoneum, pneumatosis intestinalis Notes:


PULMONARY LYMPHANGIOMATOSIS =increased number of communicating lymphatic channels smooth thickening of bronchovascular bundles + interlobular septaCT: mediastinal fat mild perihilar infiltration pleural effusion pleural thickening Notes:

diffuse increased attenuation of


PULMONARY MAINLINE GRANULOMATOSIS =PULMONARY TALCOSIS=microscopic pulmonary embolism in drug addicts from IV injection of talc-containing drugs (ground tablets)Drugs:amphetamines, methylphenidate hydrochloride ("West coast"), tripelen amine ("blue velvet"), methadone hydrochloride, dilaudid, meperidine, pentazocine, propylhexedrine, hydromorphone hydrochloride added talc (= magnesium silicate) particles incite a granulomatous foreign-body reaction + subsequent fibrosis in perivascular distribution talc retinopathy (80%) = small glistening crystals angiothrombotic pulmonary hypertension + cor pulmonaleEarly changes: widespread micronodularity of "pinpoint" size (1-3 mm) with perihilar / basilar predominance well-defined nodules predominantly in middle zonesLate changes: loss of lung volume coalescent opacities similar to progressive massive fibrosis (DDx: in silicosis away from hila)DDx of late changes: (1)Progressive massive fibrosis of silicosis / coal workers pneumoconiosis(2)Chronic sarcoidosisDx:lung biopsy

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PULMONARY THROMBOEMBOLIC DISEASE =PULMONARY EMBOLISM (PE)Prevalence:630,000 Americans/year with missed / delayed diagnosis in 400,000 causing death in 120,000; diagnosed in 1% of all hospitalized patients; in 12-64% at autopsy; in 9-56% of patients with deep venous thrombosisAge:60% >60 years of ageCause:deep vein thrombosis (DVT) of LE in >90%; PE usually occurs within first 5-7 days of thrombus formationPredisposing factors:immobilization (56%), surgery (54%) Pathophysiology:A clot from the deep veins of the leg breaks off + fragments in right side of heart + showers lung with emboli varying in size On average >6-8 vessels are embolized! Class 1 = F; whiteHisto:uniform polyhedral / round cells arranged in sheets or forming small lobules separated by fibrous septa; varying amounts of mature lymphocytesPath:large unencapsulated well-circumscribed mass asymptomatic (20-30%) chest pain / pressure, shortness of breath, weight loss, hoarseness, dysphagia, fever SVC obstruction (10%) elevated serum levels of HCG (7-18%) elevated serum levels of LDH (80%) correlate with tumor burden + rate of tumor growthMetastases:to regional lymph nodes, lung, bone, liver large bulky well-marginated lobulated mass usually NO calcification homogeneous soft-tissue density with slight enhancementPrognosis:75-100% 5-year survival rate; death from distant metastasesRx:surgery + radiation therapy (very radiosensitive) ± cisplatin2.Nonseminomatous malignant germ cell tumor (a)embryonic tissue(1)Embryonal carcinoma(b)extraembryonic tissue(1)Yolk sac = endodermal sinus tumor(2)Choriocarcinoma (least frequent)(c)combination = mixed germ cell tumorPath:large unencapsulated heterogeneous soft-tissue mass with tendency for invasion of adjacent structuresAge:during 2nd to 4th decade M:F = 9:1; in children M = FAssociated with:Klinefelter syndrome (in 20%), hematologic malignancy chest pain, dyspnea, cough, weight loss, fever, SVC syndrome (90-100%) elevated serum level of a-fetoprotein (80%) with endodermal sinus tumor / embryonal carcinoma elevated serum level of LDH (60%) elevated serum level of HCG (30%) [DDx: lung cancer; hepatocellular carcinoma; adenocarcinoma of pancreas, colon, stomach]Metastases to:lung, liver large tumor of heterogeneous texture with central hemorrhage / necrosis well circumscribed / with irregular margins enhancement of tumor periphery lobulation suggests malignancy invasion of mediastinal structures (SVC obstruction is ominous) pleural / pericardial effusion (from local invasion) Absence of primary testicular tumor / retroperitoneal mass proves primary!Rx:cisplatin-based chemotherapy + tumor resectionPrognosis: 50% long-term survivors Cx: (1)Hemorrhage(2)Pneumothorax (from bronchial obstruction with air trapping + alveolar rupture)(3)Respiratory distress (rapid increase in size from fluid production) with compression of trachea / SVC (SVC syndrome)(4)Fistula formation to aorta, SVC, esophagus(5)Rupture into bronchus (expectoration of oily substance / trichoptysis in 5-14%, lipoid pneumonia)(6)Rupture into pericardium (pericardial effusion), pleural cavity (pleural effusion)DDx:thymoma Notes:


THORACIC PARAGANGLIOMA =CHEMODECTOMA=rare neural tumor arising from paraganglionic tissue Age:3rd-5th decade; M:F = 1:1Path:extremely vascular well-marginated / irregular mass that may adhere to / envelop / invade adjacent mediastinal structures (bronchus, spinal canal)Histo:anastomosing cords of granule-storing chief cells arranged in a trabecular pattern; identical appearance for benign and malignant tumorsMay be associated with: syn- / metachronous adrenal / extrathoracic paragangliomas; multiple endocrine neoplasia type 2; bronchial carcinoid tumor asymptomatic dyspnea, cough, chest pain, hemoptysis, neurologic deficits, SVC syndrome (if tumor large) signs of excessive catecholamine production: hypertension, headache, tachycardia, palpitations, tremorLocation:base of heart + great vessels (adjacent to pericardium / heart, within interatrial septum / left atrial wall); paravertebral sulciCT: sharply marginated 5-7 cm middle / posterior mediastinal mass hypodense areas due to extensive cystic degeneration / hemorrhage exuberant enhancementMR: heterogeneous intermediate signal intensity with areas of signal void from flowing blood on T1WI high signal intensity on T2WINUC (I-123 / I-131 metaiodobenzylguanidine): useful for localization as relatively specificAngio (may precipitate cardiovascular crisis): marked hypervascularity, multiple feeding vessels homogeneous capillary blushRx:surgical excision with preoperative administration of a- or b-blockers (hypertensive crisis, tachycardia, dysrhythmia during manipulation)

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THYMIC CYST Incidence:1-2% of mediastinal massesEtiology: (1)Congenital cyst (persistent tubular remnants of 3rd pharyngeal pouch = thymopharyngeal duct, develops during 5th-8th week of gestation)(2)Acquired reactive multilocular cysts = cystic transformation of duct epithelial structures induced by an inflammatory process: eg, HIV(3)Neoplastic cyst (cystic teratoma, cystic degeneration within a thymoma), S/P radiation therapy for Hodgkin diseaseAssociated with: (1)Hodgkin disease (? thymic involvement / treatment-induced cystic degeneration)(2)myasthenia gravis (rare) commonly asymptomatic symptomatic when hemorrhage occursLocation:anterior mediastinum / lateral neck unilocular cyst with thin walls containing clear fluid / multilocular cyst with thick walls containing turbid fluid or gelatinous material may show partial wall calcification (rare) low-density fluid (0-10 HU), may be higher depending on cyst contentsUS: typically anechoicDDx:Benign thymoma, teratoma, dermoid cyst, Hodgkin disease, non-Hodgkin lymphoma, pleural fibroma

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THYMIC HYPERPLASIA Most common anterior mediastinal mass in pediatric age group through puberty Age:particularly in young individualHisto:numerous active lymphoid germinal centersEtiology: 1.Hyperthyroidism (most common), Graves disease, treatment of primary hypothyroidism, idiopathic thyromegaly2.Rebound hyperplasia in children recovering from severe illness (eg, from burns), after treatment for Cushing disorder, after chemotherapy thymus may regrow more than 50% (transient overgrowth and reducible with steroids)3.Myasthenia gravis (65%)4.Acromegaly5.Addison disease normal thymus visible in 50% of neonates 0-2 years of age notch sign = indentation at junction of thymus + heart sail sign = triangular density extending from superior mediastinum wave sign = rippled border due to indentation from ribs shape changes with respiration + position

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THYMOLIPOMA Incidence:2-9% of thymic tumorsAge:3-60 years (mean age of 22 years); M:F = 1:1Path:lobulated pliable encapsulated tumor capable of growing to large size (in 68% >500 g, in 20% >2,000 g, the largest >16 kg)Histo:benign adult adipose tissue interspersed with areas of normal / hyperplastic / atrophic thymus tissue (thymic tissue 40 years; 70% occur in 5th-6th decade; less frequent in young adults, rare in children;M:F = 1:1Associated with:parathymic syndromes (40%) such as Myasthenia gravis:=autoimmune disorder characterized by antibodies against acetylcholine receptors of the postjunctional muscle membrane progressive weakness, fatigue fatigability of skeletal muscles innervated by cranial nerves, eg, ptosis, diplopia, dysphagia, dysarthria, drooling, difficulty with chewing elevated serum level of anti-acetylcholine receptor antibodies 10-15-25% of patients with myasthenia gravis have a thymoma (in 65% due to thymic hyperplasia) 7-30-54% of patients with thymoma have myasthenia gravis; removal of thymic tumor often results in symptomatic improvement; myasthenia gravis may develop after surgical thymoma excisionRx:edrophonium chloride Pure red cell aplasia = aregenerative anemia=almost total absence of marrow erythroblasts + blood reticulocytes resulting in severe normochromic normocytic anemia 50% of patients with red cell aplasia have thymoma 5% of patients with thymoma develop red cell aplasia Acquired hypogammaglobulinemia 10% of patients with hypogammaglobulinemia have thymoma 6% of patients with thymoma have hypogammaglobulinemia Paraneoplastic syndromes occur with thymic carcinoid (10%): eg, Cushing syndrome (ACTH production) chest pain, dyspnea, cough (33%)Path: round / ovoid slow-growing primary epithelial neoplasm with smooth / lobulated surface divided into lobules by fibrous septa; areas of hemorrhage + necrosis may form cysts (a)encapsulated = thick fibrous capsule ± calcifications(b)locally invasive = microscopic foci outside capsule(c)metastasizing = benign cytologic appearance with pleural + pulmonary parenchymal seeding(d)thymic carcinomaHisto: (a)biphasic thymoma (most common)= epithelial + lymphoid elements in equal amounts (b)predominantly lymphocytic thymoma= >2/3 of cells are lymphocytic (c)predominantly epithelial thymoma= >2/3 of cells are epithelial Prognosis unrelated to cell type! asymptomatic (50% discovered incidentally) signs of mediastinal compression (25-30%):cough, dyspnea, chest pain, respiratory infection, hoarseness (recurrent laryngeal n.), dysphagia signs of tumor invasion (rare): SVC syndromeLocation:any anterior mediastinal location between thoracic inlet and cardiophrenic angle; rare in neck, other mediastinal compartments, lung parenchyma, or tracheobronchial treeSize:1-10 cm (up to 34 cm) Noninvasive = Benign Thymoma Invasive [Malignant] Thymoma Notes:

Home : CHEST : Chest disorders : THYMOMA

Noninvasive = Benign Thymoma Age peak:5th-6th decade, almost all are >25 years of age oval / round lobulated sharply demarcated asymmetric homogeneous mass of soft-tissue density (equal to muscle), usually on one side of the midline abnormally wide mediastinum displacement of heart + great vessels posteriorlyCT: homogeneous soft-tissue mass with smooth / lobulated border partially / completely outlined by fat homogeneous enhancement areas of decreased attenuation (fibrosis, cysts, hemorrhage, necrosis) amorphous, flocculent central / curvilinear peripheral calcification (5-25%)MRI: isointense to skeletal muscle on T1WI increased signal intensity (approaching that of fat) on T2WI fluid characteristics of cysts with high water content Notes:

Home : CHEST : Chest disorders : THYMOMA

Invasive [Malignant] Thymoma Malignancy defined according to extent of invasion into adjacent mediastinal fat + fascia!Frequency:in 30-35% of thymomasStageI:intact capsuleStageII:pericapsular growth into mediastinal fatStageIII:invasion of surrounding organs such as lung, pericardium, SVC, aortaStageIVa:dissemination within thoracic cavity (metastases to pleura + lung in 6%)StageIVb:distant metastases (liver, bone, lymph nodes, kidneys, brain) heterogeneous attenuation spread by contiguity along pleural reflections, extension along aorta reaching posterior mediastinum / crus of diaphragm / retroperitoneum (transdiaphragmatic tumor extension) irregular interface with lung unilateral diffuse nodular pleural thickening / pleural masses encasing lung circumferentially vascular encroachment pleural effusion UNCOMMONDDx:malignant mesothelioma, lymphoma, thymic carcinoma / malignant germ cell tumor (older male, no diffuse pleural seeding), peripheral lung carcinoma (no dominant mediastinal mass), metastatic disease (not unilateral) Rx:radical excision ± adjuvant radiation therapyPrognosis:5-year survival of 93% for stage I, 86% for stage II, 70% for stage III, 50% for stage IV; 2-12% rate of recurrence for resected encapsulated thymomas Notes:


TORSION OF LUNG Incidence:rare (29 mm), right (>20 mm) + left ( >15 mm) mainstem bronchi sacculated outline / diverticulosis of trachea on lateral CXR (= protrusion of mucous membrane between rings of trachea) may have emphysema, bullae in perihilar region Notes:


TRACHEOBRONCHOPATHIA OSTEOCHONDROPLASTICA =rare benign disease characterized by cartilaginous / osseous nodules projecting from submucosa into tracheobronchial lumenCause:unknown; may be due to chronic inflammation, degenerative process, irritation by oxygen / chemical, metabolic disturbance, amyloidosis, tuberculosis, syphilis, heredity (high prevalence in Finland)Pathogenetic theories: (1)Ecchondrosis / exostosis of cartilage rings(2)Cartilaginous / osseous metaplasia of internal elastic fibrous membrane of tracheaHisto:adipose tissue + calcified areas with foci of bone marrow; thinned normal overlying mucosa with inflammation + hemorrhageAge:in 50% >50 years (11-72 years); M:F = 3:1 usually asymptomatic (incidentally diagnosed) dyspnea, productive cough, hoarseness, hemoptysis, fever, recurrent pneumoniaLocation:distal 2/3 of trachea, larynx, lobar / segmental bronchi, entire length of trachea;spares posterior membrane of tracheaCXR: scalloped / linear opacities surrounding + narrowing the trachea (best on lateral view)CT: deformed thickened narrowed tracheal wall irregularly spaced 1-3 mm calcific submucosal nodules of trachea + bronchi (similar to plaques)Dx:bronchoscopyDDx:relapsing polychondritis, tracheobronchial amyloidosis, sarcoidosis, papillomatosis, tracheobronchomalacia

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TRANSIENT TACHYPNEA OF THE NEWBORN =NEONATAL WET LUNG DISEASE = TRANSIENT RESPIRATORY DISTRESS OF THE NEWBORN= RETAINED FETAL LUNG FLUIDIncidence:6%; most common cause of respiratory distress in newbornCause:cesarean section, precipitous delivery, breech delivery, prematurity, maternal diabetesPathophysiology: delayed resorption of fetal lung fluid (normal clearance occurs through capillaries (40%), lymphatics (30%), thoracic compression during vaginal delivery (30%) Onset: within 6 hours of life; peak at day 1 of age increasing respiratory rates during first 2-6 hours of life intercostal + sternal retraction normal blood gases during hyperoxygenation linear opacities + perivascular haze + thickened fissures + interlobular septal thickening (interstitial edema) mild hyperaeration mild cardiomegaly small amount of pleural fluidPrognosis: resolving within 1-4 days (retrospective diagnosis) DDx:(1) normal during first several hours of life(2) diffuse pneumonitis / sepsis (3) mild meconium aspiration syndrome (4) "drowned newborn syndrome" = clear amniotic fluid aspiration (5) alveolar phase of RDS (6) pulmonary venous congestion (7) pulmonary hemorrhage (8) hyperviscosity syndrome = thick blood (9) immature lung syndrome

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TRAUMATIC LUNG CYST Age:children + young adults are particularly prone thin-walled air-filled cavity (50%) ± air-fluid level preceded by homogeneous well-circumscribed mass (hematoma) oval / spherical lesion of 2-14 cm in diameter single / multiple lesions; uni- or multilocular usually subpleural under point of maximal injury persistent up to 4 months + progressive decrease in size (apparent within 6 weeks)

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TUBERCULOSIS Prevalence:10 million people worldwide, active TB develops in 5-10% of those exposedOrganism:Mycobacterium = acid-fast aerobic rods staining red with carbol-fuchsin; M. tuberculosis (95%), atypical types increasing: M. avium-intracellulare, M. kansasii, M. fortuitum Susceptible:infants, pubertal adolescents, elderly, alcoholics, Blacks, diabetics, silicosis, measles, AIDS, sarcoidosis (in up to 13%)Pathologic phases: (a)exudative reaction (initial reaction, present for 1 month)(b)caseous necrosis (after 2-10 weeks with onset of hypersensitivity)(c)hyalinization = invasion of fibroblasts (granuloma formation in 1-3 weeks)(d)calcification / ossification(e)chronic destructive form in 10% (19 years (90%); 3rd-5th decade (75%); contrasts with low incidence of varicella in this age group vesicular rash patchy diffuse airspace consolidation tendency for coalescence near hila + lung bases widespread nodules (30%) representing scarring tiny 2-3 mm calcifications widespread throughout both lungs (2%)Cx:unilateral diaphragmatic paralysisPrognosis:11% mortality rate

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VIRAL PNEUMONIA Organism:Rhinovirus (43%), respiratory syncytial virus (12%), Mycoplasma (10%), Parainfluenza virus, adenovirus, Influenza-virusPath:necrosis of ciliated epithelial cells, goblet cells, bronchial mucous glands with frequent involvement of peribronchial tissues + interlobular septaAge:most common cause of pneumonia in children under 5 years of ageDistribution:usually bilateral hyperaeration + air trapping "dirty chest" = peribronchial cuffing + opacification perihilar linear densities (bronchial wall thickening) interstitial pattern airspace pattern (from hemorrhagic edema) in 50% pleural effusion (20%) hilar adenopathy (3%) striking absence of pneumatoceles, lung abscess, pneumothorax radiographic resolution lags 2-3 weeks behind clinicalCx:bronchiectasis; unilateral hyperlucent lung Atypical measles pneumonia does NOT show the typical radiographic findings of viral pneumonias! Notes:


WEGENER GRANULOMATOSIS =probable autoimmune disease characterized by systemic necrotizing granulomatous process with destructive angiitisPath:peribronchial necrotizing granulomas + vasculitis not intimately related to arteriesMean age of onset:40 years (range of all ages);M:F = 2:1CLASSIC TRIAD: (1)respiratory tract granulomatous inflammation(2)systemic small-vessel vasculitis(3)necrotizing glomerulonephritis @Upper respiratory tract (100% involvement)(similar to midline granuloma) (a)nasal cavity: epistaxis from nasal mucosal ulceration necrosis of nasal septum saddle nose deformity progressive destruction of nasal cartilage + bone (DDx: relapsing polychondritis) granulomatous masses filling nasal cavities(b)sinuses (maxillary antra most frequently): sinus pain, purulent sinus drainage, rhinorrhea thickening of mucous membranes of paranasal sinuses@Pulmonary disease stridor (from tracheal inflammation + sclerosis) intractable cough, occasionally with hemoptysis patchy alveolar infiltrates (with acute airspace pneumonia / pulmonary hemorrhage) widely distributed multiple irregular masses / nodules of varying sizes (up to 9 cm), especially in lower lung fields thick-walled cavities with irregular shaggy inner lining (25-50%) pleural effusion in 25% lymphadenopathy exceedingly rareCx:(1)dangerous airway stenosis (15% of adults, 50% of children)(2)massive life-threatening pulmonary hemorrhage@Renal diseasefocal glomerulonephritis in 20% at presentation, as disease progresses in 83% Histo:focal necrosis, crescent formation, paucity/ absence of immunoglobulin deposits@ Other organ involvement: (a)Joints (56%): migratory polyarthropathy(b)Skin + muscle (44%): inflammatory nodular skin lesions, cutaneous purpura(c)Eyes + middle ear (29%): ocular inflammation, proptosis, otitis media(d)Heart + pericardium (28%): myocardial infarction (vasculitis)(e)CNS (22%): central / peripheral neuritis(a)involvement of abdominal visceraCx:(1) Hypertension (2) Uremia (3) Facial nerve paralysisDx:lung / renal biopsyPrognosis:death within 2 years from renal failure (83%) / respiratory failureRx:corticosteroids, cytotoxic drugs (cyclophosphamide), renal transplantation Limited Wegener Granulomatosis Midline Granuloma Notes:

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Limited Wegener Granulomatosis =Wegener granulomatosis WITHOUT renal involvement Notes:

Home : CHEST : Chest disorders : WEGENER GRANULOMATOSIS

Midline Granuloma =mutilating granulomatous + neoplastic lesions limited to nose + paranasal sinuses with very poor prognosis; considered a variant of Wegener granulomatosis WITHOUT the typical granulomatous + cellular components Notes:


WILLIAMS-CAMPBELL SYNDROME =congenital bronchial cartilage deficiency in the 4th to 6th bronchial generation either diffuse or restricted to focal areaHRCT: cystic bronchiectasis distal to 3rd bronchial generation emphysematous lung distal to bronchiectasis inspiratory ballooning + expiratory collapse of dilated segments

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WILSON-MIKITY SYNDROME =PULMONARY DYSMATURITY=similarity to bronchopulmonary dysplasia in patients breathing room air; rarely encountered anymorePredisposed:premature infants submucosa > muscularis > mesentery; mesenteric side >> antimesenteric side radiolucent clusters of cysts along contour of bowel wall (best demonstrated on CT) segmental mucosal nodularity (DDx: polyposis) ± pneumoperitoneum / pneumoretroperitoneum (asymptomatic large pneumoperitoneum may persist for months / years) ±gas in mesenteric + portal veinPrognosis: wide spectrum from innocuous to fatal; clinical outcome impossible to predict based on x-ray findings linear gas collections have probably a more severe connotation pneumatosis of the colon is likely clinically insignificant extent of pneumatosis is inversely related to severity of disease

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ABNORMAL INTRA-ABDOMINAL AIR

Soap-bubble Appearance In Abdomen Of Neonate 1.Feces in infant fed by mouth2.Meconium ileus:gas mixed with meconium, usually RLQ3.Meconium plug:gas in and around plug, in distribution of colon4.Necrotizing enterocolitis: submucosal pneumatosis5.Atresia / severe stenosis: pneumatosis6.Hirschsprung disease:impacted stool, sometimes pneumatosis

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ABDOMINAL CALCIFICATIONS & OPACITIES

Opaque Material In Bowel mnemonic:"CHIPS"Chloral hydrate Heavy metals (lead) Iron Phenothiazines Salicylates Notes:


Diffuse Abdominal Calcifications 1.Cystadenoma of ovary granular, sandlike psammomatous calcifications2.Pseudomyxoma peritonei(a)pseudomucinous adenoma of ovary(b)mucocele of appendix3.Undifferentiated abdominal malignancy4.Tuberculous peritonitis mottled calcifications, simulating residual barium5.Meconium peritonitis6.Oil granuloma annular, plaquelike Notes:


Focal Alimentary Tract Calcifications A.ENTEROLITHS1.Appendicolith: in 10-15% of acute appendicitis2.Stone in Meckel diverticulum3.Diverticular stone4.Rectal stone5.Proximal to partial obstruction (eg, tuberculosis, Crohn disease)B.MESENTERIC CALCIFICATIONS1.Dystrophic calcification of omental fat deposits + appendices epiploicae (secondary to infarction / pancreatitis / TB)2.Cysts: mesenteric cyst, hydatid cyst3.Calcified mesenteric lipomaC.INGESTED FOREIGN BODIEStrapped in appendix, diverticula, proximal to stricture 1.Calcified seeds + pits (bezoar)2.BirdshotD.TUMOR1.Mucocele of appendix crescent-shaped / circular calcification2.Mucinous adenocarcinoma of stomach / colon= COLLOID CARCINOMA small mottled / punctate calcifications in primary site ± in regional lymph node metastases, adjacent omentum, metastatic liver foci3.Gastric / esophageal leiomyoma: calcifies in 4%4.Lipoma Notes:


Abdominal Wall Calcifications A.IN SOFT TISSUES1.Hypercalcemic states2.Idiopathic calcinosisB.IN MUSCLE(a)parasites:1.Cysticercosis = Taenia solium round / slightly elongated calcifications2.Guinea worm = dracunculiasis stringlike calcifications up to 12 cm long(b)injection sitesfrom quinine, bismuth, calcium gluconate, calcium penicillin (c)myositis ossificansC.IN SKIN1.Soft-tissue nodules: papilloma, neurofibroma, melanoma, nevi2.Scar: linear density3.Colostomy / ileostomy4.Tattoo markings Notes:


Abdominal Vascular Calcifications A.ARTERIES1.Atheromatous plaques2.Arterial calcifications in diabetes mellitusB.VEINSphleboliths = calcified thrombus, generally seen below interspinous line 1.normal / varicose veins2.hemangiomaC.LYMPH NODES1.Histoplasmosis / tuberculosis2.Chronic granulomatous disease3.Residual lymphographic contrast4.Silicosis Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ABNORMAL INTRA-ABDOMINAL FLUID

Ascites A.TRANSUDATE:(1) Cirrhosis (75%): poor prognostic sign (2) Hypoproteinemia, (3) CHF, (4) Constrictive pericarditis, (5) Chronic renal failure, (6) Budd-Chiari syndrome B.EXUDATE:(1) Carcinomatosis, (2) Polyserositis, (3) TB peritonitis, (4) Pancreatitis, (5) Meigs syndrome C.HEMORRHAGIC / CHYLOUS FLUID Early signs (accumulation in pelvis): round central density in pelvis + ill-defined bladder top thickening of peritoneal flank stripe space between properitoneal fat and gut >3 mmLate signs: Hellmer sign = medial displacement of lateral liver margins medial displacement of ascending + descending colon obliteration of hepatic + splenic angles bulging flanks gray abdomen floating centralized loops separation of loops High-density Ascites 1.Tuberculosis: 20-45 HU; may be lower2.Ovarian tumor3.Appendiceal tumor Neonatal Ascites A.GASTROINTESTINAL(a)perforation of hollow viscusmeconium peritonitis (b)inflammatory lesionsMeckel diverticulum, appendicitis (c)cyst rupturemesenteric / omental / choledochal cyst (d)bile leakagebiliary obstruction / perforation B.PORTOHEPATIC(a)extrahepatic portal vein obstructionatresia of veins, compression by mass (b)intrahepatic portal vein obstructionportal cirrhosis (neonatal hepatitis), biliary cirrhosis (biliary atresia) C.URINARY TRACT Urine ascites (most common cause) from lower urinary tract obstruction + upper urinary tract rupture: posterior / anterior urethral valves, ureterovesical / ureteropelvic junction obstruction, renal / bladder rupture, anterior urethral diverticulum, bladder diverticula, neurogenic bladder, extrinsic bladder massD.GENITALruptured ovarian cyst, hydrometrocolpos E.HYDROPS FETALISimmune hydrops, nonimmune hydrops (usually cardiac causes) F.MISCELLANEOUSchylous ascites, lymphangiectasia, congenital syphilis, trauma, idiopathic Chylous Ascites IN ADULTS:1.Inflammatory process(35%)2.Tumor(30%)3.Idiopathic(23%)4.Trauma(11%)5.Congenital( 1%)IN CHILDREN:1.Congenital(39%)2.Inflammatory process(15%)3.Trauma(12%)4.Tumor( 3%)5.Idiopathic(33%) Notes:


Fluid Collections mnemonic: "BLUSCHINGS"Biloma Lymphocele, Lymphangioma, Lymphoma (almost anechoic by US) Urinoma Seroma Cyst (pseudocyst, peritoneal inclusion cyst) Hematoma (aneurysm, AVM) Infection, Infestation (empyema, abscess, Echinococcus) Neoplasm (necrotic) GI tract (dilated loops, ileus, duplication) Serosa (ascites, pleural fluid, pericardial effusion) Notes:


Intra-abdominal Cyst In Childhood 1.Omental cyst (greater omentum / lesser sac, multilocular)2.Mesenteric cyst (between leaves of small bowel mesentery)3.Choledochal cyst4.Intestinal duplication5.Ovarian cyst6.Pancreatic pseudocyst7.Cystic renal tumor8.Abscess9.Meckel diverticulum (communicates with GI tract)10.Lymphangioma11.Mesenteric lymphoma12.Intramural tumor Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders

MECHANICAL INTESTINAL OBSTRUCTION =occlusion / constriction of bowel lumen Common Causes Of Obstruction In Children Gastric Outlet Obstruction Duodenal Obstruction Jejunal And Ileal Obstruction Colonic Obstruction Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : MECHANICAL INTESTINAL OBSTRUCTION

Common Causes Of Obstruction In Children NurseryIntestinal atresia, midgut volvulus, meconium ileus, Hirschsprung disease, small bowel atresia with meconium ileus, meconium plug syndrome, small left colon syndrome, imperforate anus, obstruction from duplication cystFirst 3 monthsInguinal hernia, Hirschsprung disease, midgut volvulus6 - 24 monthsIleocolic intussusceptionChildhoodAppendicitis Notes:


Gastric Outlet Obstruction A.CONGENITAL LESION1.Antral mucosal diaphragm = antral web2.Gastric duplication: usually along greater curvature, abdominal mass in infancy3.Hypertrophic pyloric stenosisB.INFLAMMATORY NARROWING1.Peptic ulcer disease: cause in adults in 60-65%2.Corrosive gastritis3.Crohn disease, sarcoidosis, syphilis, tuberculosisC.MALIGNANT NARROWING1.Antral carcinoma: cause in adults in 30-35%2.Scirrhous carcinoma of pyloric channelD.OTHERS1.Prolapsed antral polyp / mucosa2.Bezoar3.Gastric volvulus4.Postoperative stomal edemaAbdominal plain film: large smoothly marginated homogeneous mass displacing transverse colon + small bowel inferiorly one / two air-fluid levels Notes:


Duodenal Obstruction A.CONGENITAL1.Annular pancreas2.Peritoneal bands = Ladd bands3.Aberrant vesselB.INFLAMMATORY NARROWING1.Chronic duodenal ulcer scar2.Acute pancreatitis: phlegmon, abscess, pseudocyst3.Acute cholecystitis: perforated gallstoneC.INTRAMURAL HEMATOMA1.Blunt trauma (accident, child abuse)2.Anticoagulant therapy3.Blood dyscrasiaD.TUMORAL NARROWING1.Primary duodenal tumors2.Tumor invasion from pancreas, right kidney, lymph node enlargementE.EXTRINSIC COMPRESSION1.Aortic aneurysm2.PseudoaneurysmF.OTHERS1.Superior mesenteric artery syndrome from extensive burns, body cast, rapid weight loss, prolonged bed rest2.Bezoar (in gastrectomized patient) mnemonic:"VA BADD TU BADD"childadult VolvulusTumorAtresiaUlcerBandsBandsAnnular pancreasAnnular pancreasDuplicationDuplicationDiverticulumDiverticulum Abdominal plain film: double-bubble sign = air-fluid levels in stomach + duodenum frequently normal due to absence of gas from vomiting

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Jejunal And Ileal Obstruction =SMALL BOWEL OBSTRUCTION (SBO)Technique:best evaluated by CT (95% accurate, 94% sensitive, 96% specific)A.CONGENITAL1.Ileal atresia / stenosis2.Enteric duplication: located on antimesenteric side, mostly in ileum3.Midgut volvulus from arrest in rotation + fixation of small bowel during fetal life4.Mesenteric cyst from meconium peritonitis:located on mesenteric side 5.Meckel diverticulumB.EXTRINSIC BOWEL LESIONS1.Fibrous adhesions from previous surgery / peritonitis (in 75%) 2.Hernias (inguinal, femoral, umbilical, paraduodenal, foramen of Winslow, incisional, Spigelian, obturator)3.Volvulus4.Masses: neoplasm, abscessC.LUMINAL OCCLUSION1.Swallowed foreign body, bezoar, gallstone, bolus of Ascaris lumbricoides, inspissated milk2.Meconium ileus: microcolon in cystic fibrosis 3.Meconium ileus equivalent4.Intussusception (tumor, Meckel diverticulum, chronic ulcer, adhesion)5.Tumor (rare): eg, lipomaD.INTRINSIC BOWEL WALL LESION1.Strictures from neoplasm, Crohn disease, tuberculous enteritis, parasitic disease, potassium chloride tablets, surgical anastomosis, irradiation, massive deposition of amyloid2.Intramural hemorrhage: blunt trauma, Henoch-Schönlein purpura3.Vascular insufficiency: arterial / venous occlusion Acquired Small Bowel Obstruction In Childhood mnemonic: "AAIIMM"Adhesions Appendicitis Intussusception Incarcerated hernia Malrotation Meckel diverticulum Small Bowel Obstruction In Adulthood mnemonic:"SHAVIT"Stone (gallstone ileus) Hernia Adhesion Volvulus Intussusception Tumor Plain abdominal radiograph (50-66% sensitive): "candy cane" appearance in erect position = >3 distended small bowel loops >3 cm with gas-fluid levels ( >3-5 hours after onset of obstruction) disparity in size between obstructed loops and contiguous small bowel loops of normal caliber beyond site of obstruction small bowel positioned in center of abdomen little / no gas + stool in colon with complete mechanical obstruction after 12-24 hours "stretch sign" = erectile valvulae conniventes completely encircle bowel lumen "stepladder appearance" in low obstruction (the greater the number of dilated bowel loops, the more distal the site of obstruction) "string-of-beads" indicate peristaltic hyperactivity to overcome mechanical obstruction hyperactive peristalsis / aperistalsis = fatigued small bowelCAVE:little / no gas in small bowel from fluid-distended loops may lead one to overlook obstruction Plain abdominal radiographic categories: 1.Normal=absence of small intestinal gas / gas within 3-4 variably shaped loops 3 air-fluid levels3.Probable SBO pattern=dilated multiple gas- / fluid-filled loops with air-fluid levels + moderate amount of colonic gas4.Definite SBO pattern=clearly disproportionate gaseous / fluid distension of small bowel relative to colon UGI: "snake head" appearance = active peristalsis forms bulbous head of barium column in an attempt to overcome obstruction barium appears in colon >12 hours Enteroclysis for adhesive obstruction: abrupt change in caliber of bowel with normal caliber / collapsed bowel distal to obstruction stretched folds of normal pattern angulated + fixed bowel segmentEnteroclysis categories of SBO (Shrake): (a)low-grade partial SBO=sufficient flow of contrast material through point of obstruction so that fold pattern beyond obstruction is readily defined(b)high-grade partial SBO=stasis + delay in arrival of contrast so that contrast material is diluted in distended prestenotic loop with minimal contrast in postobstructive loop leading to difficulty in defining fold pattern after transition point(c)complete SBO=no passage of contrast material 3-24 hours after start of examination CT (poor sensitivity for low-grade partial obstruction) US: small bowel loops dilated >3 cm length of dilated segment >10 cm increased peristalsis of dilated segment (may become paralytic in prolonged obstruction) colon collapsed Location of obstruction: (a)valvulae conniventes high + frequent = jejunum(b)valvulae conniventes sparse / absent = ileum Closed Loop Obstruction =bowel obstruction at two pointsCause:adhesion (75%), volvulus, incarcerated hernia U-shaped distended loop increasing intraluminal fluid fixation of bowel loop = no change in position "coffee bean sign" = gas-filled loop "pseudotumor" = fluid-filled loop U- or C-shaped dilated bowel loop on CT "beak sign" = point of obstruction on CT / UGI "whirl sign" = twisting of bowel + mesentery on CT stretched mesenteric vessels converging toward torsion Strangulated Obstruction =triad of (1) mechanical obstruction proximal to the involved segment (2) closed-loop obstruction of the involved segment (3) venous congestion of the involved segmentCT: slight circumferential thickening of bowel wall increased wall attenuation target / halo sign serrated beak at site of obstruction (32-100% specific) unusual course of mesenteric vasculature mesenteric haziness due to edema (95% specific) diffuse engorgement of mesenteric vasculature poor / no enhancement of bowel wall (100% specific) delayed prolonged enhancement of bowel wall large amount of ascites pneumatosis intestinalis

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Colonic Obstruction Incidence:25% of all intestinal obstructionsA.NEONATAL COLONIC OBSTRUCTION1.Meconium plug syndrome2.Colonic atresia3.Anorectal malformation: rectal atresia, imperforate anusB.LUMINAL OBTURATION1.Fecal impaction bubbly pattern of large mass of stool2.Fecaloma3.Gallstone (in sigmoid narrowed by diverticulitis)4.IntussusceptionC.BOWEL WALL LESION(a)malignant (60-70% of obstructions):predominantly in sigmoid (b)inflammatory1.Crohn disease2.Ulcerative colitis3.Mesenteric ischemia4.Sigmoid diverticulitis (15%) stenotic segment >6 cm5.Acute pancreatitis(c)infectious:infectious granulomatous process (actinomycosis, tuberculosis, lymphogranuloma venereum), parasitic disease (amebiasis, schistosomiasis) (d)wall hematoma:blunt trauma, coagulopathy D.EXTRINSIC (a)mass impression1.Endometriosis2.Large tumor mass: prostate, bladder, uterus, tubes, ovaries3.Pelvic abscess4.Hugely distended bladder5.Mesenteritis6.Poorly formed colostomy (b)severe constriction1.Volvulus (3rd most common cause): sigmoid colon, cecum, transverse colon, compound volvulus (= ileosigmoid knot)2.Hernia: transverse colon in diaphragmatic hernia, sigmoid colon in left inguinal hernia3.Adhesion Abdominal plain-film patterns: (a)dilated colon only = competent ileocecal valve(b)dilated small bowel (25%) = incompetent ileocecal valve(c)dilated colon + dilated small bowel = ileocecal valve obstruction secondary to cecal overdistension gas-fluid levels distal to hepatic flexure (fluid is normal in cecum + ascending colon); sign not valid with diarrhea / saline catharsis / enema cecum most dilated portion (in 75% of cases); critical at 10 cm diameter (high probability for impending perforation) The lower the obstruction, the more proximal the distension!BE:emergency barium enema of unprepared colon in suspected obstruction!contraindicated in toxic megacolon, pneumatosis intestinalis, portal vein gas, extraluminal gas

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ILEUS [ileus = stasis / inability to push fluid along (term does not distinguish between mechanical and nonmechanical causes) ] =ADYNAMIC / PARALYTIC / NONOBSTRUCTIVE ILEUS=derangement impairing proper distal propulsion of intestinal contents Cause: -in neonate:1.Hyperbilirubinemia2.Intracranial hemorrhage3.Aspiration pneumonia4.Necrotizing enterocolitis5.Aganglionosis-in child / adult:1.Postoperative ileus usually resolves by 4th postoperative day2.Visceral pain: obstructing ureteral stone, common bile duct stone, twisted ovarian cyst, blunt abdominal / chest trauma3.Intra-abdominal inflammation / infection: peritonitis, appendicitis, cholecystitis, pancreatitis, salpingitis, abdominal abscess, hemolytic-uremic syndrome, gastroenteritis4.Ischemic bowel disease5.Anticholinergic drugs: atropine, propantheline, morphine + derivatives, tricyclic antidepressants, dilantin, phenothiazines, hexamethonium bromide6.Neuromuscular disorder: diabetes, hypothyroidism, porphyria, lead poisoning, uremia, hypokalemia, amyloidosis, urticaria, sprue, scleroderma, Chagas disease, vagotomy, myotonic dystrophy, CNS trauma, paraplegia, quadriplegia7.Systemic disease: septic / hypovolemic shock, urticaria8.Chest disease: lower lobe pneumonia, pleuritis, myocardial infarction, acute pericarditis, congestive heart failure9.Retroperitoneal disease: hemorrhage (spine trauma), abscess mnemonic:"Remember the P's"Pancreatitis Pendicitis Peptic ulcer Perforation Peritonitis Pneumonia Porphyria Postoperative Potassium deficiency Pregnancy Pyelonephritis intestinal sounds decreased / absent abdominal distension large + small bowel ± gastric distension decreased small bowel distension on serial films delayed but free passage of contrast materialRx:not amenable to surgical correction Localized Ileus Intestinal Pseudoobstruction Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ILEUS

Localized Ileus =isolated distended loop of small / large bowel= SENTINEL LOOP Often associated with an adjacent acute inflammatory process Etiology: 1.Acute pancreatitis:duodenum, jejunum, transverse colon2.Acute cholecystitis:hepatic flexure of colon3.Acute appendicitis:terminal ileum, cecum4.Acute diverticulitis:descending colon5.Acute ureteral colic:GI tract along course of ureter Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ILEUS

Intestinal Pseudoobstruction A.Transient pseudoobstruction1.Electrolyte imbalance2.Renal failure3.Congestive heart failureB.Chronic pseudoobstruction1.Scleroderma2.AmyloidosisC.Idiopathic pseudoobstruction1.Chronic intestinal pseudoobstruction syndrome persistently decreased peristalsis + clinical obstructionAge:neonatal period / delayed for months + years2.Megacystis-microcolon-intestinal-hypoperistalsis syndrome

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ESOPHAGUS

Esophageal Contractions Esophageal motor activity needs to be evaluated in recumbent position without influence of gravity!PERISTALTIC EVENT = coordinated contractions of esophagus PERISTALTIC SEQUENCE = aboral stripping wave clearing esophagus A.PRIMARY PERISTALSIS=orderly peristaltic sequence with progressive aboral stripping traversing entire esophagus with complete clearance of barium; centrally mediated (medulla) swallow reflex via glossopharyngeal + vagal nerve; initiated by swallowing rapid wave of inhibition followed by slower wave of contraction Normal peristaltic sequence will be interrupted by repetitive swallowing before peristaltic sequence is complete!B.SECONDARY PERISTALSIS=local peristaltic wave identical to primary peristalsis but elicited through esophageal distension = sensorimotor stretch reflex Esophageal motility can be evaluated with barium injection through nasoesophageal tube despite patient's inability to swallow!C.TERTIARY CONTRACTIONS=nonpropulsive esophageal motor event characterized by disordered up-and-down movement of bolus without clearing of esophagus Cause: 1.Presbyesophagus2.Diffuse esophageal spasm3.Hyperactive achalasia4.Neuromuscular disease:diabetes mellitus, Parkinsonism, amyotrophic lateral sclerosis, multiple sclerosis, thyrotoxic myopathy, myotonic dystrophy5.Obstruction of cardia:neoplasm, distal esophageal stricture, benign lesion, S/P repair of hiatal hernia Tertiary activity does not necessarily imply a significant motility disturbance!Age:in 5-10% of normal adults during 4th-6th decade(a)nonsegmental = partial luminal indentationLocation: in lower 2/3 of esophagus spontaneous repetitive nonpropulsive contraction "yo-yo" motion of barium "corkscrew" appearance = scalloped configuration of barium column "rosary bead" / "shish kebab" configuration= compartmentalization of barium column no lumen-obliterating contractions(b)segmental = luminal obliteration (rare) "curling" = erratic segmental contractions "rosary-bead" appearance Notes:


Abnormal Esophageal Peristalsis A.PRIMARY MOTILITY DISORDERS1.Achalasia2.Diffuse esophageal spasm severe intermittent pain while swallowing compartmentalization of esophagus by numerous tertiary contractionsDx:extremely high pressures on manometry3.Presbyesophagus4.Chalasia5.Congenital TE fistula6.Intestinal pseudoobstructionB.SECONDARY MOTILITY DISORDERS(a)Connective tissue disease1.Scleroderma2.SLE3.Rheumatoid arthritis4.Polymyositis5.Dermatomyositis6.Muscular dystrophy(b)Chemical / physical injury1.Reflux / peptic esophagitis2.S/P vagotomy3.Caustic esophagitis4.Radiotherapy(c)InfectionFungal: candidiasisParasitic: Chagas diseaseBacterial:TB, diphtheriaViral:herpes simplex(d)Metabolic disease1.Diabetes mellitus2.Amyloidosis3.Alcoholism4.Electrolyte disturbances(e)Endocrine disease1.Myxedema2.Thyrotoxicosis(f)Neoplasm(g)Drug-relatedatropine, propantheline, curare (h)Muscle disease1.Myotonic dystrophy2.Muscular dystrophy3.Oculopharyngeal dystrophy4.Myasthenia gravis (disturbed motility only in striated muscle of upper 1/3 of esophagus) persistent collection of barium in upper third of esophagus findings reversed by cholinesterase inhibitor edrophonium (Tensilon®)(i)Neurologic disease1.Parkinsonism2.Multiple sclerosis3.CNS neoplasm4.Amyotrophic lateral sclerosis5.Bulbar poliomyelitis6.Cerebrovascular disease7.Huntington chorea8.Ganglioneuromatosis9.Wilson disease10.Friedreich ataxia11.Familial dysautonomia (Riley-Day)12.Stiff-man syndrome

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Diffuse Esophageal Dilatation =ACHALASIA PATTERN = MEGAESOPHAGUSA.ESOPHAGEAL MOTILITY DISORDER1.Idiopathic achalasia2.Chagas disease: patients commonly from South America; often associated with megacolon + cardiomegaly3.Postvagotomy syndrome4.Scleroderma5.Systemic lupus erythematosus6.Presbyesophagus7.Ehlers-Danlos syndrome8.Diabetic / alcoholic neuropathy9.Anticholinergic drugs10.Idiopathic intestinal pseudoobstruction= degeneration of innervation11.Amyloidosis: associated with macroglossia, thickened small bowel folds12.EsophagitisB.DISTAL OBSTRUCTION1.Infiltrating lesion of distal esophagus / gastric cardia (eg, carcinoma) = pseudoachalasia2.Benign stricture3.Extrinsic compression mnemonic:"MA'S TACO in a SHell"Muscular disorder (eg, myasthenia gravis) Achalasia Scleroderma Trypanosomiasis (Chagas disease) Amyloidosis Carcinoma Obstruction Stricture (lye, potassium, tetracycline) Hiatal hernia

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Air Esophagogram 1.Normal variant2.Scleroderma3.Distal obstruction: tumor, stricture, achalasia4.Thoracic surgery5.Mediastinal inflammatory disease6.S/P total laryngectomy (esophageal speech)7.Endotracheal intubation + PEEP Notes:


Abnormal Esophageal Folds A.TRANSVERSE FOLDS1.Feline esophagus frequently seen with gastroesophageal reflux; normally found in cats transient contraction of longitudinally oriented muscularis mucosae2.Fixed transverse foldsdue to scarring from reflux esophagitis stepladder appearance in distal esophagusB.LONGITUDINAL FOLDSnormally 1-2 mm wide in collapsed esophagus; >3 mm with submucosal edema / inflammation 1.Gastroesophageal reflux2.Opportunistic infection3.Caustic ingestion4.IrradiationDDx:1.Varices tortuous / serpentine folds that can be effaced by esophageal distension2.Varicoid carcinoma fixed rigid folds with abrupt demarcation due to submucosal spread

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Esophageal Inflammation A.CONTACT INJURY(a)reflux related1.Peptic ulcer disease2.Barrett esophagus3.Scleroderma (patulous LES)4.Nasogastric intubation(b)caustic1.Foreign body2.Corrosives(c)thermicHabitual ingestion of excessively hot meals / liquids B.RADIATION INJURYC.INFECTION1.Candidiasis2.Herpes simplex virus / CMV3.DiphtheriaD.SYSTEMIC DISEASE(a)dermatologic disorderspemphigoid, epidermolysis bullosa (b)others:1.Crohn disease2.Graft-versus-host disease3.Behçet disease4.Eosinophilic gastroenteritis

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Esophageal Ulceration A.PEPTIC1.Reflux esophagitis: scleroderma shallow / deep ulcers in distal esophagus2.Barrett esophagus3.Crohn disease aphthous ulcers in variable location4.Dermatologic disorders: benign mucous membrane pemphigoid, epidermolysis bullosa dystrophica, Behçet diseaseB.INFECTIOUS1.Herpes discrete superficial ulcers in midesophagus2.Cytomegalovirus large flat ulcer in mid- or distal esophagusC.CONTACT INJURY / EXTERNAL INJURY1.Corrosives: alkali, strictures in 50%2.Alcohol-induced esophagitis3.Drug-induced = "pill esophagitis":(a)antibiotics (tetracyclines), quinidine, potassium chloride discrete superficial ulcers in midesophagus(b)alendronate (= inhibitor of osteoclastic activity) long-segment involvement with severe ulceration4.Radiotherapy: smooth stricture >4500 rads shallow / deep ulcers conforming to radiation portal5.Nasogastric tube elongated stricture in middle + distal 1/36.Endoscopic sclerotherapyD.MALIGNANT1.Esophageal carcinoma Location: @Upper esophagus1.Barrett ulcer in islets of gastric [email protected] esophagitis2.CMV esophagitis3.Drug-induced esophagitis@Distal esophagus1.Reflux esophagitis2.CMV esophagitisDDx: 1.Sacculation=outpouching in distal esophagus due to asymmetric scarring in reflux esophagitis2.Esophageal intramural pseudodiverticula3.Artifact(a)tiny precipitates of barium(b)transient mucosal crinkling in inadequate distension(c)irregular Z-line

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Double-barrel Esophagus 1.Dissecting intramural hematoma from emetogenic injury2.Mallory-Weiss teartrauma, esophagoscopy (in 0.25%), bougienage (in 0.5%), ingestion of foreign bodies, spontaneous (bleeding diathesis) 3.Intramural abscess4.Intraluminal diverticulum5.Esophageal duplication (if communication with esophageal lumen present) Notes:


Esophageal Diverticulum 1.ZENKER DIVERTICULUM (pharyngoesophageal)2.INTERBRONCHIAL DIVERTICULUM=traction diverticulumresponse to pull from fibrous adhesions following lymph node infection (TB), contains all 3 esophageal layers Location:usually on right anterolateral wall of interbronchial segment calcified mediastinal nodes3.INTERAORTICOBRONCHIAL DIVERTICULUM= thoracic pulsion diverticulum Location:on left anterolateral wall between inferior border of aortic arch + upper margin of left main bronchus4.EPIPHRENIC DIVERTICULUM (rare)Location:usually on lateral esophageal wall, right > left, in distal 10 cm often associated with hiatus hernia5.INTRAMURAL ESOPHAGEAL PSEUDODIVERTICULOSIS outpouching from mucosal glands

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Tracheobronchoesophageal Fistula A.CONGENITAL1.Congenital tracheoesophageal fistulaB.MALIGNANT1.Lung cancer2.Metastases to mediastinal lymph nodes3.Esophageal cancerOften following radiation treatment of these tumors! C.TRAUMATIC1.Instrumentation (esophagoscopy, bougienage, pneumatic dilatation)2.Blunt ("crush injury") / penetrating chest trauma3.Surgery4.Foreign-body perforation5.Corrosives6.Postemetic rupture = Boerhaave syndromeD.INFECTIOUS / INFLAMMATORY1.TB, syphilis, histoplasmosis, actinomycosis, Crohn disease2.Perforated diverticulum3.Pulmonary sequestration / cyst Notes:


Long Smooth Esophageal Narrowing 1.Congenital esophageal stenosis at junction between middle + distal third weblike / tubular stenosis of 1 cm in length2.Surgical repair of esophageal atresia interruption of primary peristaltic wave at anastomosis secondary contractions may produce retrograde flow with aspiration impaction of food3.Caustic burns = alkaline burns4.Alendronate (= inhibitor of osteoclastic activity)5.Gastric acid: reflux, hyperemesis gravidarum6.Intubation: reflux + compromise of circulation7.Radiotherapy for esophageal carcinoma; tumor of lung, breast, or thymus; lymphoma; metastases to mediastinal lymph nodesOnset of stricture:usually 4-8 months post RxDose:3000-5000 rad8.Postinfectious: moniliasis (rare) Lower Esophageal Narrowing mnemonic:"SPADE"Scleroderma Presbyesophagus Achalasia; Anticholinergics Diffuse esophageal spasm Esophagitis

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Focal Esophageal Narrowing 1.Web =1- to 2-mm thick (vertical length) area of complete / incomplete circumferential narrowing2.Ring =5- to 10-mm thick (vertical length) area of complete / incomplete circumferential narrowing3.Stricture =>10 mm in vertical lengthmnemonic:"LETTERS MC"Lye ingestion Esophagitis Tumor Tube (prolonged nasogastric intubation) Epidermolysis bullosa Radiation Surgery, Scleroderma Moniliasis Congenital

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Esophageal Filling Defect A.BENIGN TUMORS duodenal location; frequently multiple + deep ulcers; commonly associated with erosions bleeding (in 1/3)4.Curling ulcer (burn) (in 0.09-2.6%)5.Retained gastric antrumB.INFLAMMATION1.Peptic ulcer disease2.Gastritis3.Radiation-induced ulcerC.BENIGN MASS1.Leiomyoma2.Granulomatous disease3.Pseudolymphoma (lymphoid hyperplasia)D.MALIGNANT MASS1.Gastric carcinoma2.Lymphoma (2% of all gastric neoplasms) multiple ulcers with aneurysmal appearance3.Leiomyosarcoma, neurogenic sarcoma, fibrosarcoma, liposarcoma4.Metastases (a)hematogenic: malignant melanoma, breast cancer, lung cancer(b)per continuum: pancreas, colon, kidneyE.DRUGSASA: greater curvature Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : STOMACH

Bulls-eye Lesions A.PRIMARY NEOPLASMS1.Leiomyoma, leiomyosarcoma2.Lymphoma3.Carcinoid4.Primary carcinomaB.HEMATOGENOUS METASTASES1.Malignant melanoma usually spares large bowel2.Breast cancer (15%) scirrhous appearance in stomach3.Cancer of lung4.Renal cell carcinoma5.Kaposi sarcoma6.Bladder carcinomaC.ECTOPIC PANCREASin duodenum / stomach D.EOSINOPHILIC GRANULOMAmost frequently in stomach

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Complications Of Postoperative Stomach 1.Filling defect of gastric remnant2.Retained gastric antrum3.Dumping syndrome4.Afferent loop syndrome5.Stomal obstruction(a)temporary reversible: edema of suture line, abscess / hematoma, potassium deficiency, inadequate electrolyte replacement, hypoproteinemia, hypoacidity(b)late mechanical: stomal ulcer (75%)mnemonic:"LOBULATING"Leaks (early) Obstruction (early) Bezoar Ulcer (especially marginal) Loop (afferent loop syndrome) Anemia (macrocytic secondary to decreased intrinsic factor) Tumor (? increased incidence) Intussusception Not feeling well after meals (dumping syndrome) Gastritis (bile reflux)

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Lesions Involving Stomach And Duodenum 1.Lymphoma: in lung > breast > choriocarcinoma > kidney > stomach, uterus, ovary, pancreasD.INTRALUMINAL1.Gallstones2.Foreign bodies, food particles, seeds, pills3.Parasites: ascariasis, strongyloidiasis, hookworm, tapeworm Sandlike Lucencies Of Small Bowel 1.Waldenström macroglobulinemia2.Mastocytosis3.Histoplasmosis4.Nodular lymphoid hyperplasia5.Intestinal lymphangiectasia6.Eosinophilic gastroenteritis7.Lymphoma8.Crohn disease9.Whipple disease10.Yersinia enterocolitis11.Cronkhite-Canada syndrome12.Cystic fibrosis13.Food particles / gas bubbles14.Strongyloides stercoralis Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : SMALL BOWEL

Small Bowel Tumors Incidence: 1:100,000; 1.5-6% of all GI neoplasmsMalignant:benign = 1:1 Symptomatic malignant:symptomatic benign = 3:1 Location of small bowel primaries: ileum (41%), jejunum (36%), duodenum (18%) ROENTGENOGRAPHIC APPEARANCE: (1)pedunculated intraluminal tumor, usually originating from mucosa smooth / irregular surface without visible mucosal pattern moves within intestinal lumen twice the length of the stalk(2)sessile intraluminal tumor without stalk, usually from tissues outside mucosa smooth / irregular surface without visible mucosal pattern(3)intra- / extramural tumor base of tumor greater than any part projecting into the lumen mucosal pattern visible, may be stretched(4)serosal tumor displacement of adjacent loops small bowel obstruction (rare) coil-spring pattern of intussusceptumCT:small bowel wall >1.5 cm thickCx:small-bowel obstruction (in up to 10%) Benign Small Bowel Tumors asymptomatic (80%) melena, pain, weakness palpable abdominal mass (20%)Types: 1.Leiomyoma (36-49%)Location:any segment2.Lipoma (14-16%)Location:duodenum (32%), jejunum (17%), ileum (51%) fat-density on CT3.Adenoma (15-20%)4.Hemangioma (13-16%)5.Lymphangioma (5%)Location: duodenum > jejunum > ileum6.Neurogenic tumor (1%) Malignant Small Bowel Tumors At risk:Crohn disease, celiac disease, polyposis syndromes, history of small-bowel diverting surgery asymptomatic (10-30%) pain due to intermittent obstruction (80%) weight loss (66%) gastrointestinal blood loss (50%) palpable abdominal mass (50%)1.Carcinoid (25-41%)Location:predominantly distal ileum calcified mesenteric mass on CT2.Adenocarcinoma (25-26%)Location:duodenum (48%), jejunum (44%), ileum (8%)3.Lymphoma (16-17%) aneurysmal dilatation4.Gastrointestinal stromal tumor (GIST)= leiomyosarcoma (9-10%) Location:ileum (50%)5.Vascular malignancy (1%)6.Fibrosarcoma (0.3%)7.Metastatic tumor Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : CECUM

Ileocecal Valve Abnormalities A.Lipomatosis: >40 years of age, female stellate / rosette patternB.NEOPLASM1.Lipoma, adenomatous polyp, villous adenoma2.Carcinoid tumor3.Adenocarcinoma: 2% of all colonic cancers4.Lymphoma: often involving terminal ileumC.INFLAMMATION1.Crohn disease2.Ulcerative colitis patulous valve, fixed in open position3.Tuberculosis4.Amebiasis terminal ileum not involved (in United States)5.Typhoid fever, anisakiasis, schistosomiasis, actinomycosis6.Cathartic abuseD.PROLAPSE(a)antegrade:indistinguishable from lipomatosis / prolapsing mucosa / neoplasm(b)retrogradeE.INTUSSUSCEPTIONF.LYMPHOID HYPERPLASIA Notes:


Coned Cecum A.INFLAMMATION1.Crohn disease involvement of ascending colon + terminal ileum2.Ulcerative colitis backwash ileitis (in 10%) gaping ileocecal valve3.Appendicitis4.Typhlitis5.Perforated cecal diverticulumB.INFECTION1.Tuberculosis colonic involvement more prominent than that of terminal ileum2.Amebiasis involvement of cecum in 90% of amebiasis thickened ileocecal valve fixed in open position reflux into normal terminal ileum skip lesions in colon3.Actinomycosis palpable abdominal mass indolent sinus tracts in abdominal wall4.Blastomycosis5.Anisakiasis from ingestion of raw fish with ascaris-like nematode 6.Typhoid, YersiniaC.TUMOR1.Carcinoma of the cecum2.Metastasis to cecum Notes:


Cecal Filling Defect A.ABNORMALITIES OF THE APPENDIX1.Acute appendicitis / appendiceal abscess2.Crohn disease3.Inverted appendiceal stump / appendiceal intussusception4.Mucocele5.Myxoglobulosis6.Appendiceal neoplasm: carcinoid tumor (90%), leiomyoma, neuroma, lipoma, adenocarcinoma, metastasisB.COLONIC LESION1.Ameboma2.Primary cecal neoplasm3.Ileocolic intussusception4.Lipomatosis of ileocecal valveC.UNUSUAL ABNORMALITIES1.Ileocecal diverticulitis (in 50% < age 30 years)2.Solitary benign ulcer of the cecum3.Adherent fecolith (eg, in cystic fibrosis)4.Endometriosis5.Burkitt lymphoma mnemonic:"CECUM TIPSALE"Carcinoma Enteritis Carcinoid Ulcerative colitis Mucocele of appendix Tuberculosis Intussusception Periappendiceal abscess Stump of the appendix Ameboma Lymphoma Endometriosis Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : COLON

Colon Cutoff Sign =abrupt cutoff of gas column at splenic flexure1.Acute pancreatitis (inflammatory exudate along transverse mesocolon)2.Colonic obstruction3.Mesenteric thrombosis4.Ischemic colitis Notes:


Colonic Thumbprinting =sharply defined fingerlike marginal indentations at contours of wall1.ISCHEMIA = Ischemic colitisocclusive vascular disease, hypercoagulability state, hemorrhage into bowel wall (bleeding diathesis, anticoagulants), traumatic intramural hematoma 2.INFLAMMATIONulcerative colitis, Crohn colitis 3.INFECTIONacute amebiasis, schistosomiasis, strongyloidiasis, cytomegalovirus (in renal transplant recipients), pseudomembranous colitis 4.MALIGNANT LESIONSlocalized primary lymphoma, hematogenous metastases 5.MISCELLANEOUSendometriosis, amyloidosis, pneumatosis intestinalis, diverticulosis, diverticulitis, hereditary angioneurotic edema mnemonic:"PSALM II"Pseudomembranous colitis Schistosomiasis Amebic colitis Lymphoma Metastases (to colon) Ischemic colitis Inflammatory bowel disease Notes:


Colonic Urticaria Pattern A.OBSTRUCTION1.Obstructing carcinoma2.Cecal volvulus3.Colonic ileusB.ISCHEMIAC.INFECTION / INFLAMMATION1.Yersinia enterocolitis2.Herpes3.Crohn diseaseD.URTICARIA Notes:


Colonic Ulcers A.IDIOPATHIC1.Ulcerative colitis2.Crohn colitisB.ISCHEMIC1.Ischemic colitisC.TRAUMATIC1.Radiation injury2.Caustic colitisD.NEOPLASTIC1.Primary colonic carcinoma2.Metastases (prostate, stomach, lymphoma, leukemia)E.INFLAMMATORY1.Pseudomembranous colitis2.Pancreatitis3.Diverticulitis4.Behçet syndrome5.Solitary rectal ulcer syndrome6.Nonspecific benign ulcerationF.INFECTION(a)protozoan1.Amebiasis2.Schistosomiasis3.Strongyloidiasis(b)bacterial1.Shigellosis, salmonellosis2.Staphylococcal colitis3.Tuberculosis4.Gonorrheal proctitis5.Yersinia colitis6.Campylobacter fetus colitis(c)fungalhistoplasmosis, mucormycosis, actinomycosis, candidiasis (d)viral1.Lymphogranuloma venereum2.Herpes proctocolitis3.Cytomegalovirus (transplants) Aphthous Ulcers 1.Crohn disease2.Amebic colitis3.Yersinia enterocolitis Organism:Gram-negative fever, diarrhea, RLQ painLocation:terminal ileum thickened folds + ulceration lymphoid nodular hyperplasia4.Salmonella, shigella infection5.Herpes virus infection6.Behçet syndrome7.Lymphoma8.Ischemia Notes:


Multiple Bulls-eye Lesions Of Bowel Wall mnemonic:"MaCK CLaN"Melanoma and Carcinoma Kaposi sarcoma Carcinoid Lymphoma and Neurofibromatosis Notes:


Double-tracking Of Colon =longitudinal extraluminal tracks paralleling the colon1.Diverticulitis: generally 3-6 cm in length2.Crohn disease: generally >10 cm3.Primary carcinoma: wider + more irregular Notes:


Colonic Narrowing A.CHRONIC STAGE OF ANY ULCERATING COLITIS(a)inflammatory:ulcerative colitis, Crohn colitis, solitary rectal ulcer syndrome, nonspecific benign ulcer (b)infectious:amebiasis, schistosomiasis, bacillary dysentery, TB, fungal disease, lymphogranuloma venereum, herpes zoster, cytomegalovirus, strongyloides (c)ischemic: ischemic colitis(d)traumatic:radiation injury, cathartic colon, caustic colitis B.MALIGNANT LESION(a)primary: colonic carcinoma (annular / scirrhous); complication of ulcerative colitis + Crohn colitis(b)metastatic:from prostate, cervix, uterus, kidney, stomach, pancreas, primary intraperitoneal sarcoma -hematogenous (eg, breast)-lymphangitic spread-peritoneal seedingC.EXTRINSIC PROCESS(a)inflammation:retractile mesenteritis, diverticulitis, pancreatitis (b)deposits:amyloidosis, endometriosis, pelvic lipomatosis D.POSTSURGICALadhesive bands, surgical anastomosis E.NORMALCannon point Localized Colonic Narrowing mnemonic:"SCARED CELL-MATE"Schistosomiasis Carcinoid Actinomycosis Radiation Endometriosis Diverticulitis Colitis Extrinsic lesion Lymphoma Lymphogranuloma venereum Metastasis Adenocarcinoma Tuberculosis Entamoeba histolytica Microcolon mnemonic:"MI MCA"Meconium ileus Ileal atresia Megacystis-microcolon-hypoperistalsis syndrome Colonic atresia (distal to atretic segment) Aganglionosis (Hirschsprung disease) Notes:


Colonic Filling Defects Submucosal Tumor 1.Lipoma2.Carcinoid3.Leiomyoma4.Lymphangioma, hemangioma Single Colonic Filling Defect A.BENIGN TUMOR1.Polyp(hyperplastic, adenomatous, villous adenoma, villoglandular); most common benign tumor 2.LipomaMost common intramural tumor; 2nd most common benign tumor; M < F Location:ascending colon + cecum > left side of colon3.Carcinoid: 10% metastasize4.Spindle cell tumor(leiomyoma, fibroma, neurofibroma); 4th most common benign tumor; rectum > cecum 5.Lymphangioma, hemangiomaB.MALIGNANT TUMOR(a)primary tumor:carcinoma, sarcoma (b)secondary tumor:metastases (breast, stomach, lung, pancreas, kidney, female genital tract), lymphoma, invasion by adjacent tumors C.INFECTION1.Ameboma2.Polypoid granuloma: schistosomiasis, TBD.INFLAMMATION1.Inflammatory pseudopolyp: ulcerative colitis, Crohn disease2.Periappendiceal abscess3.Diverticulitis4.Foreign-body perforationE.NONSESSILE INTRALUMINAL BODY1.Fecal impaction2.Foreign body3.Gallstone4.Bolus of Ascaris wormsF.MISCELLANEOUS1.Endometriosis3rd most common benign tumor Location:sigmoid colon, rectosigmoid junction (at level of cul-de-sac) may cause bleeding (after invasion of mucosa)2.Localized amyloid deposition3.Suture granuloma4.Intussusception5.Pseudotumor (adhesions, fibrous bands)6.Colitis cystica profunda Multiple Colonic Filling Defects A.NEOPLASMS(a)polyposis syndrome:familial polyposis, Gardner syndrome, Peutz-Jeghers syndrome, Turcot syndrome, juvenile polyposis syndrome, disseminated gastrointestinal polyps, multiple adenomatous polyps (b)hematogenous metastases:from breast, lung, stomach, ovary, pancreas, uterus (c)multiple tumors-benign:neurofibromatosis, colonic lipomatosis, multiple hamartoma syndrome (Cowden disease) -malignant:lymphoma, leukemia, adenocarcinoma B.INFLAMMATORY PSEUDOPOLYPSulcerative colitis, Crohn colitis, ischemic colitis, amebiasis, schistosomiasis, strongyloidiasis, trichuriasis C.ARTIFACTSfeces, air bubbles, oil bubbles, mucous strands, ingested foreign body (eg, corn kernels) D.MISCELLANEOUSnodular lymphoid hyperplasia, lymphoid follicular pattern, hemorrhoids, diverticula, pneumatosis intestinalis, colitis cystica profunda, colonic urticaria, submucosal colonic edema secondary to obstruction, cystic fibrosis, amyloidosis, ulcerative pseudopolyps, proximal to obstruction mnemonic:"MILL P3 "Metastases (to colon) Ischemia (thumbprinting) Lymphoma Lymphoid hyperplasia Polyposis Pseudopolyposis (with inflammatory bowel disease); Pneumatosis cystoides Carpet Lesions Of Colon =flat lobulated lesions with alteration of surface texture + little / no protrusion into lumenLocation:rectum > cecum > ascending colonCause: A.NEOPLASMS1.Tubular / tubulovillous / villous adenoma2.Familial polyposis3.Adenocarcinoma4.Submucosal tumor spread (from adjacent carcinoma)B.MISCELLANEOUS1.Nonspecific follicular proctitis2.Biopsy site3.Endometriosis4.Rectal varices5.Colonic urticaria

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Colonic Polyp Terminology: 1.Polyp =mass projecting into the lumen of a hollow viscus above the level of the mucosa; usually arises from mucosa, may derive from submucosa / muscularis propria(a)neoplastic: adenoma / carcinoma(b)nonneoplastic: hamartoma / inflammatory polyp2.Pseudopolyp =scattered island of inflamed edematous mucosa on a background of denuded mucosa(a)pseudopolyposis of ulcerative colitis(b)"cobblestoning" of Crohn disease3.Postinflammatory (filiform) polyp =fingerlike projection of submucosa covered by mucosa on all sides following healing + regeneration of inflammatory (most common in ulcerative colitis) / ischemic / infectious bowel disease Histologic classification: A.ADENOMATOUS POLYPS=Familial adenomatous polyposis syndrome 1.Familial polyposis2.Gardner syndrome3.Turcot syndromeB.HAMARTOMATOUS POLYPS=HAMARTOMATOUS POLYPOSIS SYNDROMES1.Peutz-Jeghers syndrome (most in small bowel)2.Cowden disease3.Juvenile polyposis4.Cronkhite-Canada syndrome5.Bannayan-Riley-Ruvalcaba syndromeC.POLYPOSIS LOOK-ALIKES1.Inflammatory polyposis2.Lymphoid hyperplasia3.Lymphoma4.Metastases5.Pneumatosis coli Polyposis Syndromes =more than 100 polyps in numberMode of transmission: A.HEREDITARY(a)autosomal dominant1.Familial (multiple) polyposis2.Gardner syndrome3.Peutz-Jeghers syndrome(b)autosomal recessive1.Turcot syndromeB.NONHEREDITARY1.Cronkhite-Canada syndrome2.Juvenile polyposis

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : RECTUM and ANUS

Rectal Narrowing 1.Pelvic lipomatosis + fibrolipomatosis2.Lymphogranuloma venereum3.Radiation injury of rectum4.Chronic ulcerative colitis

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Enlarged Presacral Space Normal width 10 mm A.RECTAL INFLAMMATION / INFECTIONulcerative colitis, Crohn colitis, idiopathic proctosigmoiditis, radiation therapy B.RECTAL INFECTION1.Proctitis (TB, amebiasis, lymphogranuloma venereum, radiation, ischemia)2.DiverticulitisC.BENIGN RECTAL TUMOR1.Developmental cyst (dermoid, enteric cyst, tailgut cyst)2.Lipoma, neurofibroma, hemangioendothelioma3.Epidermal cyst4.Rectal duplicationD.MALIGNANT RECTAL TUMOR1.Adenocarcinoma, cloacogenic carcinoma2.Lymphoma, sarcoma, lymph node metastases3.Prostatic carcinoma, bladder tumors, cervical cancer, ovarian cancerE.BODY FLUIDS / DEPOSITS1.Hematoma: surgery, sacral fracture2.Pus: perforated appendix, presacral abscess3.Serum: edema, venous thrombosis4.Deposit of fat: pelvic lipomatosis, Cushing disease5.Deposit of amyloid: amyloidosisF.SACRAL TUMOR1.Sacrococcygeal teratoma, anterior sacral meningocele2.Chordoma, metastasis to sacrumG.MISCELLANEOUS1.Inguinal hernia containing segment of colon2.Colitis cystica profunda3.Pelvic lipomatosis Notes:


Lesions Of Ischiorectal Fossa A.Congenital and developmental anomalies1.Gartner duct cyst2.Klippel-Trénaunay syndrome3.Tailgut cystB.Inflammatory and hemorrhagic lesions1.Fistula in ano2.Ischiorectal / perirectal abscess3.Extraperitoneal pelvic hematoma4.Rectal perforationC.Secondary neoplasmper direct extension / hematogenous spread: anorectal / prostatic / pelvic / sacral tumor; lung cancer; melanoma; lymphoma D.Primary neoplasm1.Aggressive angiomyxoma2.Lipoma3.Plexiform neurofibroma4.Anal adenocarcinoma5.Squamous cell carcinoma

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : PERITONEUM

Peritoneal Mass A.SOLID MASS1.Peritoneal mesothelioma2.Peritoneal carcinomatosisB.INFILTRATIVE PATTERN1.Peritoneal mesotheliomaC.CYSTIC MASS1.Cystic mesothelioma2.Pseudomyxoma peritonei3.Bacterial / mycobacterial infection Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : MESENTERY & OMENTUM

Omental Mass 33% of primary omental tumors are malignant! Secondary neoplasms are more frequent than primary!A.SOLID MASS(a)benign: leiomyoma, lipoma, neurofibroma(b)malignant: leiomyosarcoma, liposarcoma, fibrosarcoma, lymphoma, peritoneal mesothelioma, hemangiopericytoma, metastases(c)Infection: tuberculosisB.CYSTIC MASShematoma

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Mesenteric Mass A.ROUND SOLID MASSES Benign primary tumors are more common than malignant primary tumors! Secondary neoplasms are more frequent than primary! Cystic are more common than solid tumors! Malignant solid tumors have a tendency to be located near root of mesentery, benign solid tumors in periphery near bowel!1.Metastases especially from colon, ovary (most frequent neoplasm of mesentery)2.Lymphoma3.Leiomyosarcoma (more frequent than leiomyoma)4.Neural tumor (neurofibroma, ganglioneuroma)5.Lipoma (uncommon), lipomatosis, liposarcoma6.Fibrous histiocytoma7.Hemangioma8.Desmoid tumor (most common primary)B.ILL-DEFINED MASSESmetastases (ovary), lymphoma, fibromatosis, fibrosing mesenteritis (associated with Gardner syndrome), lipodystrophy, mesenteric panniculitis C.STELLATE MASSESperitoneal mesothelioma, retractile mesenteritis, fibrotic reaction of carcinoid, radiation therapy, desmoid tumor, Hodgkin disease, tuberculous peritonitis, ovarian metastases, diverticulitis, pancreatitis A mesenteric mass with calcifications suggests carcinoid tumor !D.LOCULATED CYSTIC MASSES (2/3)cystic lymphangioma (most common), pseudomyxoma peritonei, cystic mesothelioma, mesenteric cyst, mesenteric hematoma, benign cystic teratoma, cystic spindle cell tumor (= centrally necrotic leiomyoma / leiomyosarcoma)

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Mesenteric / Omental Cysts ="BUBBLES OF THE BELLY" The first step is to determine the organ of origin!1.Lymphangioma2.Nonpancreatic pseudocyst=sequelae of mesenteric / omental hematoma / abscessPath:thick-walled, usually septated cystic mass with hemorrhagic / purulent contents3.Duplication cyst4.Mesothelial cyst5.Enteric cyst Notes:


Umbilical Tumor A.PRIMARY (38%)benign / malignant neoplasm, skin tumor B.METASTASES (30%)="Sister Joseph nodule" firm painful nodule ± ulceration with serosanguinous / purulent dischargeCause:gastrointestinal cancer (50%), undetermined (25%), ovarian cancer, pancreatic cancer, small cell carcinoma of lung (very rare)Spread: (a)direct extension from anterior peritoneal surface(b)extension along embryonic remnants: falciform, median umbilical, omphalomesenteric ligaments(c)hematogenous(d)retrograde lymphatic flow from inguinal, axillary, paraaortic nodes(e)iatrogenic: laparoscopic tract, tract of percutaneous needle biopsyC.NONNEOPLASTIC1.Endometriosis (32%)2.Granuloma3.Incarcerated hernia Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ABDOMINAL LYMPADENOPATHY

Regional Patterns Of Lymphadenopathy @Retrocrural nodesAbnormal size:>6 mmCommon cause:lung carcinoma, mesothelioma, lymphoma@Gastrohepatic ligament nodes=superior portion of lesser omentum suspending stomach from liverAbnormal size:>8 mmCommon cause:carcinoma of lesser curvature of stomach, distal esophagus, lymphoma, pancreatic cancer, melanoma, colon + breast cancerDDx:coronary varices@Porta hepatis nodes=in porta hepatis extending down hepatoduodenal ligament, anterior + posterior to portal veinAbnormal size:>6 mmCommon cause:carcinoma of gallbladder + biliary tree, liver, stomach, pancreas, colon, lung, breastCx:high extrahepatic biliary obstruction@Pancreaticoduodenal nodes=between duodenal sweep + pancreatic head anterior to IVCAbnormal size:>10 mmCommon cause:lymphoma, pancreatic head, colon, stomach, lung, breast cancer@Perisplenic nodes=in splenic hilumAbnormal size:>10 mmCommon cause:NHL, leukemia, small bowel neoplasm, ovarian cancer, carcinoma of right / transverse colon@Retroperitoneal nodes=periaortic, pericaval, interaortocavalAbnormal size:>10 mmCommon cause:lymphoma, renal cell, testicular, cervical, prostatic carcinomas@Celiac and superior mesenteric artery nodes=preaortic nodesAbnormal size:>10 mmCommon cause:any intra-abdominal neoplasm@Pelvic nodes=along common, external + internal iliac vesselsAbnormal size:>15 mmCommon cause:carcinoma of bladder, prostate, cervix, uterus, rectum

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ABDOMINAL LYMPADENOPATHY

Enlarged Lymph Node With Low-density Center 1.Tuberculosis, Mycobacterium avium-intracellulare2.Pyogenic infection3.Whipple disease4.Lymphoma5.Metastatic disease after radiation + chemotherapy

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GASTROINTESTINAL HEMORRHAGE Mortality:approx. 10% Barium examination should be avoided in acute bleeders! Source: A.UPPER GASTROINTESTINAL HEMORRHAGE= bleeding site proximal to ligament of Treitz @ Esophagogastric junction 1.Esophageal varices (17%): 50% mortality2.Mallory-Weiss syndrome (7-14%): very low [email protected] hemorrhagic gastritis (17-27%)2.Gastric ulcer (10%)3.Pyloroduodenal ulcer (17-25%)Mortality: 35% if over age 60@Other causes (14%): visceral artery aneurysm, vascular malformation, neoplasm, vascular-enteric fistulaAverage mortality: 8-10% B.LOWER GASTROINTESTINAL HEMORRHAGE@Small intestinetumor (eg, leiomyoma, metastases), ulcers, diverticula (eg, Meckel diverticulum), inflammatory bowel disease (eg, Crohn disease), vascular malformation, visceral artery aneurysm, aortoenteric fistula @Colorectal (70%)1.Diverticula (most common): hemorrhage in 25% of patients with diverticulosis; spontaneous cessation of bleeding in 80%; recurrent bleeding in 25%2.Colonic angiodysplasia = dilated submucosal arteries + veins overlying mucosal thinning (? secondary to mucosal ischemia)3.Colitis, tumors, mesenteric varices INFANTILE GASTROINTESTINAL BLEEDING (1)Peptic ulcer(2)Varices(3)Ulcerated Meckel diverticulum Intramural Hemorrhage Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : GASTROINTESTINAL HEMORRHAGE

Intramural Hemorrhage A.VASCULITIS1.Henoch-Schönlein purpuraB.TRAUMAC.COAGULATION DEFECT1.Anticoagulant therapy2.Thrombocytopenia3.Disseminated intravascular coagulationD.DISEASES WITH COAGULATION DEFECT1.Hemophilia2.Leukemia, lymphoma3.Multiple myeloma4.Metastatic carcinoma5.Idiopathic thrombocytopenic purpuraE.ISCHEMIA (often fatal) abdominal pain melenaSite:submucosal / intramural / mesenteric "stacked coin" / "picket fence" appearance of mucosal folds (due to symmetric infiltration of submucosal blood) "thumbprinting" = rounded polypoid filling defect (due to focal accumulation of hematoma in bowel wall) separation + uncoiling of bowel loops narrowing of lumen + localized filling defects (asymmetric hematoma) no spasm / irritability mechanical obstruction + proximal distension of loopsPrognosis:resolution within 2-6 weeks Notes:


GI ABNORMALITIES IN CHRONIC RENAL FAILURE AND RENAL TRANSPLANTATION @Esophagus1.Esophagitis: candida, CMV, herpes@Stomach & duodenum1.Gastritis thickened gastric folds (38%) edema + erosionsCause: (a)imbalance of gastrin levels + gastric acid secretion due to (1) reduced removal of gastrin from kidney with loss of cortical mass (2) impaired acid feedback mechanism (3) hypochlorhydria(b)opportunistic infection (eg, CMV)2.Gastric ulcer (3.5%)3.Duodenal ulcer (2.4%)4.Duodenitis (47%)@ColonMore severely + frequently affected after renal transplantation 1.Progressive distention + pseudoobstructionContributing factors: dehydration, alteration of diet, inactivity, nonabsorbable antacids, high-dose steroids 2.Ischemic colitis(a)primary disease responsible for end-stage renal disease (eg, diabetes, vasculitis)(b)trauma of renal transplantation3.DiverticulitisContributing factors: chronic obstipation, steroids, autonomic nervous dysfunction 4.Pseudomembranous colitis5.Uremic colitis = nonspecific colitis6.Spontaneous colonic perforationCause:nonocclusive ischemia, diverticula, duodenal + gastric [email protected]:hypercalcemia, steroids, infection, immunosuppressive agents, [email protected] hemorrhageCause:gastritis, ulcers, colonic diverticula, ischemic bowel, infectious colitis, pseudomembranous colitis, nonspecific cecal ulceration2.Bowel perforation (in 1-4% of transplant recipients)3.Opportunistic infectionOrganism: Candida, herpes, CMV, strongyloides 4.Malignancy(a) skin tumors (b) lymphoma

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Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ENTEROPATHY

Protein-losing Enteropathy A.DISEASE WITH MUCOSAL ULCERATION1.Carcinoma2.Lymphoma3.Inflammatory bowel disease4.Peptic ulcer diseaseB.HYPERTROPHIED GASTRIC RUGAE1.Ménétrièr diseaseC.NONULCERATIVE MUCOSAL DISEASE1.Celiac disease2.Tropical sprue3.Whipple disease4.Allergic gastroenteropathy5.Gastrocolic fistula6.Villous adenoma of colonD.LYMPHATIC OBSTRUCTION1.Intestinal lymphangiectasiaE.HEART DISEASE1.Constrictive pericarditis2.Tricuspid insufficiency Notes:

Home : GASTROINTESTINAL TRACT : Differential diagnosis of gastrointestinal disorders : ENTEROPATHY

Malabsorption =deficient absorption of any essential food materials within small bowel(1)PRIMARY MALABSORPTION=the digestive abnormality is the only abnormality present1.Celiac disease = nontropical sprue2.Tropical sprue3.Disaccharidase deficiencies(2)SECONDARY MALABSORPTION=occurring during course of gastrointestinal disease(a)enteric1.Whipple disease2.Parasites: hookworm, Giardia, fish tapeworm3.Mechanical defects: fistulas, blind loops, adhesions, volvulus, short circuits4.Neurologic: diabetes, functional diarrhea5.Inflammatory: enteritis (viral, bacterial, fungal, nonspecific)6.Endocrine: Zollinger-Ellison syndrome7.Drugs: neomycin, phenindione, cathartics8.Collagen disease: scleroderma, lupus, polyarteritis9.Lymphoma10.Benign + malignant small bowel tumors11.Vascular disease12.CHF, agammaglobulinemia, amyloid, abetalipoproteinemia, intestinal lymphangiectasia(b)gastricvagotomy, gastrectomy, pyloroplasty, gastric fistula (to jejunum, ileum, colon) (c)pancreaticpancreatitis, pancreatectomy, pancreatic cancer, cystic fibrosis (d)hepatobiliaryintra- and extrahepatic biliary obstruction, acute + chronic liver disease Roentgenographic Signs In Malabsorption SMALL BOWEL WITH NORMAL FOLDS + FLUID1.Maldigestion (deficiency of bile salt / pancreatic enzymes)2.Gastric surgery3.Alactasia SMALL BOWEL WITH NORMAL FOLDS + WET1.Sprue2.Dermatitis herpetiformis DILATED DRY SMALL BOWEL1.Scleroderma2.Dermatomyositis3.Pseudoobstruction: no peristaltic activity DILATED WET SMALL BOWEL1.Sprue2.Obstruction3.Blind loop THICKENED STRAIGHT FOLDS + DRY SMALL BOWEL1.Amyloidosis (malabsorption is unusual)2.Radiation3.Ischemia4.Lymphoma (rare)5.Macroglobulinemia (rare) THICKENED STRAIGHT FOLDS + WET SMALL BOWEL1.Zollinger-Ellison syndrome2.Abetalipoproteinemia: rare inherited disease characterized by CNS damage, retinal abnormalities, steatorrhea, acanthocytosis THICKENED NODULAR IRREGULAR FOLDS + DRY SMALL BOWEL 1.Lymphoid hyperplasia2.Lymphoma3.Crohn disease4.Whipple disease5.Mastocytosis THICKENED NODULAR IRREGULAR FOLDS + WET SMALL BOWEL1.Lymphangiectasia2.Giardiasis3.Whipple disease (rare) Small Bowel Nodularity With Malabsorption mnemonic:"What Is His Main Aim? Lay Eggs, By God"Whipple disease Intestinal lymphangiectasia Histiocytosis Mastocytosis Amyloidosis Lymphoma, Lymph node hyperplasia Edema Blood Giardiasis

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Home : GASTROINTESTINAL TRACT : Anatomy and function of gastrointestinal tract : GASTROINTESTINAL HORMONES

Cholecystokinin =CCK = 33 amino acid residues (former name: Pancreozymin); the 5 C-terminal amino acids are identical to those of gastrin, causing similar effects as gastrinProduced in: duodenal + upper intestinal mucosaReleased by:fatty acids, some amino acids (phenylalanine, methionine), hydrogen ionsEffects: @Stomach(1)weakly stimulates HCl secretion(2)given alone:inhibits gastrin, which leads to decrease in HCl production(3)stimulates pepsin secretion(4)stimulates gastric motility@Pancreas(1)stimulates secretion of pancreatic enzymes(= Pancreozymin) (2)stimulates bicarbonate secretion (weakly by direct effect; strongly through potentiating effect on secretin)(3)stimulates insulin release@Liver(1)stimulates water + bicarbonate secretion@Intestine(1)stimulates secretion of Brunner glands(2)increases motility@Biliary tract(1)strong stimulator of gallbladder contraction(2)relaxation of sphincter of Oddi Notes:


Gastrin =17 amino acid peptide amide;PentagastrinPENTAGASTRIN =acyl derivative of the biologic active C-terminal tetrapeptide amideProduced in: antral cells + G-cells of pancreasReleased by: (a)vagal stimulation, gastric distension(b)short-chain alcohol (ethanol, propanol)(c)amino acids (glycine, ß-alanine)(d)caffeine(e)hypercalcemiamediated by neuroendocrine cholinergic reflexes Inhibited by: drop in pH of antral mucosa to jejunum > stomach > colon@Hormones(1)releases catecholamines from the adrenal gland that paralyze intestinal smooth muscle(2)increases serum insulin + glucose levels (mobilization of hepatic glycogen)@Biliary tract(1)increases bile flow(2)relaxes gallbladder + sphincter of Oddi Dose for radiologic imaging:1 mg maximum IV administration causes a quick response + rapid dissipation of action! IM administration prolongs onset + increases length of action!Half-life:3-6 minutesSide effects:nausea + vomiting, weakness, dizziness (delayed onset of 1.5-4 hours after IM administration)Contraindication: (1)hypersensitivity / allergy to glucagon: urticaria, periorbital edema, respiratory distress, hypotension, coronary artery spasm (?), circulatory arrest(2)known hypertensive response to glucagon(3)pheochromocytoma: glucagon stimulates release of catecholamines(4)insulinoma: insulin-releasing effect may result in hypoglycemia(5)glucagonoma(6)poorly controlled diabetes mellitus Notes:


Secretin Produced in: duodenal mucosaReleased by: hydrogen ions providing a pH 2.5 mm [email protected] mm>2.0 mm pathologicB.NORMAL NUMBER OF FOLDS@jejunum4-6 / inch@ileum3-5 / inchC.NORMAL FOLD [email protected] [email protected] mmD.NORMAL LUMEN DIAMETER@upper jejunum3.0-4.0 cm>4.5 cm pathologic@lower jejunum2.5-3.5 cm>4.0 cm [email protected] cm>3.0 cm pathologic RULE OF 3s: wall thickness 2 cm large solitary ulcer in the mid- or distal esophagus (HIV-infected cells cause alterations in cytokines resulting in infiltration of inflammatory cells into submucosa + destruction of mucosa)Rx:corticosteroids B.FUNGAL PATHOGENS1.Candidiasis The absence of thrush does not exclude the diagnosis of candidal esophagitis!Organism:commensal fungus Candida albicansPrevalence:10-20% (in United States); up to 80% in developing countriesLocation:oral cavity, esophagus discrete linear / irregular longitudinally oriented filling defects in esophagusCx:disseminated systemic candidiasis (rare + indicative of granulocytopenia from chemotherapy / direct inoculation via catheter)2.Histoplasmosis Organism:dimorphic opportunistic fungusPrevalence:10% GI involvement with disseminated histoplasmosis in AIDS patientsLocation:colon > terminal ileum segmental inflammation / applecore lesion / bowel stricture hepatosplenomegaly mesenteric lymphadenopathy diffuse hypoattenuation of spleen C.PROTOZOAN PATHOGENS1.Cryptosporidiosis One of the most common causes of enteric disease in AIDS patients!Organism:intracellular parasite CryptosporidiumPrevalence:16% (in United States) + up to 48% (in developing countries) in patients with diarrhea severe diarrhea with fluid loss of 10-17 L/dayLocation:jejunum > other small bowel > stomach > colon Cryptosporidium antritis (= area of focal gastric thickening + ulceration) small bowel dilatation (increased secretions) regular fold thickening + effacement (atrophy, blunting, fusion, loss of villi) "toothpaste" appearance of small bowel (mimicking sprue) dilution of barium (hypersecretion) marked antral narrowing (extensive inflammation)Dx:microscopic identification in stool / biopsy2.Pneumocystosis Likely to occur inpatients treated with aerosolized pentamidine!Organism:eukaryotic microbe Pneumocystis cariniiPrevalence:pulmonary infection in 75% of AIDS patients; in 80%), breast, tongue, GI tract, connective tissue small amyloid deposits3.Amyloidosis associated with multiple myeloma may precede development of multiple myelomaIncidence:10-15% primary amyloidosis with osteolytic lesions in myelomatous disease4.Tumor-forming / organ-limited amyloidosis related to primary type(a)hereditary = familial amyloidosis(b)senile amyloidosis (limited to heart / brain / pancreas / spleen) large localized masses GI involvement in primary more common than in secondary amyloidosis! malabsorption (diarrhea, protein loss) occult GI bleeding obstruction macroglossia@Esophagus (11%) loss of peristalsis megaesophagus@Stomach (37%) postprandial epigastric pain + heartburn acute erosive hemorrhagic gastritis(a)diffuse infiltrative form small-sized stomach with rigidity + loss of distensibility simulating linitis plastica (from thickening of gastric wall) effaced rugal pattern diminished / absent peristalsis marked retention of food(b)localized infiltration (often located in antrum) irregularly narrowed + rigid antrum thickened rugae superficial erosions / ulcerations(c)amyloidoma = well-defined submucosal mass@Small bowel (74%)(a)diffuse form (more common) diffuse uniform thickening of valvulae conniventes in entire small bowel broadened flat undulated mucosal folds (mucosal atrophy) "jejunalization" of ileum impaired intestinal motility small bowel dilatation(b)localized form (less common) multiple pea- / marble-sized deposits pseudoobstruction = physical + plain-film findings suggesting mechanical obstruction with patent large + small bowel on barium examination (involvement of myenteric plexus)Cx:small bowel infarction@Colon (27%): pseudopolyps in colon@ Bone: bone cysts@Spleen:Histo:(a)nodular form involving lymph follicles(b)diffuse form infiltrating red pulp discrete masses splenomegaly (4-13%)Cx:spontaneous splenic rupture (from vascular fragility + acquired coagulopathy)Dx:by rectal / gingival biopsyDDx:Whipple disease, intestinal lymphangiectasia, lymphosarcoma Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders

ANGIODYSPLASIA OF COLON =VASCULAR ECTASIA = ARTERIOVENOUS MALFORMATIONCause:?; acquired lesionAssociated with:aortic stenosis (20%) Incidence at autopsy:2%Age:majority >55 yearsLocation:(a)cecum + ascending colon (majority)(b)descending + sigmoid colon (25%) chronic intermittent low-grade bleeding occasionally massive bleeding "vascular tufts" = cluster of vessels during arterial phase along antimesenteric border early opacification of ileocolic vein densely opacified dilated tortuous ileocolic vein into late venous phase contrast extravasation (unusual) Notes:


ANISAKIASIS =parasitic disease of GI tractCause:ingestion of Anisakis larvae present in raw / undercooked fish (mackerel, cod, pollack, herring, whiting, bonito, squid) consumed as sashimi, sushi, ceviche, lomi-lomiOrganism:worm with straight / serpentine / circular threadlike appearance Site of penetration by larvae determines clinical form!@Gastric anisakiasis acute gastric pain, nausea, vomiting a few hours after ingestion (DDx: acute gastritis, peptic ulcer, food poisoning, neoplasia) eosinophilia mucosal edema about 3-cm-long threadlike filling defects (= larvae) @Intestinal anisakiasis diffuse abdominal tenderness / colicky abdominal pain, nausea, vomiting (DDx: acute appendicitis, regional enteritis, intussusception, ileus, diverticulitis, neoplasia) leukocytosis without eosinophilia (frequent)Histo:marked edema, eosinophilic infiltrates, granuloma formation thickened folds disappearance of Kerckring folds thumbprinting / saw-tooth appearance irregular luminal narrowing eosinophilic ascites (DDx: eosinophilic gastroenteritis, hypereosinophilic syndrome)Cx:ileus@Colonic anisakiasis (rare)DDx:colonic tumor Notes:


ANORECTAL MALFORMATION (1)Imperforate anus(2)Cloacal malformation(3)Cloacal exstrophyEmbryology: during weeks 3 and 4 the dorsal part of the yolk sac folds are incorporated into embryo forming the primitive hindgut consisting of distal part of transverse + descending + sigmoid colon, rectum, superior portion of anal canal, epithelium of urinary bladder, and most of the urethra; at 4 weeks the transverse rectovesical septum descends caudally between allantois and hindgut dividing the cloaca into urogenital sinus ventrally + anorectal canal dorsally; by 7th week the rectovaginal septum fuses with cloacal membrane creating a urogenital membrane ventrally + anal membrane dorsally; perineum is formed by fusion of rectovesical septum + cloacal membrane; anal membrane ruptures by 9th week In 48% associated with: (part of VACTERL syndrome) (1)GU anomalies (20%):renal agenesis / ectopia, vesicoureteral reflux, obstruction, hypospadia (3.1%); M > F; (2)Lumbosacral segmentation anomalies (30%): dysplasia, agenesis, hemivertebrae(3)GI anomalies (11%):esophageal atresia ± tracheoesophageal fistula (4%), duodenal atresia / stenosis (4)Cardiovascular anomalies (8%)(5)Abdominal wall (2%)(6)Cleft lip-cleft palate (1.6%)(7)Down syndrome (1.5%)(8)Meningomyelocele (0.5%) + occult myelodysplasia(9)Others (8%) Caudal regression syndrome: anorectal atresia, sacral agenesis, renal agenesis / dysplasia, lower limb hypoplasia, sirenomelia Notes:


ANTRAL MUCOSAL DIAPHRAGM = antral web Age range:3 months to 80 yearsAssociated with:gastric ulcer (30-50%) symptomatic if opening 2 mm diffuse hypoechogenicity (associated with higher frequency of perforation) lumen may be distended with anechoic / hyperechoic material loss of wall layers visualization of appendicolith (6%) localized periappendiceal fluid collection prominent hyperechoic mesoappendix / pericecal fatColor Doppler US: increased conspicuity (= increase in size + number) of vessels in and around the appendix = hyperemia decreased resistance of arterial waveforms continuous / pulsatile venous flowCT (87-98% sensitive, 83-97% specific, 93% accurate): abnormal appendix distended lumen circumferentially thickened ± enhancing wall appendicolith = homogeneous / ringlike calcification (25%) periappendicular inflammation linear streaky densities in periappendicular / pericecal / mesenteric / pelvic fat phlegmon = pericecal soft-tissue mass pericecal / mesenteric / pelvic abscess = poorly encapsulated single / multiple fluid collection with air / extravasated contrast material focal cecal apical thickening (80%) "arrowhead" sign = funnel of contrast medium in cecum centering about occluded orifice of appendixCx:perforation (13-30%)DDx:colitis, diverticulitis, epiploic appendagitis, small bowel obstruction, infectious enteritis, duodenal ulcer, pancreatitis, intussusception, Crohn disease, mesenteric lymphadenitis, ovarian torsion, pelvic inflammatory diseaseRx:finding of appendicolith is sufficient evidence to perform prophylactic appendectomy in asymptomatic patients (50% have perforation / abscess formation at surgery) Notes:


ASCARIASIS =most common parasitic infection in world; cosmopolitan occurrence; endemic along Gulf Coast, Ozark Mountains, Nigeria, Southeast AsiaOrganism:Ascaris lumbricoides = roundworm parasite, 15-35 cm in length; production of 200,000 eggs dailyCycle:infection by contaminated soil, eggs hatch in duodenum, larvae penetrate into venules / lymphatics, carried to lungs, migrate to alveoli and up the bronchial tree, swallowed, maturation in jejunum within 2.5 monthsAge:children age 1-10 years colic eosinophilia appendicitis hematemesis / pneumonitis jaundice (if bile ducts infested)Location:jejunum > ileum (99%), duodenum, stomach, CBD, pancreatic duct 15- to 35-cm-long tubular filling defects barium-filled enteric canal outlined within Ascaris whirled appearance, occasionally in coiled clusters ("bolus of worms")Cx:(1)Perforation of bowel(2)Mechanical obstruction Notes:


BANNAYAN-RILEY-RUVALCABA SYNDROME =RUVALCABA-MYHRE-SMITH SYNDROMECause:autosomal dominant transmission pigmented genital lesions hamartomatous intestinal polyps (in 45%): usually in distal ileum + colon macrocephaly subcutaneous and visceral lipomas + hemangiomas Notes:


BARRETT ESOPHAGUS =BARRETT SYNDROME=replacement of stratified squamous epithelium by metaplastic columnar epithelium (Barrett epithelium) containing goblet cellsCause:chronic gastroesophageal reflux with epithelial injury from esophagitisContributing factors: genetic influence, reduced LES pressure, transient LES relaxation, hiatal hernia, delayed acid clearance, reduced acid sensitivity, duodenogastroesophageal reflux, alcohol, tobacco, chemotherapy, scleroderma (37%), S/P repair of esophageal atresia / esophagogastric resection / Heller esophagomyotomy Histo:(1)specialized columnar epithelium (proximal)(2)junctional-type epithelium (distal to above)(3)fundic-type epithelium (most distally)Incidence: in general 0.3-4%; 7-10-20% of patients with symptoms of refluxAssociated with:moderate + severe esophagitis (94%), no / mild esophagitis (6%)Age:0-15 years and 40-88 years (mean of 55 years); M > F; mainly among Whites dysphagia (due to esophageal stricture) heartburn, substernal chest pain, regurgitation low-grade upper intestinal bleeding asymptomatic Location:middle to lower esophagusN.B.:the squamocolumnar junction does not coincide with the GE junction, is irregular and lies >2-3 cm orad from the gastroesophageal junctionDistribution:circumferential / focal several-cm-long stricture (71%) in midesophagus (40%) or lower esophagus (60%; DDx: peptic stricture without Barrett esophagus) large deep wide-mouthed peptic ulcer (= Barrett ulcer) at upwardly displaced squamocolumnar junction / within columnar epithelium fine reticular mucosal pattern (3-30%) located distally from stricture (DDx: gastroesophageal reflux, monilial + viral esophagitis, superficial spreading carcinoma) thickened irregular mucosal folds (28-86%) fine granular mucosal pattern (DDx: reflux esophagitis, acanthosis, leukoplakia, superficial spreading carcinoma, moniliasis / herpes simplex / CMV esophagitis) gastroesophageal reflux (45-63%) distal esophageal widening (34-66%; due to abnormal motility) hiatal hernia (75-94%) uptake of Tc-99m pertechnetate by columnar epithelium Dx:velvety pinkish red appearance of gastric-type mucosa extending from gastric mucosa into distal esophagus (endoscopy with biopsy)Cx:1.Ulceration ± penetration into mediastinum2.Stricture3.Adenocarcinoma (0-10-46%;) 40-fold higher risk than general population plaquelike / focal irregularity / nodularity / sessile polypsRx:(1)stop smoking, avoid bedtime snacks + foods that lower LES pressure, lose excess weight(2)suppress gastric acidity: antacids, H2 -receptor antagonists (cimetidine, ranitidine, famotidine), H+K+-adenosintriphosphatase inhibitor (omeprazole)(3)improve LES pressure: metoclopramide, bethanechol(4)esophageal resection in high-grade dysplasia

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BEHÇET SYNDROME =uncommon chronic multisystem inflammatory disorder of unknown etiology with relapsing course characterized by mucocutaneous-ocular symptoms as a triad of aphthous stomatitis, genital ulcers, ocular inflammationAge at onset:3rd decade; M:F = 2:1Major criteria:buccal + genital ulceration, ocular inflammation, skin lesionsMinor criteria:thrombophlebitis, GI + CNS lesions, arthritis, family history abdominal pain + diarrhea (50%)@ Mucocutaneous:aphthous stomatitis, papules, pustules, vesicles, folliculitis, erythema nodosum-like lesions@ Genital:ulcers on penis + scrotum / vulva + vagina@ Ocular:relapsing iridocyclitis, hypopyon, choroiditis, papillitis, retinal vasculitis@ Articular:mild nondestructive arthritis@ Vascular:migratory thrombophlebitis@ CNS:chronic meningoencephalitis@ Esophagus:ulceration, stenosis, perforation@ Small bowel:ulceration, perforation@ Colon:multiple discrete deep ulcers in normal mucosa (DDx: granulomatous / ulcerative colitis) Intestinal Behçet Disease Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : BEHÇET SYNDROME

Intestinal Behçet Disease =presence of intestinal ulcersIncidence:180 HULocation:paracolic gutters, pelvis "sentinel clot" sign = the highest attenuation value of blood clot marks the anatomic site of visceral injury high-density active arterial extravasation always surrounded by lower-density hematoma(DDx: extravasated oral contrast is not surrounded by lower-density material) Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : BLUNT ABDOMINAL TRAUMA

Hypovolemia

"collapsed cava" sign = persistent flattening of IVC (due to decreased venous return)N.B.: abort CT examination as shock is imminent! small

hypodense spleen (decreased enhancement) small aorta + mesenteric arteries (due to intense vasoconstriction) shock nephrogram = lack of renal contrast excretion "shock bowel" = generalized thickening of small bowel folds + increased enhancement + luminal fluid dilatation (due to vasoconstriction of mesenteric vessels) marked enhancement of adrenal gland

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Blunt Trauma To Spleen The spleen is the most frequently injured solid parenchymal organ within the abdomen!Cause:blunt trauma (most frequent)Associated with:rib fractures (in 40%), left renal injury 20% of patients with left rib fractures have a splenic injury! 25% of patients with left renal injury have a splenic injury!CECT (95% accuracy): mottled parenchymal enhancement = contusion hypoattenuating hematoma complete separation of splenic fragments (= fracture) crescentic region of low attenuation compressing normal parenchyma = subcapsular hematoma round hypodense inhomogeneous region ± hyperdense clot = intrasplenic hematoma hypoattenuating line connecting opposing visceral surfaces + perisplenic fluid = splenic laceration multiple lacerations = "shattered spleen" high-attenuation area = contrast extravasation / pseudoaneurysm hemoperitoneum (= disruption of splenic capsule)Sequelae:splenic pseudocyst (20-30 HU)Cx:delayed rupture up to 10 days laterRx:up to 91% of stable patients can be treated conservatively with observation; transcatheter embolizationDDx:(1)Normal lobulation / splenic cleft (smoothly contoured, medially located)(2)Adjacent unopacified jejunum simulating splenic tissue(3)Early differential enhancement of red and white pulp (scan obtained within 20-50 sec)(4)Perisplenic fluid from ascites / urine / succus / bile / lavage

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Blunt Trauma To Liver (20%) Second most frequently injured intra-abdominal viscusAssociated with:splenic injury in 45%Location:R > L lobeSite:perivascular, paralleling right + middle hepatic arteries + posterior branches of right portal vein, avulsion of right hepatic vein from IVC (13%) Left lobe injury more often associated with damage to duodenum, pancreas, transverse colonCECT: hypoattenuating hematoma lenticular configuration (= subcapsular hematoma) usually resolving within 6-8 weeks irregular linear branching / round regions of low attenuation = laceration focal / diffuse periportal tracking (in up to 22%) due to dissecting hemorrhage / bile / dilated periportal lymphatics (secondary to elevated central venous pressure / injury to lymphatics) alteration in distribution of vessels + ducts hypodense wedge extending to liver surface = focal hepatic devascularization focal hyperdense (80-350 HU) area = active hemorrhage / pseudoaneurysm hemoperitoneum (inability of liver veins to contract) intrahepatic / subcapsular gas (usually due to necrosis)Cx:in up to 20%(1)delayed rupture (rare)(2)hemobilia(3)arteriovenous fistula / pseudoaneurysm(4)biloma ± infection(5)superinfection of hematoma / devascularized hepatic parenchymaRx:conservative treatment in up to 80% in adults + 97% in children; transcatheter embolizationHealing:1-6-15 monthsDDx:(1)beam-hardening artifact from adjacent ribs / from air-contrast level in stomach(2)Focal fatty

infiltration

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Blunt Trauma To Gallbladder (2%) Associated with:injury to liver , duodenum pericholecystic fluid (extraperitoneal location of GB) free intraperitoneal fluidCECT: blurred contour of GB focal thickening / discontinuity of GB wall intraluminal enhancing mucosal flap hyperattenuating blood within GB lumen mass effect on adjacent duodenum collapsed GB = GB rupture focal periportal tracking = GB ruptureUS: focal hypoechoic thickening echogenic mass within GB lumen Notes:


Blunt Trauma To GI Tract (5%) Location:jejunum distal to ligament of Treitz > duodenum > ascending colon at ileocecal valve > descending colonCECT (88-92% sensitive): hypodense free fluid (85%), particularly in interloop location due to perforation focal bowel wall thickening > 3 mm = intramural hematoma (75%) ± intestinal obstruction focal discontinuity of bowel wall sentinel clot sign adjacent to bowel streaky hyperattenuating mesentery mesenteric hematoma (39%) hyperdense contrast enhancement of injured bowel wall = delayed venous transit time (20%) pneumoperitoneum (15-32%) extravasation of oral contrast material + gasN.B.:clinical signs + symptoms may be delayed for 24 hours (increasing mortality to 65%) Notes:


Blunt Trauma To Pancreas (3%) Mechanism:compression against vertebral column with shear across pancreatic neckAssociated with:injury to liver, duodenumClassification: Iminor contusion / hematoma, capsule + major duct intactIIparenchymal injury without major duct injuryIIImajor ductal injuryIVsevere crush injuryLocation:junction of body + tail posttraumatic pancreatitis edema / fluid in peripancreatic fat focal / diffuse pancreatic enlargement irregularity of pancreatic contour area of low-attenuation laceration (actual site of laceration difficult to visualize) fluid around superior mesenteric artery fluid in transverse mesocolon / lesser sac fluid between pancreas and splenic vein thickening of anterior pararenal fasciaN.B.:24-48 hours delayed scans uncover findings not present earlierRx:I + IIconservative management;III + IVneed surgery within 24 hoursCx:recurrent pancreatitis, pseudocyst, pseudoaneurysm, fistula, abscess (attendant mortality of 20%) Notes:


Blunt Trauma To Kidney Incidence:10% of injuries in emergency roomCause:motor vehicle accident, contact sports, falls, fights, assaultsMechanism:direct blow (>80%) often lacerated by lower ribs, acceleration-deceleration (renal artery tear)Associated with:other organ injury in 20% >95% hematuria 25% of patients with gross hematuria have significant injuries! 24% of patients with renal pedicle injury have no hematuria! Only 1-2% with microhematuria ( right side / hydropneumothorax pneumomediastinum (single most important plain-film finding), pneumopericardium, subcutaneous air "V-sign of Naclerio" = localized mediastinal emphysema with air between lower thoracic aorta + diaphragm mediastinal widening air-fluid level within mediastinum extravasation of contrast medium into mediastinum / pleura

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BRUNNER GLAND HYPERPLASIA Etiology:hyperplasia secondary to hyperacidityPhysiology:secrete a clear viscous alkaline substance into crypts of LieberkühnMORPHOLOGIC TYPES: 1.Diffuse nodular hyperplasia2.Circumscribed nodular hyperplasia:in suprapapillary portion 3.Single adenomatous hyperplastic polyp:in duodenal bulb Location:duodenal glands begin in vicinity of pylorus extending distally within proximal 2/3 of duodenum multiple nodular filling defects (usually limited to 1st portion of duodenum "cobblestone appearance" (most common finding) occasionally single large mass ± central ulceration

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BURKITT LYMPHOMA =most common type of non-Hodgkin lymphoma in children; initially described in AfricaEtiology:tumor from undifferentiated B-cell-derived lymphocytes; associated with Epstein-Barr virusAge:children + young adultsPath:resemblance to Hodgkin diseaseHisto:characteristic "starry sky" patternLocation:mandible (first), maxilla; multifocal (10%) jaw mass abdominal mass paraplegia NO peripheral leukemia usually intra-abdominal extranodal involvement with sparing of spleen A.ENDEMIC FORM OF BURKITT LYMPHOMAendemic in areas with malaria: tropical Africa, New Guinea 50% of all childhood cancers in central Africa Age:6-8 years@Mandible / maxilla grossly destructive lesion, spicules of bone growing at right angles large soft-tissue mass@ Other skeleton reminiscent of Ewing tumor / reticulum cell sarcoma lamellated periosteal reaction around major long bones B.NONENDEMIC FORM OF BURKITT LYMPHOMAAge:10-12 yearsLocation:abdominal involvement (69%): tumors of small bowel (terminal ileum), mesentery, retroperitoneum, ovary, uterus, salivary glands, thyroid, kidneys, bone marrow well-defined sharply marginated homogeneous tumors (75%) ascites (13%) renal masses / enlargement (5%) hydronephrosis (28%) conspicuous absence of lymph node disease pleural effusion (most common chest abnormality)Rx:dramatic response to chemotherapyPrognosis:long-term survival in 50%

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CARCINOID =most common primary tumor of small bowel + appendix (>95% of all carcinoids); belongs to APUDomas; M:F = 2:1Path:firm yellow submucosal nodule arising from argentophil Kulchitsky cells in the crypts of Lieberkühn (= argentaffinoma); invasion into mesentery incites an intense fibrotic reactionHisto:low-grade malignancy = resemble adenocarcinomas but do not have their aggressive behavior; malignant through invasion of muscularisBiochemistry: tumor elaborates (1) ACTH (2) histamine (3) bradykinin (4) kallikrein (5) serotonin = 5-hydroxytryptamine (from tryptophan over 5-hydroxytryptophan), which is metabolized in liver by monamine oxidase into 5-hydroxyindole acetic acid (5-HIAA) and excreted in urine; 5-hydroxytryptophan is destroyed in pulmonary circulation asymptomatic (66%) pain / obstruction (19%) weight loss (16%) palpable mass (14%) Carcinoid syndrome (7% of small bowel carcinoids)caused by excess serotonin levels, requires that serotonin metabolism (to 5-HIAA in liver) is bypassed (a)with liver metastases(b)with primary pulmonary / ovarian carcinoids recurrent diarrhea (70%) right-sided endocardial fibroelastosis (35%) resulting in tricuspid regurgitation + pulmonary valve stenosis + right heart failure attacks precipitated by ingestion of food / alcohol asthmatic wheezing from bronchospasm (15%) desquamative skin lesions (5%) nausea & vomiting, fever hypotension cutaneous flushing (rare)Metastases: to lymph nodes, liver (in 90% of patients with carcinoid syndrome), lung, bone (osteoblastic) (a)incidence versus tumor sizetumor of2 cm(in 5%)metastasizes in85%(b)incidence versus locationtumor in ileum(in 28%)metastasizes in35%tumor in appendix(in 46%)metastasizes in3%tumor in rectum(in 17%)metastasizes in1%Liver metastases seen: best /(only) on:(a)NECT35%(3%)(b)CECT in HAP35%(14%)(c)CECT in PVP30%(3%)HAP = hepatic arterial-dominant phase of triple phase CT PVP = portal venous-dominant phase of triple phase CT RULE OF 1/3: 1/3 occur in small bowel 1/3 have metastases 1/3 are multiple 1/3 have a second malignancyLocation:between gastric cardia and anus@Appendix (30-45%)commonly benign; surgical incidence of 0.03-0.7% Site:tip (70%), middle (20%), base (10%) of appendix@Small bowel (25-35%)Location:ileum (91%); jejunum (7%), duodenum (2%); multiple in 15-35%@Rectum (10-15%): metastasize in 10%@Colon (5%): ascending colon, often malignant@Stomach (rare)@Other organs (5%): bronchus, thyroid, pancreas, biliary tract, teratomas (ovarian, sacrococcygeal, testicular)@may be multicentricUGI: small smooth submucosal mass (usually 15 years) resulting in neuromuscular incoordination from chronically increased muscular activity + tonusAgents:castor oil, senna, phenolphthalein, cascara, podophyllum, aloinLocation:involvement of colon proximal to splenic flexure effaced mucosa with flattened smooth surface diminished / absent haustrations "pseudostrictures" = smoothly tapered areas of narrowing are typical (sustained tonus of circular muscles) poor evacuation of barium flattened + gaping ileocecal valve shortened but distensible ascending colonDDx:"burned-out" ulcerative colitis with right-sided predominance (very similar) Notes:


CHAGAS DISEASE =damage of ganglion cells by neurotoxin liberated from protozoa Trypanosoma cruzi resulting in aperistalsis of GI tract + dilatation Endemic to Central + South America (esp. eastern Brazil) Histo:decreased number of cells in medullary dorsal motor nucleus + Wallerian degeneration of vagus + decrease / loss of argyrophilic cells in myenteric plexus of AuerbachPeak age:30-50 years; M:F = 1:1 intermittent / persistent dysphagia odynophagia (= fear of swallowing) foul breath, regurgitation, aspiration Mecholyl test: abnormal response indicative of deficient innervation; 2.5-10 mg methacholine subcutaneously followed by severe tetanic nonperistaltic contraction 2-5 minutes after injection, commonly in distal half of esophagus, accompanied by severe pain@Dilatative cardiomyopathy (myocarditis)@Megacolon (bowels move at intervals of 8 days to 5 months)Cx:impacted feces, sigmoid volvulus@Esophagus: changes as in achalasia Notes:


CHALASIA =continuously relaxed sphincter with free reflux in the absence of a sliding herniaEtiology:elevated submerged segmentCauses:(1)Delayed development of esophagogastric region in newborns(2)Scleroderma, Raynaud disease(3)S/P forceful dilatation / myotomy for achalasia free / easily induced reflux Notes:


CHRONIC IDIOPATHIC INTESTINAL PSEUDOOBSTRUCTION =nonpropulsive intestine characterized by impaired response to intestinal dilatation without definable cause; ? autosomal dominantAge:all ages, M:F = 1:1 recurrent attacks of abdominal distension, periumbilical pain, nausea, vomiting, constipation mild to marked gaseous distension of duodenum + proximal small bowel esophageal dilation + hypoperistalsis (lower third) excessive duodenal dilation (DDx: megaduodenum, superior mesenteric artery syndrome) ligament of Treitz may be placed lower than usual delayed transit of barium through affected segments disordered motor activity (fluoroscopy) Notes:


COLITIS CYSTICA PROFUNDA =rare benign condition characterized by submucosal mucus-containing cysts lined by normal colonic epitheliumEtiology:probably related to chronic inflammationAge:primarily disease of young adults brief periods of bright red rectal bleeding mucous / bloody discharge intermittent diarrheaLocation:(a)localized to rectum (most commonly) / sigmoid(b)generalized colonic process (less common) nodular polypoid / cauliflower-like lesions 50 years of age: annual fecal occult-blood test + sigmoidoscopy / BE every 3 to 5 years(b)for first-degree relatives of patients with colon cancer screening should start at age 40Incidence:15% of all newly diagnosed cancers; 13% of all cancer deaths; 156,000 new cases/year with 61,300 deaths; 6.5% lifetime probability of any White person to develop colorectal cancer; 3/100,000 in 30- to 34-year-olds; 532/100,000 for >85-year-olds Age:median age of 71 years for colon cancer; median age of 69 years for rectal cancer; M:F = 3:2Histo:(1)Adenocarcinoma with varied degrees of differentiation(2)Mucinous carcinoma (uncommon)(3)Squamous cell carcinoma + adenoacanthoma (rare) Staging (modified Dukes = Astler-Coller classification): Alimited to mucosaBinvolvement of muscularis propria B1 extension into muscularis propriaB2 extension through muscularis propria into serosa / mesenteric fat (35%)Clymph node metastases (50%)C1 + growth limited to bowel wallC2 + growth extending into adipose tissueDdistant metastases Staging (UICC-AJCC Colorectal Cancer Staging System): StageGrouping5-year survival0TisN0M0>95%IT1N0M0T2N0M075-100%IIT3N0M0T4N0M050-75%IIIany TN1M0any TN2,3M030-50%IVany Tany NM14 pericolic LnnN3any Lnn along course of a vascular trunk Metastases (lymphatic / hematogenous venous): 1.liver (75%; 15-20% at time of surgery)2.retroperitoneal + mesenteric nodes (10-15%)3.adrenal (10-14%)4.lung (5-50%)5.ovary (3-8%)6.psoas muscle tumor deposit7.malignant ascites8.bone (5%)9.brain (5%) Because of absence of lymphatics in lamina propria colon cancer will not metastasize until it penetrates the muscularis mucosa! rectal bleeding, iron deficiency anemia change in caliber of stools obstruction (poor prognostic indicator) hydronephrosis (13%) positive fecal occult blood testing (2-6% positive-result rate ; 5-10% positive predictive value; fails to detect 30 -50% of colorectal carcinomas + up to 75% of adenomas): Hemoccult (hematein), Hemoquant (porphyrins), Haemselect (hemoglobin) progressive elevation of carcinoembryonic antigen (CEA) >10 µg/L indicative of recurrent / metastatic diseaseLocation:rectum (15-33 -41%), sigmoid (20-37%), descending colon (10-11%), transverse colon (12%), ascending colon (8-16%), cecum (8-10%); "aging gut" = number of right-sided lesions increasing with age Colonoscopy: cecum not visualized in 10-36%; fails to detect 12% of colonic polyps (10% in areas never reached by colonoscope) Cx:perforation in 0.2% (0.02% for BE); death in 1:5,000 (1:50,000 for BE) BE (sensitivities for polyps >1 cm: single contrast 77-94%, double contrast 82-98%; for polyps 50% of tumor composed of extracellular mucin) psammomatous calcifications in mucinous adenocarcinoma signs of Lnn involvement: single lymph node >1 cm in diameter / cluster of >3 nodes 50%); colon (100%)Prognosis:rapidly fatal in women within 6-18 months (cachexia); tendency toward remission in men Notes:


DESMOID TUMOR =uncommon benign tumor consisting of fibrous tissue with insidious growth [desmos = "band / tendon"]=subgroup of fibromatosesTypes: 1.ABDOMINAL DESMOIDLocation:mesentery (most common mesenteric primary), musculoaponeurosis of rectus, internal oblique muscle; occasionally external oblique muscle2.EXTRA-ABDOMINAL DESMOID=musculoaponeurotic fibromatosisLocation:pelvis, chest wall, mediastinum Age:peak age in 3rd decade, 70% between 20 and 40 years of age; M:F = 1:3Path:poorly circumscribed coarsely trabeculated tumor resembling scar tissue, confined to musculature + overlying aponeurosisHisto:elongated spindle-shaped cells of uniform appearance, septated by dense bands of collagen, infiltration of adjacent tissue (DDx: low-grade fibrosarcoma, reactive fibrosis)Associated with:Gardner syndrome, multiple pregnancies, prior trauma firm slowly growing deep-seated massSize:5-20 cm in diameterMR: hypointense to muscle on T1WI + variable intensity on T2WICT: ill-defined / well-circumscribed mass usually higher attenuation than muscle ± enhancement retraction, angulation, distortion of small / large bowel with mesenteric infiltrationUS: sharply defined + smoothly marginated mass of low / medium / high echogenicityCx:compression / displacement of bowel / ureter, intestinal perforationPrognosis:locally aggressive growth; 25-65% recurrence rateRx:local resection + radiotherapy, antiestrogen therapyDDx:(1)Malignant tumor: metastasis, fibrosarcoma, rhabdomyosarcoma, synoviosarcoma, liposarcoma, fibrous histiocytoma, lymphoma, (2)Benign tumor: neurofibroma, neuroma, leiomyoma(3)Acute hematoma

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DIAPHRAGM DISEASE =small bowel webs due to NSAIDsEffect of NSAID:gastric irritation, ulceration of small intestinesFrequency:in 10% of patients receiving long-term NSAID therapyPath:foci of submucosal fibrosis with interruption of adjacent muscularis mucosae blood + protein loss intermittent intestinal obstructionLocation:ileum > jejunumEnteroclysis: multiple concentric diaphragm-like stricturesDDx:Crohn disease

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DISACCHARIDASE DEFICIENCY =enzyme deficiencies for any of the disaccharides (maltose, lactose, etc.)A.PRIMARYB.SECONDARY to other diseases (eg, Crohn disease)Pathophysiology: (a)unabsorbed disaccharides produce osmotic diarrhea(b)bacterial fermentation produces short-chain volatile fatty acids causing further osmotic + irritant diarrhea normal small bowel series without added lactose abnormal small bowel series done with lactose (50 g added to 600 cm3 of barium suspension) small + large bowel distension dilution of barium shortening of transit time Notes:


DISTAL INTESTINAL OBSTRUCTION SYNDROME =MECONIUM ILEUS EQUIVALENT=impaction of inspissated stool in distal part of ileum + proximal part of colonPrevalence:7-15-41% of children / adolescents with cystic fibrosis; 2% in patients ascending colon > transverse colon > descending colon (contiguous involvement) diffuse colonic thickening mural striation (50%) mesenteric soft-tissue infiltration (100%) increased pericolonic fat (60%)Cx:intussusception, volvulusRx:stool softeners, oral polyethylene glycol-electrolyte solution (Go-lytely®), increasing dose of pancreatic enzyme supplements, mucolytic agents (N-acetylcysteine) orally / with Gastrografin® enemaDDx:appendicitis, partial intestinal obstruction (adhesion / stricture from previous bowel surgery)

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DIVERTICULAR DISEASE OF COLON =overactivity of smooth muscle causing herniation of mucosa + submucosa through muscle layersIncidence:5-10% in 5th decade; 33-48% over age 50; 50% past 7th decade; M:F = 1:1; most common affliction of colon in developed countriesCause:decreased fecal bulk (diet high in refined fiber + low in roughage)Location:in 80% in sigmoid (= narrowest colonic segment with highest pressure); in 17% distributed over entire colon; in 4-12% isolated to cecum / ascending colon

Cross Section through Colon

Prediverticular Disease Of Colon Colonic Diverticulosis Colonic Diverticulitis Colonic Diverticular Hemorrhage Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : DIVERTICULAR DISEASE OF COLON

Prediverticular Disease Of Colon =longitudinal + circular smooth muscle thickening with redundancy of folds secondary to myostatic contracture "saw-tooth sign" = crowding + thickening of haustral folds (shortening of colonic segment) plump marginal indentations superimposed muscle spasm (relieved by antispasmodics)DDx:hemorrhage; ischemia; radiation changes; pseudomembranous colitis

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Colonic Diverticulosis =acquired herniations of mucosa + muscularis mucosae through the muscularis propria with wall components of mucosa, submucosa, serosa = false diverticula of pulsion typeSite: (a)lateral diverticula arise between mesenteric + antimesenteric teniae on opposite sides(b)antimesenteric intertaenial diverticula opposite of mesenteric sideIntramural type vasa recta (= nutrient arteries) pass through the circular muscle (weakness in muscular wall) and are carried over the fundus of the diverticula as it enlarges size: initially tiny (3- to 10-mm) V-shaped protrusions increasing up to several cm in diameter bubbly appearance of air-containing diverticula residual barium within diverticula from previous study spiky irregular outline (antimesenteric intertaenial ridge is typical site for intramural diverticula ) smooth dome-shaped appendages with a short neck may be pointed, attenuated, irregular with variable filling circular line with sharp outer edge + fuzzy blurred inner edge (en face view in double contrast BE) Giant sigmoid diverticulum = large gas-containing cyst (air entrapment secondary to ball-valve mechanism) arising in left iliac fossaCT: diverticula distorted luminal contour + muscular hypertrophy Notes:


Colonic Diverticulitis =perforation of diverticulum with intramural / localized pericolic abscessIncidence:5% of population; in 10-35% of diverticular disease; increasing frequency with agePathogenesis:mucosal abrasion from inspissated fecal material leads to perforation of thin wall pain + local tenderness + mass in LLQ fever (25%), leukocytosis (36%) Location:sigmoid colon (most commonly) localized ileus ± pattern of small bowel obstruction (kinking / edema if small bowel adheres to abscess) gas in abscess / fistula pneumoperitoneum (rare)BE (77-86% sensitive): focal area of eccentric luminal narrowing caused by pericolic / intramural inflammatory mass marked thickening + distortion of mucosal folds mucosal tethering extraluminal contrast = PERIDIVERTICULITIS "double-tracking" = pericolonic longitudinal sinus tract pericolonic collection = peridiverticular abscess fistula to bladder / small bowel / vaginaCT (79-93% sensitive, 77% specific): poorly marginated hazy area of increased attenuation ± fine linear strands within pericolic fat (98%) diverticula (84%) = flask-shaped structures projecting through colonic wall + filled with air / barium / fecal material circumferential bowel wall thickening of >4 mm (70%) frank abscess (47%) = central liquid / gas fluid ± air of peritonitis (16%) fluid at root of mesentery fistula formation (14%):most commonly colovesical, also colovaginal, coloenteric, colocutaneous colonic obstruction (12%) intramural sinus tracts (9%) ureteral obstruction (7%)US (85-98% sensitive, 80-97% specific): thickening of bowel wall = >4 mm distance between echogenic lumen interface and serosa diverticula = round / oval hypo- / hyperechoic foci protruding from colonic wall with focal disruption of normal layer continuity ± internal acoustic shadowing inflammatory pericolic fat = regionally increased echogenicity adjacent to colonic wall ± ill-defined hypoechoic zones pericolic abscessPrognosis:(a)self-limiting (usually)(b)transmural perforation(c)superficial ulceration(d)chronic abscessDDx:(1)Colonic neoplasm (shorter segment, heaped-up margins, ulcerated mucosa)(2)Crohn colitis (double-tracking longer than 10 cm)Rx:antibiotics, surgery (in 25%), percutaneous abscess drainage Notes:


Colonic Diverticular Hemorrhage Not related to diverticulitis Incidence:in 3-47% of diverticulosisLocation:75% located in ascending colon (larger neck + dome of diverticula) massive rectal hemorrhage without pain extravasation of radionuclide tracers angiographic contrast pooling in bowel lumenRx:(1)transcatheter infusion of vasoconstrictive agents (Pitressin®)(2)embolization with Gelfoam® Notes:


DUMPING SYNDROME =early postprandial vascular symptomatology of sweating, flushing, palpitation, feeling of weakness and dizzinessPathophysiology:rapid entering of hypertonic solution into jejunum resulting in fluid shift from blood compartment into small bowelIncidence:1-5%; M:F = 2:1 Roentgenologic findings not diagnostic! rapid emptying of barium into small bowel (= loss of gastric reservoir function)Rx:lying down, dietDDx:late postprandial hypoglycemia (90-120 minutes after eating) Notes:


DUODENAL ATRESIA =most common cause of congenital duodenal obstruction; second most common site of gastrointestinal atresias after ileumIncidence:1:10,000; M:F = 1:1Etiology:defective vacuolization of duodenum between 6th-11th weeks of fetal life; rarely from vascular insult (extent of obstruction usually involves larger regions with vascular insult)Age at presentation:first few days of life persistent bilious vomiting a few hours after birth / following 1st feeding rapid deterioration secondary to loss of fluids + electrolytesIsolated sporadic anomaly (30-52%) Associated anomalies (in 60%): (1)Down syndrome (20-33%); 25% of fetuses with duodenal atresia have Down syndrome! 3 cm (rare) with higher morbidity + mortality; may be overlooked by simulating a normal / deformed duodenal bulb "cloverleaf deformity, hourglass stenosis" (healed stage) with prestenotic dilatation of recessesCx:(1)Obstruction (5%)(2)Perforation ( posterior wall; fistula to gallbladder(3)Penetration ( hematemesisRx:antral resection (Billroth I) + vagotomy

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DUODENAL VARICES =dilated collateral veins secondary to portal hypertension (posterior superior pancreaticoduodenal vein) lobulated filling defects (best demonstrated in prone position, maximal luminal distension will obliterate them) commonly associated with fundal + esophageal varices

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DUPLICATION CYST =uncommon congenital anomaly found anywhere along alimentary tract from tongue to anusIncidence:15% of pediatric abdominal masses are gastrointestinal duplication cystsTheories of formation: (1)Abortive twinning(2)Persistent embryologic diverticula(3)Split notochord(4)Aberrant luminal recanalization(5)Intrauterine vascular accidentassociated with alimentary tract atresia in 9% Age:presentation often in infancy / early childhoodPath:spherical cyst / tubular structure located in / immediately adjacent to gastrointestinal tract; shares a common muscle wall + blood supply; has a separate mucosal lining; cyst contents are usually serousHisto:smooth muscle wall + lined with alimentary tract mucosa; ectopic mucosa squamous, transitional, ciliated mucosa; lymphoid aggregates; ganglion cells Gastric mucosa + pancreatic tissue are the only ectopic tissues of clinical importance! respiratory distress (with esophageal duplication) palpable abdominal mass nausea, emesisLocation:ileum (30-33%), esophagus (17-20%), colon (13-30%), jejunum (10-13%), stomach (7%), pylorus (4%), duodenum (4-5%), ileocecal junction (4%), rectum (4%); In 7-15% concomitant duplications elsewhere in the alimentary tract!Site:on mesenteric aspect of alimentary canalMorphology: (a)large spherical / saccular cyst (82%)(b)small intramural cyst(c)tubular sausage-shaped cyst (18%): commonly along small + large bowel; frequently communicates with lumen of adjacent gut elongated tubular / spherical cystic mass muscular rim sign (= echogenic inner mucosal lining + hypoechoic outer rim) in 47% cyst paralleling normal bowel lumenCx:bowel obstruction, intussusception, bleeding (due to presence of gastric mucosa / pressure necrosis of adjacent mucosa by cyst expansion / from intussusception)DDx: (1)Omental cyst (greater omentum / lesser sac, multilocular)(2)Mesenteric cyst (between leaves of small bowel mesentery)(3)Choledochal cyst(4)Ovarian cyst(5)Pancreatic pseudocyst(6)Cystic renal tumor(7)Abscess(8)Meckel diverticulum (communicates with GI tract)(9)Lymphangioma(10)Mesenteric lymphoma(11)Intramural tumor

Colonic Duplication Cyst Duodenal Duplication Cyst Esophageal Duplication Cyst Gastric Duplication Cyst Rectal Duplication Cyst Small Bowel Duplication Cyst Thoracoabdominal Duplication Notes:

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Colonic Duplication Cyst Incidence:13% of all alimentary tract duplicationsA.CYSTIC COLONIC DUPLICATION (7%)Path:closed spherical cyst; contains gastric mucosa in 2% + ectopic pancreatic tissue in 5% abdominal mass, bowel obstruction, GI hemorrhageLocation:cecum (40%) ± intussusceptionB.COLORECTAL TUBULAR DUPLICATION (6%)=DUPLICATION OF THE HINDGUT=double-barreled duplication involving part / all of large bowel with "twin" segment on mesenteric / antimesenteric sideSymptomatic age:neonatal period / infancy;M:F = 1:2 May be associated with: rectogenital / rectourinary fistula, duplication of internal / external genitalia, vertebral anomalies, multisystem congenital anomaly complex bowel obstruction passage of feces through vagina simultaneous opacification of true + twin colon duplication may terminate at(a)2nd functional anus(b)imperforate perineal orifice(c)fistulous communication with GU tractC.DOUBLE APPENDIX Notes:


Duodenal Duplication Cyst Incidence:5% of all alimentary tract duplicationsPath:noncommunicating spherical cyst; may contain ectopic gastric mucosa in 21%, small bowel mucosa, pancreatic tissue obstruction, palpable abdominal mass hemorrhage (due to peptic ulceration) jaundice (due to biliary obstruction) pancreatitis (due to ectopic pancreatic tissue)Site:on mesenteric side of anterior wall of 1st + 2nd portion of duodenum mass in concavity of duodenal C-loop compression + displacement of 1st / 2nd portion of duodenum superiorly + anteriorlyCx:pancreatitis from perforation of duplication cyst DDx:pancreatic cyst, pancreatic pseudocyst, choledochal cyst, choledochocele, duodenal intramural tumor, pancreatic tumor Notes:


Esophageal Duplication Cyst arises from foregut Incidence:10-20% of all alimentary tract duplications; 0.5-2.5% of all esophageal masses;M:F = 2:1Path:contains ectopic gastric mucosa in 43%Histo:contains no cartilage, lined by gastrointestinal tract epitheliumAssociated with: vertebral anomalies, esophageal atresia, small bowel duplication (18%)Location:adjacent to esophagus / within esophageal musculature at any level, paraspinal position; R:L = 2:1; in right pleural space detached from esophagus (rare)A.CERVICAL ESOPHAGUS (23%) asymptomatic enlarging lateral neck mass upper airway obstruction in newbornDDx:thyroglossal duct cyst, branchial cleft cyst, cystic hygroma, cervical tumor, cervical lymphadenopathyB.MIDESOPHAGUS (17%) severe upper airway obstruction in early infancyDDx:bronchogenic cyst, neurenteric cyst, intramural esophageal tumorC.DISTAL ESOPHAGUS (60%) frequently asymptomaticLocation:paraspinalDDx:bronchogenic cyst, neurenteric cyst, intramural esophageal tumor closed spherical cyst, almost never communicatingCXR: posterior mediastinal mass ± air-fluid level lobar consolidation + central cavitation (from autodigestion of lung tissue by gastric secretions) thoracic vertebral anomaliesUGI: displacement of esophagus by paraesophageal mass intramural extramucosal massUS: hypoechoic fluid-filled cyst + inner mucosal liningCx:(1)Peptic ulceration (secondary to gastric mucosa)(2)Perforation (secondary to penetrating ulcer)(3)Hematemesis (from erosion into esophagus)(4)Hemoptysis + autodigestion of pulmonary tissue (from erosion into tracheobronchial tree) Notes:


Gastric Duplication Cyst =intramural gastric cyst lined with secretory epitheliumIncidence:7% of all alimentary tract duplicationsPath:noncommunicating spherical cyst (majority); may communicate with aberrant pancreatic duct; ectopic pancreatic tissue found in 37%Symptomatic age:infancy; in 75% detected before age 12; M:F = 1:2 pain (from overdistension of cyst, rupture with peritonitis, peptic ulcer formation, internal pancreatitis) vomiting, anemia, fever symptoms mimicking congenital hypertrophic pyloric stenosis (if duplication in antrum / pylorus)Most common site:greater curvature (65%) paragastric cystic mass up to 12 cm in size, indenting greater curvature seldom communicates with main gastric lumen at one or both ends may enlarge + ulcerate Tc-99m uptakeUS: cyst with two wall layers (inner echogenic layer of mucosa, outer hypoechoic layer of muscle) clear / debris-containing fluidCx:(1)Partial / complete small bowel obstruction(2)Relapsing pancreatitis (with ductal communication)(3)Ulceration, perforation, fistula formationDDx:pancreatic cyst, pancreatic pseudocyst, mesenteric cyst, leiomyoma, adenomatous polyp, hamartoma, lipoma, neurofibroma, teratoma Notes:


Rectal Duplication Cyst Incidence:4% of all alimentary tract duplicationsPath:spherical fluid-filled cyst; may contain duodenal / gastric mucosa + pancreatic tissueSite:posterior to rectum / anus communication with rectum / perianal fistula (in 20%)Symptomatic age:childhood constipation + fecal soiling palpable retrorectal / retroanal mass intractable excoriation of perianal skin (with chronic perianal fistula) cystic mass; may be echogenic (due to solid material ± gas from communication with rectum)DDx:anterior meningocele, sacrococcygeal teratoma, retrorectal abscess, pilonidal cyst, sacral bone tumor Notes:


Small Bowel Duplication Cyst Incidence:most common of all alimentary tract duplicationsSymptomatic age:neonatal period (1/3); 10 mm (12-85% accuracy) hepatic, pulmonary, adrenal metastases direct erosion of vertebral body tumor >3 cm wide = high frequency of extra-esophageal spreadHisto: (1)Squamous cell carcinoma (81-95%)(2)Adenocarcinoma (4-19%) arising from mucosal / submucosal glands or heterotopic gastric mucosa or columnar-lined epithelium (Barrett)(a)in 70% from Barrett esophagus(b)at gastroesophageal junction(3)Mucoepidermoid carcinoma, adenoid cystic carcinoma(4)Carcinosarcoma = pseudosarcoma = spindle-cell squamous carcinomaAge:in men >45 yearsLocation:usually middle third of esophagus large bulky polypoid smooth, lobulated, scalloped intraluminal mass, may be pedunculated(5)Leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma, malignant lymphoma dysphagia (87-95%) of 4 mm (if tumor located in upper third of esophagus) widened mediastinum tracheal deviation posterior tracheal indentation / mass retrocardiac mass esophageal air-fluid level lobulated mass extending into gastric air bubble repeated aspiration pneumonia (with tracheoesophageal fistula) Cx:fistula formation to trachea (5-10%) / bronchi / mediastinum Prognosis:3-5-20% 5-year survival rate Mean survival time: 90 dayswith subdiaphragmatic lymphadenopathy180 dayswith local invasion + abdominal metastases480 dayswithout evidence of invasion / metastases Rx:(1)chemotherapy (fluorouracil, cisplatin, bleomycin sulfate, mitomycin) + surgery(2)chemotherapy + irradiation (~4,000 cGy)(3)chemotherapy + irradiation + surgeryOperative mortality:3-8%

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ESOPHAGEAL INTRAMURAL PSEUDODIVERTICULOSIS =dilated excretory ducts of deep esophageal adnexal mucous glandsEtiology:uncertainIncidence:about 100 cases in world literatureSite:diffuse / segmental involvementIn 90% associated with: any severe esophagitis (most often reflux / Candida), esophageal stricture multiple tiny rounded / flask-shaped barium collections in longitudinal rows parallel to long axis of esophagus appear to "float" outside esophagus without apparent communication with lumen commonly associated with strictures in distal esophagus

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ESOPHAGEAL PERFORATION Cause: (1)Iatrogenic injury (most common cause, 55%): complication of endoscopy, dilatation of stricture, bougie, disruption of suture line following surgical anastomosis, attempted intubation(2)Spontaneous rupture = Boerhaave syndrome (15%): emetogenic injury of the esophagus from sudden increase in intra-abdominal pressure + relaxation of distal esophageal sphincter in the presence of a moderate to large amount of gastric contents(3)Closed chest trauma (10%)(4)Esophageal carcinoma(5)Retained foreign body (14%): coin, aluminum pop-tops, metallic button, safety pin, invisible plastic toy) leading to perforation (in pediatric age group)(6)Barrett ulcer pain, dysphagia, odynophagia rapid onset of overwhelming sepsis: fever, tachycardia, hypotension, shockPlain film (normal in 9-12%): pneumomediastinum subcutaneous emphysema of the neck delayed widening of the mediastinum (secondary to mediastinitis) hydrothorax (after rupture into pleural cavity), usually unilateral hydropneumothorax (often not initially seen) confirmation with contrast study (90% of contrast esophagrams are positive)CT: extraluminal air (92%; most useful sign) periesophageal / mediastinal fluid (92%) pleural effusion (75%) esophageal thickening extravasation of oral contrast materialEsophagography with: (1)water-soluble contrast material (10% false-negative results)(2)barium (if result with water-soluble material negative)A.UPPER / MID-ESOPHAGEAL PERFORATIONLocation:at level of cricopharyngeus muscle (most frequent) widening of upper mediastinum right-sided hydrothoraxB.DISTAL ESOPHAGEAL PERFORATION (more common)Cause:biopsy, dilatation of stricture, Boerhaave syndrome left-sided hydrothorax little mediastinal changesCx:(1) Acute mediastinitis (2) Obstruction of SVC(3) Mediastinal abscessPrognosis:20-60% mortality Notes:


ESOPHAGEAL VARICES =dilated submucosal veins due to increased collateral blood flow from portal venous system to azygos systemA.UPHILL VARICES=collateral blood flow from portal vein via azygos vein into SVC (usually lower esophagus drains via left gastric vein into portal vein)Cause: (a)intrahepatic obstruction from cirrhosis(b)splenic vein thrombosis (usually gastric varices)(c)obstruction of hepatic veins(d)IVC obstruction below hepatic veins(e)IVC obstruction above hepatic vein entrance / CHF(f)marked splenomegaly / splenic hemangiomatosis (rare) varices in lower half of esophagusB.DOWNHILL VARICES=collateral blood flow from SVC via azygos vein into IVC / portal venous system (upper esophagus usually drains via azygos vein into SVC)Cause:obstruction of superior vena cava distal to entry of azygos vein most commonly due to lung cancer, lymphoma, retrosternal goiter, thymoma, mediastinal fibrosis varices in upper 1/3 of esophagus EXAMINATION TECHNIQUE (a)small amount of barium (not to obscure varices)(b)relaxation of esophagus (not to compress varices): refrain from swallowing because succeeding swallow initiates a primary peristaltic wave that lasts for 10-30 seconds; sustained Valsalva maneuver precludes from swallowing(c)in LAO projection with patient recumbent / in Trendelenburg position ± Valsalva maneuver / deep inspirationPlain film: lobulated masses in posterior mediastinum (visible in 5-8% of patients with varices) silhouetting of descending aorta abnormal convex contour of azygoesophageal recessUGI: thickened sinuous interrupted mucosal folds (earliest sign) tortuous radiolucencies of variable size + location "worm-eaten" smooth lobulated filling defects findings may be accentuated after sclerotherapyCT: thickened esophageal wall + lobulated outer contour scalloped esophageal luminal masses right- / left-sided soft-tissue masses(= paraesophageal varices) marked enhancement following dynamic CTCx:bleeding in 28% within 3 years; exsanguination in 10-15%DDx:varicoid carcinoma of esophagus Notes:


ESOPHAGEAL WEB =ringlike esophageal constriction caused by thin mucosal membrane projecting into lumen; covered by squamous epithelium on superior + inferior surfacesAge:middle-aged females? association with: Plummer-Vinson syndrome = Paterson-Kelly syndrome (iron deficiency anemia, stomatitis, glossitis, dysphagia, thyroid disorder, spoon-shaped nails) Cause:mnemonic:"BIEP"B-ring (Schatzki ring) Idiopathic (= transverse mucosal fold) Epidermolysis bullosa Plummer-Vinson disease Path:hyperkeratosis + chronic inflammation of submucosa mostly asymptomatic (unless severely stenosing)Location:in cervical esophagus near cricopharyngeus (most common) > thoracic esophagus; occasionally multiple visualized during maximal distension (in one tenth of a second) arises at right angles from anterior esophageal wall thin delicate membrane of uniform thickness of 3-mm-wide folds with irregular lobulated contour mucosal nodularity (= multiple ulcerations + intervening edema) erosions vertically oriented ulcers usually 3-10 mm in length inflammatory esophagogastric polyp = proximal gastric fold extending across esophagogastric junction (rare) abnormal motility Notes:

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Candida Esophagitis =MONILIASIS = CANDIDIASIS Most common cause of infectious esophagitis!Organism:C. albicans, C. tropicalis; endogenous (majority) / transmitted by another human / animal; often discovered in diseased skin, GI tract, sputum, female genital tract, urine with an indwelling Foley catheterPredisposed: (a)individuals with depressed immunity: hematologic disease, renal transplant, leukemia, chronic debilitating disease, diabetes mellitus, steroids, chemotherapy, radiotherapy, AIDS Most common type of fungi found with opportunistic infections!(b)delayed esophageal emptying: scleroderma, strictures, achalasia, S/P fundoplication(c)antibioticsPath:patchy, creamy-white plaques covering a friable erythematous mucosaHisto:mucosal plaques = necrotic epithelial debris + fungal colonies dysphagia (= difficulty swallowing) severe odynophagia (= painful swallowing from segmental spasm) intense retro- / substernal pain associated with thrush (= oropharyngeal moniliasis) in 20-50-80%Location:predilection for upper 1/2 of esophagus involvement of long esophageal segments longitudinal plaques = grouping of tiny 1-2 mm nodular filling defects with linear orientation(= heaped-up areas of mucosal plaques) "cobblestone" appearance = mucosal nodularity in early stage (from growth of colonies on surface) shaggy / fuzzy / serrated contour (from coalescent plaques, pseudomembranes, erosions, ulcerations, intramural hemorrhage) in fulminant candidiasis narrowed lumen (from spasm, pseudomembranes, marked edema) "intramural diverticulosis" = multiple tiny indentations + protrusions sluggish / absent primary peristalsis strictures (rare) mycetoma resembling large intraluminal tumor (rare)Diagnostic sensitivity:endoscopy (97%), double contrast (88%), single contrast (55%)Cx:(1)systemic candidiasis ("microabscesses" in liver, spleen, kidney)(2)gastric bezoar due to large fungus ball (after long-standing esophageal candidiasis)Rx:Mycostatin®DDx:glycogen acanthosis, reflux esophagitis, superficial spreading carcinoma, artifacts (undissolved effervescent crystals, air bubbles, retained food particles), herpes esophagitis, acute caustic ingestion, intramural pseudo-diverticulosis, squamous papillomatosis, Barrett esophagus, epidermolysis bullosa, varices Notes:


Caustic Esophagitis =CORROSIVE ESOPHAGITISCorrosive agents: lye (sodium hydroxide), washing soda (sodium carbonate), household cleaners, iodine, silver nitrate, household bleaches, Clinitest® tablets (tend to be neutralized by gastric acid) Severity of injury dependent on contact time + concentration of corrosive material!Associated with:injury to pharynx + stomach (7-8%): antral burns more common with acid (buffering effect of gastric acid on alkali)Location:middle + lower thirds of esophagusStageI:acute necrosis from protein coagulation mucosal blurring (edema) diffusely atonic + dilated esophagus tertiary contractionsStageII:frank ulceration in 3-5 days ulceration + pseudomembranesStageIII:scarring + stricture from fibroblastic activity long segmental stricture after 10 days when acute edema subsides (7-30%)Cx:(1)Esophageal / gastric perforation during ulcerative stage(2)Squamous cell carcinoma in injured segment Notes:


Chronic Esophagitis Notes:

luminal narrowing with tapered transition to normal + proximal dilatation circumferential / eccentric stricture sacculations = pseudodiverticula


Cytomegalovirus Esophagitis Organism:member of herpesvirus groupAssociated with:AIDS

severe odynophagia diffusely normal mucosal background one /

more large ovoid flat ulcers (up to several cm in size) near gastroesophageal junction discrete small superficial ulcers indistinguishable from herpes esophagitis (uncommon)Rx:ganciclovir (relatively toxic) Dx:endoscopic brushings, biopsy specimen, cultures Notes:


Drug-induced Esophagitis Agents:tetracycline, doxycycline, potassium chloride, quinidine, aspirin, ascorbic acid, alprenolol chloride, emepronium bromide severe odynophagia history of taking medication with little / no water immediately before going to bedLocation:midesophagus at site of compression by aortic arch / left mainstem bronchus superficial solitary / several discrete / localized clusters of tiny ulcers distributed circumferentially dramatic healing of lesion 7-10 days after withdrawal of offending agentDDx:herpes esophagitis (less localized) Notes:


Herpes Esophagitis 2nd most common cause of opportunistic infection!Organism:Herpes simplex virus type I (DNA core virus) secreted in saliva of 2% of healthy populationAge:15-30 years; usually males history of recent exposure to sexual partners with herpetic lesions on lips / buccal mucosa flulike prodrome of 3-10 days (headaches, fever, sore throat, upper respiratory symptoms, myalgia) severe acute dysphagia / odynophagiaMay be associated with:oropharyngeal herpetic lesions / oropharyngeal candidiasisLocation:midesophagus (level of left main bronchus) initially vesicles / blisters that subsequently rupture multiple small discrete superficial punctate / linear / stellate (often "diamond shaped") ulcers surrounded by radiolucent halos of edematous mucosa intervening mucosa normal (without plaques) multiple plaquelike lesions (only with severe infection)Rx:oral / intravenous acyclovirDx:rising serum titer for HSV type 1, viral culture, biopsy (immunofluorescent staining for HSV antigen, demonstration of intranuclear inclusions)DDx:drug-induced esophagitis, Crohn disease, esophageal intramural pseudodiverticulosis Notes:


Human Immunodeficiency Virus Esophagitis maculopapular rash + ulcers of soft palate one / more giant flat ovoid / diamond-shaped ulcers (at time of seroconversion) indistinguishable from CMV esophagitisDx:ONLY per exclusionDDx:CMV esophagitis, mycobacterial esophagitis, actinomycosis, potassium chloride, quinidine, caustic ingestion, nasogastric intubation, radiation therapy, endoscopic sclerotherapy Notes:


Reflux Esophagitis =esophageal inflammation secondary to reflux of acid-peptic contents of the stomach; reflux occurs if resting pressure of LES 4% stomach activity Cx of reflux: (a)from acid + pepsin acting on esophageal mucosa:1.Motility disturbance2.Stricture3.Schatzki ring4.Barrett esophagus5.Iron-deficiency anemia6.Reflux / peptic esophagitis(b)from aspiration of gastric contents1.Acute aspiration pneumonia2.Mendelson syndrome3.Pulmonary fibrosis Notes:


Viral esophagitis Predisposed:immunocompromised, eg, underlying malignancy, debilitating illness, radiation treatment, steroids, chemotherapy, AIDS

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FAMILIAL ADENOMATOUS POLYPOSIS =FAMILIAL MULTIPLE POLYPOSIS = autosomal dominant disease with 80% penetrance (gene for familial polyposis localized on chromosome 5); sporadic occurrence in 1/3Incidence:1:7,000 to 1:24,000 livebirthsHisto:tubular / villotubular adenomatous polyps; usually about 1,000 adenomasAge:polyps appear around puberty family history of colonic polyps (66%) Screening of family members after puberty! clinical symptoms begin during 3rd-4th decade (range 5-55 years) vague abdominal pain, weight loss diarrhea, bloody stools protein-losing enteropathy (occasionally)Associated with:(1)Hamartomas of stomach in 49%(2)Adenomas of duodenum in 25%(3)Periampullary carcinoma "carpet of polyps" = myriad of 2-3 mm (up to 2 cm) polypoid lesions@Colon (100%): more numerous in distal colon; always affecting rectum normal haustral pattern@Stomach (5%)@Small bowel ( stomach > small bowel (in 12% by 5 years; in 30% by 10 years; in 100% by 20 years after diagnosis; age at carcinomatous development usually 20-40 years; multiple carcinomas in 48%) Periampullary carcinoma is the most common cause of death after prophylactic colectomy!Rx:prophylactic total colectomy in late teens / early twenties before symptoms develop +(1)Permanent ileostomy(2)Continent endorectal pull-through pouch(3)Kock pouch (= distal ileum formed into a one-way valve by invaginating the bowel at skin site) DDx:other polyposes, lymphoid hyperplasia, lymphosarcoma, ulcerative colitis with inflammatory pseudopolyps Notes:


GALLSTONE ILEUS Incidence:0.4-5% of all intestinal obstructions (20% of obstruction in patients >65 years; 24% of obstructions in patients >70 years); develops in 2.5 cm in diameter change in position of previously identified gallstoneUGI / BE: well-contained localized barium collection lateral to first portion of duodenum (barium-filled collapsed GB + possibly biliary ducts)Fistulous communication: CHOLECYSTODUODENAL (60%), choledochoduodenal, cholecystocolic, choledochocolic, cholecystogastric identification of site of obstruction: terminal ileum (60-70%), proximal ileum (25%), distal ileum (10%), pylorus, sigmoid, duodenum (Bouveret syndrome)Cx:recurrent gallstone ileus in 5-10% (additional silent calculi more proximally)Prognosis:high mortality

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GANGLIOCYTIC PARAGANGLIOMA =rare benign tumor of the GI tractFrequency:3 cm from primary along branches of celiac axisN3paraaortic, hepatoduodenal, retropancreatic, mesenteric nodesM1distant metastases Location:mostly distal third of stomach + cardia; 60% on lesser curvature, 10% on greater curvature; esophagogastric junction in 30% Probability of malignancy of an ulcer: at lesser curvature 10-15%, at greater curvature 70%, in fundus 90% Morphology: 1.Polypoid / fungating carcinoma2.Ulcerating / penetrating carcinoma (70%)3.Infiltrating / scirrhous carcinoma (5-15%)= linitis plastica Histo:frequently signet ring cell type + increase in fibrous tissueLocation:antrum, fundus + body (38%) firmness, rigidity, reduced capacity of stomach, aperistalsis in involved area granular / polypoid folds with encircling growth4.Superficial spreading carcinoma=confined to mucosa / submucosa; 5-year survival of 90% patch of nodularity little loss of elasticity5.Advanced bulky carcinoma GI bleeding, abdominal pain, weight loss UGI: rigidity filling defect amputation of folds ± ulceration ± stenosis calcifications (mucinous adenocarcinoma)CT: irregular nodular luminal surface asymmetric thickening of folds mass of uniform density / varying attenuation wall thickness >6 mm with gas distension + 13 mm with positive contrast material distension increased density in perigastric fat enhancement exclusively in linitis plastica type nodules of serosal surface (= dilated surface lymphatics) diameter of esophagus at gastroesophageal junction larger than adjacent aorta (DDx: hiatal hernia) lymphadenopathy below level of renal pedicle (3%) Metastases: 1.along peritoneal ligaments(a)gastrocolic lig.: transverse colon, pancreas(b)gastrohepatic + hepatoduodenal lig.: liver2.local lymph nodes3.hematogenous: liver (most common), adrenals, ovaries, bone (1.8%), lymphangitic carcinomatosis of lung (rare) 4.peritoneal seeding:on rectal wall=Blumer shelfon ovaries=Krukenberg tumor5.left supraclavicular lymph node = Virchow nodePrognosis: overall 5-year survival rate of 5-18%, mean survival time of 7-8 months; -85% 5-year survival in stage T1-52%5-year survival in stage T2-47%5-year survival in stage T3-17%5-year survival in stage N1-2- 5%5-year survival in stage N3

Early Gastric Cancer (20%) Advanced Gastric Cancer (T2 lesion and higher) Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : GASTRIC CARCINOMA

Early Gastric Cancer (20%) =invasion limited to mucosa + submucosa (T1 lesion)Classification of Japan Research Society for Gastric Cancer: TypeIProtruded type = >0.5 cm height with protrusion into gastric lumen (10-20%)TypeIISuperficial type = 60 years of agePath:surface epithelial damage + inflammation with mucosal infiltration by neutrophils, plasma cells, and lymphoid nodulesLocation:gastric antrum > proximal half of stomachSite:beneath mucus layer on surface epithelial cells asymptomatic (vast majority) dyspepsia, epigastric pain gastritis thickened gastric folds polypoid gastritis mimicking malignant tumor enlarged areae gastricae gastric ulcer (60-80% prevalence of H. pylori) duodenal ulcer (90-100% prevalence of H. pylori)Dx:(1) Endoscopic brushings + biopsy(2)Breath test measuring urease activity after ingestion of carbon-14-labeled urea(3)Serologic test for IgG antibodiesRx:triple therapy (= bismuth + metronidazole + tetracycline / amoxicillin) results in 95% cure rate after 2 weeks of therapy Notes:


HEMANGIOMA OF SMALL BOWEL Increased incidence in:Turner syndrome, tuberous sclerosis, Osler-Weber-Rendu diseaseLocation:duodenum (2%), jejunum (55%), ileum (42%) multiple sessile compressible intraluminal filling defects nodular segmental mucosal abnormality phleboliths in intestinal wall Notes:


HENOCH-SCHÖNLEIN PURPURA =most common systemic allergic vasculitis in children precipitated by bacterial / viral infection, allergies, insect sting, drugs (eg, penicillin, sulfonamides, aspirin)Cause:deposition of IgA-dominant immune complexes in venules, capillaries, and arteriolesAge:children (peak age of 5 years) + adults most frequent manifestations: purpuric skin rash on legs + extensor surfaces on arms colicky abdominal pain + GI bleeding microscopic hematuria + proteinuria in 50% (from proliferative glomerulonephritis with IgA deposits demonstrated by immunofluorescence) often begins as an upper respiratory tract infection arthralgias thickened valvulae conniventes (due to hemorrhage + edema)Cx:renal insufficiency (10-20%), end-stage renal disease (5%)Rx:high doses of corticosteroids + azathioprine

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Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : HERNIA

External Hernia =bowel extending outside the abdominal cavityIncidence:95% of all herniasLocation: 1.Inguinal hernia2.Femoral hernia3.Spigelian hernia Frequency:2% of anterior abdominal hernias=acquired ventrolateral hernia through defect in aponeurosis between transverse and rectus muscle of abdomen at junction of semilunar + arcuate lines below umbilicus hernia sac dissects laterally to rectus abdominis muscle through a fibrous groove (= semicircular / spigelian line) hernia sac lies beneath an intact external oblique aponeurosis4.Petit lumbar triangle5.Obturator foramen6.Sciatic notch7.Diaphragmatic hernia (foramen of Bochdalek + Morgagni)8.Richter hernia = entrapment of antimesenteric border of bowel in hernia orifice, usually seen in older women with femoral hernias9.Perineal hernia (rare)(a)anterior perineal hernia = defect of urogenital diaphragm anterior to superficial transverse perineal m. + lateral to bulbocavernosus m. + medial to ischiocavernosus m. (only in females)(b)posterior perineal hernia = defect in levator ani m. / between levator ani m. and coccygeus m. posterior to superficial transverse perineal m. defecating proctography

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Internal Hernia Incidence:5% of all hernias, responsible for 2 cm peristalsis ceases above hiatus (end of peristaltic wave delineates esophagogastric junction) tortuous esophagus having an eccentric junction with hernia numerous coarse thick gastric folds within suprahiatal pouch (>6 longitudinal folds) ± gastroesophageal refluxCT: dehiscence of diaphragmatic crura >15 mm pseudomass within / above esophageal hiatus increase in fat surrounding distal esophagus(= herniation of omentum through phrenicoesophageal ligament) DDx:normal temporary cephalad motion of esophagogastric junction by 1-2 cm into chest due to contraction of longitudinal muscle during esophageal peristalsis Paraesophageal Hernia (1%) =ROLLING HIATAL HERNIA = PARAHIATAL HERNIA = portion of stomach superiorly displaced into thorax with esophagogastric junction remaining in subdiaphragmatic position cardia in normal position herniation of portion of stomach anterior to esophagus frequently nonreducible may be associated with gastric ulcer of lesser curvature at level of diaphragmatic hiatus Totally Intrathoracic Stomach =defect in central tendon of diaphragm in combination with slight volvulus in transverse axis of stomach behind heart cardia may be intrathoracic (usually) / subdiaphragmatic great gastric curvature either on right / left side Congenitally Short Esophagus (not true hernia, very rare) =gastric ectopy by lack of lengthening of esophagus nonreducible intrathoracic gastric segment (in erect / supine position) cylindrical / round intrathoracic segment with large sinuous folds short straight esophagus circular narrowing at gastroesophageal junction, frequently with ulcer gastroesophageal reflux Notes:


Umbilical Hernia =protrusion of abdominal contents / fat into anterior abdominal wall via umbilical ringPrevalence:4% of all hernias; M 2 lymph node regions on same side of diaphragmIIIlymph node involvement on both sides of diaphragmIVdiffuse / disseminated involvement of > 1 extralymphatic organs / tissues ± associated lymph node involvementE=extralymphatic siteS=splenic involvementA=absence of fever, night sweats, >10% weight loss in past 6 monthsB=presence of fever, night sweats, >10% weight loss in past 6 months painless lymphadenopathy alcohol-induced pain unexplained fevers, night sweat, weight loss generalized pruritusLocation:intestinal involvement uncommon (10-15%); duodenum + jejunum (67%); terminal ileum (20%) narrow rigid obstructive lesion abundance of desmoplastic reaction (DDx from NHL) infiltrating (60%); polypoid (26%); ulcerated (14%)Prognosis: excellent for isolated / localized disease Notes:


HYPERPLASTIC POLYP OF COLON =intestinal metaplasia consisting of mucous glands lined by a single layer of columnar epithelium; NO malignant potentialPath:infolding of epithelium into the glandular lumenLocation:rectum usually 10 mL (92% sensitive, 86% specific)UGI (95% sensitivity): Precautions:(1)empty stomach via nasogastric tube before study(2)remove contrast at end of study pyloric wall thickness >10 mm elongation + narrowing of pyloric canal (2-4 cm in length) "double / triple track sign" = crowding of mucosal folds in pyloric channel "string sign" = passing of small barium streak through pyloric channel Twining recess = "diamond sign" = transient triangular tentlike cleft / niche in midportion of pyloric canal with apex pointing inferiorly secondary to mucosal bulging between two separated hypertrophied muscle bundles on the greater curvature side within pyloric channel "pyloric teat" = outpouching along lesser curvature due to disruption of antral peristalsis "antral beaking" = mass impression upon antrum with streak of barium pointing toward pyloric channel Kirklin sign = "mushroom sign" = indentation of base of bulb (in 50%) gastric distension with fluid active gastric hyperperistalsis "caterpillar sign" = gastric hyperperistaltic waves

US: "target sign" = hypoechoic ring of hypertrophied pyloric muscle around echogenic mucosa centrally on cross-section "cervix sign" = indentation of muscle mass on fluid-filled antrum on longitudinal section "antral nipple sign" = redundant pyloric channel mucosa protruding into gastric antrum pyloric volume >1.4 cm3 (= 1/4 ÷ x [maximum pyloric diameter]2 x pyloric length); most criteria independent of contracted or relaxed state (33% false negative) pyloric length (mm) + 3.64 x muscle thickness (mm) > 25 pyloric muscle wall thickness >3 mm pyloric transverse diameter >13 mm with pyloric channel closed elongated pyloric canal >17 mm in length exaggerated peristaltic waves delayed gastric emptying of fluid into duodenumCx:hypochloremic metabolic alkalosisDDx: 1.Infantile pylorospasm muscle thickness between 1.5 and 3 mm variable caliber of antral narrowing antral peristalsis delayed gastric emptying elongation of pylorusPrognosis:resolves in several days / ? early stage of evolving pyloric stenosisRx:effective with metoclopramide hydrochloride2.Milk allergy3.Eosinophilic gastroenteritis

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Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : HYPERTROPHIC PYLORIC STENOSIS

Adult Form Of Hypertrophic Pyloric Stenosis (secondary to mild infantile form) acute obstructive symptoms uncommon nausea, intermittent vomiting postprandial distress, heartburnAssociated with: (1) peptic ulcer disease (in 50-74%) (prolonged gastrin production secondary to stasis of food)(2)chronic gastritis (54%) persistent elongation (2-4 cm) + concentric narrowing of pyloric channel parallel + preserved mucosal folds antispasmodics show no effect on narrowing proximal benign ulcer (74%), usually near incisura

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Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : HYPERTROPHIC PYLORIC STENOSIS

Focal Pyloric Hypertrophy =TORUS HYPERPLASIA=localized muscle hypertrophy on the lesser curvature=milder atypical form of HPS flattening of distal lesser curvature

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IMPERFORATE ANUS Prevalence:1:5,000 live births A.LOW ANOMALY (55%)=bowel has passed through levator sling fistula to perineum / vulvaRx:readily reparableB.INTERMEDIATE DEFECT (least common)=bowel ends within levator muscle as a result of abnormality in posterior migration of rectum fistula opening low in vagina / vestibuleRx:2- / 3-stage operationC.HIGH ANOMALY=bowel ends above levator sling; M > F fistulous connection to perineum / vagina / posterior urethra (air in bladder in males; air in vagina in females)Cx:associated malformations more common + more severeRx:multiple surgical procedures distance between rectal air and skin will not accurately outline the extent of atretic rectum and anus (varying length during crying with increase in abdominal pressure + contraction of levator ani muscle)US: 6 years of ageAge:peak incidence between 6 months and 2 years; 3-9 months (40%); colocolicCx:vascular compromise secondary to incorporation of mesentery (hemorrhage, infarction, acute inflammation)B.IN ADULTS (6%)Etiology: (1)specific cause (80%): benign tumor (1/3), malignant tumor (1/5), lipoma, Meckel diverticulum, prolapsed gastric mucosa, aberrant pancreas, adhesions, foreign body, feeding tube, chronic ulcer (TB, typhoid), prior gastroenteritis, gastroenterostomy, traumawithout anatomic lead point: celiac disease, scleroderma, Whipple disease, fasting, anxiety, agonal state (2)idiopathic (20%) recurrent episodes of colicky pain, nausea, vomitingLocation:ileoileal (40%) > ileocolic (13%) Plain film (no abnormality in 25%): abdominal soft-tissue mass (50-60%), usually in RUQ loss of inferior hepatic margin small bowel obstruction (25%) with nipplelike termination of gas shadowAntegrade barium study: "coil spring" appearance beaklike abrupt narrowing of barium column demonstrating a central channelRetrograde barium study: convex intracolic mass + "coiled spring" patternUS (close to 100% sensitive): "doughnut / target / bulls eye sign" (on transverse scan) = concentric rings of alternating hypoechoic + hyperechoic layers (= intussuscipiens) with central hyperechoic portion (= mesentery of intussusceptum) "pseudokidney / sandwich / hay fork sign" (on longitudinal scan) = hypoechoic layers on each side of echogenic center of mesenteric fat peritoneal fluid trapped inside intussusception (associated with irreducibility + ischemia) color Doppler demonstrates mesenteric vessels dragged between entering + returning wall of intussusceptum Absence of blood flow suggests bowel necrosis!CT: "multiple concentric rings" = 3 concentric cylinders (central cylinder = canal + wall of intussusceptum; middle cylinder = crescent of mesenteric fat; outer cylinder = returning intussusceptum + intussuscipiens) proximal obstruction HYDROSTATIC / PNEUMATIC REDUCTION 3 mmAngio (findings similar to inflammatory disease): normal / slightly attenuated arterial supply mild acceleration of arteriovenous transit time small tortuous ectatic draining veinsPrognosis: (1)Transient ischemia = complete resolution within 1-3 months(2)Stricturing ischemia = incomplete delayed healing narrowed foldless segment of several cm in length with smooth tapering margins(3)Gangrene with necrosis + perforation (extremely uncommon) Notes:


JEJUNOILEAL DIVERTICULAR DISEASE =JEJUNAL DIVERTICULOSIS=rarest form of gastrointestinal diverticular diseaseCause:acquired mucosal herniation (= pulsion diverticulum) Incidence:0.5-2.3% on UGI; 0.3-4.5% of autopsy series; M > FAge:6th-7th decadesLocation:80% in jejunum, 15% in ileum (usually solitary), 5% in jejunum + ileumSite:on mesenteric border near entrance of vasa recti intermittent upper abdominal pain, flatulence, episodes of diarrhea (30%)Plain film: air-fluid levels in multiple diverticula slight dilatation of intestinal loops in area of diverticulaBE: may not fill (narrow neck / stagnant secretions) trapped barium on delayed film after 24 hoursCx: (1)Blind loop syndrome with bacterial overgrowth steatorrhea, diarrhea, malabsorption, weight loss megaloblastic anemia (overgrowth of coliform bacteria leads to deconjugation of bile acids + intraluminal metabolism of vitamin B12)(2)Free perforation = leading cause of pneumo-peritoneum without peritonitis (21-40% mortality)(3)Hemorrhage (few cases)(4)Diverticulitis(5)Intestinal obstruction Notes:


JUVENILE POLYPOSIS =rare autosomal dominant disease with variable penetrance characterized by development of multiple (>5) juvenile polyps in GI tract Most common familial / nonfamilial colonic polyp in children (75%)!Categories: A.Juvenile polyposis of infancyAge:4-6 years (range 1-10 years); M:F = 3:2 protein-losing enteropathy, diarrhea, hemorrhage rectal prolapse intussusceptionB.Colonic & generalized juvenile polyposisAge:in 85% manifested by 20 years of age prolapse of polyp / rectum rectal bleeding, anemia Path:hamartomatous polyps; adenomas may coexistHisto:little / no smooth muscle; hyperplasia of mucous glands; retention cysts develop with obstruction of gland orifices (multiple mucin-filled spaces); edematous inflamed expanded lamina propriaDDx:familial adenomatous polyposis, Peutz-Jeghers syndrome rectal bleeding (95%) most commonly as intermittent bright red hematochezia anemia, pain diarrhea, constipation abdominal pain (from intussusception) rectal prolapse (rare)Location:rectosigmoid (80%); rare in small bowel + stomach; not in esophagus solitary polyp (75%); multiple polyps (1/3) of smooth round contour lesion of pinpoint size / up to several cm in diameter invariably on stalk of variable lengthDx:(1)any number of polyps with family history(2)polyps throughout the GI tract(3)>5-10 polyps in colonCx:colorectal cancer by 35 years of age (in 15%)DDx:solitary juvenile polyps ( F usually asymptomatic (due to slow growth) dysphagia, odynophagia, dyspepsia hematemesis if large (rare)Site:frequently lower + mid 1/3 of esophagus;intramural; multiple leiomyomas in 3-4% 2-15 cm large smooth well-defined intramural mass causing eccentric thickening of wall + deformity of lumen may have coarse calcifications Leiomyoma is the only calcifying esophageal tumor! ulceration uncommonCT: uniform soft-tissue density diffuse contrast enhancementCAVE:high percentage misdiagnosed as extrinsic lesion! Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : LEIOMYOMA

Leiomyoma Of Small Bowel Most common benign tumor of small bowel Location:duodenum (21%), jejunum (48%), ileum (31%); single in 97%Site:mainly serosal (50%), mainly intraluminal (20%), intramural (10%)Size:10 cm (25%) small ulcer + large barium-filled cavity (central necrosis + communication with lumen) hypervascular Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : LEIOMYOMA

Leiomyoma Of Stomach 2nd most common benign gastric tumor (after gastric polyp), most common of calcified benign tumors Location: pars media (39%), antrum (26%), pylorus (12%), fundus (12%), cardia (10%)Site:intraluminal submucosal (60%), exophytic subserosal (35%), combined intramural-extramural dumbbell type mass (5%) average size of 4.5 cm ovoid mass with smooth margin + smooth surface (most frequently) forms right angle with gastric wall ulcerated in 50% pedunculated intraluminal tumor in submucosal growth (rare) "iceberg phenomenon" = large extraluminal component in subserosal growth calcifies in 4%Cx:(1)Hemorrhage (acute / chronic)(2)Obstruction (tumor bulk / intussusception)(3)Infection(4)Fistulization / perforation(5)Malignant degeneration (benign:malignant = 3:1) Notes:

Home : GASTROINTESTINAL TRACT : Gastrointestinal disorders : LEIOMYOSARCOMA

Leiomyosarcoma Of Small Bowel Location:duodenum (26%), jejunum (34%), ileum (40%) usually >6 cm in size nodular mass: intraluminal (10%), intraluminal pedunculated (5%), intramural (15%), chiefly extrinsic (66%) mucosa may be stretched + ulcerated (50%) may show central ulcer pit / fistula communicating with a large necrotic center intussusception Notes:


Leiomyosarcoma Of Stomach Incidence:0.1-3% of all gastric malignanciesAge:10-73 years; M > FHisto:pleomorphism, hypercellularity, mitotic figures, cystic degeneration, necrosis GI bleeding (from ulceration) obstructionMetastases: (a)hematogenous to liver, lung, peritoneum; rarely to bone + soft tissue(b)direct extension into omentum, retroperitoneum(c)lymph nodes (rare)Location:anterior / posterior wall of body of stomach average size of 12 cm intramural mass may be pedunculated large masses tend to be exogastric very frequently ulceratedCT: lobulated irregular outline central zones of low density (necrosis with liquefaction) air / positive contrast within tumor (= ulceration) dystrophic calcifications Notes:


Carney Syndrome Triad of(1)Gastric epitheloid leiomyosarcoma(2)Functioning extraadrenal paraganglioma(3)Pulmonary chondromasIncidence:24 patients reported; M:F = 1:11

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LIPOMA Most common submucosal tumor in colon Incidence:in colon in 0.25% (autopsy)Location:colon (particularly cecum + ascending colon) > duodenum > ileum > stomach > jejunum > esophagus asymptomatic crampy pain, hemorrhage (rare) smooth, sharply outlined, round / ovoid globular mass of 1-3 cm in diameter short thick pedicle in 1/3 caused by repeated peristaltic activity (prone to intussuscept) marked radiolucency change in shape + size on compression due to softness "squeeze sign" = sausage-shaped mass on postevacuation radiographsCT: sharply defined intramural mass of fat densityCx:intussusception (rare) / ulceration (rare)Prognosis:NO liposarcomatous degeneration

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LYMPHANGIOMA =congenital malformation of lymphatic vesselsPath:usually multiloculated large thin-walled cystic mass with chylous / serous / hemorrhagic fluid contentsLocation:mesentery proximal bowel dilatation (in partial bowel obstruction)US: multiseptated cystic mass with lobules fluid anechoic / with internal echoes / sedimentationCT: cystic mass with contents of water- to fat-densityMR: serous contents: hypointense on T1WI + hyperintense on T2WI hemorrhage / fat: hyperintense on T1WI + T2WIRx:surgery (difficult due to intimate attachment to bowel wall) Notes:


LYMPHOGRANULOMA VENEREUM =LGV = sexually transmitted disease caused by virus Chlamydia trachomatis producing a nonspecific granulomatous inflammatory response in infected mucosa (mononuclear cells + macrophages), perirectal lymphatic invasionLocation:rectum, may extend to sigmoid + descending colonM:F = 3.4:1 narrowing + shortening + straightening of rectosigmoid widening of retrorectal space irregularity of mucosa + ulcerations paracolic abscess fistula to pericolic area, rectum, vagina (common)Rx:tetracyclines effective in acute phase before scarring has occurred

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LYMPHOID HYPERPLASIA Incidence:normal variant in 13% of BE examinationsHisto:hyperplastic lymph follicles in lamina propria (Peyer patches), probably compensatory attempt for immunoglobulin deficiencyEtiology: (1)Normal in child / young adult(2)Self-limiting local / systemic inflammation / infection / allergy(3)May be related to immunodeficiency / dysgammaglobulinemia with small bowel involvementAge:(a)generally in children colon > esophagus; multicentric in 10-50% enlargement of spleen bulky enlargement of regional lymph nodes@Esophagusleast common site of GI involvement (in 6 years Location:ileum (51%), jejunum (47%), duodenum (2%),Site:arising from lymphoid patches of PeyerTypes: 1.Infiltrating lymphoma with plaquelike involvement of wall >5 cm in length (80%) / >10 cm in length (20%) (DDx: Crohn disease) ± ulceration (considerable excavation) desmoplastic response thickened valvulae with corrugated appearance aneurysmal dilatation (secondary to destruction of autonomic nerve plexus + muscle / tumor necrosis)2.Single / multiple polypoid mucosal / submucosal masses cobblestone defects due to lymphomatous polyps nodules may ulcerate may cause intussusception sprue pattern3.Endoexoenteric mass large mass with only small intramural component ± ulcer + fistulae + aneurysmatic dilatation4.Mesenteric / retroperitoneal adenopathy single / multiple extraluminal masses displacing bowel ill-defined confluent mass engulfing + encasing multiple loops of adjacent bowel "sandwich configuration" = mass surrounding mesenteric vessels that are separated by perivascular fat conglomerate mantle of retroperitoneal + mesenteric mass@ Colon Less commonly involved than stomach / small bowel; 1.5% of all abdominal lymphomas Location:cecum most commonly involved (85%) single mass > diffuse infiltration > polypoid lesion paradoxical dilatation gross mural circumferential / focal soft-tissue thickening (average size of 5 cm) slight enhancement massive regional + distant mesenteric + retroperitoneal adenopathyDDx:frequently resembles inflammatory disease / polyposis Prognosis:(a)71-82% 2-year survival rate in isolated bowel lymphoma(b)0% 2-year survival rate in stage IV disease with bowel involvement Cx during chemotherapy:perforation (9-40%), hemorrhage

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MALIGNANT MELANOMA =develops from melanocytes derived from neural crest cells, arising in preexisting benign neviIncidence:1% of all cancers @Skin primaryClark staging: LevelIall tumor cells above basement membrane (in situ lesion)LevelIItumor extends to papillary dermisLevelIIItumor extends to interface between papillary + reticular dermisLevelIVtumor extends between bundles of collagen of reticular dermisLevelVtumor invasion of subcutaneous tissue (in 87% metastatic)Breslow staging: thin4 mm depth of invasion METASTASES: latent period of 2-20 years after initial diagnosis (most commonly 2-5 years) Primary site:head + neck (79%), eye (77%), GU system (67%), GI tract (in up to 60%)@Lymphadenopathy-in 23% with level II + IV-in 75% with level V@Bone (11-17%) often initial manifestation of recurrence poor prognosisLocation:axial skeleton (80%), ribs (38%)@Lung (70% at autopsy)most common site of relapse; respiratory failure most common cause of death @Liver (17-23%; 58-66% at autopsy) single / multiple lesions 0.5-15 cm in size larger lesion often necrotic may be partially calcified@Spleen (1-5%; 33% at autopsy) single / multiple lesions of variable size solid / cystic@GI tract + mesentery (4-8%) abdominal pain, GI bleedingLocation:small intestine (35-50%), colon (14-20%), stomach (7-20%) multiple submucosal nodules ± "bulls-eye / target" appearance = central ulceration irregular amorphous cavity (exoenteric growth) intussusception (10-20%)@Kidney (up to 35% at autopsy)@Adrenal (11%, up to 50% at autopsy)@SubcutisPrognosis:30-40% eventually die from this tumor Notes:


MALLORY-WEISS SYNDROME =mucosal + submucosal tear with involvement of venous plexusPathophysiology:violent projection of gastric contents against lower esophagusAge:30-60 years; M > FPredisposed:alcoholics history of repeated vomiting prior to hematemesis massive painless hematemesis Location:at / above / below (76%) esophagogastric junction longitudinal single tear in 77%, in 23% multiple tears extravasation of bariumAngio: bleeding site at gastric cardiaDDx:peptic ulcer / ulcerative gastritis Notes:


MALROTATION =abnormal position of gut secondary to a narrow mesenteric attachment as a result of arrest in the embryologic development of gut rotation + fixation

Embryology: duodenojejunal + ileocolic segments of primitive digestive tube rotate by 270° in a counterclockwise direction about the omphalomesenteric vessels to cross beneath the vessels (future SMA + SMV); LUQ fixation at ligament of Treitz (an extension of the right crus of diaphragm) + fibrous tissue around celiac artery, located to left of L2) + RLQ fixation of cecum Definition:nonrotation < 90°; malrotation = 90-270° Associated with:urinary pseudoobstruction, prune-belly syndrome, cloacal exstrophy Barium meal & barium enema: Purpose:guess the location of abnormal peritoneal fixation from position of bowel! clearly abnormal position of duodenum (81%): duodenum + jejunum to the right of spine (30%) corkscrew duodenum + jejunum (29%) duodenojejunal junction low + in midline (22%) unusual abnormal position of duodenum (16%): duodenojejunal junction over right pedicle duodenojejunal junction to left of spine but low duodenal redundancy to right of spine Z-shape configuration of duodenum + jejunum nonrotation = small bowel on right + colon on left (in 0.2% incidental finding in adults) abnormal position of duodenum + cecum (84%) normal position of duodenum (3%) normal position of cecum (in 5-20%) DDx:mobile cecum (15%) CT: SMV positioned to left of SMA (80%) aplastic / hypoplastic uncinate process of pancreas Cx:midintestinal volvulus, duodenal obstruction, internal herniation Notes:


MASTOCYTOSIS =systemic disease with mast cell proliferation in skin + RES (lamina propria of small bowel; bone; lymph nodes; liver; spleen) associated with eosinophils + lymphocytes Age:85% sensitivity, >95% specificity, >83-88% accuracy): accumulation of radiotracer in right lower quadrant coinciding with uptake of tracer in stomach N.B.:sensitivity drops after adolescence, because patients asymptomatic throughout childhood are less likely to have ectopic gastric mucosa Tc-99m pertechnetate is excreted by mucoid cells of gastric mucosa, excretion is not dependent on presence of parietal cells Preparation: (1)No irritative measures for 48 hours (contrast studies, endoscopy, cathartics, enemas, drugs irritating GI tract) (2)Fasting for 3-6 hours (results in decreased gastric secretion + diminished bowel peristalsis) (3)Evacuation of bowel + bladder prior to study Dose:5-20 mCi (100 µCi/kg) Tc-99m pertechnetate Radiation dose:0.54 rad/2 mCi for thyroid; 0.3 rad/2 mCi for large intestine; 0.2 rad/2 mCi for stomach Imaging:serial images in 5- to 10-minute intervals for 1 hour improved visualization through (a)pentagastrin = stimulates uptake (6 µg/kg SC 20 min prior to pertechnetate) (b)cimetidine = inhibits secretion (maximum 300 mg/dose IV 1 hour prior) (c)glucagon = decreases peristalsis (50 µg/kg IM 5-10 minutes prior) poor visualization with use of perchlorate + atropine (= depressed uptake) False-positive results: (1)Ectopic gastric mucosa in gastrogenic cyst, enteric duplication, normal small bowel, Barrett esophagus (2)Increased blood pool in AVM, hemangioma, hypervascular tumor, aneurysm (3)Duodenal ulcer, ulcerative colitis, Crohn disease, appendicitis, laxative abuse (4)Intussusception, intestinal obstruction, volvulus (5)Urinary tract obstruction, caliceal diverticulum (6)Anterior meningomyelocele (7)Poor technique mnemonic:"HA GUIDI" Hemangioma Appendicitis Gastric ectopia Urinary obstruction Intussusception Duplication of bowel Inflammatory bowel disease False-negative results: (1)Insufficient mass of ectopic gastric mucosa (2)Dilution of intraluminal activity (hemorrhage / hypersecretion) mnemonic:"MIS" Malrotation of ileum Irritable bowel in RLQ (rapid transit) Small amount of ectopic gastric mucosa Enteroclysis: elongated, smoothly marginated, clublike, intraluminal mass parallel to long axis of distal ileum = inverted Meckel diverticulum (20%) 0.5-20-cm-long blind pouch on the antimesenteric border of ileum with junctional fold pattern Angio (59% accuracy): presence of vitelline artery (= anomalous end branch of superior mesenteric artery) is PATHOGNOMONIC Cx (in 20%): (1)GI bleeding secondary to ulceration (in 95% due to ectopic gastric mucosa) (2)Acute diverticulitis (3)Intestinal obstruction secondary to intussusception (diverticulum acts as lead point) / volvulus (when omphalomesenteric diverticulum attached to umbilicus by fibrous band) (4)Malignant tumor (rare): carcinoma, sarcoma, carcinoid (5)Chronic abdominal pain Notes:


MECONIUM ILEUS =small bowel obstruction secondary to desiccated meconium pellets impacted in distal ileum Age:may develop in utero (in 15%) Associated with: cystic fibrosis with thick + sticky meconium due to deficiency of pancreatic secretions (in almost 100%) Earliest clinical manifestation of cystic fibrosis! Virtually all infants with meconium ileus prove to have cystic fibrosis 10-15% of infants with cystic fibrosis present with meconium ileus! abdominal distension, bilious emesis failure to pass meconium within 48 hours numerous dilated small bowel loops without air-fluid levels (fluid not present) "bubbly" / "frothy" appearance of intestinal contents "soap-bubble" / "applesauce" appearance in RLQ (in 50-66%) multiple round / oval filling defects in distal ileum + colon microcolon (unused colon in antenatal obstruction) OB-US: unusual echogenic intraluminal areas in small bowel (DDx: normal transient inspissated meconium) usually polyhydramnios fluid-filled dilated small bowel Cx (in 40-50%):volvulus, ischemia, necrosis, stenosis, atresia, perforation, meconium peritonitis, pseudocyst Rx:(1)Nonionic contrast media enema (because of risk of bowel perforation) (2)17% Hypaque / Conray enema mixed with acetylcysteine (Mucomyst®) (3)Gastrografin® enema with Tween 80 (attention to fluid + electrolyte balance) DDx:Hirschsprung disease, small bowel atresia with meconium ileus, meconium plug syndrome, small left colon syndrome, imperforate anus, obstruction from duplication cyst Notes:


MECONIUM PERITONITIS =sterile chemical peritonitis secondary to perforation of bowel proximal to high-grade / complete obstruction that seals in utero due to inflammatory response Incidence:1:35,000 livebirths Age:antenatal perforation after 3rd month of gestation Cause: (1)Atresia (secondary to ischemic event) (50%) (a)of small bowel (usually ileum or jejunum) (b)of colon (uncommon) (2)Bowel obstruction (46%) (a)meconium ileus (b)volvulus, internal hernia (c)intussusception, congenital bands, Meckel diverticulum (3)Hydrometrocolpos Meconium peritonitis due to cystic fibrosis diagnosed in utero in 8% + at birth in 15-40%! Intraperitoneal meconium may calcify within 24 hours! Types: (a)fibroadhesive type (most common): =intense chemical reaction of peritoneum, which seals off the perforation no evidence for active leak at birth dense mass with calcium deposits calcific plaques scattered throughout peritoneal cavity (b)cystic type: =cystic cavity formed by fixation of bowel loops surrounding the perforation site, which continues to leak meconium cyst outlined by calcific rim (c)generalized type: perforation occurs immediately antenatally active leakage of bowel contents complicated ascites intra-abdominal calcifications (conspicuously absent in cystic fibrosis) peripherally calcified pseudocysts small flecks of calcifications scattered throughout abdomen larger aggregates of calcifications along inferior surface of liver / flank / processus vaginalis / scrotum obstructive roentgen signs following birth separation of bowel loops by fluid microcolon = "unused colon" meconium hydrocele producing labial mass OB-US: polyhydramnios (64-71%) fetal ascites (54-57%) bowel dilatation (27-29%) intra-abdominal bright echogenic mass multiple linear / clumped foci of calcifications (84%); may develop within 12 hours after perforation meconium pseudocyst = well-defined hypoechoic mass surrounded by an echogenic calcified wall (= contained perforation) DDx:(1)Intra-abdominal teratoma (2)Fetal gallstones (3)Isolated liver calcifications Mortality:up to 62% Prognosis:generally good; surgery may not be required when perforation site is completely healed Notes:


MECONIUM PLUG SYNDROME =local inspissation of meconium leading to low colonic obstruction Age:newborn infant (symptomatic within first 24 hours of life) Cause:cystic fibrosis (25%), Hirschsprung disease, prematurity, maternal magnesium sulfate treatment abdominal distension vomiting failure to pass meconium distended transverse + ascending colon + dilated small bowel (proximal to obstruction) occasionally bubbly appearance in colon (DDx: submucosal air in necrotizing enterocolitis) presacral pseudotumor (no gas in rectum) double-contrast effect = barium between meconium plug + colonic wall Rx:water-soluble enema DDx:Hirschsprung disease Notes:


MELANOSIS COLI =benign brown-black discoloration of colonic mucosa Incidence:10% of autopsies Cause:? chronic anthracene cathartic usage malignant potential Notes:

asymptomatic Prognosis:no


MÉNÉTRIÈR DISEASE =GIANT HYPERTROPHIC GASTRITIS =HYPERPLASTIC GASTROPATHY =characterized by excessive mucus production and TRIAD of(1)Giant mucosal hypertrophy (2)Hypoproteinemia (3)Hypochlorhydria Histo:hyperplasia of glandular tissue + microcyst formation, mucosal thickness up to 6 mm (normal range: 0.6-1.0 mm) Age:20-70 years; M:F = 2:1 Associated with: benign gastric ulcer (13-72%) protein-losing enteropathy with hypoproteinemia + peripheral edema weight loss gastrointestinal bleeding absent / decreased acid secretion (>50%) epigastric pain vomiting Location:throughout fundus + body, particularly prominent along greater curvature, antrum usually spared (DDx to lymphoma: usually in antrum) markedly enlarged + tortuous gastric folds in spite of adequate gastric distension relatively abrupt demarcation between normal + abnormal areas marked hypersecretion (mucus) preserved pliability CT: wall thickening of proximal stomach nodular symmetric folds DDx:lymphoma, polypoid variety of gastric carcinoma, acute gastritis, chronic gastritis, gastric varices Notes:


MESENTERIC LYMPHADENITIS =clinical entity whose symptoms relate to benign inflammation of lymph nodes in the bowel mesentery Cause:Yersinia enterocolitica, Y pseudotuberculosis, viral infection Age:children, young adults nausea, vomiting, diarrhea, fever diffuse / RLQ pain + tenderness Location:usually RLQ (immediately anterior to right psoas muscle in 78%, small bowel mesentery in 56%) enlarged mesenteric lymph nodes isolated ileal wall thickening (33%) colonic wall thickening (18%) N.B.:visualization of entire normal appendix is necessary to differentiate from acute appendicitis! DDx:appendicitis (enlarged nodes immediately anterior to right psoas muscle in 40-82%, nodes less numerous + smaller), Crohn disease Notes:


MESENTERIC ISCHEMIA Acute Mesenteric Ischemia Etiology: (a)arterial:atheromatous disease, embolic disease, dissecting aortic aneurysm, fibromuscular hyperplasia, arteritis, endotoxin shock, hypoperfusion (shock, hypovolemia), disseminated intravascular coagulation, direct trauma, radiation -occlusive mesenteric infarction (90% mortality) 1.embolus (40-50%) just distal to middle colic a. 2.SMA thrombosis (20-40%) at origin + site of atherosclerotic narrowing (ostium stenosis) -nonocclusive mesenteric ischemia (10% mortality) = preexisting atherosclerosis with systemic low-flow state (cardiac failure / intraoperative hypotension, bowel vasospasm) (b)venous ( inferior mesenteric vein > portal vein (c)incarceration of hernia, volvulus, constriction by adhesive bands, intussusception Prevalence:5% for SMA; 4% for celiac artery; 11% for inferior mesenteric artery Pathophysiology:mucosa is most sensitive area to anoxia from arterial / venous occlusion with early ulcerations leading to formation of strictures first crampy, then continuous abdominal pain with acute event cardiac disease predisposing to embolization gut emptying (vomiting / diarrhea) WBC >12,000/µl with left shift (80%) gross rectal bleeding Location:(a)any segment of small bowel (b)distal transverse colon, splenic flexure, cecum (most common) Consequences: dependent on magnitude of insult, duration of process, adequacy of collaterals (a)reversible ischemia 1.Complete restitution of bowel wall secondary to abundant collaterals 2.Healing with fibrosis + stricture formation (b)irreversible ischemia 1.Transmural infarction with bowel perforation Plain film: gasless abdomen (= fluid-filled loops from exudation) (21%) bowel distension to splenic flexure (= perfusion territory of SMA) in 43% "thumbprinting" (36%) = thickening of bowel wall + valvulae (edema) small bowel pseudoobstruction (most frequently in thrombosis) pneumatosis = dissection of luminal gas into bowel wall (28%) mesenteric + portal vein gas (14%) ascites (14%) Barium: "scalloping / thumbprinting" = thickening of wall + valvulae "picket fencing" separation + uncoiling of loops narrowed lumen circumferential ulcer CT (26-73-82% sensitive): focal / diffuse bowel dilatation (10-56-71%) with gas (43%) / fluid (29%) portal venous gas (5-13-36%) / mesenteric vein gas (28%) pneumoperitoneum (7%) ascites (43%) mesenteric edema (a)arterial occlusion: thrombosis of SMA (4-18%) pneumatosis intestinalis (22-30%) thumbprinting (26%) = thickening of bowel wall lack of bowel wall enhancement with arterial occlusion (b)venous thrombosis: SMV / portal vein thrombosis (15%) thickened intestinal wall (64%) marked contrast enhancement Angio: occlusion / vasoconstriction / vascular beading embolus lodged at major branching points distal to first 3 cm of SMA NUC: (a)IV / IA Tc-99m sulfur colloid / labeled leukocytes, Ga-citrate, Tc-99m pyrophosphate: tracer accumulation 5 hours after onset of ischemia (more intense uptake with transmural infarcts) (b)intraperitoneal injection of Xe-133 in saline is absorbed by intestine: decreased washout with abnormal perfusion of strangulated bowel Prognosis: (1)Massive infarction of small + large bowel if mesenteric embolization occurs proximal to middle colic artery (= limited collateral flow) (2)Focal segments of intestinal ischemia if mesenteric embolization occurs distal to middle colic artery (= good collateral flow) Mortality:70-80-92% for intestinal infarction Chronic Mesenteric Ischemia =ABDOMINAL ANGINA = intermittent mesenteric ischemia in severe arterial stenosis with inadequate collateralization provoked by food ingestion postprandial abdominal pain 15-20 minutes after food intake (due to "gastric steal" diverting blood flow away from intestine) fear of eating large meals weight loss, malabsorption reflex emptying of bowel after eating Barium: (a)Subacute: flattening of one border pseudosacculation / pseudodiverticula on antimesenteric border (b)Chronic: 7- to 10-cm-long smooth pliable strictures dilatation of gut between strictures thinned + atrophic valvulae Cx:obstruction Duplex US: celiac trunk occlusion + retrograde perfusion of hepatic artery through SMA PSV >300 cm/sec and EDV >45 cm/sec in SMA peak systolic velocity >160 cm/sec in celiac trunk for >50% stenosis (57% sensitivity, 100% specificity) during fasting state Notes:


MESOTHELIAL CYST =MESENTERIC / OMENTAL CYST Etiology:failure of mesothelial peritoneal surfaces to coalesce Path:unilocular thin-walled cyst usually with serous, occasionally chylous / hemorrhagic fluid contents Histo:lined by mesothelial cells + surrounded by thin layer of fibrous tissue Location:small bowel, mesentery (78%), mesocolon asymptomatic single cyst up to several cm in size omental cysts may be pedunculated CT: near-water density / soft-tissue density ± fluid levels related to fat + water components Cx:torsion, hemorrhage, intestinal obstruction DDx:lymphangioma (septations) Notes:


METASTASES TO SMALL BOWEL Origin:colon > stomach > breast > ovary > uterine cervix > melanoma > lung > pancreas Spread: (1)Intraperitoneal seeding: primary mucinous tumor of ovary, appendix, colon; breast cancer (2)Hematogenous dissemination with submucosal deposits: malignant melanoma, breast carcinoma, lung carcinoma, Kaposi sarcoma (3)Direct extension from adjacent neoplasm: ovary, uterus, prostate, pancreas, colon, kidney fixation + tenting + transverse stretching (= across long axis) of folds secondary to mesenteric + peritoneal infiltration (most common form) UGI: single mass protruding into lumen resembling annular carcinoma "bulls-eye" lesions = multiple polypoid masses with sizable ulcer craters obstruction from kinking / annular constriction / large intraluminal mass compression by direct extension of primary tumor / involved nodes CT: soft-tissue density nodules / masses sheets of tissue causing thickening of bowel wall + mesenteric leaves fixation + angulation of bowel loops (in tumors with desmoplastic response) ascites Notes:


METASTASES TO STOMACH Organ of origin:malignant melanoma, breast, lung, colon, prostate, leukemia, secondary lymphoma GI bleeding + anemia (40%) (50%) multiple nodules (30%) linitis plastica (20%): especially breast multiple umbilicated nodules: melanoma Notes:

epigastric pain

solitary mass


MIDGUT VOLVULUS =torsion of entire gut around SMA due to a short mesenteric attachment of small intestine in malrotation Age:neonate / young infant; occasionally older child / adult In 20% associated with:(1)Duodenal atresia (2)Duodenal diaphragm (3)Duodenal stenosis (4)Annular pancreas Pathophysiology: degree of twisting can change due to natural movement of bowel + determines symptomatology; severe volvulus (= twist of 3 and a half turns) causes bowel necrosis acute symptoms in newborn (medical emergency): bile-stained vomiting (intermittent, postprandial, projectile); abdominal distension; shock intermittent obstructive symptoms in older child: recurring attacks of nausea, vomiting, and abdominal pain failure to thrive (hypoproteinemic gastroenteropathy as a result of lymphatic + venous obstruction) Plain film: dilated air-filled duodenal bulb + paucity of gas distally "double bubble sign" = air-fluid levels in stomach + duodenum isolated collection of gas-containing bowel loops distal to obstructed duodenum = gas-filled volvulus = closed-loop obstruction (from nonresorption of intestinal gas secondary to obstruction of mesenteric veins) Barium studies: duodenojejunal junction (ligament of Treitz) located lower than duodenal bulb + to the right of expected position spiral course of midgut loops = "apple-peel / twisted ribbon / corkscrew" appearance (in 81%) duodenal-fold thickening + thumbprinting (mucosal edema + hemorrhage) abnormally high position of cecum CT: whirl-like pattern of small bowel loops + adjacent mesenteric fat converging to the point of torsion (during volvulus) SMV to the left of SMA (NO volvulus) chylous mesenteric cyst (from interference with lymphatic drainage) US: clockwise whirlpool sign = color Doppler depiction of mesenteric vessels moving clockwise with caudal movement of transducer distended proximal duodenum with arrowhead-type compression over spine superior mesenteric vein to the left of SMA thick-walled bowel loops below duodenum + to the right of spine associated with peritoneal fluid Angio: "barber pole sign" = spiraling of SMA tapering / abrupt termination of mesenteric vessels marked vasoconstriction + prolonged contrast transit time absent venous opacification / dilated tortuous superior mesenteric vein Cx:intestinal ischemia + necrosis in distribution of SMA (bloody diarrhea, ileus, abdominal distension) DDx:pyloric stenosis (same age group, no bilious vomiting) Notes:


MUCOCELE OF APPENDIX Mucocele =distension of appendix with sterile mucus Etiology: (a)(perhaps) cystic dilatation of lumen secondary to obstruction by fecolith, foreign body, carcinoid, endometriosis, adhesions, volvulus (b)mucosal hyperplasia (c)mucinous cystadenoma (d)mucinous cystadenocarcinoma Incidence:0.07-0.3% of appendectomies Mean age:55 years; M:F = 1:4 Associated with:colonic adenocarcinoma (6-fold risk), mucin-secreting tumor of ovary asymptomatic (25%) acute / chronic right lower quadrant pain globular, smooth-walled, broad-based mass invaginating into cecum nonfilling of the appendix on BE peripheral rimlike calcifications frequent CT: round sharply defined mass with homogeneous content of near-water / soft-tissue attenuation US: purely cystic / cystic with fine internal echoes / complex cystic mass with high-level echoes gravity-dependent echoes = layering of protein macroaggregates / inspissated mucoid material NUC: intense early gallium uptake (affinity to acid mucopolysaccharides of mucus) Cx:(1)Rupture with pseudomyxoma peritonei (2)Torsion with gangrene + hemorrhage (3)Herniation into cecum with bowel obstruction Myxoglobulosis =rare variant of mucocele of the appendix characterized by clusters of pearly white mucous balls intermixed with mucus usually asymptomatic may appear as acute appendicitis multiple 1- to 10-mm small rounded annular, nonlaminated calcified spherules (PATHOGNOMONIC) DDx:inverted appendiceal stump, acute appendicitis, carcinoma of the cecum Notes:


NECROTIZING ENTEROCOLITIS =NEC = ischemic bowel disease secondary to hypoxia, perinatal stress, infection (endotoxin), congenital heart disease Incidence:most common GI emergency in premature infants Age:develops >48-72 hours after birth; in 90% within first 10 days of life Path:acute inflammation + mucosal ulceration + widespread transmural necrosis Organism:not yet isolated; often occurs in miniepidemics within nursery Predisposed:premature infant (50-80%), Hirschsprung disease, bowel obstruction (small bowel atresia, pyloric stenosis, meconium ileus, meconium plug syndrome) blood-streaked stools (in 50%); explosive diarrhea bile emesis mild respiratory distress generalized sepsis Location:usually in terminal ileum (most commonly involved), cecum, right colon; rarely in stomach, upper bowel disarrayed bowel gas pattern (no longer normal array of polygons) distension of small bowel and colon (loops wider than vertebral body L1) ± air-fluid levels, commonly in RLQ (1st sign) tubular loops of bowel bowel wall thickening + "thumbprinting" "fixed" bowel = persistent abnormal loop of bowel without change on supine vs. prone films / for >24 hours pneumatosis intestinalis (80%) -in curvilinear shape (= subserosal) or -bubbly / cystic (= submucosal gas collection from gasforming organisms / dissection of intraluminal gas) "bubbly" appearance of bowel due to gas in wall / intraluminal gas / fecal matter (intraluminal contents are composed of blood, sloughed colonic mucosa, intraluminal gas, some fecal material) gas in portal venous system (frequently transient, does not imply hopeless outcome) ascites pneumoperitoneum (immediate surgery required) N.B.:Barium enema is contraindicated! May be used judiciously in selected cases with radiologic + clinical doubt! Cx:(1)Inflammatory stricture after healing (BE follow-up in survivors) (2)Bowel perforation in 12-32% Notes:


PELVIC LIPOMATOSIS + FIBROLIPOMATOSIS =nonmalignant overgrowth of adipose tissue with minimal fibrotic + inflammatory components compressing soft-tissue structures within pelvis Age:9-80 years (peak 25-60 years); M:F = 10:1; NO racial predominance for Blacks; obesity NOT contributing factor often incidental finding urinary frequency, flank pain, suprapubic tenderness recurrent urinary tract infections low back pain, fever elongation + narrowing of rectum elevation of rectosigmoid + sigmoid colon out of pelvis increase in sacrorectal space >10 mm stretching of sigmoid colon elongation + elevation of urinary bladder with symmetric inverted pear shape elongation of posterior urethra pelvic lucency; CT confirmatory medial / lateral displacement of ureters Cx of fibrolipomatosis: (1)Ureteral obstruction (40% within 5 years) (2)IVC obstruction Notes:


PERITONEAL MESOTHELIOMA =only primary tumor of peritoneum arising from mesothelial cells lining peritoneal cavity Age:55-66 years; M >> F Associated with:asbestos exposure Spread:intraperitoneal along serosal surfaces; direct invasion of liver, pancreas, bladder, bowel Location:pleura (67%), peritoneum (30-40%), pericardium (2.5%), processus vaginalis (0.5%) thickening of mesentery, omentum, peritoneum, bowel wall nodular masses in anterior parietal peritoneum becoming confluent cakelike disproportionately small amount of ascites areas of calcification (rare) CT: nodular irregular thickening of peritoneal surfaces localized masses infiltrating sheets of tissue foci of calcifications ascites of near-water density stellate configuration of neurovascular bundles pleated thickening of mesenteric leaves NUC: diffuse uptake of gallium-67 Prognosis:extremely poor due to advanced disease at presentation (most patients die within 1 year) Cystic Mesothelioma =rare benign neoplasm without metastatic potential but tendency for local recurrence (in 27-50%) Path:multiple thin-walled cysts lined by mesothelial cells + filled with watery fluid; intermediate form between benign adenomatoid tumor + malignant peritoneal mesothelioma Not associated with asbestos exposure! Median age:37 years; M duodenum) > colon > stomach; mouth + esophagus spared @ Small bowel (>95%) multiple usually broad-based polyps separated by wide areas of intervening flat mucosa multilobulated surface of larger polyps myriad of 1- to 2-mm nodules of up to several cm = carpet of polyps intussusception usually confined to small bowel @ Colon + rectum (30%) multiple scattered 1- to 30-mm polyps; NO carpeting @ Stomach + duodenum (25%) diffuse involvement with multiple polyps @ Respiratory + urinary tract adenoma of bronchus + bladder Cx: (1)Transient intussusception (pedunculated polyp) (2)Carcinoma of GI tract (2-3%) (3)Carcinoma of pancreas (13%) (4)Carcinoma of breast (commonly bilateral + ductal) (5)Ovarian tumor (5%): ovarian sex cord tumor, mucinous cystic tumor, cystadenoma, granulosa cell tumor (6)Endometrial cancer: adenoma malignum of cervix (7)Testicular tumor: feminizing Sertoli cell tumor Rx:(1)Endoscopic removal of all polyps >5 mm (2)Surgery is reserved for obstruction, severe bleeding, malignancy Prognosis:decreased life expectancy (risk of cancer approaching 40% by 40 years of age) DDx:familial adenomatous polyposis, juvenile polyposis (similar age), Cowden syndrome, Cronkhite-Canada syndrome Notes:


POSTCRICOID DEFECT =variable defect seen commonly in the fully distended cervical esophagus; no pathologic value Etiology:redundancy of mucosa over rich postcricoid submucosal venous plexus Incidence:in 80% of normal adults Location:anterior aspect of esophagus at level of cricoid cartilage tumor- / weblike lesion with variable configuration during swallowing DDx:submucosal tumor, esophageal web (persistent configuration) Notes:


POSTINFLAMMATORY POLYPOSIS =PSEUDOPOLYPOSIS =reepithelialized inflammatory polyps as sequelae of mucosal ulceration Etiology:ulcerative colitis (10-20%); granulomatous colitis (less frequent); schistosomiasis (endemic); amebic colitis (occasionally); toxic megacolon Location:most common in left hemicolon, may occur in stomach / small intestine sessile + frondlike appearance (often) filiform polyposis = multiple wormlike projections only attached at their bases (CHARACTERISTIC) Pathogenesis:ulcerative undermining of strips of mucosa with reepithelialization of denuded surfaces of tags + bowel wall Prognosis:NO malignant potential DDx:familial polyposis (polyps terminate in bulbous heads) Notes:


PRESBYESOPHAGUS =defect in primary peristalsis + LES relaxation associated with aging Incidence:15% in 7th decade; 50% in 8th decade; 85% in 9th decade Associated with:hiatus hernia, reflux usually asymptomatic impaired / no primary peristalsis often repetitive nonperistaltic tertiary contractions in distal esophagus mild / moderate esophageal dilatation poor LES relaxation DDx:diabetes, diffuse esophageal spasm, scleroderma, esophagitis, achalasia, benign stricture, carcinoma Notes:


PROGRESSIVE SYSTEMIC SCLEROSIS =PSS = multisystem connective tissue disorder (collagen-vascular disease) of unknown etiology characterized by widespread disorder of the microvasculature causing exuberant interstitial fibrosis with atrophy + sclerosis of many organ systems =SCLERODERMA = variety of skin disorders associated with hardening of skin; by extent of cutaneous involvement divided into: (a)DIFFUSE SCLERODERMA tends to involve older women; interstitial pulmonary fibrosis more severe; organ failure more likely (b)SYSTEMIC SCLEROSIS WITH LIMITED SCLERODERMA (formerly CREST syndrome) CREST features more common; pulmonary arterial hypertension more common + more severe) May be associated with: other connective tissue diseases (especially SLE and polymyositis/dermatomyositis) Cause:autoimmune condition with genetic predisposition, may be initiated by environmental antigen (eg, toxic oil syndrome in Spain through ingestion of adulterated rape seed oil / ingestion of L-tryptophan) Peak age:30-50 years; M:F = 1:3 Histo:vasculitis + submucosal fibrosis extending into muscularis, smooth muscle atrophy (initially hypertrophy and finally atrophy of collagen fibers) CREST:Calcinosis of skin Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia antinuclear antibodies (30-80%): centromere antibody (ACA) specific for limited disease anti-topoisomerase-1 (= antiScl-70) identifies patients with diffuse cutaneous disease antibodies to extracellular matrix proteins and type I + IV collagen rheumatoid factor (35%) LE cells (5%) weakness, generalized debility Prognosis:50-67% 5-year survival rate Gastrointestinal Scleroderma (in 40-45%) Third most common manifestation of scleroderma (after skin changes + Raynaud phenomenon) May precede other manifestations! abdominal pain, diarrhea multiple episodes of pseudoobstruction hepatomegaly @Esophagus (in 42-95%) First GI tract location to be involved! dysphagia (50%) heartburn (30%) normal peristalsis above aortic arch (striated muscle in proximal 1/3 of esophagus) hypotonia / atony + hypokinesia / aperistalsis in lower 2/3 of esophagus (>50%) deficient emptying in recumbent position thin / vanished longitudinal folds mild to moderate dilatation of esophagus chalasia (= patulous lower esophageal sphincter) gastroesophageal reflux (70%) erosions + superficial ulcers (from asymptomatic reflux esophagitis: NO protective esophageal contraction) fusiform stricture usually 4-5 cm above gastroesophageal junction (from reflux esophagitis) esophageal shortening + sliding hiatal hernia Cx:peptic stricture, aspiration, Barrett esophagus, adenocarcinoma @ Stomach (less frequent involvement) gastric dilatation decreased motor activity + delayed emptying @ Small bowel (in up to 45%) PSS is rapidly progressing once small intestine is involved! malabsorption (delayed intestinal transit time + bacterial overgrowth) marked dilatation of small bowel (in particular duodenum = megaduodenum, jejunum) simulating small bowel obstruction CAVE:misdiagnosis of obstruction may lead to exploratory surgery! abrupt cutoff at SMA level (atrophy of neural cells with hypoperistalsis) prolonged transit time with barium retention in duodenum up to 24 hours "hidebound / accordion" pattern (60%) = sharply defined folds of normal thickness with decreased intervalvular distance (tightly packed folds) within dilated segment (due to predominant involvement of circular muscle) pseudodiverticula (10-40%) = asymmetric sacculations with squared tops + broad bases on mesenteric side (due to eccentric smooth muscle atrophy) pneumatosis cystoides intestinalis + pneumoperitoneum (occasionally) excess fluid with bacterial overgrowth (= "pseudo-blind loop syndrome") normal mucosal fold pattern Cx:intussusception without anatomic lead point @ Colon (up to 40-50%) constipation (common), may alternate with diarrhea pseudosacculations + wide-mouthed "diverticula" on antimesenteric side (formed by repetitive bulging through atrophic areas) in transverse + descending colon eventually complete loss of haustrations (simulating cathartic colon) marked dilatation (may simulate Hirschsprung disease) stercoral ulceration (from retained fecal material) Cx:life-threatening barium impaction DDx:(1)Dermatomyositis (similar radiographic findings) (2)Sprue (increased secretions, segmentation, fragmentation, dilatation most significant in midjejunum, normal motility) (3)Obstruction (no esophageal changes, no pseudodiverticula) (4)Idiopathic intestinal pseudoobstruction (usually in young people) Pulmonary Scleroderma (in 10-25%) Path:almost 100% involvement in autopsy series Histo:thickening of basement membrane of alveoli + small arteries and veins slightly productive cough + progressive dyspnea hematemesis pulmonary function abnormalities in the absence of frank roentgenographic changes (typical dissociation of clinical, functional, and radiologic evidence) pericarditis Location:most prominent at both lung bases (where blood flow greatest) fine / coarse reticulations / diffuse interstitial infiltrates subpleural fibrocystic spaces (honeycombing) low lung volumes from progressive volume loss alveolar changes (secondary to aspiration of refluxed gastric contents with disturbed esophageal motility / mineral oil taken to combat constipation) air esophagram (DDx: achalasia, mediastinitis) pleural reaction / effusion distinctly uncommon Cx:(1)Pulmonary arterial hypertension (6-60%) (2)Increased incidence of lung cancer @ Heart: sclerosis of cardiac muscle ± cor pulmonale Renal Scleroderma (25%) Onset:common within 3 years Histo:fibrinoid necrosis of afferent arterioles (also seen in malignant hypertension) renal cortical necrosis spotty inhomogeneous nephrogram (constriction + occlusion of arteries) concomitant arterial ectasia Cx:renal failure (from nephrosclerosis) Musculoskeletal Scleroderma edema of distal portion of extremities thickened inelastic waxy skin most prominent about face + extremities symmetrical polyarthralgias (50-80%) Raynaud phenomenon (may proceed other symptoms by months / years) atrophy + thickening of skin and musculature (78%) @Fingers "sausage digit" = edema of digits associated with loss of transverse skin folds + lack of definition of subcutaneous fat "tapered fingers" = sclerodactyly = atrophy + resorption of soft tissues of fingertips + soft-tissue calcifications acroosteolysis = "penciling" / "autoamputation" = resorption of distal phalanges of hand (63%) beginning at volar aspect of terminal tufts with proximal progression calcinosis (25%) = punctate soft-tissue calcifications of fingertips, axilla, ischial tuberosity, forearm, elbow (over pressure area), lower leg, face calcifications around tendons. bursae, within joints @Arthritis stiffness in small joints, occasionally in knee, shoulder, wrist lack of motility, eventually contractures arthritis of interphalangeal joints of hands (25%) Location:1st CMC, MCP, DIP, PIP central / marginal erosions (50%) resorption of palmar aspect of terminal phalanges (most frequent sign) bony erosions of carpal bones (trapezium), distal radius + ulna, mandible, ribs, lateral aspect of clavicle, humerus, acromion, mandible, cervical spine joint-space narrowing (late) DDx:rheumatoid, psoriatic, erosive arthritis soft-tissue swelling ± periarticular osteoporosis NO significant osteoporosis ± flexion contractures of fingers (from tendon sheath inflammation + fibrosis) erosion of superior aspect of ribs widening of periodontal membrane Notes:


PROLAPSED ANTRAL MUCOSA =prolapse of hypertrophic + inflammatory mucosa of gastric antrum into duodenum resulting in pyloric obstruction mushroom- / umbrella- / cauliflower-shaped filling defect at duodenal base filling defect varies in size + shape redundant gastric rugae can be traced from pyloric antrum through pyloric channel gastric hyperperistalsis Notes:


PSEUDOMEMBRANOUS COLITIS =CLOSTRIDIUM DIFFICILE DISEASE (more appropriate name because pseudomembranes are uncommon) Cause:overgrowth of Gram-positive Clostridium difficile in response to a decrease in normal intestinal flora Etiologic agent:cytotoxin produced by C. difficile Predisposed: (a)complication of antibiotic therapy with tetracycline, penicillin, ampicillin, clindamycin, lincomycin, amoxicillin, chloramphenicol, cephalosporins (b)complication of some chemotherapeutic agents: methotrexate, fluorouracil (c)following surgery / renal transplantation / irradiation; intestinal vascular insufficiency (d)shock, uremia (e)proximal to large bowel obstruction (f)debilitating diseases: lymphosarcoma, leukemia (g)immunosuppressive therapy with actinomycin D Histo:pseudomembranes (exudate composed of leukocytes, fibrin, mucin, sloughed necrotic epithelium held in columns by strands of mucus) on a partially denuded colonic edematous mucosa (mucosa generally intact); reactive edema in lamina propria, submucosa, and eventually subserosa profuse watery diarrhea, abdominal cramps, tenderness fever, fecal blood, leukocytosis less common: chronic diarrhea, toxic megacolon, hyperpyrexia, leukemoid reaction, hypoalbuminemia with anasarca confluent small yellow plaques (= pseudomembranes) adherent to mucosal surface seen on endoscopy (50%) Location:rectum (95%); confined to right + transverse colon (5-27%) Plain film: adynamic ileus pattern = moderate gaseous distension of small bowel + colon "transverse banding" = marked thickening + distortion of haustral folds "thumbprinting" most prominent in transverse colon diffusely shaggy + irregular surface (confluent pseudomembranes) BE (CONTRAINDICATED in severe cases): "accordion-like" haustral thickening = contrast material trapped between distorted thickened closely spaced transverse edematous folds (simulating intramural tracts) pseudoulcerations = barium filling clefts between pseudomembranes irregular ragged polypoid contour of colonic wall discrete multiple plaquelike lesions of 2-4 mm in size (DDx: polyposis, nodular form of lymphoma) N.B.:Risk of colonic perforation! CT (85% sensitive, 48% specific): colonic wall thickening of 4-22 mm (61-88%) smooth circumferential thickening (44%) accordion sign (51-70%) = alternating bands of edematous haustral folds separated by intraluminal contrast material nodular thickening (17%) homogeneous enhancement due to hyperemia pericolonic stranding (42%) ascites (15-25%) NO colonic abnormality (12-39%) Dx:(1)Stool assay for Clostridium difficile cytotoxin (detects toxin B): cumbersome to perform (2)Enzyme immunoassay test (up to 33% false-negative results): detects toxin A + B (3)Stool culture (95% sensitive): not available for 2 days (4)Pseudomembranes on proctosigmoidoscopy Cx:peritonitis Prognosis:15% mortality; most patients recover within 2 weeks Rx:discontinuation of suspected antibiotic + administration of vancomycin / metronidazole with attention to fluid and electrolyte balance Notes:


PSEUDOMYXOMA PERITONEI ="jelly belly" = "gelatinous ascites" = slow insidious accumulation of large amounts of intraperitoneal gelatinous material Etiology:rupture of mucinous cystadenoma / cystadenocarcinoma of appendix (male) / ovary (female); rarely associated with malignancy of colon (1-2 years following irradiation Predisposed:women (cancer of cervix, endometrium, ovary), patients with bladder cancer crampy abdominal pain (from intermittent obstruction) persistent diarrhea occult intestinal hemorrhage Location:ileum; concomitant radiation damage to colon / rectum irregular nodular thickening of folds with straight transverse course ± ulcerations serrated bowel margin thickened bowel wall with luminal narrowing multiple strictures + partial mechanical obstruction separation of adjacent bowel loops by >2 mm shortening of small bowel fixation + immobilization of bowel loops with similar radiographic appearance between examinations (from dense desmoplastic response to irradiation) CT: increased attenuation of mesentery DDx:Crohn disease, lymphoma, ischemia, hemorrhage Radiation Injury Of Rectum Manifestation of radiation colitis can occur up to 15 years following irradiation Predisposed:90% in women (carcinoma of cervix) tenesmus, diarrhea, bleeding, constipation ridgelike appearance of mucosa (submucosal fibrosis) irregularly outlined ulcerations (rare) CT: narrowed partially distensible rectum thick homogeneous rectal wall "target sign" = submucosal circumferential lucency proliferation of perirectal fat >10 mm thickening of perirectal fascia "halo sign" = increase in pararectal fibrosis Cx:(1) Obstruction (2) Colovaginal / coloenteric fistula formation Notes:


RETAINED GASTRIC ANTRUM Cause:retention of endocrinologically active gastric antrum in continuity with pylorus + duodenum Pathophysiology:bathing of antrum in alkaline duodenal juice stimulates secretion of gastrin Associated with:gastric ulcers in 30-50% duodenogastric reflux of barium through pylorus (diagnostic) giant marginal ulcer / several marginal ulcers usually on jejunal side of anastomosis (large false-negative + false-positive rates; correct-positive rate of 28-60%) large amount of secretions edematous mucosa of jejunal anastomotic segment lacy / cobweblike small bowel pattern (hypersecretion) Cx:gastrojejunocolic fistula Notes:


RETRACTILE MESENTERITIS =CHRONIC FIBROSING MESENTERITIS = CHRONIC SUBPERITONEAL SCLEROSIS = MESENTERIC PANNICULITIS = LIPOSCLEROTIC MESENTERITIS = MESENTERIC LIPODYSTROPHY = MESENTERIC WEBER-CHRISTIAN DISEASE =rare disorder of unknown etiology characterized by fibrofatty thickening of small bowel mesentery Etiology:? trauma, previous surgery, ischemia Path:spectrum ranging from mesenteric lipodystrophy through mesenteric panniculitis to mesenteric fibrosis Histo:chronic inflammation with a dense collection of lymphocytes + plasma cells + lipid-laden macrophages; desmoplastic reaction; fat necrosis; calcifications Associated with: (1)Gardner syndrome, familial polyposis (2)Fibrosing mediastinitis, retroperitoneal fibrosis (3)Lymphoma, lymphosarcoma (4)Carcinoid tumor (5)Metastatic gastric / colonic carcinoma (6)Whipple lipodystrophy (7)Weber-Christian disease Age:most common in 6th decade; M:F = 2:1 crampy abdominal pain nausea + vomiting; mild weight loss low-grade fever Location:root of mesentery extending toward mesenteric border of bowel Plain film: soft-tissue mass with calcifications ± thumbprinting (from vascular congestion) UGI: compression / distortion of duodenum near ligament of Treitz separation of small bowel loops with fixation, kinking, and angulation CT: mass of fat density interspersed with soft-tissue density (fibrous tissue) + calcifications mesenteric thickening with fine stellate pattern extending to bowel border retraction of small bowel loops single mesenteric soft-tissue mass (fibroma) multiple nodules throughout mesentery (fibromatosis) Prognosis:usually benign course DDx:metastatic gastric / colonic adenocarcinoma; carcinoid tumor; mesenteric lymphoma; liposarcoma of mesentery Notes:


SCHATZKI RING =LOWER ESOPHAGEAL MUCOSAL RING = constant lower esophageal ring (mucosal thickening) presumed to result from reflux esophagitis = thin annular peptic stricture Incidence:6-14% of population; old age > young age; M > F Histo:usually squamous epithelium on upper surface + columnar epithelium on undersurface; may be covered totally by squamous epithelium or columnar epithelium asymptomatic (if ring >20 mm) dysphagia (if ring remainder of small bowel Small bowel follow-through: small bowel dilatation is HALLMARK in untreated celiac disease (70-95%), best seen in mid + distal jejunum (due to intestinal hypomotility); degree of dilatation related to severity of disease hypersecretion-related artifacts: air-fluid levels in small bowel (rare) segmentation = breakup of normal continual column of barium creating large masses of barium in dilated segments separated by stringlike strands from adjacent clumps due to excessive fluid; best seen on delayed films flocculation = coarse granular appearance of small clumps of disintegrated barium due to excess fluid best seen at periphery of intestinal segment; occurs especially with steatorrhea fragmentation = scattering = faint irregular stippling of residual barium resembling snowflakes associated with segmentation due to excessive fluid "moulage sign" (50%) = smooth contour with effaced featureless folds resembling tubular wax mold (due to atrophy of the folds of Kerckring); CHARACTERISTIC of sprue if seen in duodenum + jejunum long / normal / short transit time nonpropulsive peristalsis (flaccid + poorly contracting loops) normal / thickened / effaced mucosal folds (depending on degree of hypoproteinemia) colonlike haustrations in well-filled jejunum (secondary to spasm + cicatrization from transverse ulcers) "jejunization" of ileal loops (= adaptive response to decreased jejunal mucosal surface) = SPECIFIC transient nonobstructive intussusception (20%) without anatomic lead point "bubbly bulb" = peptic duodenitis = mucosal inflammation, gastric metaplasia, Brunner gland hyperplasia Enteroclysis: decreased number of folds in proximal jejunum (5 folds per inch) tubular featureless lumen mosaic pattern = 1-2 mm polygonal islands of mucosa surrounded by barium-filled distinct grooves (10%) CT: small bowel dilatation + increased fluid content ± mucosal fold thickening mild to moderate lymphadenopathy in mesentery / retroperitoneum (up to 12%) Dx:(1)Jejunal / duodenal biopsy (2)Improvement of small bowel abnormalities after a few months on a gluten-free diet Cause for relapse:hidden dietary gluten, diabetes, bacterial overgrowth, intestinal ulceration, development of lymphoma Cx: (1)Ulcerative jejunoileitis =multiple chronic benign ulcers (sausage appearance of small bowel) with hemorrhage, perforation + obstruction Age:5th-6th decade Location:jejunum > ileum > colon response to gluten-free diet ceases Prognosis:frequently fatal Rx:small bowel resection (2)Hyposplenism (30-50%) small atrophic spleen (3)Cavitary mesenteric lymph node syndrome characterized by: (a)mesenteric lymph node cavitation (b)splenic atrophy (c)villous atrophy of small intestinal mucosa enlarged lymph nodes of low attenuation ± fat-fluid levels (filled with lipid-rich hyaline material) within jejunoileal mesentery Prognosis:usually fatal disorder (4)Malignant tumors (a)lymphoma (in 8%): commonly diffuse + nodular and of C-cell type Peak prevalence:7th decade enlarged nodular folds, ulcers, extrinsic mass effect (b)adenocarcinoma of small bowel (6%), rectum, stomach (c)squamous cell carcinoma of pharynx / esophagus (in 6%) during 6th-7th decade (5)Generalized lymphadenopathy with lymphocytosis (mimicking lymphoma) (6)Sigmoid volvulus (rare) DDx: (1)Esophageal hypoperistalsis: scleroderma, idiopathic pseudoobstruction (2)Gastric abnormalities: Zollinger-Ellison syndrome, chronic granulomatous disease, eosinophilic enteritis, amyloidosis, malignancy (3)Tiny nodular defects on thickened folds: Whipple disease, intestinal lymphangiectasia, Waldenström macroglobulinemia (4)Small 1- to 3-mm nodules: lymphoid hyperplasia associated with giardiasis and immunoglobulin deficiency disease, diffuse lymphoma (5)Small nodules of varying sizes: systemic mastocytosis, amyloidosis, eosinophilic enteritis, Cronkhite-Canada syndrome (6)Bowel wall narrowing, kinking, scarring, ulceration: regional enteritis, bacterial / parasitic infection, carcinoid, vasculitis, ischemia, irradiation Notes:


STRONGYLOIDIASIS Organism:helminthic parasite Strongyloides stercoralis (2.2 mm long, 50µm in diameter); capable of reproducing within human host Prevalence:100 million cases globally; 4% in U.S. Country:tropical + subtropical regions, parts of Europe, southeastern U.S. (eastern Kentucky, rural Tennessee), Puerto Rico Infection:filiform larva enters body through skin / mucous membranes (from contaminated soil) Cycle:larva passes from subcutaneous / submucosal sites via venous circulation to lung; larva breaks into alveolar spaces and ascends via bronchi + trachea; larva swallowed; settles in duodenum + upper jejunum (lives in tunnels between enterocytes); parasitic adult female worms release eggs containing mature larvae into the intestinal lumen; ova hatch immediately into rhabditiform larvae and are passed to the environment Path:edema + inflammation of intestinal wall secondary to invasion by larvae; flattening of villi; ova in mucosal crypts asymptomatic for many years (in majority) larva currens = recurrent allergic cutaneous skin lesions of autoinfection severe malnutrition (malabsorption, steatorrhea) weight loss worms, larvae, eggs in stool peripheral eosinophilia elevated levels of immunoglobulin E paralytic ileus (massive invasion) edematous irregular mucosal folds, spasm, dilatation of proximal 2/3 of duodenum ulcerations stricture of 3rd + 4th part of duodenum rigid pipestem appearance + irregular narrowing of duodenum (in advanced cases) Rx:thiabendazole (90% efficacy rate) Prognosis:high mortality in undernourished patients HYPERINFECTION SYNDROME = extensive tissue invasion by larvae in patients with malignancy, autoimmune disease, malnutrition bacteremia, septicemia crampy abdominal pain, persistent vomiting, diarrhea CXR: fine miliary nodules / diffuse reticular opacities Notes:


SUPERIOR MESENTERIC ARTERY SYNDROME =VASCULAR COMPRESSION OF DUODENUM =WILKIE SYNDROME = CHRONIC DUODENAL ILEUS =BODY CAST SYNDROME =vascular compression of 3rd portion of duodenum within aortomesenteric compartment; probably representing a functional reflex dilatation Etiology:narrowing of angle between SMA + aorta to 10-22° (normal 45-65°): congenital, weight loss, visceroptosis due to loss of abdominal muscle tone (as in pregnancy), asthenic built, exaggerated lumbar lordosis, prolonged bed rest in supine position (body cast, whole-body burns, surgery) repetitive vomiting abdominal cramping megaduodenum = pronounced dilatation of 1st + 2nd portion of duodenum + frequently stomach, best seen in supine position vertical linear compression defect in transverse portion of duodenum overlying spine abrupt change in caliber distal to compression defect relief of compression by postural change into prone knee-elbow position Notes:


TAILGUT CYST =RETRORECTAL CYSTIC HAMARTOMA Cause:incomplete regression of embryonic tailgut (= the portion distal to future anus) Average age:35 years; M5.5 cm in transverse colon (damage to entire colonic wall + neuromuscular degeneration) Etiology: 1.Ulcerative colitis (most common) 2.Crohn disease 3.Amebiasis, salmonellosis 4.Pseudomembranous colitis 5.Ischemic colitis Histo:widespread sloughing of mucosa + thinning of frequently necrotic muscle layers systemic toxicity profuse bloody diarrhea colonic ileus with marked dilatation of transverse colon few air-fluid levels increasing caliber of colon on serial radiographs without redundancy loss of normal colonic haustra + interhaustral folds coarsely irregular mucosal surface pseudopolyposis = mucosal islands in denuded ulcerated colonic wall pneumatosis coli ± pneumoperitoneum CT: distended colon filled with large amounts of fluid + air distorted haustral pattern irregular nodular contour of thin wall intramural air / small collections BE:CONTRAINDICATED due to risk of perforation Prognosis:20% mortality Notes:


TUBERCULOSIS Rarely encountered in Western Hemisphere, increased incidence in AIDS; usually associated with pulmonary tuberculosis (in 6-38%) Etiology: (1)Ingestion of tuberculous sputum (2)Hematogenous spread from tuberculous focus in lung to submucosal lymph nodes, associated with radiographic evidence of pulmonary TB in ascending colon > jejunum > appendix > duodenum > stomach > sigmoid > rectum @Tuberculous peritonitis (in 1/3) Most common presentation Cause:hematogenous spread / rupture of mesenteric node (a)wet type = exudative ascites with high protein contents + leukocytes (b)dry type = caseous adenopathy + adhesions (c)fibrotic type = omental cakelike mass with separation + fixation of bowel loops CT: high-density ascites (20-45 HU) enlarged lymph nodes (90%) with low-density centers in 40% (due to caseous necrosis) Location:peripancreatic + mesentery, retroperitoneum irregular masses of soft-tissue density in omentum + mesentery (common) Cx:small bowel obstruction (adhesions from serosal tubercles) @ Ileocecal area (80-90%) Most commonly affected bowel Cause:relative stagnation of intestinal contents + abundance of lymphoid tissue (Peyer patches) Stierlin sign = rapid emptying of narrowed terminal ileum (due to persistent irritability) on BE thickened ileocecal valve (mass effect) Fleischner sign = "inverted umbrella" defect = wide gaping patulous ileocecal valve associated with narrowing of the immediately adjacent ileum + narrowed rigid cecum deep fissures + ulcers with sinus tracts / enterocutaneous fistulas / perforation DDx:Crohn disease, cecal carcinoma @Colon Site:segmental colonic involvement, esp. on right side rigid contracted cone-shaped cecum (spasm / transmural fibrosis) spiculations + wall thickening diffuse ulcerating colitis + pseudopolyps shortening + short hourglass strictures DDx:ulcerative colitis, Crohn disease, amebiasis (spares terminal ileum), colitis of bacillary dysentery, ischemic colitis, pseudomembranous colitis @Gastroduodenal Site:simultaneous involvement of pylorus + duodenum stenotic pylorus with gastric outlet obstruction narrowed antrum (linitis plastica appearance) antral fistula multiple large and deep ulcerations on lesser curvature thickened duodenal folds with irregular contour / dilatation DDx:carcinoma, lymphoma, syphilis @Esophagus Least common GI tract manifestation Cause:secondary involvement from adjacent tuberculous lymphadenitis / primary TB deep ulceration stricture mass intramural dissection / fistula formation =sinus tract formation Notes:


TURCOT SYNDROME =autosomal recessive disease with (a)colonic polyposis (b)CNS tumors (especially supratentorial glioblastoma, occasionally medulloblastoma) Age:symptomatic during 2nd decade Histo:adenomatous polyps diarrhea seizures multiple 1-30 mm polyps in colon + rectum Cx:malignant transformation of colonic polyps in 100% Prognosis:death from brain tumor in 2nd + 3rd decade Notes:


TYPHLITIS =ILEOCECAL SYNDROME = NEUTROPENIC COLITIS =acute inflammation of cecum, appendix, and occasionally terminal ileum; initially described in children with leukemia + severe neutropenia; typhlos = "blind sac" = cecum Cause:leukemic / lymphomatous infiltrate, ischemia, focal pseudomembranous colitis, infection Histo:edema + ulceration of entire bowel wall; transmural necrosis with perforation possible Organism:CMV, Pseudomonas, Candida, Klebsiella, E. coli, B. fragilis, Enterobacter Predisposed:common in childhood leukemia, aplastic anemia, lymphoma, immunosuppressive therapy (eg, renal transplant), clinical AIDS abdominal pain, may be localized to RLQ watery diarrhea fullness / palpable mass in RLQ fever, neutropenia hematochezia / occult blood Location:cecum + ascending colon, appendix + distal ileum may become secondarily involved fluid-filled masslike density in RLQ distension of nearby small bowel loops thumbprinting of ascending colon circumferential thickening of cecal wall >4 mm occasionally pneumatosis CT (preferable examination due to risk of perforation): circumferential wall thickening (>1-3 mm) of cecum ± terminal ileum decreased bowel wall attenuation (edema) increased attenuation of adjacent fat + thickening of fascial planes (pericolonic inflammation) ± pericolonic fluid + intramural pneumatosis Cx:(1)Perforation (BE is a risky procedure) (2)Abscess formation Rx:early aggressive medical support (high doses of antibiotics + IV fluids) prior to development of transmural necrosis DDx:(1)Leukemic / lymphomatous deposits (more eccentric thickening) (2)Appendicitis with periappendicular abscess (normal cecal wall thickness) (3)Diverticulitis (4)Inflammatory bowel disease Notes:


ULCERATIVE COLITIS =common idiopathic inflammatory bowel disease with continuous concentric + symmetric colonic involvement Etiology:? hypersensitivity / autoimmune disease Prevalence:50-80:100,000 In high incidence areas of North America, Northern Europe, Australia Path:predominantly mucosal + submucosal disease with exudate + edema + crypt abscesses (HALLMARK) resulting in shallow ulceration Age peak:20-40 years + 60-70 years; M:F = 1:1 alternating periods of remission + exacerbation bloody diarrhea electrolyte depletion, fever, systemic toxicity abdominal cramps Extracolonic manifestations: iritis, erythema nodosum, pyoderma gangrenosum pericholangitis, chronic active hepatitis, primary sclerosing cholangitis, fatty liver spondylitis, peripheral arthritis, coincidental rheumatoid arthritis (10-20%) thrombotic complications Location:begins in rectum with proximal progression (rectum spared in 4%) (a)rectosigmoid in 95% (diagnosed by rectal biopsy); continuous circumferential involvement often limited to left side of colon (b)colitis extending proximally to splenic flexure = universal colitis (c)terminal ileum in 10-25% ("backwash ileitis") Plain film: hyperplastic mucosa, polypoid mucosa, deep ulcers diffuse dilatation with loss of haustral markings toxic megacolon free intraperitoneal gas complete absence of fecal residue (due to inflammation) BE: (a)acute stage narrowing + incomplete filling (spasm + irritability) fine mucosal granularity = stippling of barium coat (from diffuse mucosal edema + hyperemia + superficial erosions) spicules + serrated bowel margins (tiny superficial ulcers) "collar button" ulcers (= undermining of ulcers) "double-tracking" = longitudinal submucosal ulceration over several cm hazy / fuzzy quality of bowel contour (excessive secretions) "thumbprinting" = symmetric thickening of colonic folds pseudopolyps = scattered islands of edematous mucosa + reepithelialized granulation tissue within areas of denuded mucosa widening of presacral space obliterated rectal folds = valves of Houston (43%) (b)subacute stage distorted irregular haustra inflammatory polyps = sessile frondlike / rarely pedunculated lesions (= localized mucosal inflammation resulting in polypoid protuberance) coarse granular mucosa (= mucosal replacement by granulation tissue) (c)chronic stage shortening of colon (= reversible spasm of longitudinal muscle) with depression of flexures "leadpipe" colon = rigidity + symmetric narrowing of lumen widening of haustral clefts / complete loss of haustrations (DDx: cathartic colon) "burnt-out colon" = fairly distensible colon without haustral markings + without mucosal pattern hazy / fuzzy quality of bowel contour (excessive secretions) postinflammatory polyps (12-19%) = small sessile nodules / long wormlike branching + bridging outgrowths (= filiform polyposis) "backwash ileitis" (5-30%) involving 4-25 cm of terminal ileum with patulous ileocecal valve + absent peristalsis + granularity CT: wall thickening 75% of tumor, diagnosis can be made on BE) apparent decrease in size on postevacuation films Cx:malignant transformation / invasion (in 36%) related to size of tumor 5 cm (55%); >10 cm (100%) Villous Adenoma Of Duodenum More common in colon + rectum; fewer than 50 cases in world literature sessile, soft nonobstructive mass "lace" / "soap bubble" pattern preservation of peristaltic activity + bowel distensibility Notes:


WALDENSTRÖM MACROGLOBULINEMIA =low-grade lymphoid malignancy composed of mature plasmacytoid lymphocytes with production of abnormal monoclonal IgM protein Incidence:0.53 / 100,000 annually; frequency 10-15% that of multiple myeloma Histo:macroglobulin proteinaceous hyaline material fills lacteals in lamina propria of small bowel villi with secondary lymphatic distension + edema Mean age:63 years; M > F fatigue, weight loss diarrhea, steatorrhea, malabsorption anemia, bleeding diathesis IgM elevation hyperviscosity syndrome (20%) = bleeding, visual changes, neurologic abnormalities @Small bowel (rarely involved) small bowel dilatation uniform diffuse thickening of valvulae conniventes with spikelike configuration (jejunum + proximal ileum) granular surface of punctate filling defects (distended villi) @Bone marrow involvement (91-98%) (a)diffuse replacement of bone marrow (56%) (b)variegated replacement of bone marrow (35%) compression fractures of spine (48%) diffuse demineralization of spine lytic lesions on bone surveys (in up to 20%) MR (pre- and postcontrast T1WI preferred): marrow iso- / hypointense to muscle on T1WI enhancement of abnormal marrow on T1WI @Lymph nodes lymphadenopathy (43%) @Liver & spleen hepatosplenomegaly Dx:(1)characteristic M-spike in serum / urine electrophoresis (2)abnormal lymphplasmacytoid cells in bone marrow / lymph nodes DDx:multiple myeloma (lymphadenopathy rare, lytic lesions in 31%) Notes:


WHIPPLE DISEASE =INTESTINAL LIPODYSTROPHY =sporadically occurring chronic multisystem disease Etiology:thought to be caused by infection with an as yet unidentified gram-positive bacterium (Tropheryma whippelii) closely related to actinobacteria Histo:PAS-positive material (periodic acid Schiff) = glycoprotein within foamy macrophages in the submucosa of the jejunum (bacterial cell wall) + fat deposits within intestinal submucosa and lymph nodes causing lymphatic obstruction + dilatation Age:4th-6th decade (mean age of onset, 50 years); M:F = 8:1; Caucasians recurrent and migratory arthralgias / nondeforming arthritis (65-95%); arthritis may precede Whipple disease in 10% up to 10 years malabsorption, steatorrhea, abdominal pain weight loss, low-grade fever polyserositis generalized peripheral lymphadenopathy (50%) hyperpigmentation of skin similar to Addison disease pale shaggy yellow plaques / erosions in postbulbar duodenum on endoscopy Organ involvement:virtually every organ system, liver, intestines, joints, heart, lung, CNS, eyes, skin moderate thickening of jejunal + duodenal folds (from mucosal + submucosal infiltration by PAS-positive macrophages combined with lymphatic obstruction) micronodularity (= swollen villi) and wild mucosal pattern hypersecretion, segmentation, fragmentation (occasionally if accompanied by hyperproteinemia) NO / minimal dilatation of small bowel NO rigidity of folds NO ulcerations normal transit time (approximately 3 hours) hepatosplenomegaly CT: bulky 3-4 cm large low-density lymph nodes in mesenteric root + retroperitoneum (due to extracellular neutral fat + fatty acids) thickening of bowel wall splenomegaly ascites pleuropericarditis sacroiliitis Dx:endoscopically guided biopsy of small bowel mucosa, abdominal / peripheral lymph node biopsy Rx:long-term broad-spectrum antibiotics (tetracycline) DDx:(1)Sprue (marked dilatation, no fold thickening, pronounced segmentation + fragmentation) (2)Intestinal lymphangiectasia (thickened folds throughout small bowel) (3)Amyloidosis (4)Lymphoma Pseudo-Whipple disease in AIDS similar clinical picture caused by Mycobacterium avium intracellulare lymphadenopathy Notes:

wall + fold thickening of small bowel loops

mesenteric


ZENKER DIVERTICULUM =PHARYNGOESOPHAGEAL DIVERTICULUM =outpouching of posterior hypopharyngeal wall = pulsion diverticulum with herniation of mucosa + submucosa through oblique + transverse muscle bundles (pseudodiverticulum) of the cricopharyngeal muscle Prevalence:0.01-0.11% (overall); higher in elderly women (50% occur in 7th-8th decade) Etiology:cricopharyngeal dysfunction (cricopharyngeal achalasia / premature closure) results in increased intraluminal pressure Associated with:hiatal hernia, gastroduodenal ulcer, midesophageal diverticulum, esophageal spasm, achalasia compressible neck mass upper esophageal dysphagia (98%) regurgitation + aspiration of undigested food noisy deglutition halitosis (= foul breath) Location:at pharyngoesophageal junction in midline of Killian dehiscence / triangle of Laimer, at level of C5/6 posterior barium extension in upper half of semilunar depression on the posterior wall of esophagus (cricopharyngeal muscle) barium-filled sac extending caudally behind + usually to left of esophagus partial / complete obstruction of esophagus from external pressure of sac contents partial barium reflux from diverticulum into hypopharynx continual growth with successive enlargement CXR: air-fluid level in superior mediastinum Cx:aspiration pneumonia (30%); esophageal perforation; carcinoma (0.48%) Rx:surgical excision Notes:


ZOLLINGER-ELLISON SYNDROME =peptic ulcer diathesis associated with marked hypersecretion of gastric acid + gastrin-producing non-b islet cell tumor of pancreas Cause: A.GASTRINOMA (90%) = non-b islet cell tumor with continuous gastrin production B.PSEUDO Z-E SYNDROME = COWLEY SYNDROME = antral G-cell hyperplasia (10%) (increase in number of G-cells in gastric antrum) lack of gastrin elevation after secretin injection exaggerated gastrin elevation after protein meal Age:middle age; M > F Clinical tetrad: (1)Gastric hypersecretion: refractory response to histamine stimulation test concerning HCl concentration; increased basal secretion (>60% of augmented secretion is diagnostic) (2)Hypergastrinemia >1000 ng/L (during fasting) (3)Hyperacidity with basal acid output >15 mEq/h (4)Diarrhea (30%), steatorrhea (40%): may be sole complaint in 10%, frequently nocturnal; secondary to inactivation of pancreatic enzymes by large volumes of HCl severe intractable pain (90%) ulcer perforation (30%) positive secretin test = increase in serum gastrin level by >200 ng/L after administration of 2 IU/kg of secretin ulcers (atypical location + course should suggest diagnosis): Location:duodenal bulb (65%) + stomach (20%), near ligament of Treitz (25%), duodenal C-loop (5%), distal esophagus (5%) Multiplicity: solitary ulcer (90%), multiple ulcers (10%) recurrent / intractable ulcers marginal ulcers in postgastrectomy patient (a) on gastric side of anastomosis (b) on mesenteric border of efferent loop prominence of area gastricae (hyperplasia of parietal cell mass) enlargement of rugal folds sluggish gastric peristalsis (? hypokalemia) "wet stomach" = dilution of barium by excess secretions in nondilated nonobstructed stomach gastroesophageal reflux (common) + esophagitis dilatation of duodenum + upper small bowel (fluid overload) thickened folds in duodenum + jejunum (edema) rapid small-bowel transit time mnemonic:"FUSED" Folds (thickened, gastric folds) Ulcers (often multiple, postbulbar) Secretions increased (refractory to histamine) Edema (of proximal small bowel) Diarrhea Cx:(1)Malignant islet cell tumor (in 60%) (2)Liver metastases will continue to stimulate gastric secretion Rx: (1)Control of gastric hypersecretion: (a)H2-receptor antagonist: cimetidine, ranitidine, famotidine (b)Hydrogen-potassium adenosine triphosphatase inhibitor (omeprazole) (2)Resection of gastrinoma if found (because of malignant potential) (3)Total gastrectomy Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders

RENAL FAILURE =reduction in renal function

rise in serum creatinine >2.5 mg/dL

Acute Renal Failure Chronic Renal Failure Musculoskeletal Manifestations Of CRF Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : RENAL FAILURE

Acute Renal Failure = clinical condition associated with rapid steadily increasing azotemia ± oliguria (3 insufficiency fractures with predominant involvement of ribs) avascular necrosis lack of osteosclerosis less evidence of subperiosteal resorption3.Amyloid depositionPath:amyloid consists of b2 -microglobulinOrgans:bone, tenosynovium (carpal tunnel syndrome), vertebral disk, articular cartilage + capsule, ligament, muscle4.Destructive spondyloarthropathy (15%) discovertebral junction erosion + sclerosis vertebral body compression disk space narrowing Schmorl node formation lack of osteophytosis facet involvement with subluxation5.Tendon rupture6.Crystal deposition diseaseType:calcium hydroxyapatite, CPPD, calcium oxalate, monosodium urate7.Osteomyelitis + septic arthritis8.Avascular necrosis (in up to 40%)

Notes:


DIABETES INSIPIDUS A.Hypothalamic Diabetes Insipidus=vasopressin production is reduced to 100,000 organisms/mL urinePrevalence:3% of girls + 1% of boys during first 10 years of lifeUnderlying radiologic abnormality: 1.Vesicoureteral reflux = VUR (30-40%)2.Obstructive uropathy (8%)3.Reflux nephropathy / scar formation (6%) The prevalence of an underlying radiologic abnormality depends on age, sex, and frequency of previous infections!Imaging objective: 1.Identify patients at risk for reflux nephropathy2.Detect reflux nephropathy / scars3.Detect obstructive uropathy4.Minimize radiation, morbidity, and costVCUG: for children 1 cm )3.Sacral agenesis=segmental defect (below S2) with deficiency of nerves that innervate bladder, urethra, rectum, feet Children of diabetic mothers are affected in 17%!4.Extravesical infrasphincteric ectopic ureter only affects girls as boys do NOT have infrasphincteric ureteral orifices (a)ureter draining upper pole of duplex system exits below urethral sphincter (90%)(b)ureter draining single system with ectopic extravesical orifice (10%)5.Synechia vulvae =adhesive fusion of minor labia directs urine primarily into vagina from where it dribbles out post micturition6.Vaginal reflux in obese older girls with fat thighs and fat labia 7.Miscellaneousposterior urethral valves, urethral stricture, urethral diverticula Notes:


MALE INFERTILITY A.CONGENITAL(a)Wolffian duct anomalies1.Renal agenesis / atrophy2.Vas deferens agenesis / cyst3.Seminal vesicle agenesis / cyst4.Ejaculatory duct cyst(b)Müllerian duct anomalies1.Müllerian duct cyst2.Utricle cystB.ACQUIRED1.Cowper duct cyst2.Prostatic cyst in peripheral zoneC.INFECTIOUS1.ProstatitisD.HORMONAL semen low in volume, acid pH, without fructose1.Seminal vesicle atrophy=seminal vesicles duodenum > stomach > small bowel > appendix1.Inflammation: chronic purulent renal infection, diverticulitis, Crohn disease2.Neoplastic: colonic carcinoma Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : KIDNEY

Absent Renal Outline On Plain Film A.ABSENT KIDNEY1.Congenital absence2.S/P nephrectomyB.SMALL KIDNEY1.Renal hypoplasia2.Renal atrophyC.RENAL ECTOPIA1.Pelvic kidney2.Crossed fused ectopia3.Intrathoracic kidneyD.OBLITERATION OF PERIRENAL FAT1.Perirenal abscess2.Perirenal hematoma3.Renal tumors Notes:


Nonvisualized Kidney On Excretory Urography A.ABSENCE OF KIDNEY1.Agenesis2.EctopiaB.LOSS OF PERFUSION1.Chronic infarction2.Unilateral renal vein thrombosis3.Fractured kidneyC.URINARY OBSTRUCTION1.Hydronephrosis2.Ureteropelvic junction obstructionD.REPLACED NORMAL RENAL PARENCHYMA1.Multicystic dysplastic kidney2.Unilateral polycystic kidney disease3.Renal tumor (RCC, TCC, Wilms tumor)4.Xanthogranulomatous pyelonephritis Notes:


Unilateral Large Smooth Kidney A.PRERENAL(a)arterial:acute arterial infarction(b)venous:acute renal vein thrombosisB.INTRARENAL(a)congenital:duplicated pelvicaliceal system, crossed fused ectopia, multicystic dysplastic kidney, adult polycystic kidney (in 8% unilateral)(b)infectious:acute bacterial nephritis(c)adaptation:compensatory hypertrophyC.POSTRENAL(a)collecting system:obstructive uropathy mnemonic:"AROMA"Acute pyelonephritis Renal vein thrombosis Obstructive uropathy Miscellaneous (compensatory hypertrophy, duplication) Arterial obstruction (infarction) Notes:


Bilateral Large Kidneys Average renal length by x-ray: M = 13 cm; F = 12.5 cm 1.PROTEIN DEPOSITIONamyloidosis, multiple myeloma 2.INTERSTITIAL FLUID ACCUMULATIONacute tubular necrosis, acute cortical necrosis, acute arterial infarction, renal vein thrombosis 3.CELLULAR INFILTRATION(a)Inflammatory cells: acute interstitial nephritis, acute bacterial nephritis(b)Malignant cells: leukemia / lymphoma4.PROLIFERATIVE / NECROTIZING DISORDERS(a)Glomerulonephritis (GN)acute (poststreptococcal) GN, rapidly progressive GN, idiopathic membranous GN, membrano-proliferative GN, lobular GN, IgA nephropathy, glomerulosclerosis, glomerulosclerosis related to heroin abuse (b)Multisystem diseasepolyarteritis nodosa, systemic lupus erythematosus, Wegener granulomatosis, allergic angiitis, diabetic glomerulosclerosis, Goodpasture syndrome (lung hemorrhage + glomerulonephritis), Schönlein-Henoch syndrome (anaphylactoid purpura), thrombotic thrombocytopenic purpura, focal glomerulonephritis associated with subacute bacterial endocarditis 5.URINE OUTFLOW OBSTRUCTIONbilateral hydronephrosis: congenital / acquired 6.HORMONAL STIMULUSacromegaly, compensatory hypertrophy, nephromegaly associated with cirrhosis / hyperalimentation / diabetes mellitus 7.DEVELOPMENTALbilateral duplication system, horseshoe kidney, polycystic kidney disease 8.MISCELLANEOUSacute urate nephropathy, glycogen storage disease, hemophilia, sickle cell disease, Fabry disease, physiologic response to contrast material and diuretics mnemonic:"FOG P"Fluid:= edema of kidney (ATN, acute cortical necrosis)Other:leukemia, acromegaly, sickle cell anemia, bilateral duplication, acute urate nephropathyGlomerular disease:acute GN, lupus, polyarteritis nodosa, diabetes mellitusProtein deposition:multiple myeloma, amyloidosis

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Bilateral Small Kidneys A.PRERENAL = VASCULAR1.Arterial hypotension (acute)2.Generalized arteriosclerosis3.Atheroembolic disease4.Benign & malignant nephrosclerosisB.INTRARENAL1.Hereditary nephropathies:medullary cystic disease, hereditary chronic nephritis (Alport syndrome) 2.Chronic glomerulonephritis3.Amyloidosis (late)C.POSTRENAL1.Papillary necrosisD.CAUSES OF UNILATERAL SMALL KIDNEYoccurring bilaterally mnemonic:"CAPE HANA"Chronic glomerulonephritis Arteriosclerosis Papillary necrosis Embolic disease (secondary to atherosclerosis) Hypotension Alport syndrome Nephrosclerosis Amyloidosis (late) Notes:


Unilateral Small Kidney A.PRERENAL = VASCULAR1.Lobar infarction2.Chronic infarction3.Renal artery stenosis4.Radiation nephritisB.INTRARENAL = PARENCHYMAL1.Congenital hypoplasia 2.Multicystic dysplastic kidney (in adult)3.Postinflammatory atrophyC.POSTRENAL = COLLECTING SYSTEM1.Reflux nephropathy = chronic atrophic pyelonephritis2.Postobstructive atrophy mnemonic:"RIP R HIP"Reflux atrophy Ischemia (renal artery stenosis) Postobstructive atrophy Radiation therapy Hypoplasia (congenital) Infarction Postinflammatory atrophy Notes:


Increased Echogenicity Of Renal Cortex =RENAL MEDICAL DISEASE = diffuse increase in cortical echogenicity with preservation of corticomedullary junctionPath:deposition of collagen / calcium in interstitial, glomerular, tubular, vascular disease echointensity of cortex greater than liver / spleen ± equal to renal sinus renal size may be normal; enlarged kidneys suggest active stage of renal disease; small kidneys suggest chronic + often end-stage renal disease 1.Acute / chronic glomerulonephritis2.Renal transplant rejection3.Lupus nephritis4.Hypertensive nephrosclerosis5.Renal cortical necrosis6.Methemoglobulinuric renal failure7.Alport syndrome8.Amyloidosis9.Diabetic nephrosclerosis10.Nephrotoxin-induced acute tubular necrosis11.End-stage renal disease Notes:


Hyperechoic Renal Pyramids In Children A.NEPHROCALCINOSIS(a)iatrogenic (most common cause):furosemide (Rx for BPD), vitamin D (Rx for hypophosphatemic rickets) (b)noniatrogenic:1.Idiopathic hypercalcemia2.Williams syndrome3.Absorptive hypercalcemia4.Hyperparathyroidism5.Milk-alkali syndrome6.Kenny-Caffey syndrome7.Distal renal tubular acidosis8.Malignant tumors9.Chronic glomerulonephritis10.Sjögren syndrome (distal RTA)11.SarcoidosisB.METABOLIC DISEASE1.Gout2.Lesch-Nyhan syndrome (urate)3.Fanconi syndrome4.Glycogen storage disease (distal RTA)5.Wilson disease (distal RTA)6.Alpha-1-antitrypsin deficiency7.Tyrosinemia8.Cystinosis9.Oxalosis10.Crohn diseaseC.HYPOKALEMIA1.Primary aldosteronism2.Pseudo-Bartter syndromeD.PROTEIN DEPOSITS1.Infant dehydration with presumed Tamm-Horsfall proteinuria2.Toxic shock syndromeE.VASCULAR CONGESTION1.Sickle cell anemiaF.INFECTION1.Candida / CMV nephritis2.AIDS-associated Mycobacterium avium-intracellulareG.FIBROSIS OF RENAL PYRAMIDSH.CYSTIC MEDULLARY DISEASE1.Medullary sponge kidney2.Congenital hepatic fibrosis with tubular ectasiaI.INTRARENAL REFLUX1.Chronic pyelonephritis

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Iron Accumulation In Kidney A.RENAL CORTEX1.Paroxysmal nocturnal hemoglobulinuria(= intravascular extrasplenic hemolysis) 2.Sickle cell anemiaB.RENAL MEDULLA1.Hemorrhagic fever with renal syndrome (uncommon viral illness caused by Hanta virus)Triad:(1)renal medullary hemorrhage(2)right atrial hemorrhage(3)necrosis of anterior pituitary Notes:


Depression Of Renal Margins 1.Fetal lobation notching between normal calices2.Splenic impression flattened upper outer margin of left kidney3.Chronic atrophic pyelonephritis indentation over clubbed calices4.Renal infarct normal calices5.Chronic renal ischemia normal calices Notes:


Enlargement Of Iliopsoas Compartment A.INFECTION(a)from retroperitoneal organs1.Renal infection2.Complicated pancreatitis3.Postoperative aortic graft infection(b)from spine1.Osteomyelitis / postoperative complication of bone surgery2.Discitis / postoperative complication from disk surgery(c)from GI tract1.Crohn disease2.Appendicitis(d)others1.Pelvic inflammatory disease / postpartum infection2.SepsisB.HEMORRHAGE1.Coagulopathy and anticoagulant therapy2.Ruptured aortic aneurysm3.Postoperative aneurysm repair / other surgery / traumaC.NEOPLASTIC DISEASE(a)Extrinsic1.Lymphoma2.Metastatic lymphadenopathy3.Bone metastases with soft-tissue involvement4.Retroperitoneal sarcoma(b)Intrinsic1.Muscle tumors2.Nervous system tumors3.Lipoma / liposarcomaD. MISCELLANEOUS1.Pseudoenlargement of psoas musclecompared to de facto atrophy of contralateral side in neuromuscular disease 2.Fluid collectionsurinoma, lymphocele, pancreatic pseudocyst, enlargement of iliopsoas bursa 3.Pelvic venous thrombosis diffuse swelling of all muscles (edema) Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : RENAL MASS

Bilateral Renal Masses A.MALIGNANT TUMOR1.Malignant lymphoma / Hodgkin disease2.Metastases3.Renal cell carcinoma4.Wilms tumorB.BENIGN TUMOR1.Angiomyolipoma2.NephroblastomatosisC.CYSTS1.Adult polycystic kidney disease2.Acquired cystic kidney disease Notes:


Renal Mass In Neonate A.UNILATERAL1.Multicystic kidney (15%)2.Hydronephrosis (25%)(a)UPJ obstruction(b)upper moiety of duplication3.Renal vein thrombosis4.Mesoblastic nephroma5.Rare: Wilms tumor, teratomaB.BILATERAL1.Hydronephrosis2.Polycystic kidney disease3.Multicystic kidney + contralateral hydronephrosis4.Nephroblastomatosis5.Bilateral multicystic kidney Notes:


Renal Mass In Older Child A.SINGLE MASS1.Wilms tumor2.Multilocular cystic nephroma3.Focal hydronephrosis4.Traumatic cyst, abscess5.Renal cell carcinoma6.Malignant rhabdoid tumor7.Teratoma8.Clear cell sarcoma of kidney9.Intrarenal neuroblastomaB.MULTIPLE MASSES1.Nephroblastomatosis2.Multiple Wilms tumors3.Angiomyolipoma4.Lymphoma5.Leukemia6.Adult polycystic kidney disease7.Abscesses

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Growth Pattern Of Renal Tumors In Adults A.EXPANSILE GROWTH1.Renal cell carcinoma2.Oncocytoma3.Angiomyolipoma4.Juxtaglomerular tumor5.Metastatic tumor (eg, lymphoma)6.Mesenchymal tumorB.INFILTRATIVE GROWTH1.Lymphoma / leukemia2.Invasive transitional cell carcinoma3.Metastatic tumor4.Renal cell carcinoma (unusual)5.Xanthogranulomatous pyelonephritis Notes:


Local Bulge In Renal Contour A.CYST1.Simple renal cystB.TUMOR1.Adenocarcinoma2.Angiomyolipoma3.PseudotumorC.INFECTION1.Subcapsular abscess2.XGPD.TRAUMA1.Subcapsular hematomaE.DILATED COLLECTING SYSTEM Notes:


Unilateral Renal Mass Solid Renal Mass A.TUMORS(a)primary malignant:adenocarcinoma (83%), chromophobe carcinoma (4%), papillary neoplasm (14%), renal collecting duct carcinoma = Bellini duct carcinoma (1%), transitional cell carcinoma (8%), renal neuroendocrine tumors (carcinoid, small cell carcinoma), Wilms tumor (6%), renal sarcoma (2%) in horseshoe kidney: adenocarcinoma (45%), Wilms tumor (28%), transitional cell carcinoma (20%) (b)secondary malignant:malignant lymphoma / Hodgkin disease, metastases, invasive transitional cell carcinoma (c)benign:adenoma, oncocytoma, hamartoma (mesoblastic nephroma, angiomyolipoma, myolipoma, lipoma, leiomyoma, fibroma), hemangioma B.INFLAMMATORY MASSESacute focal bacterial nephritis, renal abscess, xanthogranulomatous pyelonephritis, malacoplakia, tuberculoma Fluid-filled Mass A.CYSTS1.Simple renal cyst2.Inherited cystic disease:multicystic dysplastic kidney disease (Potter type II), multilocular cystic nephroma 3.Focal hydronephrosisB.VASCULAR1.Arteriovenous malformation2.Arteriovenous fistula=single dilated artery + vein tortuous varices over time enlargement of renal veinCx:hydronephrosis Lesions 80% stenosis); reduced pressure stimulates release of renin followed by angiotensin-II, and aldosterone causing (a)constriction of efferent glomerular arterioles(b)increase in systemic hypertension(c)sodium retentionCause: 1.Atherosclerosis (60-90%) in individuals >50 years of age2.Fibromuscular dysplasia (10-35%) in women 4 cm large focal mass with central necrosis in one adrenal gland + atrophy of contralateral gland = adrenal adenocarcinomaB.BILATERAL ADRENAL ENLARGEMENT diffuse uniform thickening = Cushing diseaseC.MULTIPLE BILATERAL ADRENAL NODULES macronodules = multinodular hyperplasia of long-standing Cushing disease large nodules (autonomous ACTH-independent) = massive macronodular hyperplasia small nodules = primary pigmented nodular adrenal disease Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : ADRENAL GLAND

Bilateral Large Adrenals mnemonic:"4 H PM"Hodgkin disease Hyperplasia Hemorrhage Histoplasmosis / TB Pheochromocytoma Metastasis Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : ADRENAL GLAND

Unilateral Adrenal Mass CT attenuation15HU=indeterminate on 15-minute-delayed CECT scan:25 HU malignant lesion Cause:rapid contrast washout from benign lesionsmnemonic:"PLAN My HAM"Pheochromocytoma Lymphoma Adenoma Neuroblastoma Myelolipoma Hemorrhage Adenocarcinoma Metastasis Small Unilateral Adrenal Tumor Incidental discovery of adrenal mass in 1% of all CT!(a)mass 5 cm in diameter is likely malignant1.Cortical adenoma (in 1-9% of autopsies) breast > kidney > lung solitary / multiple nodules2.Lymphomabladder involved at autopsy: for NHL in 15%, for Hodgkin disease in 5% 3.Leukemiamicroscopic involvement in 22% at autopsy 4.Direct extension (common)from prostate, rectum, sigmoid, cervix, ovary

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Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : URINARY BLADDER

Bladder Wall Calcification A.INFLAMMATION1.Schistosomiasis (50%) relatively normal distensibility2.Tuberculosis bladder markedly contracted3.Postirradiation cystitis4.Bacillary UTI (extremely uncommon)B.NEOPLASMTCC, squamous cell carcinoma, leiomyosarcoma, hemangioma, neuroblastoma, osteogenic sarcoma mnemonic:"SCRITT"Schistosomiasis Cytoxan Radiation Interstitial cystitis Tuberculosis Transitional cell carcinoma

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Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : URINARY BLADDER

Masses Extrinsic To Urinary Bladder A.NORMAL / ENLARGED ORGANS1.Uterus, leiomyomatous uterus, pregnant uterus2.Distended rectosigmoid3.Ectopic pelvic kidney4.Prostate cancer / BPHB.SOLID PELVIC TUMORS1.Lymphadenopathy2.Bone tumor from sacrum / coccyx3.Rectosigmoid mass4.Hip arthroplasty5.Neurogenic neoplasm, meningomyelocele6.Pelvic lipomatosis / liposarcoma C.CYSTIC PELVIC LESIONS(a) congenital / developmental 1.Urachal cyst2.Müllerian duct cyst3.Gartner duct cyst4.Anterior meningocele5.Hydrometrocolpos(b) related to trauma 1.Hematoma (eg, rectus sheath hematoma)2.Urinoma3.Lymphocele4.Abscess5.Aneurysm6.Mesenteric cyst(c) cyst of genitalia 1.Prostatic cyst2.Cyst of seminal vesicle3.Cyst of vas deferens4.Ovarian cyst5.Hydrosalpinx6.Vaginal cyst(d)cyst of urinary bladder1.Bladder diverticulum(e) cyst of GI tract 1.Peritoneal inclusion cyst2.Fluid-filled bowel Notes:


VOIDING DYSFUNCTION A.FAILURE TO STORE URINE urinary frequency, urgency, incontinence(a)bladder causes1.involuntary detrusor contractions-detrusor instability (idiopathic / neurogenic)-detrusor hyperreflexia (upper cord lesion)2.poor bladder compliance-detrusor hyperreflexia-bladder wall fibrosis3.sensory urgency-infection, inflammation, irritation-neoplasia4.vesicovaginal fistula5.psychogenic condition(b)sphincter causes1.Stress incontinence2.Sphincteric incontinence(c)extravesical ectopic insertion of ureter in femalesB.FAILURE TO EMPTY BLADDER poor flow, straining, hesitancy inability to completely empty bladder(a)bladder causes1.Detrusor areflexia (sacral arc lesion)2.Impaired detrusor contractility (myogenic)3.Psychogenic condition(b)bladder outlet obstruction:1.Bladder neck contracture2.Prostatic enlargement3.Detrusor-external sphincter dyssynergia4.Scarring from surgery / radiation therapy5.Ectopic ureterocele6.Urethral stenosis7.Urethral kinking (eg, due to cystocele) Incontinence Prostatic Obstruction Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : VOIDING DYSFUNCTION

Incontinence 1.Stress incontinence2.Vesicovaginal / ureterovaginal fistula3.Overflow incontinencesecondary to lesions of sacral spinal cord / sacral reflex arc or severe outlet obstruction 4.Reflex voiding(a)hyperreflexive lesion (lesion of upper spinal cord)(b)uninhibited / unstable bladder5.Urge incontinence6.Continual dribbling(extravesical ectopic termination of ureter)7.Psychogenic incontinence Stress Incontinence =SPHINCTER WEAKNESS INCONTINENCECause: A.Female: congenital bladder neck weakness, pregnancy, childbirth, aging (secondary to changes in anatomic relationship of urethra + bladder base)B.Male: S/P prostatectomy with damage to distal sphincter frequency, urgency (involuntary filling of bladder neck) opening of bladder neck during coughing impairment of milk-back mechanism (= retrograde emptying of urethra during interruption of voiding phase does not occur) urethrovesical descent (in types I + II)Chain cystography: posterior urethrovesical angle (= angle between posterior urethra + bladder base) increased >100° upper urethral axis (= angle between upper urethra + vertical line) increased >35° Detrusor Instability =MOTOR URGE INCONTINENCE = UNSTABLE BLADDERCondition resembles that of immature bladder before toilet training Patient groups: (1)symptoms of nocturnal enuresis + frequency / incontinence dating back to childhood(2)idiopathic instability occurring in middle age(3)outflow obstruction commonly in men(4)degenerative instability secondary to cardiovascular + neurologic disease later in life frequency, urgency, urge incontinence, occasionally nocturia hesitancy + difficulty in voiding may occur in men without significant prostatic hypertrophy involuntary bladder contractions with no relationship to bladder distension progressively vigorous contractions during bladder filling postural instability limited to upright position impaired milk-back due to high bladder pressure strong aftercontractions following bladder emptyingCx:thickening of bladder wall, bladder diverticulaRx:treatment of obstruction, anticholinergic drug (oxybutynin), operative increase in bladder capacity Sensitive Bladder (Sensory Urgency) Cause: cystitis (reduced compliance), some cases of stress incontinence (filling of bladder neck induces urgency) frequency, urgency, sometimes nocturia patient uncomfortable with low bladder filling no abnormal rise in bladder pressure normal voiding function Detrusor-sphincter Dyssynergia =overactivity of bladder neck muscle with failure to relax at beginning of voidingCause:spinal cord lesion / trauma above level of sacral outflow difficulty in voiding ± frequency lifelong history of poor stream collarlike indentation of bladder neck during voiding(= persistent / intermittent narrowing of membranous urethra) may have high voiding pressure + reduced flow trapping of contrast in urethra during interruption of flow massive reflux into prostatic ducts during voiding (due to high pressure within prostatic urethra) severely trabeculated "Christmas-tree" bladder + bilateral hydroureteronephrosisRx:bladder neck incision Hinman Syndrome = NONNEUROGENIC NEUROGENIC BLADDER [NNNB] = DETRUSOR-SPHINCTER DYSSYNERGIA Cause:no neurologic / anatomic obstructive disease; distinctly abnormal family dynamics (in 50%)Age:some time after toilet training with onset during early / late childhood / puberty clinical criteria:(1)intact perineal sensation + anal tone(2)normal anatomy + function of lower extremities(3)absence of skin lesions overlying sacrum(4)normal lumbosacral spine at plain radiography(5)normal spinal cord at MR imaging high-pressure uninhibited detrusor contractions lack of coordination between detrusor contraction + periurethral striated sphincter relaxation inability to suppress bladder contractions normal response of detrusor muscle to reflex stimulation increased bladder capacity + pressure sphincter activity may increase paradoxically during detrusor contractionUS: trabeculated bladder dilatation of upper urinary tracts renal damageVCUG: urethra normal during early voiding urethral distension after contraction of external sphincter as voiding progresses ureterovesical obstruction / refluxRx:suggestion therapy + hypnosis, bladder retraining, biofeedback, anticholinergic drugs Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : VOIDING DYSFUNCTION

Prostatic Obstruction =urethral compression by hypertrophic prostatic tissue flow increase in bladder capacity with reduced contractility (late) Notes:

difficulty in voiding

reduction in flow rate high-pressure bladder slow + prolonged


BLADDER TRAUMA 1.Bladder contusion (most common injury)2.Interstitial bladder injury (uncommon)=bladder tear without serosal involvement3.Bladder rupture(a)intraperitoneal rupture (30%)(b)extraperitoneal rupture(c)combined intra- and extraperitoneal rupture (5%)

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Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : MALE GENITAL TRACT

Acutely Symptomatic Scrotum =acute unilateral scrotal swelling ± painCause: epididymitis:torsion = 3:220 years of age A.TORSION1.Torsion of testis (20%)=most common acute process in prepubertal age2.Torsion of testicular appendagesaccounts for 5% of scrotal pathology; both located near upper pole of testes Frequency: appendix testis:appendix epididymis = 9:1 8-9 mm complex mass in superior aspect of scrotum without color Doppler flow signals mildly enlarged epididymis (75%) blood flow increased in epididymis (60%), scrotal wall (53%), testis (13%) simulating acute epididymo-orchitis3.Scrotal fat necrosis4.Strangulated herniaB.INFECTION / INFLAMMATION (75-80%)1.Acute epididymitis=most common acute process in postpubertal age2.Orchitis Etiology: (a)bacterial infection(b)complication of mumps in 20%:in adolescents + young adults; usually developing 4-5 days later; unilateral involvement in >90%; parotitis precedes orchitis in 84%, simultaneous in 3%, later in 4%, without parotitis in 10% 3.Intrascrotal abscessC.HEMORRHAGE1.Testicular traumaLocation:hematoma in scrotal wall, between layers of tunica vaginalis (= hematocele), in epididymis, in testis rapid change in echo character over time disruption of tunica albuginea (= testicular rupture)2.Hemorrhage into testicular tumorD.STRANGULATED HERNIA

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Scrotal Wall Thickening 1.Acute idiopathic scrotal edemaIncidence:20-30% of all acute scrotal disordersAge:5-11 years (range 18 months to 14 years) subcutaneous scrotal edema, erythema minimal pain, afebrile, peripheral eosinophilia2.Epididymo-orchitis3.Testicular torsion4.Torsion of testicular / epididymal appendage5.Trauma6.Henoch-Schönlein purpura7.Cx of ventriculoperitoneal shunt8.Cx of peritoneal dialysis (? leakage of fluid into the anterior abdominal wall + dissection into scrotum)

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Scrotal Gas 1.Fournier gangrene2.Scrotal abscess3.Scrotal hernia with gas-containing bowel4.Scrotal emphysema from bowel perforation5.Extension of subcutaneous emphysema6.Air leakage + dissection due to faulty chest tube positioning Notes:


Scrotal Mass Most frequent conditions: 1.Inflammation(48%)2.Hydrocele(24%)3.Torsion( 9%)4.Varicocele( 7%)5.Spermatocele( 4%)6.Cysts( 4%)7.Malignant tumor( 2%)8.Benign tumor(0.7%) Sonographic differentiation of intra- from extratesticular mass is 80-95% accurate! A.INTRATESTICULAR MASS 90-95% of testicular tumors are malignant!1.Malignant tumor2.Inflammation: focal orchitis3.Abscess4.Testicular infarction: torsion, endocarditis, trauma, leukemia, vasculitis, embolus soft to palpation hypoechoic wedge-shaped peripheral defect5.Hematoma6.Benign gonadal tumor7.Granulomatous disease: sarcoidosis8.Testicular cyst / tunica albuginea cyst9.Postbiopsy defect10.Adrenal rest increase in circulating corticotropin bilateral eccentric nodular masses ± acoustic shadowing B.MULTIPLE INTRATESTICULAR MASSES1.Primary testicular tumor2.Lymphoma / leukemia3.Chronic infections4.Metastases5.Sarcoidosis The prevalence of synchronous / metachronous bilateral testicular neoplasms is 1-3%! C.EXTRATESTICULAR FLUID COLLECTION1.Hydrocele, pyocele, hematocele (surgery, trauma, neoplasm)2.Varicocele3.Spermatocele=cyst filled with fluid + spermatozoa + cellular debris frequently following vasectomyLocation:commonly in head of epididymis up to a few cm in size ± septations4.Epididymal cyst=cyst without spermatozoaLocation:anywhere within epididymis5.Scrotal hernia D.PARATESTICULAR MASS Only 4% of all scrotal tumors!(a)inflammatory mass1.Sarcoidosis of epididymis2.Inflammatory nodule of epididymitis3.Sperm granulomaCause:sperm extravasation with granuloma formation4.Scrotal calculi = "scrotal pearls"Cause:fibrinous debris in long-standing hydrocele / following torsion of appendix testis or epididymis(b)paratesticular tumor The majority of paratesticular tumors are derived from the spermatic cord!-Benign Paratesticular tumor (70%)1.Cord lipoma (vast majority)2.Adenomatoid tumor (30%)=benign slow-growing mesothelial neoplasm Age:2nd-4th decadeHisto:epithelial-like cells + fibrous stromaLocation:epididymis (particularly in globus minor), tunica albuginea, spermatic cord (rare) well-marginated solid mass with echogenicity equal to / greater than testis 0.4-5.0 cm in size3.Epidermoid inclusion cyst4.Polyorchidism5.Others: herniated omentum, adrenal rest, carcinoid, papillary cystadenoma of epididymis, cord leiomyoma, cord fibroma (= reactive nodular proliferation of paratesticular tissues), adrenal rest, cholesteatoma-Malignant Paratesticular Tumor (3-16%)1.Sarcomas: Sarcomas are the most common spermatic cord tumors after lipomas!(a)primarily in adults: undifferentiated sarcoma (30%), leiomyo-, lipo-, fibro-, myxochondro-sarcoma(b)children: embryonal sarcoma, rhabdomyosarcoma (20%)2.Mesothelioma of tunica (in 15% malignant)3.Metastases Prepubertal Testicular Mass A.Germ cell tumors (70-90%): yolk sac tumor, teratomaB.Interstitial cell tumors: Leydig / Sertoli cell tumor, gonadoblastomaC.Leukemia, lymphoma, metastasesD.Others: adrenal rest, lipoma, hematoma, histiocytosis, tuberculous orchitis

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Calcification Of Male Genital Tract A.VAS DEFERENS1.Diabetes mellitus: in muscular outer layer2.Degenerative changes3.TB, syphilis, nonspecific UTI: intraluminalB.SEMINAL VESICLESgonorrhea, TB, schistosomiasis, bilharziasis C.PROSTATEcalcified corpora amylacea, TB Notes:


Cystic Lesions Of Testis Incidence:4-10% (increasing with age) asymptomaticA.NONNEOPLASTIC1.Testicular cyst nonpalpableOften associated with:spermatoceleLocation:related to rete testis (in 92%)2.Tunica albuginea cyst palpable solitary small marginally located cyst3.Intratesticular tubular ectasia =DILATATION OF RETE TESTISAge:middle-aged to elderlyOften associated with:spermatocele nonpalpableLocation: mediastinum testis elliptical hypoechoic mass with branching tubular structures ± cysts4.Congenital cystic dysplasia of testis (extremely rare)B.NEOPLASTIC 24% of all testicular tumors have cystic component! palpable in combination with solid elements DDx:hematoma, inflammation, seminoma, Leydig cell tumor

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Epididymal Enlargement With Hypoechoic Foci 1.Epididymitis2.Sperm granulomas3.Tuberculosis4.Lymphogranuloma venereum5.Granuloma inguinale6.Filarial granuloma7.Fungal disease8.Lymphoproliferative disease9.Metastases Notes:


Cystic Lesions Of Epididymis 1.Epididymal cystIncidence:in up to 40%May be associated with:intratesticular tubular ectasia single / multiple / bilateralDDx:loculated hydrocele2.Spermatocele may contain low-level echoes3.Cystic degeneration of epididymis

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Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : PROSTATE and URETHRA

Seminal Vesicle Cyst A.CONGENITALassociated with:renal dysgenesis, collecting system duplication, ectopic ureter, vas deferens agenesisB.ACQUIRED1.Autosomal dominant polycystic kidney disease bilateral seminal vesicle cysts2.Invasive bladder tumor3.Infection4.Benign prostatic hypertrophy5.Ejaculatory duct obstruction

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Large Utricle 1.Prune belly syndrome2.Imperforate anus of high type3.Down syndrome4.Hypospadia5.Posterior urethral valves

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Prostatic Cysts 1.Müllerian duct cyst from remnants of paramesonephric (= müllerian) duct which has regressed by 3rd fetal month Prevalence:4-5% of male newborns; in 1% of menAge:discovered in 3rd-4th decade obstructive / irritative urinary tract symptoms suprapubic / rectal pain hematuria infertility (most common cause of ejaculatory duct obstruction)Location:arise from region of verumontanum slightly lateral to midline No communication with genital tract / urethra large intraprostatic cyst usually with extension superolaterally above prostate aspirate contains serous / mucous clear brown / green fluid (hemorrhage + debris), NOT spermatozoa rarely contains calculiCx:infection, hemorrhage, carcinomatous transformation2.Utricle cyst Secondary to dilatation of prostatic utricle (sometimes believed to be a remnant of the müllerian duct) Age:1st-2nd decade postvoid dribbling obstructive / irritative urinary tract symptoms suprapubic / rectal pain hematuriaOften associated with: hypospadia, intersex disorders, incomplete testicular descent, ipsilateral renal agenesis Location:arise in midline from verumontanum Free communication with urethra 8- to 10-mm long cyst usually NO extension above prostateDx:endoscopic catheterization with aspiration of white / brown fluid occasionally containing spermatozoaCx:infection, hemorrhage, carcinomatous metaplasia3.Ejaculatory duct cyst Cause:congenital / acquired obstruction of ejaculatory duct perineal pain, dysuria, ejaculatory pain hematospermiaLocation:along expected course of ejaculatory duct intraprostatic cyst within central zone aspirate contains spermatozoa with normal testicular function cyst commonly contains calculi cystic dilatation of ipsilateral seminal vesicle contrast injection into cyst outlines seminal vesicle4.Cystic degeneration of BPH Most common cystic lesion of prostate Location: transition zone usually small cyst within nodules of benign prostatic hyperplasia 5.Retention cyst =dilatation of glandular aciniCause:acquired obstruction of glandular ductuleAge:5th-6th decadeLocation: transition / central / peripheral zone 1- to 2-cm smooth-walled unilocular cyst6.Cavitary / diverticular prostatitis Cause:fibrosis of chronic prostatitis constricts ducts leading to stagnation of exudate + breakdown of intraacinar septa with cavity formation history of long-standing inflammatory condition "Swiss cheese" prostate7.Prostatic abscess Age:5th-6th decade fever, chills urinary frequency, urgency, dysuria, hematuria perineal / lower back pain focally enlarged tender prostate hypo- / anechoic mass with irregular wall + septations8.Parasitic cyst (Echinococcus, bilharziasis)9.Cystic carcinoma hemorrhagic aspirate solid tissue invaginating into cyst

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Hypoechoic Lesion Of Prostate 1.Adenocarcinoma (35%)2.Benign prostatic hyperplasia (18%) rarely may originate in the peripheral zone3."Normal" prostatic tissue (18%)(a)cluster of prostate retention cysts(b)prominent ejaculatory ducts4.Acute / chronic prostatitis (14%)5.Granulomatous prostatitis (0.8%): most frequently due to Calmette-Guérin bacillus (BCG)6.Atrophy (10%) occurs in 70% of young healthy men May be confused with carcinoma histologically!7.Prostatic dysplasia (6%) Notes:


Cowper (Bulbourethral) Gland Lesions Analogous to Bartholin glands in females Prevalence:2.3% (autopsy)Location:within urogenital diaphragm1.Retention cystCx:prenatal death from urinary obstruction2.Infectious / traumatic cyst asymptomatic (most) hematuria, bloody urethral discharge postvoid dribbling

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Urethral Tumors Benign Urethral Tumor 1.Fibroepithelial polyp in child / young adult; transitional cell epithelium solitary, pedunculated fingerlike filling defect attached near verumontanumCx:bladder outlet obstruction2.Transitional cell papilloma older patient; in prostatic / bulbomembranous urethra; frequently associated with concomitant bladder papillomas 3.Adenomatous polyp young men; adjacent to verumontanum Histo:columnar epithelium from aberrant prostatic epithelium hematuria4.Penile squamous papilloma / condyloma acuminata in 5% of patients with cutaneous disease (glans penis) verrucous lesion in distal urethra, rarely extension into bladder5.Others:caruncle, urethral mucosal prolapse, inflammatory tags (in female) Malignant Urethral Neoplasm Incidence:6th-7th decade, M:F = 1:5A.FEMALE urethral bleeding obstructive symptoms dysuria mass at introitus1.Squamous cell carcinoma (70%):distal 2/3 of urethra 2.Transitional cell carcinoma (8-24%):posterior 1/3 of urethra 3.Adenocarcinoma (18-28%):from periurethral glands of Skene B.MALE palpable urethral mass periurethral abscess obstructive symptoms cutaneous fistula bloody dischargeSite:bulbomembranous urethra (60%); penile urethra (30%); prostatic urethra (10%)1.Squamous cell carcinoma (70%)secondary to chronic urethritis from venereal disease (44%) + urethral strictures (88%) 2.Transitional cell carcinoma (16%)part of multifocal urothelial neoplasia, in 10% after cystectomy for bladder tumor 3.Adenocarcinoma (6%)in bulbous urethra originating in glands of Cowper / Littre 4.Melanoma, rhabdomyosarcoma, fibrosarcoma (rare)5.Metastases from bladder / prostatic carcinoma (rare)

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AMBIGUOUS GENITALIA =external genitalia that are not clearly of either sexPrevalence:1:1,000 live births cryptorchidism labial fusion clitoromegaly epi- / hypospadiaCause: A.Abnormal hormone levels1.congenital adrenal hyperplasia2.transplacental passage of hormones3.true hermaphroditismB.Anomalies of external genitalia not hormonally mediated (eg, micropenis) SEX=what a person is biologically; sex assignment based on (1) karyotype (2) gonadal biopsy (3) genital anatomyGENDER=what a person becomes socially Female Pseudohermaphroditism Male Pseudohermaphroditism Gonadal Dysgenesis True Hermaphroditism Notes:

Home : UROGENITAL TRACT : Differential diagnosis of urogenital disorders : AMBIGUOUS GENITALIA

Female Pseudohermaphroditism =FEMALE INTERSEXCause:exposure to excessive androgens in 1st trimester due to(a) congenital adrenogenital syndrome (b)maternal drug ingestion (progestational agents, androgens)(c) masculinizing ovarian tumor Karyotype: 46,XX masculinized external genitalia penislike clitoris (due to prominent corpora cavernosa + corpus spongiosum) rugose labioscrotum uterus + vagina may be filled with urine through urogenital sinus normal ovaries, fallopian tubes, uterus, vagina enlarged adrenal glands (adrenal hyperplasia) no testicular tissue / internal wolffian duct derivatives Notes:


Male Pseudohermaphroditism Cause:within fetal testis(a)decreased testosterone synthesis(b)decreased dihydrotestosterone production(= substance responsible for masculinization of external genitalia) due to 5a-reductase deficiency(b)no testosterone production due to early destruction / dysgenesis of testes(c)complete / incomplete androgen insensitivity due to androgen receptor defect (= testicular feminization)Karyotype:46,XY incompletely masculinized / ambiguous external genitalia[ apparent hypergonadotropic primary amenorrhea] commonly undescended normal / mildly defective bilateral testes prostatic tissue no müllerian duct derivatives (production of müllerian regression factor by testes not affected) occasionally blind-ending vaginal pouch emptying into perineum (= pseudovagina) / through urethra (= urogenital sinus)

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Gonadal Dysgenesis characterized by abnormal gonadal organization and function with gonads often partially / completely replaced by fibrous stroma (1)Mixed gonadal dysgenesis=testis on one side + gonadal streak on other sideKaryotype:45,XO/46,XY karyotype or other mosaics with a Y chromosome ambiguous external genitalia small / rudimentary uterus + vagina fallopian tube present on side of streak gonad urogenital sinus commonly empties at base of phallus dysgenetic gonads (with inability to secrete müllerian regression factor)Cx:gonadal neoplasia(2)Pure XY gonadal dysgenesisKaryotype:46, XY bilateral streak gonads / dysgenetic testes müllerian + wolffian duct derivatives both absent / partially developed(3)XY gonadal agenesis= vanishing testes syndrome = testicular resorption in early fetal life of unknown cause Karyotype:46,XY ambiguous external genitalia / female phenotype absent testes müllerian + wolffian duct derivatives both absent / partially developed Notes:


True Hermaphroditism = TRUE INTERSEX =condition characterized by presence of ovarian + testicular tissue either separate or in same gonad(= ovotestis in 64%)Gonads:(a)ovary on one + testis on other side (30%)(b)ovary / testis on one + ovotestis on other side (50%)(c)bilateral ovotestes (20%)Location:in pelvis (predominantly ovarian tissue); in scrotum / inguinal region (predominantly testicular tissue)Incidence:rare (500 cases in world literature); spleen > renal cortex > renal medulla-renal sinus echogenicity less prominent in neonate because of paucity of fat Notes:

Home : UROGENITAL TRACT : Anatomy and function of urogenital tract : RENAL ANATOMY

Renal Vascular Anatomy 1st order:main renal artery2nd order:anterior + posterior division at / before hilum3rd order:5 segmental branches for each divisionAccessory renal artery =segmental arteries originating from the aortaAberrant renal artery =segmental artery arising from superior mesenteric artery / internal spermatic arteryResistive index:8 nm), threshold at molecular weight of approximately 40,000 Glomerular Filtration Rate (GFR) [P] x GFR = [U] x Uvol GFR = {[U] x Uvol} / [P] = 125 mL/min = 20% of RPF Substrate: inulin; Tc-99m DTPA Tubular Secretion (Tm) [U] x Uvol = [P] x GFR + Tm Tm = [U] x Uvol - [P] x GFR Substrate: p-aminohippurate (PAH); I-131 Hippuran Renal Plasma Flow (RPF) [P] x RPF = [U] x Uvol RPF = [U] x Uvol / [P] Substrate: p-aminohippurate [P]= concentration in plasmaGFR= glomerular filtration rate[U]= concentration in urineUvol= urine volumeTm= transport maximum (across tubular cells)RPF= renal plasma flow Renal Acidification Mechanism Renal Imaging In Newborn Infant Contrast Excretion Notes:

Home : UROGENITAL TRACT : Anatomy and function of urogenital tract : RENAL PHSYIOLOGY

Renal Acidification Mechanism Proximal tubule: reabsorption of 90% of filtered bicarbonate by luminal Na+/H+ exchange and Na+/HCO3 - cotransport at basolateral membrane regulated by:luminal carbonic anhydraseinfluenced by:luminal HCO3 - concentration, extracellular fluid volume, parathormone, K+, aldosteroneDistal nephron: active secretion of H+ against a steep urine-to-blood gradient across luminal cell membrane by H+-ATPase pump facilitated by Na+ reabsorption resulting in reabsorption of 10% of filtered bicarbonate, formation of ammonium (NH4 +) and titratable acidity Ammonium excretion: Ammonia (NH3 ) is formed in proximal tubule as a product of catabolism of glutamine + other amino acids; combination with secreted H+ to NH4 + takes place in distal nephron Titratable acidity: divalent basic phosphate is converted into monovalent acid form in distal tubule

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Renal Imaging In Newborn Infant low glomerular filtration rate (GFR):-on first day of life:21% of adult values-by 2 weeks of age:44% of adult values-at end of 1st year:close to adult values limited capacity to concentrate urineIVP: occasional failure of renal visualizationNUC: improved visualization on radionuclide studies

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Contrast Excretion UROGRAPHIC DENSITY depends on [U] = [P] x GFR / Uvol 1. Concentration of contrast material in plasma [P] is a function of (a)total iodine dose(b)contrast injection rate(c)volume distributionRapid decline of concentration of contrast material in vessels is due to: (1)rapid mixing within vascular compartment(2)diffusion into extravascular extracellular fluid space (capillary permeation)(3)renal excretion2. Glomerular filtration rate (GFR): 99% filtered 3. Urine volume (Uvol), ie, activity of ADH: (a)in dehydrated state with increased ADH activity concentrations of contrast material are higher Dehydration is considered a risk-potentiating factor for nephrotoxicity!(b)in volume-expanded state with decreased ADH activity concentrations of contrast material are lower Patients with CHF require higher doses of contrast material!A.MEGLUMINEno metabolization, excreted by glomerular filtration alone Meglumine effect of osmotic diuresis: (a) lower concentration of urinary iodine per mL urine (b) greater distension of collecting system N.B.:Avoid meglumine in "at risk" patients (higher incidence of contrast reactions than sodium!)B.SODIUMextensive reabsorption by tubules with delayed excretion Sodium effect of reabsorption: (a)increased concentration of urinary iodine (improved visualization)(b)less distension of collecting system (ureteral compression necessary) Notes:

Home : UROGENITAL TRACT : Anatomy and function of urogenital tract : DEVELOPMENTAL RENAL ANOMALIES

Numerary Renal Anomaly 1.Supernumerary kidney2.Complete / partial renal duplication3.Abortive calix4.Unicaliceal (unipapillary) kidney Notes:


Renal Underdevelopment 1.Congenital renal hypoplasia2.Renal agenesis3.Renal dysgenesis Notes:


Renal Ectopia Normal location of kidneys: 1st-3rd lumbar vertebra Incidence:0.2% (autopsy series) Longitudinal Renal Ectopia Location:pelvic, sacral, lower lumbar level, intrathoracic; L > R must demonstrate aberrant arteriesDDx:displacement through diaphragmatic hernia (nonaberrant); hypermobile kidney Pelvic kidney =ectopic kidney due to failure of renal ascentIncidence:1:725 birthsMay be associated with: (1)vesicoureteral reflux(2)hydronephrosis due to abnormally high insertion of ureter into renal pelvis(3)hypospadia (common)(4)contralateral renal agenesis blood supply via iliac vessels / aorta nonrotation = anteriorly positioned renal pelvis (common) Crossed Renal Ectopia =kidney located on opposite side of midline from its ureteral orifice; usually L > R and crossed kidney inferior to normal kidneyCause:? faulty development of ureteral bud, vascular obstruction of renal ascentAssociated with:obstruction urolithiasis, infection, reflux, megaureter, hypospadia, cryptorchidism, urethral valves, multicystic dysplasia(a)fused (common)(b)separate (rare) invariably aberrant renal arteries distal ureter inserts into trigone on the side of origin Renal Fusion ="lump, cake, disk, horseshoe"Cx:aberrant arteries may cross and obstruct ureter Discoid / pancake kidney =bilateral fused pelvic kidneysAssociated with: abnormal testicular descent, tetralogy of Fallot, vaginal agenesis, sacral agenesis, caudal regression, anal anomalies Renal Malrotation collecting structures may be positioned ventrally (most common), lateral (rare), dorsal (rarer), transverse (along AP axis) "funny-looking calices" = developmental usually nonobstructive ectasia

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Home : UROGENITAL TRACT : Anatomy and function of urogenital tract

ADRENAL ANATOMY from periphery to centrum: (a)renin-angiotensin-dependent outer adrenal cortex:zona glomerulosa=mineralocorticoid (aldosterone)(b)corticotropin-dependent inner adrenal cortex:zona fasciculata=cortisolzona reticularis=sex hormones (androgen, estrogen)(c)medulla=norepinephrine, epinephrine mnemonic:"Glomerular Filtration Rate May Give Answers"Glomerulosa Fasciculata Reticulosa Mineralocorticoids Glucocorticoids Androgens Normal size:3-5 x 3 x 1 cmNormal weight:3-5 gVisualizationby CT:Left side 100%, Right side 99%by US:Left side 45%, Right side 80%

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SCROTAL ANATOMY Scrotal wall thickness: 2-8 mm (3-6 mm in 89%) Tunica vaginalis =inferior extension of processus vaginalis of the peritoneumHydrocele: small to moderate in 14% of normals

Testis Epididymis Spermatic Cord Notes:

Home : UROGENITAL TRACT : Anatomy and function of urogenital tract : SCROTAL ANATOMY

Testis Average size of testis: 3.8 x 3.0 x 2.5 cm (decreasing with age) Length of testis:3-5.5 cm (mature);1-1.5 cm (newborn)Testicular cysts:in 8% of normals (average size 2-3 mm), numbers increasing with age Appendix Testis =small stalked appendage at upper pole of testis= remnant of paramesonephric duct Tunica Albuginea =fibrous covering of testis, invaginating into testicular parenchyma at mediastinum testis; externally covered by visceral layer of tunica vaginalis; internally applied to tunica vasculosa carrying the capsular artery Mediastinum Testis =converging point of ~400 cone-shaped lobules separated by fibrous septa + seminiferous tubules forming tubuli recti and the rete testis within the mediastinum linear echogenic region extending longitudinally 5-8 mm from the edge Blood

Flow To Testis PSV:4-10-19 cm/sEDV:2-5-8 cm/sRI:0.44-0.60-0.75

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Epididymis =tortuous tightly folded canal forming the efferent route from testis; consists of head (= globus major), body, tail (= globus minor)Size of globus major:11 x 7 x 6 mm (decreasing with age)Epididymal cysts:occur in 30% of normals (average size of 4 mm)Epididymal calcification:in 3%Appendix epididymis= occasionally duplicated, small stalked appendage of globus major

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Spermatic Cord =testicular + deferential + cremasteric aa., pampiniform plexus of veins, vas deferens, nerves, lymphatics Notes:


ZONAL ANATOMY OF PROSTATE Normal weight:20 ± 6 gNormal size:2.8 cm (craniocaudad), 2.8 cm (anteroposterior), 4.8 cm (width)A.Outer gland1.Central zone:surrounds ejaculatory ducts from their entrance at prostatic base to verumontanum; 25% of glandular tissue2.Peripheral zone:extends from base of prostate to apex along rectal surface;70% of glandular tissueB.Inner gland1.Transition zone:on each side of internal sphincter; 4% of glandular tissue; enlarges with BPH2.Periurethral zone:surrounding urethra; 1% of

glandular tissue Notes:

Home : UROGENITAL TRACT : Anatomy and function of urogenital tract : ANATOMY OF URETHRA

Male Urethra extends through corpus spongiosum (composed of large venous sinuses) A.Posterior urethra1.Prostatic urethra = from vesical neck to triangular ligament-orifices of ducts from prostatic acini on floor-verumontanum = colliculus seminalis = prostatic utricle (fused end of müllerian ducts)-orifice of the two ejaculatory ducts2.Membranous urethra = portion traversing urogenital diaphragm-pea-sized bulbourethral glands of Cowper lie laterally + posteriorly between fasciae and sphincter urethrae within urogenital diaphragmB.Anterior = cavernous urethra1.Bulbous urethra2.Penile = pendulous urethra-many small branched tubular periurethral glands of Littré terminate in recesses (lacunae of Morgagni)Cx:recurring urethral discharge following chronic urethritis, latent gonorrheal urethritis, stricture

formation3.Fossa navicularis

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Home : UROGENITAL TRACT : Anatomy and function of urogenital tract : ANATOMY OF URETHRA

Female Urethra 3-5 cm in length, 6 mm in diameter urethral crest = posteriorly located prominent fold Two sets of glands: (a)urethral glands = terminate separately along entire length of urethra(b)paraurethral glands = glands of Skene (homologues of prostatic ducts) are formed by an interdependent conducting system and exit on either side of midline just posterior to urethral meatus draining into vaginal vestibuleCx:chronic gonorrheal urethritis1. Intrapelvic urethra =upper 2/3 of urethra that lies behind symphysis pubis2.Membranous urethrasurrounded by sphincter membranacea urethrae (weaker less important structure than in male) 3.Perineal urethralower 1/3 extending from superior fascia of urogenital diaphragm to meatus between labia minora

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Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders

ABORTIVE CALYX = developmental anomaly with short blind-ending outpouching of pyramid without papillary invaginationLocation:(a) renal pelvis(b) infundibulum (mostly upper pole)

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ACQUIRED CYSTIC KIDNEY DISEASE =ACQUIRED CYSTIC DISEASE OF UREMIA=development of numerous fluid-filled renal cysts in patients with chronic renal failure undergoing hemodialysis Successful transplant probably stops development of additional cysts, but does not affect malignant potential!Prevalence:in 10-20% after 1-3 years, in 40-60% after 3-5 years, in 90% after 5-10 years of hemodialysis;in 25% of renal allograft recipientsProposed etiologies: (a)altered compliance of tubular basement membrane(b)intraand extratubal obstruction due to focal proliferation of tubular epithelium(c)obstruction of ducts by interstitial fibrosis / oxalate crystals(d)toxicity from circulating metabolites (endogenous / exogenous toxins, mutagens, mitogens, growth factors)(e)vascular insufficiencyAt increased risk:older menHisto:cysts lined by flattened cuboidal / papillary epitheliumIn 13-20% associated with: (a)small papillary / tubular / solid clear-cell adenomas1 cm in diameter(b)renal cell carcinoma (in 3-6%): 7-year interval between transplantation + detection of RCC small end-stage kidneys (3 cysts + NO history of hereditary cystic diseaseCx:spontaneous hemorrhage into cyst (macrohematuria / retroperitoneal hemorrhage from cyst rupture)

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AIDS azotemia, proteinuria, hematuria, pyuria (in 38-68% sometime during illness) progressive renal failure (10%)1.HIV nephropathy (40%)=characterized by nephrotic-range proteinuria + rapidly progressive renal failure, primarily occurring in Black patientsHisto:focal + segmental glomerulosclerosis, sparse interstitial infiltrates, severe tubular degenerative changes, interstitial tubular microcystic ectasia containing protein casts mild hypertension early + rapidly progressive renal failure with 100% mortality within 6 months global enlargement of both kidneysUS (best screening test): increased cortical echogenicity (33-68%)CT: medullary hyperattenuation (14%) striated nephrogram on CECTMRI: loss of corticomedullary differentiationPrognosis:death within 6 months2.Renal infection with Pneumocystis carinii (8%) more frequent since introduction of prophylactic aerosolized pentamidine therapy encouraging extrapulmonic spread ( Burkitt lymphoma, Hodgkin disease bilateral multiple renal masses direct extension of retroperitoneal lymphadenopathy engulfing kidney, renal sinus, ureter4.Cystitis (22%)Organism:routine Gram-negative species, Candida, beta-hemolytic streptococci, Salmonella, CMV bladder wall thickening

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ACUTE CORTICAL NECROSIS =rare disorder with patchy / universal necrosis of renal cortex + proximal convoluted structures secondary to distension of glomerular capillaries with dehemoglobulinized RBCs; medulla and 1-2 mm of peripheral cortex are sparedEtiology: (a)Obstetric patient (most often): abruptio placentae= premature separation of placenta with concealed hemorrhage (50%), septic abortion, placenta previa (b)Children: severe dehydration + fever, infection, hemolytic uremic syndrome, transfusion reaction(c)Adults: sepsis, dehydration, shock, myocardial failure, burns, snakebite, abdominal aortic surgery, hyperacute renal transplant rejection protracted + severe oliguria / anuria A.EARLY SIGNS diffusely enlarged smooth kidneys absent / faint nephrogramUS: loss of normal corticomedullary region with hypoechoic outer rim of cortexNUC: severely impaired renal perfusionB.LATE SIGNS small kidney (after a few months) "tramline" / punctate calcifications along margins of viable and necrotic tissue (as early as 6 days)US: hyperechoic cortex with acoustic shadowingPrognosis:poor chance of recovery

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ACUTE DIFFUSE BACTERIAL NEPHRITIS =ACUTE SUPPURATIVE PYELONEPHRITIS=more severe and extensive form of acute pyelonephritis, which may lead to diffuse necrosis (phlegmon)Organism:Proteus, Klebsiella > E. coliPredisposed:diabetics (60%)

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ACUTE INTERSTITIAL NEPHRITIS =infiltration of interstitium by lymphocytes, plasma cells, eosinophils, few PMNs + edemaCause:allergic / idiosyncratic reaction to drug exposure (methicillin, sulfonamides, ampicillin, cephalothin, penicillin, anticoagulants, phenindione, diphenylhydantoin) eosinophilia (develops 5 days to 5 weeks after exposure) large smooth kidneys with thick parenchyma normal / diminished contrast densityUS: normal / increased echogenicity

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ACUTE TUBULAR NECROSIS =temporary reversible marked reduction in tubular flow rateEtiology: (a)DRUGS: bichloride of mercury, ethylene glycol (antifreeze), carbon tetrachloride, bismuth, arsenic, uranium, urographic contrast material (especially when associated with glomerulosclerosis in diabetes mellitus), aminoglycosides (gentamicin, kanamycin)(b)ISCHEMIA: major trauma, massive hemorrhage, postpartum hemorrhage, crush injury, myoglobulinuria, compartmental syndrome, septic shock, cardiogenic shock, burns, transfusion reaction, severe dehydration, pancreatitis, gastroenteritis, renal transplantation, cardiac surgery, biliary surgery, aortic resectionPathophysiology:profound reduction in renal blood flow due to elevated arteriolar resistance smooth large kidneys, especially increase in AP diameter >4.63 cm (due to interstitial edema) diminished / absent opacification of collecting system immediate persistent dense nephrogram (75%) increasingly dense persistent nephrogram (25%) diffuse calcifications (rare)US: normal to diminished echogenicity of medulla sharp delineation of swollen pyramids normal (89%) / increased (11%) echogenicity of cortex elevated resistive index >0.75 (in 91% excluding patients with hepatorenal syndrome); unusual in prerenal azotemiaAngio: normal arterial tree with delayed emptying of intrarenal vessels slightly delayed / normal venous opacificationNUC: poor concentration of Tc-99m glucoheptonate / Tc-99m DTPA well-maintained renal perfusion better renal visualization on immediate postinjection images than on delayed images progressive parenchymal accumulation of I-131 Hippuran / Tc-99m MAG3 no excretion

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ADDISON DISEASE =PRIMARY ADRENAL INSUFFICIENCY 90% of adrenal cortex must be destroyed!Course:acute (adrenal apoplexy), subacute (disease present for L; bilateral in 10%B.ADULT1.Anticoagulant therapy: during initial 3 weeks2.Stress caused by sepsis: Waterhouse-Friderichsen syndrome3.Surgery: orthotopic liver transplantation4.Adrenal venous sampling5.Tumor6.Blunt abdominal traumaPrevalence:2% (in 28% of autopsies)Location:R:L = 9:1, bilateral in 20% round / oval hematoma (in 83%) located in medulla + stretching cortex around hematoma obliteration of gland by diffuse irregular hemorrhage (in 9%) uniform adrenal enlargement (in 9%) periadrenal hemorrhage causes ill-defined adrenal margin + stranding + asymmetric thickening of diaphragmatic crus mass displacing renal axis gradual decrease in size peripheral calcification occurring after 1 weekUS: initially echogenic becoming progressively hypoechoic (degeneration, lysis)CT: high-attenuation mass (50-90 HU) in acute / subacute stage Notes:


ADRENOCORTICAL ADENOMA A.NONHYPERFUNCTIONINGcharacterized by (a)normal lab values of adrenal hormones(b)NO pituitary shutdown of the contralateral gland(c)activity on NP-59 radionuclide scansIncidence:incidental finding in 0.6 -1.5% of CT examinations, in 3-9% at autopsy surveillance CT to confirm lack of growthRx:surgical removal for masses 3-5 cm as indeterminate potentially malignant neoplasmsDDx:metastasis B. HYPERFUNCTIONING 1.Primary hyperaldosteronism (= Conn syndrome)Pathophysiology:secretion of aldosterone by an adenoma is pulsatile ACTH infusion incites a dramatic increase in levels of cortisol + aldosterone for venous sampling2.Cushing syndrome (10%)3.Virilization:(a) hirsutism + clitoromegaly in girls (b) pseudopuberty in boys most common type of hormone elevation in children elevated testosterone levels >0.55 ng/mL4.Feminization (estrogen production) contralateral atrophic gland (secondary to ACTH suppression with autonomous adenoma) unilateral focus of I-131 NP-59 radioactivity + contralateral absence of iodocholesterol accumulation (DDx: hyperplasia [bilateral activity]) well-defined sharply marginated mass 4.5 mg/dL causes acute renal failure in 60% of nondiabetics + 100% of diabetics!Previously considered but no longer accepted risk factors: dehydration, hypertension, proteinuria, peripheral vascular disease, age >65 years, multiple myeloma Mechanism: increase in renal perfusion by vasodilatation (via prostaglandin I2 ± E2) followed by vasoconstriction (via angiotensin II, norepinephrine, vasopressin) Time course: (a)rise in serum creatinine within 1-2 days(b)peak at 4-7 days(c)return to normal by 10-14 days persistent nephrogram on plain film cortical attenuation >140 HU on CT with 24-hour delayRecommendation: Employ nonionic contrast media (LOCM appears safe in patients without renal dysfunction / underlying risk factors in doses as large as 800 mL [300 mg iodine per mL]) Do not exceed maximum allowed dose (Cigarroa formula for HOCM): Contrast limit (mL) 60% by weight = ( 5 mL x body weight (kg) ) / ( serum creatinine (mg/100 mL) ) Notes:


CUSHING SYNDROME =HYPERCORTISOLISM = excessive glucocorticoid secretion from either exogenous / endogenous sourcesEtiology: A.ACTH-INDEPENDENT1.Exogenous cortisol2.Primary adrenal abnormality (20%):(a)primary pigmented nodular adrenocortical hyperplasia (children, young adults)(b)adrenocortical adenoma (10-20% of cases; 10% in adults, 15% in children)(c)adrenocortical carcinoma (5-10% of cases; 10% in adults, 66% in children)B.ACTH-DEPENDENT=overproduction of corticotropin with adrenal hyperplasia (in up to 85%)1.Exogenous ACTH2.Paraneoplastic ectopic ACTH production (20%): oat cell carcinoma of lung (8%), liver cancer, prostate cancer, ovarian cancer, breast cancer, bronchial / thymic carcinoid, bronchial adenoma, pancreatic islet cell tumor (10%), medullary carcinoma of thyroid, thymoma, pheochromocytoma Bronchial + thymic carcinoids are often 50 years; M:F = 1:2Predisposed:diabetes mellitus, neurogenic bladder, bladder outlet obstruction, chronic UTIOrganism:E. coli, E. aerogenes, P. mirabilis, S. aureus, streptococci, Clostridium perfringens, Nocardia, CandidaMay be associated with:emphysematous pyelitis / pyelonephritis pneumaturia (rare)Plain film: translucent streaky irregular area / ring of air bubbles in bladder wall intraluminal air-fluid levelUS: shadowing echogenic foci within area of bladder wall thickeningCT (most specific modality) DDx:(a)Gas within bladder:trauma, urinary tract instrumentation, enterovesical fistula (b)Gas extern to bladder:rectal gas, emphysematous vaginitis, pneumatosis cystoides intestinalis, gas gangrene of uterus Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : CYSTITIS

Granulomatous Cystitis = Tuberculous Cystitis irritable hypertonic bladder with decreased capacity disease process usually starts at trigone spreading upward and laterally calcification of bladder wall (rare) Notes:


Hemorrhagic Cystitis Cause:unclear(a)nonspecific: negative culture(b)bacterial: E. coli (in 17%)(c)viral (adenovirus in 19%): negative culture, viral exanthem(d)cytotoxic: cyclophosphamide (Cytoxan®), in 15% of patients within 1st year of treatment echogenic mobile clumps of solid material(= intraluminal blood clots) Notes:


Interstitial Cystitis Age:postmenopausal female Notes:

pink pseudoulceration of bladder mucosa characteristically at vertex of bladder (= Hunner ulcer)


Bullous Edema Of Bladder Wall Cause:continuous internal contact with Foley catheter, involvement of bladder wall by external contact in pelvic inflammatory conditions (eg, Crohn disease, appendicitis, diverticulitis) smoothly thickened / polypoid redundant hypoechoic mucosa DDx:bladder neoplasm, ureterocele, pseudoureterocele, neurofibromatosis, pseudosarcomatous myofibroblastic proliferations

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DIABETES MELLITUS =multisystem disorderPrevalence:14 million patients in United StatesPath:macro- and microvascular disease; neuropathy increased susceptibility to infectionA.CHRONIC EFFECTS1.Papillary necrosis2.Renal artery stenosis3.Vas deferens calcificationB.URINARY TRACT INFECTIONS1.Renal and perirenal abscess2.Emphysematous pyelonephritis3.Emphysematous cystitis4.Fungal infection: Candida, Aspergillus5.Xanthogranulomatous pyelonephritisC.GENITAL INFECTION1.Fournier gangrene2.Postmenopausal tubo-ovarian abscess

Diabetic Nephropathy Diabetic Cystopathy Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : DIABETES MELLITUS

Diabetic Nephropathy =defined as persistent proteinuria (>500 mg of albumin/24 hours) + retinopathy + elevated blood pressure Most common cause of end-stage renal disease!Incidence:35-45% of IDDM; FHisto:diffuse intercapillary glomerulosclerosisMortality:90% after 40 yearsEarly: renal enlargement (renal hypertrophy with glomerular expansion)Late: progressive decrease in size diffuse cortical hyperechogenicity with gradual loss of corticomedullary differentiation resistive index >0.7 (very late)IVP: contrast material may induce renal failure (= rise in serum creatinine level 1-5 days after exposure) Keep patient well hydrated with 0.45% saline! Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : DIABETES MELLITUS

Diabetic Cystopathy Cause:autonomous peripheral neuropathyHisto:vacuolation of ganglion cells in bladder wall, giant sympathetic neurons, hypochromatic ganglion cells, demyelination insidious impairment of bladder sensation decreased reflex detrusor activity enlarged postvoid residual urine volumeCx:vesicoureteral reflux, recurrent pyelonephritis, pyohydronephrosis, overflow incontinence Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : EPIDIDYMITIS

Acute Epididymitis =ACUTE EPIDIDYMO-ORCHITIS=most common acute pathologic process in postpubertal age secondary to ascending infection (usually beginning as prostatitis)Incidence:634,000 cases/year; 35 years of age Escherichia coli + Proteus mirabilis(b)15 cm/s with PSV ratio >1.9 compared with normal side detection of venous flow diastolic flow reversal in testicular artery (due to epididymal edema with obstruction of venous outflow)NUC (true positive rate of 99%): symmetric perfusion of iliac + femoral vessels markedly increased perfusion through spermatic cord vessels (testicular + deferential arteries) curvilinear increased activity laterally in hemiscrotum on static images (also centrally if testis involved) increased activity of scrotal contents on static images (hyperemia + increased capillary permeability)Rx:antimicrobial therapy, scrotal elevation, bed rest, analgesics, ice packsCx:(1)Focal / diffuse orchitis (20-40%)(2)Epididymal abscess (6%) / testicular abscess (6%)(3)Testicular infarction (3%) from extrinsic compression of testicular blood flow(4)Late testicular atrophy (21%)(5)Hydropyocele(6)Fournier gangreneDDx:(1)Testicular abscess (increased perfusion with centrally decreased uptake)(2)Hydrocele (normal perfusion, no uptake)(3)Testicular tumor (slightly increased perfusion; in- / decreased uptake; no associated epididymal hyperemia on CFI; positive tumor markers: HCG, AFP) Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : EPIDIDYMITIS

Chronic Epididymitis US: Notes:

enlarged hyperechoic epididymis


ERECTILE DYSFUNCTION =IMPOTENCE (term replaced due to negative connotation)=inability to obtain / maintain a penile erection sufficient for vaginal penetration in 50% or more attempts during intercoursePhysiology: Psychogenic phase: stimuli from thalamic nuclei, rhinencephalon, limbic system converge in medial preoptic anterior hypothalamic area Neurologic phase: sacral nerve roots (S2-S4) contribute fibers to pelvic sympathetic plexus stimulation of cavernous n. (parasympathetic nerve) causes changes in blood flow resulting in full erection stimulation of pudendal n. (motor nerve) causes contraction of bulbocavernosus + ischiocavernosus muscle resulting in occlusion of veins + rigid erection Risk factors:hypertension, diabetes, smoking, CAD, peripheral vascular disease, pelvic trauma / surgery, blood lipid abnormalities, Cause: A.Organic cause (50%)1.Endocrine disorder (reducing serum testosterone / increasing serum prolactin2.Vascular disease (10-20%): increasing with age3.Neurologic disorder (10%): multiple sclerosis, spinal cord trauma, cervical spondylosis, spinal arachnoiditis, pelvic trauma, temporal lobe / idiopathic epilepsy, Alzheimer disease, Parkinson disease, tabes dorsalis, amyloidosis, primary autonomic insufficiency, cerebrovascular accidents, primary / metastatic tumor4.Chronic disease: diabetes mellitus, drugs (antihypertensives, anticonvulsants, alcohol, narcotics, psychotropic agents)5.Surgery: damage to pelvic sympathetic nerves / cavernous n. during radical prostatectomy / cystectomy Penile-brachial index (normal > 1.0) =highest penile artery pressure over mean brachial pressure 8 mm at 20-30 weeks, >10 mm after 30 weeks MA ratio of AP diameter of renal pelvis to kidney >50% caliceal distension communicating with renal pelvis Postnatal evaluation after 4-7 days of age (because of decreased GFR + relative dehydration in first days of life)!Prognosis:parenchymal atrophy + renal impairment (dependent on severity + duration) Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : HYDRONEPHROSIS

Focal Hydronephrosis =HYDROCALICOSIS = HYDROCALYX = obstructed drainage of one portion of kidneyCause:(1)Congenital: partial / complete duplication(2)Infectious stricture: eg, TB(3)Infundibular calculus(4)Tumor(5)Trauma unifocal mass, commonly in upper pole absent polar group of calices (early) dilated polar group (late) with displacement of adjacent calices delayed opacification in obstructed group focally replaced nephrogram US: anechoic cystic lesion with smooth marginsCT: focal area of water density with smooth margin and thick wall Notes:


IMPOTENCE =inability to have + maintain an erection adequate for sexual intercourseIncidence:10 million AmericansCause: A.ORGANIC (majority): diabetes (2 million), vascular disease, cancer surgery, spinal cord injury, pelvic trauma, endocrine problem, multiple sclerosis, alcoholism, drug-associated impotence(a)failure to initiate (neurogenic)(b)failure to fill (arteriogenic)(c)failure to store (venogenic)(d)end organ diseaseB.PSYCHOGENICRx:(1)Vascular reconstructive surgery(2)Oral / intracavernous pharmacotherapy(3)Vacuum erection devices(4)Penile prosthesis placement(a)nonhydraulic: semirigid, malleable, positionable(b)hydraulicalso see ERECTILE DYSFUNCTION

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JUXTAGLOMERULAR TUMOR =RENINOMA = very rare tumor arising from renin-producing juxtaglomerular cellsIncidence: ureter; bilateral in 10% corrugated / striated irregularities of pelvicaliceal walls, localized / generalized plaquelike intraluminal mass with "onion skin" pattern of contrast material in interstices caliectasis + pyelectasis common (with obstruction) ridging / filling defects of ureter associated with calculi in 25-50%Cx:premalignant condition for epidermoid carcinoma in 12% (controversial!) Notes:


LOBAR NEPHRONIA = ACUTE FOCAL BACTERIAL NEPHRITIS = focal variant of acute pyelonephritis with single / multiple areas of suppuration + necrosisOrganism:E. coli > Proteus > KlebsiellaPredisposed:patients with altered host resistance (diabetes [60%], immunosuppression), chronic catheterization, mechanical / functional obstruction, trauma fever, flank pain, pyuriaSite:usually involves entire renal lobe focal area of absent nephrogram / distorted pyelogram renal arteries displaced, renal veins compressed hypoechoic mass with ill-defined margins and disruption of corticomedullary border, NO fluid collection low attenuation zone with poorly defined transition to surrounding parenchyma Ga-67 uptake vesicoureteral reflux often presentCx:scarring, abscess Notes:


LOCALIZED CYSTIC DISEASE =multiple simple cysts involving only one portion of the kidney progressive Notes:

no family historyHisto:dilated ducts and tubules varying in size from mm to several cmPrognosis:not


LYMPHOMA Incidence:in 2.7-6% renal involvementTypes: A.NON-HODGKIN LYMPHOMArenal involvement detected in 5% of abdominal CT, in 33-65% of autopsies; occurs usually late in diseaseB.HODGKIN DISEASErenal involvement in 13% of autopsiesPatterns of involvement: (a)primary renal lymphoma (very rare)(b)hematogenous dissemination:-single / multiple foci-diffuse infiltration(c)contiguous extension from adjacent pararenal lymphomatous disease, usually extranodal clinically silent (50%) flank pain, palpable mass, weight loss hematuria compromise of renal function (urinary tract obstruction, renal vein compression, diffuse infiltration of kidney, superimposed infarct, amyloidosis, hypercalcemia) unilateral:bilateral = 3:1 multiple nodular masses (29-61%) invasion from retroperitoneal disease (11%) with involvement by transcapsular / transsinus extension single bulky tumor (7%), small solitary tumor (7-48%) diffuse infiltration (6-19%), microscopic infiltration (7%)CECT: usually homogeneous poorly marginated masses less dense than renal parenchymaUS: single / multiple anechoic / hypoechoic masses renal enlargement + decreased parenchymal echoes loss of renal sinus echoesAngio: neovascularity, encasement, vascular displacement (occasionally palisade-like configuration) Notes:


MALACOPLAKIA =uncommon chronic inflammatory response to Gram-negative infectionOrganism:E. coli (in 94%);diabetes mellitus predisposesHisto:submucosal histiocytic granulomas containing large foamy mononuclear cells (Hansemann macrophages) with intracytoplasmic basophilic PAS-positive inclusion bodies (Michaelis-Gutmann bodies) consisting of incompletely destroyed E. coli bacterium surrounded by lipoprotein membranesPeak age:5th-7th decade; M:F = 1:4 hematuria raised yellow lesion lower 2/3 of ureter > upper ureter > renal pelvis; multifocal in 75%; bilateral in 50% multiple dome-shaped smooth mural filling defects scalloped appearance if lesions confluent generalized pelviureteral dilatation (if obstructive) displacement of pelvicaliceal system + distorted central sinus complex multifocal parenchymal masses may cause diminished / absent nephrogramDDx:pyeloureteritis cystica

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MALPOSITIONED TESTIS =MALDESCENDED TESTIStestes are normally within scrotum by 28-32 weeks MAIncidence:early 3rd trimester in 10%; at birth in 3.7% (in babies >2,500 g in 3.4%; in premature babies in 30%); beyond 3 months of age in 1%Test sensitivity: MR:modality of choiceUS:20-88%; very sensitive in inguinal canal need to identify mediastinum testis (DDx: lymph node)CT:95% (testis > FMay be associated with:primary megaureter normal glomerular filtration rateSite:entire kidney / part of kidney; unilateral / bilateral kidney usually enlarged with prominent fetal lobation reduced parenchymal thickness (medulla affected, NOT cortex) mosaic-like arrangement of dilated calices (polygonal + faceted appearance, NOT globular as in obstruction) increased number of calices ABSENT caliceal cupping (semilunar instead of pyramidal configuration of papillae) NO dilatation of pelvis / ureters, NORMAL contrast excretionCx:(1) Hematuria (2) Stone formation Notes:


MEGACYSTIS-MICROCOLON SYNDROME =MEGALOCYSTIS-MICROCOLON-INTESTINAL HYPOPERISTALSIS SYNDROME (MMIH)= functional obstruction of bladder + colon characterized by (1)enlarged urinary bladder(2)small colon(3)strikingly short small intestine suspended on a primitive dorsal mesentery(4)markedly enlarged hydronephrotic kidneys with little remaining parenchymaIncidence:26 cases reported; M:F = 1:7May be associated with: diaphragmatic hernia, PDA, teeth at birth distended abdomen (large bladder + dilated small bowel loops) overflow incontinence intestinal pseudo-obstruction (poor emptying of stomach, NO peristaltic activity of small bowel)OB-US: normal amount of amniotic fluid / polyhydramnios (in spite of dilated bladder = "nonobstructive obstruction") massive + progressive bladder distension with poor emptying bilateral megaloureters ± hydronephrosis female sexBE: microcolon (transient feature of "unused colon") with narrow rectum + sigmoid malrotation / malfixation or foreshortening of small bowelVCUG distended unobstructed bladder with poor / absent muscular functionPrognosis:lethal in most cases (a few months of age) Notes:


MEGALOURETER =CONGENITAL PRIMARY MEGAURETER = TERMINAL URETERECTASIS = ACHALASIA OF URETER= URETEROVESICAL JUNCTION OBSTRUCTION = intrinsic congenital dilatation of lower juxtavesical orthotopic ureter Cause:aperistaltic juxtavesical (1.5 cm long) segment secondary to faulty development of muscle layers of ureter (functional, NOT mechanical obstruction)Incidence:all ages; second most common cause of hydronephrosis in fetus and newborn;M:F = 2-5:1 Associated disorders (in 40%): (a)contralateral: UPJ obstruction, reflux, ureterocele, ureteral duplication, renal ectopia, renal agenesis(b)ipsilateral: caliceal diverticulum, megacalicosis, papillary necrosis asymptomatic (mostly) pain abdominal mass hematuria infectionLocation:L:R = 3:1, bilateral in 15-40% prominent localized dilatation of pelvic ureter (up to 5 cm in diameter) usually not progressive, but may involve entire ureter + collecting system vigorous nonpropulsive to-and-fro motion in dilated segment functional smoothly tapered narrowing of intravesical ureter NO reflux, NO stenosis Notes:


MESOBLASTIC NEPHROMA =FETAL RENAL HAMARTOMA = LEIOMYOMATOUS HAMARTOMA = BENIGN CONGENITAL WILMS TUMOR = BENIGN FETAL HAMARTOMA = FETAL MESENCHYMAL TUMOR = BOLANDE TUMOR = CONGENITAL FIBROSARCOMA = FIBROMYXOMA=nonfamilial benign fibromyomatoid mass arising from renal connective tissueIncidence:most common renal neoplasm in neonate; 3% of all renal neoplasms in childrenAge:3 months mean age at presentation; may occasionally go undetected until adulthood; M > FHisto:smooth muscle cells + immature fibroblasts resembling leiomyoma containing trapped islands of embryonic glomeruli, tubules, vessels, hematopoietic cells, cartilageIn 14% associated with:prematurity, polyhydramnios, GI + GU tract malformations, neuroblastoma large flank mass hematuria (20%) / hypertension (4%), anemia usually replaces 60-90% of renal parenchyma usually solid but may produce multiple cystic spaces NO sharp cleavage plane toward normal parenchyma, may extend beyond capsule calcifications (rare) NO venous extension (DDx from Wilms tumor)IVP: large noncalcified renal mass with distortion of collecting system usually NO herniation into renal pelvis (DDx from MLCN)US: evenly echogenic tumor with concentric echogenic + hypoechoic rings resembling uterine fibroids complex mass with hemorrhage + cyst formation + necrosisAngio: hypervascular mass with neovascularity + displacement of adjacent vesselsCx:transformation to metastasizing spindle cell sarcoma (rare)Rx:complete resectionPrognosis:excellent

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METASTASES TO KIDNEY Most common malignant tumor of the kidney (2-3 times as frequent as primaries in autopsy studies)! 5th most common site of metastases (after lung, liver, bone, adrenals)!most common primaries: bronchus, breast, opposite kidney, non-Hodgkin lymphoma, colon less common primaries: stomach, cervix, ovary, pancreas, prostate, chloroma, myeloblastoma, myeloblastic sarcoma, melanoma (45% incidence), osteogenic sarcoma, choriocarcinoma (10-50% incidence), Hodgkin lymphoma, rhabdomyosarcoma usually asymptomatic bilateral multiple small masses (due to brief survival of patient)DDx on CT:lymphoma, bilateral RCC, multiple renal infarcts, acute focal bacterial nephritis, infiltrating TCC Notes:


MULTICYSTIC DYSPLASTIC KIDNEY =MULTICYSTIC DYSGENETIC KIDNEY (MCDK)= MULTICYSTIC KIDNEY (MCK) = Potter Type II Second most common cause of an abdominal mass in neonate (after hydronephrosis)! Most common form of cystic disease in infants!Incidence:1:10,000 (for bilateral MCDK); M:F = 2:1 (for unilateral MCDK); more common among infants of diabetic mothersRisk of recurrence:2-3%Etiology: (sporadic) generalized interference with ureteral bud function before 8-10 weeks of fetal lifePathophysiology:ureteral obstruction / atresia interferes with ureteral bud division + inhibits induction and maturation of nephrons; collecting tubules enlarge into cystsHisto:immature glomeruli + tubules reduced in number + whirling mesenchymal tissue, cartilage (33%), cysts abdominal mass asymptomatic if unilateral (may go undetected until adulthood) recurrent urinary tract infections, intermittent abdominal pain, nausea + vomiting, hematuria, failure to thrive fatal due to pulmonary hypoplasia if bilateralFatal form:bilateral MCDK (4.5-21%), contralateral renal agenesis (0-11%)Location: 1.UNILATERAL multicystic dysplastic kidneymost common form (80-90%); L:R = 2:1 secondary to pelvoinfundibular atresia Associated withanomalies of contralateral side in 20-40-50%:(1)Ureteropelvic junction obstruction (7-27%)(2) Horseshoe kidney (5-9%)(3)Ureteral anomalies (5%)(4)Renal hypoplasia (4%)(5)Vesicoureteral reflux(6)Malrotation(7)Renal agenesisAssociated withipsilateral anomalies:(1)Vesicoureteral reflux (25%)(2)Ectopic ureter2.SEGMENTAL / focal renal dysplasia="multilocular cyst" secondary to(a)high-grade obstruction of upper pole moiety in duplex kidney from ectopic ureterocele(b)single obstructed infundibulum3.BILATERAL cystic dysplasiain the presence of severe obstruction in utero from posterior urethral valves / urethral atresia with oligohydramnios + pulmonary hypoplasia Types: (1)Multicystic kidney (Potter IIa) large kidney with multiple large cysts + little visible renal parenchyma(2)Hypoplastic / diminutive form (Potter IIb) echogenic small kidney APPEARANCE RELATED TO SITE OF OBSTRUCTION @ureteropelvic junction single / several large / multiple medium-sized cysts in large kidney@distal ureter / urethra small / no cysts in small kidney APPEARANCE RELATED TO TIME OF INSULT (a)early onset between 8th-11th week small / atretic renal pelvis + calices 10-20 cysts + loss of reniform appearance(b)late onset = HYDRONEPHROTIC FORM large central cyst (= dilated pelvis) often communicating with cysts some renal function may be demonstrated large kidney with lobulated contour in infancy incidental finding of small kidney in adults (secondary to arrested growth) ipsilateral atretic ureter contralateral renal hypertrophy calcification: curvilinear / ringlike in wall of cysts in30% of adults, rarely in childrenIVP + NUC: NUC preferred over IVP in first month of life as concentrating ability of even normal neonatal kidneys is suboptimal! no function (rarely faint contrast accumulation)US: normal renal architecture replaced random cysts of varying shape + size ("cluster of grapes") with largest cyst in peripheral nonmedial location (100% accurate) cysts separated by septa (100% accurate) central sinus complex absent (100% accurate) no communication between multiple cysts (93% accurate) no identification of parenchymal rim or corticomedullary differentiation (74% accurate) cysts begin to disappear in infancy kidney may be small + atrophic (as little as 1 g) / normal / large oligohydramnios in bilateral MCDK / unilateral MCDK + contralateral urinary obstructionAngio: absent / hypoplastic renal artery; angiography unnecessary since a DDx to long-standing functionless kidney is not possible OB-management: (1)Routine antenatal care + evaluation by pediatric urologist following delivery if unilateral(2)Option of pregnancy termination if 24 weeks GA Cx:(1)Renin-dependent hypertension (rare)(2)Malignancy in 30 years (30%); M:F = 8:1 90% of tumors in males occur in first 2 years of life (peak 3-24 months)! Most of the lesions in females occur between ages 4 and 20 or 40 and 60!Path:solitary large well-circumscribed multiseptated mass of noncommunicating fluid-filled loculi, surrounded by thick fibrous capsule + compressed renal parenchyma; cyst size between mm up to 4 cmHisto:(gross anatomic features are identical)1.Cystic nephroma fibrous tissue septa of undifferentiated mesenchymal and primitive glomerulotubular elements surround cysts lined by flattened cuboidal epithelium; NO blastemal / other embryonal elements 2.Cystic partially differentiated nephroblastoma =CPDNpredominantly cystic lesion with septa containing blastemal / other embryonal elements commonly asymptomatic painless abdominal mass ± sudden and rapid enlargement pain, hematuria, urinary tract infectionLocation:unilateral, often replacing an entire renal pole (usually lower pole) Size:average size of 10 cm (few cm to 33 cm) sharply well-circumscribed (characteristic) multiseptated cystic renal mass tumor surrounded by thick fibrous capsule cluster of noncommunicating "honeycombed" cysts of various sizes separated by thick septa smaller closely spaced cysts appear as solid nodules contrast enhancement of septations (secondary to tortuous fine vessels coursing through septa) curvilinear to flocculent calcification of septa / capsuleIVP: distortion of calices / hydronephrosis secondary to nonfunctional mass tendency for herniation of tumor cysts into renal pelvis (nonspecific, also seen with Wilms tumor + RCC)US: cluster of cysts separated by thick septa (SUGGESTIVE PATTERN) occasionally solid echogenic character (due to very small cysts / jellylike contents)CT: cysts with attenuation equal to / higher than water (gelatinous fluid) Cx:local recurrence / coexistent Wilms tumor (extremely rare)Rx:nephrectomyDDx:(1)Cystic Wilms tumor (overlapping age)(2)Clear cell sarcoma (poor prognosis)(3)Cystic mesoblastic nephroma (most common renal tumor of infancy)(4)Cystic RCC (mean age of 10 years)(5)Segmental form of multicystic dysplastic kidney Notes:


MULTIPLE MYELOMA It is essential that dehydration is avoided!Impairment of renal function: (1)Precipitation of abnormal proteins (Bence-Jones ± Tamm-Horsfall protein casts) into tubule lumen (30-50%)(2)Toxicity of Bence-Jones proteins on tubules(3)Impaired renal blood flow secondary to increased blood viscosity(4)Amyloidosis(5)Nephrocalcinosis from hypercalcemia Contrast-induced renal failure in multiple myeloma is not seen with greatly increased frequency! Tamm-Horsfall proteinuria (tubular cell secretion) smooth normal to large kidneys (initially), become small with time occasionally attenuated pelvo-infundibulo-caliceal system normal to diminished contrast material density; increasingly dense in acute oliguric failureUS: normal to increased echogenicityNUC in bone scintigraphy: nonspecific increased parenchymal activity

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MYCETOMA =FUNGUS BALLOrganism:typically Candida, Aspergillus, Mucor, Cryptococcus, Phycomycetes, Actinomycetes mostly mycelial (M-form) or occasionally yeast cells (Y-form)Predisposed:diabetics, debilitating illness, prolonged antibiotic therapy, leukemia, lymphoma, thymoma, immunosuppression flank pain, passing of tissue, hematuria (extremely rare) renal candidiasis associated with candidemia Candida cystitis preceded by vaginal candidiasis unilateral nonvisualization of kidney (most frequent) large irregular filling defect extending into dilated calices (retrograde contrast study) necrotizing papillitis from Candida nephritis (common) lacelike pattern (on antegrade contrast study) Notes:


NEPHROBLASTOMATOSIS =multiple / diffuse nephrogenic rests (= abnormally persistent nephrogenic cells with potential to form Wilms tumor)Incidence:in 41% with unilateral Wilms tumor, in 94% with metachronous contralateral Wilms tumor, in 99% with bilateral Wilms tumorPathogenesis:primitive renal tissue (metanephrogenic blastema) normally present up to 36 weeks of gestational age; embryonal renal tissue in mature kidney after birth retains potential to form nephroblastomatosis / Wilms tumorHisto:contains only primitive epithelial cell line without mesenchymal elements (as seen in Wilms tumor)Age:neonatal period, infancy, childhoodAssociated with: hemihypertrophy, sporadic aniridia, Klippel-Trénaunay syndrome, Beckwith-Wiedemann syndrome, trisomy 18, pseudohermaphroditism, splenic agenesis with hepatic malformation, Drash syndrome Site:(a)at periphery of renal lobe = perilobar nephrogenic rest associated with a 1-2% risk of Wilms tumor(b)within renal lobe = intralobar nephrogenic restassociated with 4-5% risk of Wilms tumor A.Multifocal (juvenile) nephroblastomatosis most common form =discrete islands of rests in cortex / columns may escape detection with imaging ± deformation of pelvicaliceal structures kidneys may be enlargedB.Superficial diffuse (late infantile) nephroblastomatosis =superficial continuous peripheral ring of rests around normal medulla + pyramidAge:2 cm in size; up to 40% in extraadrenal location are missed by CT; 93-100% sensitivity solid / cystic / complex mass with low-density areas secondary to hemorrhage / necrosis IV injection of iodinated contrast material may precipitate hypertensive crisis in patients not on alpha-adrenergic blockers!NUC:I-131 / I-123 MIBG (metaiodobenzylguanidine) scan (80-90% sensitivity; 98% specificity)Useful: (a)with clear clinical / laboratory evidence of tumor but no adrenal abnormality on CT / MRI(b)in detecting extraadrenal pheochromocytomas by whole-body scintigraphyUS: well-marginated purely solid (68%) / complex (16%) / cystic tumor (16%) homo- (46%) / heterogeneously (54%) solid tumor: isoechoic + hypoechoic (77%) / hyperechoic (23%) to renal parenchymaMRI: iso- / slightly hypointense to liver on T1WI extremely hyperintense on T2WI marked homo- / inhomogeneous enhancementAngio:intraarterial injection CONTRAINDICATED(induces hypertensive crisis) localization by aortography in >91% usually hypervascular lesion with intense tumor blush slow washout of contrast material enlarged feeding arteries + neovascularity ("spoke-wheel" pattern) parasitization from intrarenal perforating branches venous blood sampling (at different levels in IVC) Cx: malignancy in 2-14%; metastases (may be hormonally active) to bone, lymph nodes, liver, lungRx:(1)Surgical removal curative(2)Alpha-adrenergic blocker (phenoxybenzamine / phentolamine)(3)Beta-adrenergic blocker (propranolol)(4)I-131 MIBG used to treat metastasesDDx:nonfunctioning adrenal adenoma, adrenocortical carcinoma, adrenal cyst Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : POLYCYSTIC KIDNEY DISEASE

Autosomal Dominant Polycystic Kidney Disease =ADULT POLYCYSTIC KIDNEY DISEASE=Potter Type III=slowly progressive disease with nearly 100% penetrance and great variation in expressivityCause:gene located on short arm of chromosome 16 (in 90%); spontaneous mutation in 10%Incidence:1:1,000 people carry the mutant gene; 3rd most prevalent cause of chronic renal failureRisk of recurrence:50%Histo:abnormal rate of tubule divisions (Potter Type III) with hypoplasia of portions of tubules left behind as the ureteral bud advances; cystic dilatation of Bowman capsule, loop of Henle, proximal convoluted tubule, coexisting with normal tissueMean age at diagnosis: 43 years (neonatal / infantile onset has been reported); M:F = 1:1 Onset of cyst formation: -54% in 1st decade-72% in 2nd decade-86% in 3rd decademorphologic evidence in all patients by age 80 Associated with: (1)Cysts in: liver (25-50%), pancreas (9%); rare in lung, spleen, thyroid, ovaries, uterus, testis, seminal vesicles, epididymis, bladder(2)Aneurysm: saccular "berry" aneurysm of cerebral arteries (3-13%)(3)Mitral valve prolapse symptomatic at mean age of 35 years (cysts are growing with age) hypertension (50-70%) azotemia hematuria, proteinuria lumbar / abdominal pain bilaterally large kidneys with multifocal round lesions; unilateral enlargement may be the first manifestation of the disease cysts may calcify in curvilinear rim- / ringlike irregular amorphous fashion elongated + distorted + attenuated collecting system nodular puddling of contrast material on delayed images "Swiss cheese" nephrogram = multiple lesions of varying size with smooth margins polycystic kidneys shrink after beginning of renal failure, after renal transplantation, or on chronic hemodialysisNUC: poor renal function on Tc-99m DTPA scan multiple areas of diminished activity, cortical activity only in areas of functioning cortexUS: multiple cysts in cortical region (usually not seen prior to teens) diffusely echogenic when cysts small (children) renal contour poorly demarcatedOB-US: large echogenic kidneys similar to infantile PCKD (usually in 3rd trimester, earliest sonographic diagnosis at 14 weeks), can be unilateral macroscopic cysts (rare) normal amount of amniotic fluid / oligohydramnios (renal function usually not impaired) Atypical rare presentation: (a)unilateral adult PCKD(b)segmental adult PCKD(c)adult PCKD in utero / neonatal period Cx: (1)Death from uremia (59%) / cerebral hemorrhage (secondary to hypertension or ruptured aneurysm [13%]) / cardiac complications (mean age 50 years)(2)Renal calculi (3)Urinary tract infection(4)Cyst rupture(5)Hemorrhage(6)Renal cell carcinoma (increased risk)DDx: (1)Multiple simple cysts (less diffuse, no family history)(2)von Hippel-Lindau disease (cerebellar hemangioblastoma, retinal hemangiomas, occasionally pheochromocytomas)(3)Acquired uremic cystic disease (kidneys small, no renal function, transplant)(4)Infantile PCKD (usually microscopic cysts)

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Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : POLYCYSTIC KIDNEY DISEASE

Autosomal Recessive Polycystic Kidney Disease =INFANTILE POLYCYSTIC KIDNEY DISEASE=POLYCYSTIC DISEASE OF CHILDHOOD=Potter Type IIncidence:1: 6,000 to 1:50,000 livebirths; F > M; carrier frequency of 1:112Path: @kidney: abnormal proliferation + dilatation of collecting tubules resulting in multiple 1to 2-mm cysts@liver: periportal fibrosis often with abnormal proliferation + dilatation of bile ducts@pancreas: pancreatic fibrosis A.ANTENATAL FORM (most common)90% of tubules show cystic changes onset of renal failure in utero Potter sequence oligohydramnios and dystocia (large abdominal mass)Prognosis:death from renal failure / respiratory insufficiency (pulmonary hypoplasia) within 24 hours in 75%, within 1 year in 93%; uniformly fatalB.NEONATAL FORM60% of tubules show ectasia + minimal hepatic fibrosis + bile duct proliferation onset of renal failure within 1st month of lifePrognosis:death from renal failure / hypertension / left ventricular failure within 1st year of lifeC.INFANTILE FORM20% of renal tubules involved + mild / moderate periportal fibrosis disease appears by 3-6 months of agePrognosis:death from chronic renal failure / systemic arterial hypertension / portal hypertensionD.JUVENILE FORM10% of tubules involved + gross hepatic fibrosis + bile duct proliferation disease appears at 1-5 years of agePrognosis:death from portal hypertension The less severe the renal findings, the more severe the hepatic findings! @Lung severe pulmonary hypoplasia pneumothorax / pneumomediastinum@Liver portal venous hypertension tubular cystic dilatation of small intrahepatic bile ducts increase in liver echogenicity (from congenital hepatic fibrosis)@Kidneys bilateral gross renal enlargement faint nephrogram + blotchy opacification on initial images increasingly dense nephrogram poor visualization of collecting system "sunburst nephrogram" = striated nephrogram with persistent radiating opaque streaks (collecting ducts) on delayed images prominent fetal lobationCT: prolonged corticomedullary phaseUS: hyperechoic enlarged kidneys (unresolved 1- to 2-mm cystic / ectatic dilatation of renal tubules increase number of acoustic interfaces) increased renal through-transmission (high fluid content of cysts) loss of corticomedullary differentiation, poor visualization of renal sinus + renal borders occasionally discrete macroscopic cysts 0.30 hyperechoic renal parenchyma nonvisualization of urine in fetal bladder (in severe cases) oligohydramnios (33%) small fetal thoraxOB management: (1)Chromosome studies to determine if other malformations present (eg, trisomy 13 / 18)(2)Option of pregnancy termination 24 weeks if severe oligohydramnios presentRisk of recurrence:25%DDx:Meckel-Gruber syndrome, adult polycystic kidney disease

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POSTERIOR URETHRAL VALVES =congenital thick folds of mucous membrane located in posterior urethra (prostatic + membranous portion) distal to verumontanum Type I:(most common) mucosal folds (vestiges of Wolffian duct) extend anteroinferiorly from the caudal aspect of the verumontanum, often fusing anteriorly at a lower levelType II:(rare) mucosal folds extend anterosuperiorly from the verumontanum toward the bladder neck (nonobstructive normal variant, probably a consequence of bladder outlet obstruction)Type III:diaphragm-like membrane located below the verumontanum (= abnormal canalization of urogenital membrane)Incidence:1:5,000-8,000 boys; most common cause of urinary tract obstruction + leading cause of end-stage renal disease among boysTime of discovery:prenatal (8%), neonatal (34%), 1st year (32%), 2nd-16th year (23%), adult (3%) urinary tract infection (fever, vomiting) in 36% obstructive symptoms in 32% (hesitancy, straining, dribbling [20%], enuresis [20%]) palpable kidneys / bladder in neonate (21%) failure to thrive (13%) hematuria (5%)VCUG: vesicoureteral reflux, mainly on left side (75% have stage C + D! Escape routes through prostatic capsule are: (1) apex, (2) capsular margin at neurovascular bundle posterolaterally, (3) seminal vesicles!Staging (American Joint Committee on Cancer): T0No evidence of primary tumorT1Clinically inapparent nonpalpable nonvisible tumorT1a3.8 cm3 ! Metastases to lymph nodes: 0% in stage A 1 , 3-7% in stage A2, 5% in stage B1 , 10-12% in stage B2 , 54-57% in stage C; 10% with Gleason grade 15 years(3)Radiation therapy for(a)disease confined to capsule, life expectancy Proteus > Klebsiella, Enterobacter, PseudomonasAge:any; M > diffuse involvement of kidney delayed opacification of collecting system compression of collecting system (edema) nonobstructive ureteral dilatation (rare, effect of endotoxins) immediate persistent dense nephrogram, rarely striated diminished nephrographic density (global / wedge-shaped / patchy) nonvisualization of kidney (in severe pyelonephritis, rare) "tree-barking" = mucosal striations (rare)CT: area of high attenuation on unenhanced scan (= hemorrhagic bacterial nephritis) thickening of Gerota fascia + thickened bridging septa / stranding (= perinephric inflammation) generalized renal enlargement / focal swelling obliteration of renal sinus caliceal effacement thickening of walls of renal pelvis + calices mild dilatation of renal pelvis + ureter soft-tissue filling defect in collecting system (= papillary necrosis, inflammatory debris, blood clot)CECT: hypoattenuating wedge-shaped area of cortex extending from papilla to renal capsule during nephrographic phase (= lobar segments of hypoperfusion + edema) poor corticomedullary differentiation streaky linear bands of alternating hyper- and hypoattenuation parallel to axis of tubules + collecting ducts during excretory phase (diminished concentration of contrast material in tubules from ischemia + tubular obstruction by inflammatory cells + debris) persistent enhancement on delayed scans in area of earlier diminished enhancement contrast material staining of parenchyma on 3-6 hours delayed scan (= functioning renal parenchyma)US: majority of kidneys appear normal swollen kidney with decreased echogenicity loss of central sinus echoes wedge-shaped hypo- / isoechoic zones, rarely hyperechoic (due to hemorrhage) thickened sonolucent corticomedullary bands blurred corticomedullary junctions localized increase in size + echogenicity of perinephric fat ± fat within renal sinus localized perinephric exudate thickening of wall of renal pelvisMR: wedge-shaped foci of high signal intensity on contrast-enhanced fast multiplanar IR imagesRenal cortical scintigraphy (Tc-99m DMSA): focal areas of diminished uptake (in 90%)Prognosis: (1)Quick response to antibiotic treatment will leave no scars(2)Delayed treatment of acute pyelonephritis during first 3 years of life can severely affect renal function later in life: decreased renal function, hypertension (33%), end-stage renal disease (10%)Cx:(1)Renal abscess (near-water density lesion without enhancement)(2)Scarring of affected renal lobes often in children + in up to 43% in adults(3)Maternal septic shock (3%)(4)Premature labor (17%) Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : PYELONEPHRITIS

Emphysematous Pyelitis =gas confined to renal pelvis + calicesOrganism:E. coliPredisposed:diabetes mellitus (50%); M:F = 1:3May be associated with: emphysematous cystitis (rare) pyuria gas pyelogram outlining pelvicaliceal system dilated renal collecting system (frequent) ± gas in uretersDDx:reflux of gas / air from bladder or urinary diversion

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Emphysematous Pyelonephritis =life-threatening acute fulminant necrotizing infection of kidney and perirenal tissues associated with gas formationOrganism:E. coli (68%), Klebsiella pneumoniae (9%), Proteus mirabilis, Pseudomonas, Enterobacter, Candida, Clostridia (exceptionally rare)Path:acute and chronic necrotizing pyelonephritis with multiple cortical abscessesMechanism:pyelonephritis leads to ischemia + low O2 tension with anaerobic metabolism; facultative anaerobe organisms form CO2 with fermentation of necrotic tissue / tissue glucosePredisposed:immunocompromised patients, esp. diabetics (in 87-97% of cases); ureteral obstruction (in 20-40%)Average age:54 years; M:F = 1:2May be associated with:XGP features of acute severe pyelonephritis (chills, fever, flank pain, lethargy, confusion) not responding to Rx positive blood + urine cultures (in majority) urosepsis, shock fever of unknown origin + NO localizing signs in 18% multiple associated medical problems: uncontrolled hyperglycemia, acidosis, dehydration, electrolyte imbalanceLocation:in 5-7% bilateralType I (33%): streaky / mottled gas in interstitium of renal parenchyma radiating from medulla to cortex crescent of subcapsular / perinephric gas NO fluid collection (= no effective immune response)Prognosis: 69% mortality Type II (66%): bubbly / loculated intrarenal gas (infers presence of abscess) renal / perirenal fluid collection gas within collecting system (85%)Prognosis: 18% mortality parenchymal destruction absent / decreased contrast excretion (due to compromised renal function)US: high-amplitude echoes within renal sinus / renal parenchyma associated with "dirty" shadowing / "comet tail" reverberationsCAVE:(1)kidney may be completely obscured by large amount of gas in perinephric space (DDx: surrounding bowel gas)(2)gas may be confused with renal calculiCT (most reliable + sensitive modality): mottled areas of low attenuation extending radially along the pyramids extensive involvement of kidney + perinephric space air extending through Gerotas fascia into retroperitoneal space occasionally gas in renal veinsMR: signal void on T1WI + T2WI (DDx: renal calculi, rapidly flowing blood)Mortality:60-75% under antibiotic Rx;21-29% after antibiotic Rx + nephrectomy;80% with extension into perirenal spaceRx:antibiotic therapy + nephrectomy; drainage procedure with coexisting obstructionDDx:emphysematous pyelitis (gas in collecting system but not in parenchyma, diabetes in 50%, less grave prognosis) Notes:


Xanthogranulomatous Pyelonephritis =chronic suppurative granulomatous infection in chronic obstruction (calculus, stricture, carcinoma) originating in medullaIncidence:681,000 surgically proven cases of chronic pyelonephritisOrganism:Proteus mirabilis, E. coli, S. aureusPath:replacement of corticomedullary junction with soft yellow nodules; calices filled with pus and debrisHisto:diffuse infiltration by plasma cells + histiocytes + lipid-laden macrophages (xanthoma cells)Peak age:45-65 years; all ages affected, may occur in infants; M:F = 1:3-1:4 pyuria(95%) flank pain(80%) fever(70%) palpable mass(50%) weight loss(50%) microscopic hematuria(50%) reversible hepatic dysfunction with elevated liver function tests (50%) Symptomatic for 6 months prior to diagnosis in 40%! A.DIFFUSE XGP (83-90%)B.SEGMENTAL / FOCAL XGP (10-17%)=tumefactive form due to obstructed single infundibulum / one moiety of duplex systemDDx:renal cell carcinoma kidney globally enlarged (smooth contour uncommon) / focal renal mass contracted pelvis with dilated calices totally absent / focally absent nephrogram central obstructing calculus: staghorn calculus in 75% extension of inflammation into perirenal space, pararenal space, ipsilateral psoas muscle, colon, spleen, diaphragm, posterior abdominal wall, skinRetrograde: complete obstruction at ureteropelvic junction / infundibulum / proximal ureter contracted renal pelvis, dilated deformed calices + nodular filling defects irregular parenchymal masses with cavitationCT: low attenuation masses replacing renal parenchymaUS: hypoechoic dilated calices with echogenic rim hypoechoic masses frequently with low-level internal echoes replacing renal parenchyma loss of corticomedullary junction parenchymal calcifications are uncommonAngio: stretching of segmental / interlobar arteries around large avascular masses hypervascularity / blush around periphery of masses in late arterial phase (= granulation tissue) venous encasement + occlusionDDx:hydronephrosis, avascular tumorRx:nephrectomy

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PYELOURETERITIS CYSTICA =hyperplastic transitional epithelial cell collections projecting into ureteral lumen Indicative of past / present urinary tract infection!Cause:chronic urinary tract irritant (stone / infection)Histo:numerous small submucosal epithelial-lined cysts representing cystic degeneration of epithelial cell nests within lamina propria (cell nests of von Brunn) formed by downward proliferation of buds of surface epithelium that have become detached from the mucosa Organism:E. coli > M. tuberculosis, Enterococcus, Proteus, schistosomiasisPredisposed:diabeticsAge:6th decade; more prevalent in women no specific symptoms; ± hematuriaLocation:bladder >> proximal 1/3 of ureter > ureteropelvic junction; unilateral >> bilateral multiple small round smooth lucent defects of 1-3 mm in size; scattered discrete / clustered persist unchanged for years in spite of antibiotic therapyCx:increased incidence of transitional cell carcinomaDDx:(1)Spreading / multifocal TCC(2)Vascular ureteral notching(3)Multiple blood clots(4)Multiple polyps(5)Allergic urticaria of mucosa(6)Submucosal hemorrhage (eg, anticoagulation)

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PYONEPHROSIS =presence of pus in dilated collecting system secondary to infected hydronephrosisPath:purulent exudate composed of sloughed urothelium + inflammatory cells from early formation of microabscesses + necrotizing papillitisOrganism:most commonly E. coliUS: dispersed / dependent internal echoes within dilated pelvicaliceal system shifting urine-debris level dense peripheral echoes in nondependent location + shadowing (gas from infection)Cx:1.XGP2.Renal abscess3.Perinephric abscess4.Fistula to duodenum, colon, pleura

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RADIATION NEPHRITIS Histo:interstitial fibrosis, tubule atrophy, glomerular sclerosis, sclerosis of arteries of all sizes, hyalinization of afferent arterioles, thickening of renal capsuleThreshold dose:2,300 rads over 5 weeks clinically resembling chronic glomerulonephritis normal / small smooth kidney consistent with radiation field parenchymal thickness diminished (globally / focally;related to radiation field) diminished nephrographic density Notes:


REFLUX ATROPHY Cause:increased hydrostatic pressure of pelvicaliceal urine with atrophy of nephrons secondary to long-standing vesicoureteral reflux small smooth kidney with loss of parenchymal thickness widened collecting system with effaced papillae longitudinal striations from redundant mucosa when collecting system is collapsed Do NOT confuse with reflux nephropathy! Notes:


REFLUX NEPHROPATHY =CHRONIC ATROPHIC PYELONEPHRITIS = ascending bacterial urinary tract infection secondary to reflux of infected urine from lower tract + tubulointerstitial inflammation in childhood (hardly ever endangers adult kidney); most common cause of small scarred kidneyEtiology:3 essential elements:(1) Infected urine (2) Vesicoureteral reflux (3) Intrarenal reflux Age:usually young adults (subclinical diagnosis starting in childhood); M < F fever, flank pain, frequency, dysuria hypertension, renal failure may have no history of significant symptomsSite:predominantly affecting poles of kidneys secondary to presence of compound calyces having distorted papillary ducts of Bellini (= papillae with gaping openings instead of slitlike openings of interpolar papillae) normal / small kidney; uni- / bilateral; uni- / multifocal focal parenchymal thinning with contour depression in upper / lower pole (more compound papillae in upper pole), scar formation only up to age 4 retracted papilla with clubbed calyx subjacent to scar contralateral / focal compensatory hypertrophy (= pseudotumor) dilated ureters (secondary to reflux) sometimes with linear striations (redundant / edematous mucosa)US: focally increased echogenicity within cortex (scar)Angio: small tortuous intrarenal arteries, pruning of intrarenal vessels vascular stenoses, occlusion, aneurysms inhomogeneous nephrographic phaseNUC (Tc-99m glucoheptonate / DMSA with SPECT most sensitive method): focal / multifocal photon-deficient areasCx:1.Hypertension2.Obstetric complications3.Renal failure

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RENAL / PERIRENAL ABSCESS =usually complication of renal inflammation with liquefactive necrosis; 2% of all renal massesPathway of infection: (a)ascending (80%): associated with obstruction (UPJ, ureter, calculus)(b)hematogenous (20%): infection from skin, teeth, lung, tonsils (S. aureus), endocarditis, intravenous drug abuseOrganism:E. coli, ProteusPredisposed:diabetics (twice as frequent compared with nondiabetics) positive urine culture in 33% positive blood culture in 50% pyuria, hematuria (absent if abscess isolated within parenchyma) Renal Abscess Carbuncle Perinephric Abscess Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RENAL / PERIRENAL ABSCESS

Renal Abscess

may have negative urine analysis / culture (in up to 20%)IVP:

focal mass displacing collecting systemCT:

hypoattenuating focal renal mass with

thick irregular enhancing wall / pseudocapsule ± presence of gas thickened septa + Gerota fascia perinephric fat obliteration US: slightly hypoechoic (early), hypo- to anechoic (late) mass with irregular margins + increased through-transmission ± septations ± microbubbles of gasNUC (Ga-67 citrate / In-111 leukocytes): hot spotDDx: cystic renal cell carcinoma Notes:


Carbuncle =multiple coalescent intrarenal abscesses Term should not be used in radiology reports!

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Perinephric Abscess Cause:acute pyelonephritis / extension of renal abscess through capsulePredisposed:diabetics (in 30%), urolithiasis, septic emboli 14-75% of patients with perinephric abscess have diabetes mellitus! loss of psoas margin / obscuration of renal contour renal displacement focal renal mass scoliosis concave to involved side respiratory immobility of kidney = renal fixation occasionally gas in renal fossa unilateral impaired excretion pleural effusion

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RENAL ADENOMA Small adenoma 80 years of age); increased frequency in tobacco users + patients on long-term dialysisAge:usually >30 years; M:F = 3:1Types: (1)Papillary / cystadenoma (38%)(2)Tubular adenoma (38%)(3)Mixed type adenoma (21%)(4)Alveolar adenoma (3%) = precursor of RCC solitary in 75%, multiple in 25% usually R visualization of single kidney (DDx: additional kidney in ectopic location) absent adrenal gland (11%) absent / rudimentary renal vessels colon occupies renal fossa compensatory contralateral renal hypertrophy (50%) B. BILATERAL RENAL AGENESIS (= Potter syndrome) Incidence:1:3,000 to 1:10,000 pregnancies; M:F = 2.5:1Risk of recurrence:1.5:1(b)presence of collateral vessels(c)greater than 70% stenosis with poststenotic dilatation(d)transstenotic pressure gradient >40 mm Hg(e)decrease in renal size 15-20% of patients remain hypertensive after restoration of normal renal blood flow (= renal artery stenosis without renovascular hypertension)!Cause: 1.Atherosclerosis (60-90%) mostly in proximal 2 cm of main renal artery Any of multiple renal arteries (occurring in 14-28% of the population) may be affected!2.Fibromuscular dysplasia (10-30%)3.Others (105 mm Hg2.Patients 48 hours for captopril / lisinopril!(a)captopril (Capoten®):Dose:1 mg/kg PO for pediatric patient, 25 or 50 mg PO for adult patientTechnique:radiopharmaceutical injected 60 minutes after ingestion of captopril(b)enalaprilat (Vasotec®):Dose:0.04 mg/kg IV (up to 2.5 mg maximum)Technique: }10 mL fluid/kg body weight PO over 1 hour (to ensure adequate hydration)}5 mCi IV Tc-99m MAG3 + 20 mg IV furosemide}image acquisition for 22 minutes}postvoid image (or Foley catheter with PVR)}0.04 mg/kg IV enalaprilat (up to a maximum of 2.5 mg) infused over 5 minutes}5 mCi IV Tc-99m MAG3 + 20 mg IV furosemide injected 15 minutes after injection of enalaprilat}image acquisition for another 22 minutes

Semiquantitative interpretation of renograms: delay in the time to peak activity + elevation of 3rd phase of curve residual cortical activity (= activity remaining at 20 minutes expressed as percent of peak) >30% with increase by 10% over baseline following ACEI challenge asymmetry of renal uptake 150 cm/sec for angles 70° (with many false positives due to suboptimal Doppler angles) ratio of peak renal artery velocity to peak aortic center stream velocity >3.5 (for >60% stenosis; 0-91% sensitive, 37-97% specific) poststenotic spectral broadening ± flow reversal absence of blood flow during diastole (for >50% stenosis)Problems: (a)technically inadequate examination (gas, corpulence, respiratory motion) in 6-49%; usually limited to children + thin adults(b)multiple renal arteries in 16-28%(c)"false" tracings from large collateral vessels / reconstituted segments of main renal artery(d)need to visualize entire length of renal artery(e)transmitted cardiac / aortic pulsations obscure renal artery waveform recordings(2)indirect signs = measurement of distal arterial segments tardus-parvus pulse:(a)gradual (= tardus) slope of Doppler waveform during systole = delay in acceleration / pulse rise time of >0.07-0.12 sec(b)attenuated (= parvus) Doppler waveform amplitude = decrease in peak systolic velocity to 3 m/sec2 (single most sensitive screening parameter; 76% sensitive + 95% specific at 20% disease prevalence) RI 5% between both kidneys (82% sensitive + 92% specific for stenosis >50%, 100% sensitive + 94% specific for stenosis >60%) absent early systolic peak (ESP) segmental arterial flow detectable with renal artery occlusionFalse-negative:stenosis in accessory renal arteryFalse-positive:coarctation

Renal Artery Waveforms with Normal Early Systolic Peaks AT = acceleration time; DV = velocity difference between early systolic peak velocity and late diastolic velocity; ESP = early systolic peak; LSP = late systolic peak; Acceleration index (AI) = DV/AT; 98% PPV for exclusion of renal artery stenosis with a normal spectral tracing from each renal pole

Tardus-Parvus Pattern Results for >60% renal artery stenosis: sensitivityspecificityaccuracyAT > 0.07 sec81%95%91%AI < 30 cm/sec2 89%86%87%absent ESP92%96%95% Arteriosclerotic Renal Artery Disease Fibromuscular Dysplasia Of Renal Artery Neurofibromatosis Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RENAL ARTERY STENOSIS

Arteriosclerotic Renal Artery Disease Incidence:in up to 6% of hypertensive patients; most common cause of secondary hypertensionAge:>50 years; M > FPath:lesion primarily involving intima worsening of preexistent hypertension abrupt onset of severe hypertension >180/110 mm Hg vascular bruit in 40-50% (present in 20% of hypertensive patients without renal artery stenosis)Associated with:severe arteriosclerosis of aorta, cerebral, coronary, peripheral arteriesLocation:main renal artery (93%) + additional stenosis of renal artery branch (7%); bilateral in 31% eccentric stenosis in proximal 2 cm of renal artery, frequently involving orifice decrease in renal length over time (= high-grade renal artery stenosis with risk for occlusion)Prognosis:progression of atherosclerotic lesion (40-45%) to renal atrophy, arterial occlusion, ischemic renal failureCx:azotemia with(a)bilateral renal artery stenoses(b)unilateral renal artery stenosis + poorly functioning contralateral kidney Reversible azotemia may be induced by treatment with angiotensin-converting enzyme inhibitors / sodium nitroprusside!Rx:(1)Three-step antihypertensive therapy (control of hypertension difficult)(2)Angiotensin-converting enzyme inhibitors (eg, Captopril PO, Enalaprilat IV)(3)Renal artery angioplasty (80% success for nonostial lesion, 25-30% for ostial lesion)(4)Surgical revascularization (80-90% success for any lesion location)

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Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RENAL ARTERY STENOSIS

Fibromuscular Dysplasia Of Renal Artery Incidence:35% of renal artery stenoses; 1,100 patients reported (by 1982) with involvement of renal artery in 60% + extracranial carotid artery in 30%; 25% of all cases of renovascular hypertensionAge:most common cause of renovascular hypertension in children + young adults gastroduodenal Mortality rate:approaches 75% (because of delayed diagnosis) Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RENAL TRANSPLANT

Hypertension With Renal Transplant Leading cause of death in renal transplant recipient! Prevalence:up to 60% 1 year after transplantation Cause: A.TRANSPLANT RELATED 1.Acute transplant rejection 2.Chronic rejection 3.Cyclosporine toxicity 4.Ureteral obstruction 5.Renal artery stenosis (a)accelerated atherosclerosis (b)postsurgical fibrosis at anastomosis B.NOT TRANSPLANT RELATED 1.Renin production of native kidney 2.Original renal disease involving transplant 3.Development of essential hypertension Notes:


Aseptic Necrosis With Renal Transplant Most common long-term disabling complication; femoral head most common site, bilateral in 59-80% Frequency:6-15-29% within 3 years after surgery Time of onset:symptoms develop 5-126 (mean 9-19) months after transplantation Risk factors: dose + method of glucocorticoid administration, duration + quality of dialysis before transplantation, secondary hyperparathyroidism, allograft dysfunction, liver disease, previous transplantation, iron overload, increased protein catabolism during dialysis Pathophysiology of corticosteroid therapy: (1)Fat embolism (fat globules occlude subchondral end arteries) (2)Increase in fat cell volume in closed marrow space (increase in intramedullary pressure leads to diminished perfusion) (3)Osteopenia (increased bone fragility) (4)Reduced sensibility to pain (loss of protection against excessive stress) Histo:fragmentation, compression, resorption of dead bone, proliferation of granulation tissue, revascularization, production of new bone 40% asymptomatic joint pain restriction of movement Sites:femoral head, femoral condyles (lateral > medial condyle), humeral head subchondral bone resorption patchy osteosclerosis collapse / fragmentation of bone MR with abbreviated T1WI protocol = test of choice! see page 37 AVASCULAR NECROSIS Notes:


Posttransplant Lymphoproliferative Disease =abnormal proliferation of B-cell lymphocytes strongly associated with Epstein-Barr virus infection (in 80%); up to 11% may arise from T-cell lymphocytes Incidence: 0.6%after bone marrow transplantation, 1-6%after kidney transplantation (in 20% NHL, especially affecting CNS) 1.8-20%after cardiac transplantation Prevalence of NHL is 35 x greater than in general population! Cause:sequela of chronic immunosuppression with limited ability to suppress neoplastic activity Types: 1.Polyclonal B-cell hyperplasia (nearly identical to infectious mononucleosis) 2.Monoclonal non-Hodgkin lymphoma Time of onset:as early as 1 month after transplantation depending on immunosuppressive regimen Location: @Lymph nodes: tonsils, cervical neck nodes @Gastrointestinal tract Cx:visceral perforation (frequent) @Thorax multiple / solitary well-circumscribed pulmonary nodules ± mediastinal lymphadenopathy (DDx: cryptococcosis, fungus, Kaposi sarcoma) patchy airspace consolidation (DDx: edema, infection, rejection) DDx:lymphoid hyperplasia (spontaneous resolution) Rx:(1)Antiviral agents (controversial) (2)Reduction / cessation of immunosuppressive agents (3)Surgical resection of tumor mass (complete resolution in 63%) Notes:


RENAL TRAUMA Classification: 1.Superficial cortical laceration (75-85%) (a)Subcapsular hematoma lenticular-shaped area + flattening of subjacent parenchyma (b)Renal contusion poorly defined area of low attenuation (c)Small cortical laceration without caliceal disruption Rx:observation 2.Complete cortical laceration / fracture communicating with caliceal system (10%) extravasation of contrast material separation of renal poles (= fracture) Rx:clinical judgement required 3.Shattered kidney / injury to the renal vascular pedicle (5%) multiple separate renal fragments (= shattered kidney) lack of enhancement of part / all of kidney ± "rim sign" (= enhancement of renal periphery through intact capsular / collateral vessels) extravasation of contrast material Rx:surgery DDx:respiratory motion artifact (low-attenuation area surrounding kidney) Notes:


RENAL TUBULAR ACIDOSIS =clinical syndrome characterized by tubular insufficiency to resorb bicarbonate, excrete hydrogen ion, or both (= nonanion gap metabolic acidosis) Proximal Renal Tubular Acidosis Distal Renal Tubular Acidosis Notes:

failure to thrive

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RENAL TUBULAR ACIDOSIS

Proximal Renal Tubular Acidosis =TYPE 2 RTA =impaired capacity to absorb HCO3 - in proximal tubule leads to presence of bicarbonate in urine at lower plasma levels than normal Pathogenesis: ? defect in Na+/HCO3- cotransport at basolateral membrane; deficit of carbonic anhydrase; parathyroid hormone activates cyclic AMP which inhibits carbonic anhydrase (hypocalcemia of hyperparathyroidism + various types of Fanconi syndrome) self-limited acidosis (bicarbonate loss stops once bicarbonate threshold of about 15 mEq/L is reached) unimpaired ability to lower urine pH (pH 4.5-7.8 depending on level of plasma bicarbonate) by normal excretion of hydrogen ions hypokalemia (due to hyperaldosteronism secondary to decreased proximal resorption of NaCl) rickets / osteomalacia N.B.:NEVER nephrocalcinosis / nephrolithiasis (due to normal urinary citrate excretion, low urine pH, self-limited less severe acidosis with less calcium release from bone Dx:bicarbonate titration test, large requirement of alkali to sustain plasma bicarbonate level at 22 mmol/L Rx:administration of alkali ± potassium ± hydrochlorothiazide 1.INFANTILE TYPE OF PRIMARY PROXIMAL RTA Age:diagnosed within first 18 months of life; usually male patients excessive vomiting in early infancy growth retardation (5.5-6.0) Pathophysiology: primary defect of nonacidification of urine followed by (a)hyperchloremia small constant loss of serum sodium bicarbonate (NaHCO3 ) without concomitant loss of chloride (NaCl retention) leads to shrinkage of ECF volume (b)chronic severe + progressive acidosis (due to inability to excrete the usual endogenously produced nonvolatile acid) leads to -mobilization of calcium + phosphate from bone (osteomalacia) -growth retardation -hypercalciuria (+ 2° hyperparathyroidism) -loss of phosphate (osteomalacia / rickets) (c)nephrocalcinosis + nephrolithiasis (due to combination of hypercalciuria + elevated urine pH + marked reduction in urinary citrate) (d)potassium wastage with hyperkaliuria + hypokalemia (due to constant small loss of sodium bicarbonate in urine, reduction of ECF space, 2° hyperaldosteronism, increase in sodium-potassium exchange in distal tubule) Path:calcium deposits accompanied by chronic interstitial nephritis with cellular infiltration, tubular atrophy, glomerular sclerosis muscle weakness, hyporeflexia, paralysis (due to hypokalemia) bone pain (due to osteomalacia) polyuria (from defect in urinary concentrating ability as a result of nephrocalcinosis + potassium deficiency) low plasma bicarbonate hyperchloremic acidosis (from impaired ability to excrete the usual endogenous load of nonvolatile acid) alkaline urine (pH >5.0-5.5) hypokalemia, loss of sodium hypercalciuria (continued mobilization of calcium phosphate from bone due to metabolic acidosis) hypocitraturia (increased proximal tubular reabsorption of citrate) Dx:acid load test with ammonium chloride (NH4 Cl) Rx:administration of mixture of sodium + potassium bicarbonate Cx:interstitial nephritis, chronic renal failure (damage from nephrocalcinosis + secondary pyelonephritis), bone lesions, nephrocalcinosis, nephrolithiasis 1.PERMANENT DISTAL RTA =ADULT TYPE OF PRIMARY DISTAL RTA =BUTLER-ALBRIGHT SYNDROME Genetics:mostly sporadic, may be autosomal dominant Age:children + adults (usually not diagnosed before age 2); F > M vomiting, constipation, polyuria, dehydration failure to thrive, growth retardation, anorexia polyuria (due to renal concentrating defect) potassium loss resulting in flaccid paralysis bone pain + pathologic fractures in adolescents + adults (from osteomalacia) low serum pH, low bicarbonate concentration elevation of chloride urinary pH of 6.0-6.5 rickets / osteomalacia moderately retarded bone age medullary nephrocalcinosis / nephrolithiasis (as early as 1 month of age) 2.SECONDARY DISTAL RTA (a)systemic conditions: -starvation, malnutrition, sickle cell disease -primary hyperthyroidism + nephrocalcinosis, 1° hyperparathyroidism + nephrocalcinosis, vitamin D intoxication, idiopathic hypercalcemia, idiopathic hypercalciuria + nephrocalcinosis -amphotericin B nephropathy, toxicity to lithium, toluene sniffing -hepatic cirrhosis, fructose intolerance with nephrocalcinosis, Ehlers-Danlos syndrome, Marfan syndrome, elliptocytosis (b)renal conditions: renal tubular necrosis, renal transplantation, medullary sponge kidney, obstructive uropathy (c)hypergammaglobulinemic states (? autoimmune process): idiopathic hypergammaglobulinemia, chronic active hepatitis, hyperglobulinemic purpura, Sjögren syndrome, cryoglobulinemia, systemic lupus erythematosus, lupoid hepatitis, fibrosing alveolitis [TRANSIENT DISTAL RENAL TUBULAR ACIDOSIS =INFANTILE TYPE OF PRIMARY DISTAL RTA =LIGHTWOOD SYNDROME =transient self-limited form in infancy (only observed within 1st year of life) with unclear pathophysiology, probably due to vitamin D intoxication] Notes:


RENAL VEIN THROMBOSIS Prevalence:0.5% (autopsy) Causes: A.Intrinsic =thrombotic process begins intrarenally within small intrarenal veins due to acidosis, hemoconcentration, disseminated intravascular coagulation, intrarenal arteriolar constriction reducing venous flow (a)antenatally: abruptio placentae (b)newborns: advanced maternal age, glycosuria in infants of diabetic mothers, dehydration from vomiting, diarrhea, enterocolitis, sepsis, polycythemia, birth trauma, left adrenal hemorrhage, prematurity (c)adults: membranous GN, pyelonephritis, amyloidosis, polyarteritis nodosa, sickle cell anemia, thrombosis of IVC, renal neoplasia (50%), low flow states (CHF, constrictive pericarditis), diabetic nephropathy, lupus nephropathy, sarcoidosis, hypercoagulable states, trauma B.Extrinsic umbilical vein catheterization, thrombosis of IVC with extension into renal vein, malpositioned IVC filter, carcinoma of pancreatic tail invading renal vein (in 75%), pancreatitis, lymphoma, retroperitoneal sarcoma, retroperitoneal fibrosis, metastases to retroperitoneum (bronchogenic carcinoma) mnemonic:"TEST MAN" Thrombophlebitis Enterocolitis (dehydration) Sickle cell disease, Systemic lupus erythematosus Trauma Membranous glomerulonephritis Amyloidosis Neoplasm Radiographic appearance varies with: (1) rapidity of venous occlusion (2) extent of occlusion (3) availability of collateral circulation (4) site of occlusion in relation to collateral pathways Pathophysiology:formation of collateral channels develops at 24 hours + peaks at 2 weeks after onset of occlusion Collaterals:ureteral v. to vesicular vv., pericapsular vv. to lumbar vv., azygos v., portal v. on left:in addition gonadal v., adrenal v., inferior phrenic vv. Acute Renal Vein Thrombosis Subacute Renal Vein Thrombosis Chronic Renal Vein Thrombosis Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RENAL VEIN THROMBOSIS

Acute Renal Vein Thrombosis Path:hemorrhagic renal infarction from ruptured venules + capillaries without time for effective development of collaterals gross hematuria, proteinuria asymptomatic / painful flank mass consumptive thrombocytopenia anuria, hypertension smooth enlargement of kidney (edema + hemorrhage) initially faint + delayed dense nephrogram little / no pyelocaliceal visualization focal hemorrhagic infarction + capsular rupture US: enlarged kidney of variably altered echotexture thrombus within distended renal vein / IVC Doppler-US: venous flow present in segmental veins + collateral veins overlying renal hilum mimicking patency of main renal vein steady / less pulsatile venous flow compared with contralateral main renal vein main renal vein not traceable into IVC on color Doppler elevated resistive index >0.70 ± reversed end-diastolic renal arterial flow in native kidney CT: prolonged cortical nephrographic phase + persistent corticomedullary differentiation thickened renal fascia + perirenal stranding retroperitoneal hemorrhage Angio: poorly filling cortical arteries absent inflow from renal vein into IVC thrombus extending into IVC NUC: no characteristic pattern on sequential functional study Cx:(1) Pulmonary emboli (50%) (2)Severe renal atrophy (may show complete recovery) Notes:


Subacute Renal Vein Thrombosis =good collateral drainage; impaired function with steady state or recanalization enlarged edematous boggy kidney slightly diminished / normal nephrographic density (may increase over time) compression of collecting system ("spidery calices") increased renal cortical echogenicity collateral veins allow venous efflux normalizing arterial waveform main renal vein appears small due to recanalization Notes:


Chronic Renal Vein Thrombosis =indolent stage 80-90% asymptomatic nephrotic syndrome (proteinuria, hypercholesterolemia, anasarca) normal excretory urogram in 25% (with good collateral circulation especially if left side affected) notching of collecting system + proximal ureter retroperitoneal dilated collaterals lacelike intrarenal pattern of calcifications US: branching linear calcifications (calcified thrombus) small echogenic kidney CT: renal vein + IVC thrombus (24%); perirenal collaterals prolonged corticomedullary differentiation delayed / absent pyelocaliceal opacification + attenuated collecting system thickening of Gerota fascia Arteriography: enlarged venous collaterals on delayed images Notes:


RETROCAVAL URETER =CIRCUMCAVAL URETER = abnormality in embryogenesis of IVC with abnormal persistence of right subcardinal vein ventral to ureter (instead of right supracardinal vein, which is dorsal to right ureter) Incidence:0.07%; M:F = 3:1 symptoms of right ureteral obstruction ureteral course swings medially over pedicle of L3/4, passing behind IVC, and then exiting anteriorly between IVC and aorta returning to its normal position varying degrees of hydronephrosis + proximal hydroureteronephrosis Notes:


RETROPERITONEAL FIBROSIS =ORMOND DISEASE = CHRONIC PERIAORTITIS Path:dense hard fibrous tissue enveloping the retroperitoneum with effects on ureter, lymphatics, great vessels Causes: A.PRIMARY RETROPERITONEAL FIBROSIS (2/3) Probably autoimmune disease with antibodies to ceroid (by-product of aortic plaque, which has penetrated into media) leading to systemic vasculitis; Associated with fibrosis in other organ systems (in 8-15%): mediastinal fibrosis, Riedel fibrosing thyroiditis, sclerosing cholangitis, fibrotic orbital pseudotumor Age:31-60 years (in 70%); M:F = 2:1 Rx:responsive to corticoids B.SECONDARY RETROPERITONEAL FIBROSIS (1/3) (1)Drugs (12%): methysergide, b-blocker, phenacetin, hydralazine, ergotamine, methyldopa, amphetamines, LSD (2)Desmoplastic response to malignancy (8%): lymphoma, Hodgkin disease, carcinoid, retroperitoneal metastases (breast, lung, thyroid, GI tract, GU organs) (3)Retroperitoneal fluid collection: from trauma, surgery, infection (4)Aneurysm of aorta / iliac arteries (desmoplastic response) (5)Connective tissue disease: eg, polyarteritis nodosa (6)Radiation therapy Peak age:40-60 years; M:F = 2:1 weight loss, nausea, malaise dull pain in flank, back, abdomen (90%) renal insufficiency (50-60%) hypertension leg edema, fever, hydrocele (10%) claudication (occasionally) Location: plaque typically begins around aortic bifurcation extending cephalad to renal hilum / surrounding kidney; rarely extends below pelvic rim, but may extend caudad to bladder + rectosigmoid IVP Classic TRIAD: (1)ureterectasis above L4/5 (interference with peristalsis) (2)medial deviation of ureters in middle third, typically bilateral (3)gradual tapering of ureter (extrinsic compression) usually mild pyelocaliectasis US: hypoechoic homogeneous mass in para-aortic region / perinephric space CT: periaortic mass of attenuation similar to muscle may show contrast enhancement (active inflammation) MR: low to medium homogeneous signal intensity on T1WI heterogeneous high signal intensity on T2WI (with malignancy / associated inflammatory edema) low signal intensity on T2WI (in dense fibrotic plaque) NUC: gallium uptake during active inflammation DDx:lymphoma, retroperitoneal adenopathy Rx:(1)Withdrawal of possible causative agent (2)Interventional relief of obstruction (3)Corticosteroids Notes:


RETROPERITONEAL LEIOMYOSARCOMA Incidence:2nd most common primary retroperitoneal malignancy (after liposarcoma) Origin: (a)retroperitoneal space without attachment to organs (b)wall of inferior vena cava Age:5th-6th decade; M:F = 1:6 abdominal mass, pain, weight loss, nausea, vomiting abdominal distension, change in defecation habits, leg edema, back / radicular pain, frequency of urination hemoperitoneum, GI bleeding, dystocia, paraplegia Metastases: frequently hematogenous, less commonly lymphatic dissemination (a)common sites: liver, lung, brain, peritoneum (b)rare sites: skin, soft tissue, bone, kidney, omentum Distant metastases present at time of diagnosis in 40% A.EXTRAVASCULAR LEIOMYOSARCOMA (62%) Path:extraluminal (= completely extravascular) large tumor with extensive necrosis IVP: large soft-tissue mass with (a)displacement of kidney + ureter (b)gas-containing ascending / descending colon well-defined fat plane between mass and kidney obstruction of kidney (ureteral involvement) usually not calcified US: solid mass isoechoic to liver / rarely hyperechoic complex mass with cystic spaces + irregular walls CT: lobulated mass often >10 cm in size large cystic areas of tumor necrosis in center of mass areas of high attenuation with recent hemorrhage MR: intermediate intensity on T1WI with low-intensity areas of necrosis inhomogeneous intermediate intensity on T2WI Angio: hypervascular tumor with blood supply from lumbar, celiac, mesenteric, renal arteries avascular center surrounded by thick hypervascular rind B.INTRAVASCULAR LEIOMYOSARCOMA (6%) Path:intraluminal (= completely intravascular) polypoid mass firmly attached to vessel wall Location:between diaphragm + renal veins, may extend along entire length of IVC + into heart small solid mass within IVC gradually dilatation / obstruction of IVC intratumoral vascularity confirmed by Doppler irregular enhancement (CT bolus injection) Cx: (1)Budd-Chiari syndrome (extension into hepatic veins) (2)Nephrotic syndrome (extension into renal veins) (3)Edema of lower extremities (extension into lower IVC without adequate collateralization) (4)Tumor embolus to lung C.EXTRA- AND INTRAVASCULAR LEIOMYOSARCOMA (33%) solid / necrotic extraluminal mass not originating from a retroperitoneal organ with contiguous intravascular enhancing component (PATHOGNOMONIC) D.INTRAMURAL LEIOMYOSARCOMA (extremely rare) DDx:(1)Liposarcoma (fat content) (2)Malignant fibrous histiocytoma (not as necrotic) (3)Lymphoma (nonnecrotic, tends to envelop IVC + aorta) (4)Primary adrenal tumor (5)IVC thrombus (no luminal enlargement, no neovascularity) Rx:(1) Complete excision (resectable in 10-75%) (2)Partial resection (reduction in tumor size) (3)Adjuvant chemotherapy / radiotherapy Prognosis:local recurrence in 40-70%; death within 5 years in 80-87% with extraluminal tumors Notes:


RETROPERITONEAL LIPOSARCOMA =slow-growing tumor that displaces rather than infiltrates surrounding tissue and rarely metastasizes Incidence:2nd most common primary retroperitoneal tumor (after malignant fibrous histiocytoma), 95% of all fatty retroperitoneal tumors Histo: rarely arising from lipoma (a)myxoid form (most common): varying degrees of mucinous + fibrous tissue + relatively little lipid =intermediate differentiation radiodensity between water + muscle (b)lipogenic form: malignant lipoblasts with large amounts of lipid + scanty myxoid matrix =well-differentiated radiodensity of fat (c)pleomorphic type (least common): marked cellular pleomorphism, paucity of lipid + mucin =highly undifferentiated radiodensity of muscle Age:most commonly 40-60 years; M > F Sites:lower extremity (45%), abdominal cavity + retroperitoneum (14%), trunk (14%), upper extremity (7.6%), head & neck (6.5%), miscellaneous (13.5 %) CT: solid pattern: inhomogeneous poorly marginated infiltrating mass with contrast enhancement mixed pattern: focal fatty areas (-40 to -20 HU) + areas of higher density (+ 20 HU) pseudocystic pattern: water-density mass (averaging of fatty + solid connective-tissue elements) calcifications in up to 12% Angio: hypovascular without vessel dilatation / capillary staining / laking Prognosis:most radiosensitive of soft-tissue sarcomas; 32% overall 5-year survival DDx:malignant fibrous histiocytoma, leiomyosarcoma, desmoid tumor Notes:


RHABDOMYOSARCOMA, GENITOURINARY Frequency: 4-8% of all malignant solid tumors in children bone marrow, liver Metastases in 10-20% at time of diagnosis! nonspecific imaging features: homogeneous echogenicity similar to muscle ± hypoechoic areas (hemorrhage / necrosis) hyperemia with high diastolic flow component bulky pelvic mass of heterogeneous attenuation hypointense on T1WI + hyperintense on T2WI with heterogeneous enhancement diffuse tumor vascularity on angio Prognosis: (a)14-35% 5-year survival with radical surgery (b)60-90% 3-year survival with chemotherapy added Local recurrence is common! Bladder-prostate Rhabdomyosarcoma Rhabdomyosarcoma Of Female Genital Tract Paratesticular Rhabdomyosarcoma Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : RHABDOMYOSARCOMA, GENITOURINARY

Bladder-prostate Rhabdomyosarcoma Age:in first 3 years of life Location:trigone of urinary bladder / prostate (tumor infiltrating both) abdominal pain + distension (from bladder outlet obstruction) urinary frequency + dysuria (from urinary tract infection) palpable bladder hematuria (unusual late manifestation) strangury (= painful urge to void without success) polyploid intraluminal tumor mass elevation of bladder floor with obstruction of bladder neck + large postvoid residual ± invasion of periurethral / perivesical tissues retroperitoneal lymph node enlargement DDx:polyp, hemangioma, ectopic ureterocele, cystitis Notes:


Rhabdomyosarcoma Of Female Genital Tract Location:vulva / vagina (infancy), cervix (reproductive years), uterine corpus (postmenopausal) vaginal mass vaginal bleeding / discharge / protruding grapelike mass DDx:polyp, urethral prolapse, hydrometrocolpos, neoplasm Notes:

vulvar / perineal /


Paratesticular Rhabdomyosarcoma Age:childhood, 2nd age peak in adolescence Location:spermatic cord, testis, penis, epididymis painless scrotal swelling palpable nontransilluminating intrascrotal tumor bulky abdominal (lymphadenopathy) displacement / compression / infiltration of adjacent testis Prognosis:73-89% 3-year survival rate DDx:hydrocele, epididymitis, testicular neoplasm Notes:


SCHISTOSOMIASIS =BILHARZIASIS Organism:trematodes of species: S. haematobium (GU tract) >95%; S. mansoni, S. japonicum (GI tract) R), most commonly in intravesical portion with cobra-head configuration = pseudoureterocele); Makar stricture = focal stricture at L3 multiple inflammatory pseudopolyps in ureter secondary to granulomas (= bilharziomas) ureteritis cystica ureterolithiasis / ureteritis calcinosa (= punctate / linear calcifications) vesicoureteral reflux polypoid filling defects + mucosal irregularities in urinary bladder (pseudotubercles, papillomas) thick-walled fibrotic "flat-topped" bladder with high insertion of ureters reduced bladder capacity with significant postvoid residual (fibrotic stage) urethral stricture with perineal fistulas Cx:Squamous cell carcinoma of bladder Age:30-50 years (exposed early in childhood with 20-to-30-year latency period) Location:posterior bladder wall, rarely trigone irregular filling defect discontinuous calcifications @GI tract portal hypertension (ova migrating into portal venous system incite fibrosing granulomatous reaction within presinusoidal portal veins) esophageal varices (from portal hypertension) polypoid calcifying bowel lesions (from eggs of S. mansoni trapped in bowel wall + inciting granulomatous reaction) @Chest enlargement of RV + pulmonary artery + azygos vein (from portal hypertension) diffuse granulomatous lung lesions Rx:praziquantel Notes:


SCROTAL ABSCESS Etiology: (1)Complication of epididymo-orchitis (often in diabetics), missed testicular torsion, gangrenous tumor, infected hematoma, primary pyogenic orchitis (2)Systemic infection: mumps, smallpox, scarlet fever, influenza, typhoid, syphilis, TB (3)Septic dissemination from: sinusitis, osteomyelitis, cholecystitis, appendicitis NUC: marked increase in perfusion, hot hemiscrotum with photon-deficient area representing the abscess on Tc-99m pertechnetate scan (DDx: chronic torsion) increased scrotal uptake with leukocyte imaging US: hypoechoic / complex fluid collection with low-level echoes (differentiation of intra- from extratesticular abscess location possible) Cx:(1)Pyocele (2)Fistulous tract to skin Notes:


SEMINAL VESICLE CYST 1.ACQUIRED SEMINAL VESICLE CYST 2.CONGENITAL SEMINAL VESICLE CYST Associated with:anomalies of ipsilateral mesonephric duct: (1)Ectopic insertion of ipsilateral ureter (92%) into bladder neck / posterior prostatic urethra / ejaculatory duct / seminal vesicle (2)Ipsilateral renal dysgenesis (80%) (3)Duplication of collecting system (8%) Symptomatic age:21-41 years abdominal / flank / pelvic / perineal pain exacerbated by ejaculation dysuria, frequent urination epididymitis in prepubertal boy recurrent urinary tract infection cystic mass posterior to urinary bladder (DDx: müllerian duct cyst) dilated ejaculatory duct Notes:


SINUS LIPOMATOSIS =PERIPELVIC LIPOMATOSIS =PELVIC FIBROLIPOMATOSIS =PERIPELVIC FAT PROLIFERATION Etiology: (1)Normal increase with aging and obesity (2)Vicarious proliferation of sinus fat with destruction / atrophy of kidney (= replacement lipomatosis) (3)Extravasation of urine leading to proliferation of fatty granulation tissue (4)Normal variant Age:6th-7th decade kidney may be enlarged elongated "spiderlike / trumpetlike" pelvicaliceal system infundibula arranged in "spoke-wheel" pattern parenchymal thickness diminished with underlying disease occasionally focal fat deposit with localized deformity of collecting system Plain film: diminished sinus density CT: unequivocal fat values US: echodense / patchy hypoechoic sinus complex Notes:


SQUAMOUS CELL CARCINOMA OF KIDNEY Incidence:15% of all urothelial tumors Path:flat ulcerating mass + extensive induration Associated with:previous chronic renal infection + calculi (25-60%) stricture that may simulate extrinsic cause ureteropelvic junction obstruction (common) presence of faceted calculi thickening of pelvicaliceal wall (with superficial spread over large areas) arterial encasement + occlusion + neovascularity enlarged pelvic + ureteric arteries occlusion of renal vein / branches (41%) Prognosis:poor due to early metastases Notes:


SUPERNUMERARY KIDNEY =aberrant division of nephrogenic cord into two metanephric tails (rare) Associated with:horseshoe kidney, vaginal atresia, duplicated female urethra, duplicated penis Location: most commonly on left side of abdomen caudal to normal kidney supernumerary ureter may insert into ipsilateral kidney / directly into bladder / ectopic site Cx:hydronephrosis, pyonephrosis, pyelonephritis, cysts, calculi, carcinoma, papillary cystadenoma, Wilms tumor Notes:


TESTICULAR INFARCTION Etiology:torsion, trauma, leukemia, bacterial endocarditis, polyarteritis nodosa, Henoch-Schönlein purpura (hemorrhage / fibrosis) Notes:

diffusely hypoechoic small testis

hyperechoic regions


TESTICULAR MICROLITHIASIS Etiology:formation of microliths from degenerating cells in the seminiferous tubules + absence of phagocytosis by Sertoli cells Prevalence:0.05-0.60% May be associated with: Klinefelter syndrome, cryptorchidism, testicular infarcts, granulomas, subfertility, infertility, testicular germ cell tumor (40%), male pseudohermaphroditism, Down syndrome, pulmonary alveolar microlithiasis asymptomatic, uncommon incidental finding 1- to 2-mm hyperechoic foci scattered throughout the testicular parenchyma (PATHOGNOMONIC) Cx:concurrent germ cell tumor in 40% DDx:postinflammatory changes, scars, granulomatous changes, benign adenomatoid tumor, hemorrhage with infarction, large-cell calcifying Sertoli cell tumor Notes:


TESTICULAR RUPTURE Testicular rupture is indication for immediate surgical intervention! Cause:scrotal trauma Salvageability: 80-90% if surgical repair occurs 72 hours after trauma areas of decreased / increased echogenicity (hemorrhage ± necrosis) loss of testicular outline thickened scrotal wall (= hematoma) visualization of fracture plane hematocele, may show thickening + calcification of tunica vaginalis if chronic uriniferous hydrocele from perforated bulbous urethra avascular region on color duplex Cx:torsion (due to stimulation of a forceful cremasteric contraction) DDx:laceration, contusion, hemorrhage Notes:


TESTICULAR TORSION =SPERMATIC CORD TORSION Most common scrotal disorder in children, 20% of acute scrotal pathology Incidence:1:160, 10-fold risk in undescended testis compared with normal annual incidence of 1:4,000 males Etiology: (1)"Bell and clapper" deformity = high insertion of tunica vaginalis on spermatic cord (2)Abnormally loose mesorchium between testis + epididymis (3)Extravaginal torsion involving testis + tunica vaginalis due to loose attachment of testicular tunics to scrotum during in utero + perinatal period Peak age:newborn period + puberty (13-16 years); 21 years in 26%; >30 years in 9% sudden severe pain in 100% (frequently at night) negative urine analysis (98%) history of similar episode in same / contralateral testis (42%) nausea + vomiting (50%) scrotal swelling + tenderness (42%) leukocytosis (32%) low-grade fever (20%) history of trauma / extreme exertion (13%) Location:in 5% bilateral (anomalous suspension of contralateral testis found in 50-80%) Salvage rate: versus time interval between onset of pain and surgery 80-100%24 hours spontaneous detorsion in 7% Irreversible ischemic damage in only 3-6 hours! Cx:testicular atrophy (in 33-45%) Acute Testicular Torsion Subacute Testicular Torsion Chronic Testicular Torsion Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : TESTICULAR TORSION

Acute Testicular Torsion 70% of patients present within first 6 hours from onset of pain US (80-90% sensitivity): normal grey-scale appearance (within 6 hours) testicular + epididymal enlargement with decreased echogenicity (within 8-24 hours) increase in size of spermatic cord scrotal skin thickening hydrocele (occasionally) loss of spermatic cord Doppler signal (sensitivity 44%, specificity 67%) Color duplex (86% sensitive, 100% specific, 97% accurate): absence of testicular + epididymal flow (DDx: global testicular infarction) false-negative:torsion-detorsion sequence, incomplete torsion 24 hours + less than 10 days US: enlarged / normal-sized testis with heterogeneous texture increased peritesticular flow without parenchymal blood flow NUC: normal NUC angiogram / nubbin sign "doughnut" sign = decreased testicular activity with rim hyperemia of dartos perfusion MRI: enlarged spermatic cord without increase in vascularity whirlpool pattern (twisting of spermatic cord) torsion knot = low-signal-intensity focus at point of twist (displacement of free protons from epicenter of twist) Notes:

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Chronic Testicular Torsion Notes:

small homogeneously hypoechoic testis

enlarged echogenic epididymis


TESTICULAR TUMOR Most common neoplasm in males between ages 25-34 years; 1-2% of all cancers in males; 4-6% of all male genitourinary tumors; 1.5% of all childhood malignancies; 4th most common cause of death from malignancy between ages 15-34 years (12%) Incidence per year:3-5:100,000 Peak age:25-35 years; prior to puberty: yolk sac tumor + teratoma Risk factors: (a)Caucasian race, Jewish religion (b)family history of testicular cancer, previous testicular neoplasm (c)testicular maldescent / atrophy (10 x risk); abdominal site affected in 5%, inguinal site affected in 1.25% chronic pain, "heaviness" acute scrotal pain (10%, from intratumoral hemorrhage) enlarging testis, mass gynecomastia, virilization Location:mostly unilateral; contralateral tumor develops eventually in 8% Staging: StageIlimited to testis + spermatic cord StageIImetastases to lymph nodes below diaphragm II Anonpalpable II Bbulky mass StageIIImetastases to lymph nodes above diaphragm III Aconfined to lymphatic system III Bextranodal metastases Metastases:at presentation in 4-14% to lung, liver, bones, brain, lymph nodes Tumor activity:monitored by levels of a-fetoprotein + b-HCG Color duplex: tumor 1.6 cm hypervascular in 95% (DDx: orchitis associated with epididymal hyperemia) distortion of vessels Prognosis:>93% 5-year survival rate for stage I; 85-90% 5-year survival rate for stage II; complete remission under chemotherapy in 65-75%; relapse in 10-20% within 18 months Germ Cell Tumors (95%) Stromal Cell Tumors = Interstitial Cell Tumors Metastases To Testis (0.06%) Lymphoma / Leukemia Of Testis Burned-out Tumor Of Testis Second Testicular Tumor Notes:

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Germ Cell Tumors (95%) (a)one histologic type in 65% (b)mixed lesion in 35-40% 1.Teratocarcinoma (= teratoma + embryonal cell carcinoma) 2nd most common after seminoma, may occasionally undergo spontaneous regression 2.Embryonal cell carcinoma + seminoma 3.Seminoma + teratoma mnemonic:"YES CT" Yolk sac tumor Embryonal cell carcinoma Seminoma Choriocarcinoma Teratoma A.SEMINOMA (40-50%) Most common tumor in undescended testis Peak age: 30-40 years Spread:in 25% metastasized on initial presentation, pulmonary metastases develop in 19% serum a-fetoprotein usually normal b-HCG elevation in 10-15% usually uniformly hypoechoic + confined within tunica albuginea may be multifocal Rx:sensitive to radiation + chemotherapy Prognosis:10-year survival rate of 75-85% B.NONSEMINOMATOUS TUMOR Age:20-30 years 1.Embryonal cell carcinoma (20-25%) Most common component of mixed testicular tumors; often associated with teratoma Peak age:2nd-3rd decade and GI tract, bladder, thyroid, melanoma More common than germ cell tumors in males >50 years of age! (b)in children: neuroblastoma, Wilms tumor, rhabdomyosarcoma often multiple and bilateral mostly hypoechoic, occasionally echogenic masses Notes:


Lymphoma / Leukemia Of Testis Incidence:6.7% of all testicular tumors Lymphoma:most common testicular tumor in men > age 50; bilateral in 40% Leukemia:60-92% incidence of testicular involvement on autopsy, 8-16% on clinical examination during therapy, up to 41% on clinical examination after therapy Occult testicular tumor often found in patients in bone marrow remission ("gonadal barrier" to chemotherapy) uni- / bilateral diffuse / focal process of decreased echogenicity Notes:


Burned-out Tumor Of Testis = AZZOPARDI TUMOR = spontaneous regression of testicular malignancy (teratocarcinoma) highly echogenic focal lesion ± shadowing (= scarred tumor residue) metastases to retroperitoneum, mediastinum, cervical / axillary / supraclavicular lymph nodes, lung, liver Notes:


Second Testicular Tumor Risk for second tumor in cryptorchidism: 15% for inguinal, 30% for abdominal location Risk for second contralateral tumor: 500-1,000 x ; bilaterality in 1.1-4.4%; Development interval between 1st + 2nd tumor: 4 months to 25 years Detected in 47% by 2 years; in 60% by 5 years, in 75% by 10 years Synchronous contralateral tumor in 1-3% US:a testicular abnormality is malignant in only 50%! Notes:


TRANSITIONAL CELL CARCINOMA Prevalence:85% of all urothelial tumors / primary renal pelvic tumors; 7% of all renal neoplasms Mean age:64 years; M:F = 3:1 Pathogenesis:chemical carcinogens act locally on epithelium (= field of change), action enhanced by length of contact time (eg, stasis / diverticulum) Risk factors: (1)tobacco (2-3 x) (2)aniline dye, benzidine, aromatic amines, azo dyes in textile, rubber, printing, plastic manufacturing (lag time of 10 years) (3)cyclophosphamide therapy (lag time of 6.5 years) (4)analgesic abuse (8 x increase): phenacetin (5)Balkan nephritis (= progressive renal failure + development of bilateral and multiple tumors) (6)recurrent / chronic urinary tract infection Classification: (a)exophytic papillary lesion (85%) = frondlike structure with central fibrovascular core lined by epithelial layer -broad based -pedunculated (b)infiltrating: usually higher grade + less common (c)carcinoma in situ Grade:usually correlates with stage 1=cells slightly anaplastic 2=intermediate features 3=marked cellular pleomorphism frank / microscopic hematuria (72%) dull flank pain (22%) acute renal colic (due to obstruction) Location:bladder 30-50 x more common than upper urinary tract SYNCHRONOUS TCC (a)both renal pelves (in 1-2%) (b)both ureters (in 2-9%) (c)bladder-in 24% of primary renal pelvic involvement -in 39% of primary ureteral involvement -in 2% of primary bladder involvement Renal And Ureteral TCC Bladder TCC Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : TRANSITIONAL CELL CARCINOMA

Renal And Ureteral TCC Staging: TNMAJCCDescription Tis0in situ lesion Ta...noninvasive papillary carcinoma T1Iinvasion of subepithelial connective tissue T2IIconfined to muscularis layer T3IIIinvasion of renal parenchyma / peripelvic soft tissues T4IVextension beyond renal capsule METACHRONOUS TCC IN UPPER TRACT (a)in 12% of pelvic + ureteral primaries (in 25 months) (b)in 4% of bladder primaries (2/3 within 2 years, up to 20 years later) @Kidney Site:extrarenal part of renal pelvis > infundibulocaliceal region IVP: single / multiple filling defects in renal pelvis (35%) "stipple sign" = contrast material trapped in interstices (DDx: blood clot, fungus ball) dilated calyx with filling defect (26%) due to partial / complete obstruction of infundibulum "phantom calyx" = failure to opacify from obstruction ± focal delayed increasingly dense nephrogram "oncocalyx" = caliceal distension with tumor caliceal amputation (19%) absent / decreased excretion with renal atrophy (13%) due to long-standing obstruction of ureteropelvic junction hydronephrosis with renal enlargement (6%) due to tumor obstruction of ureteropelvic junction US: bulky hypoechoic (similar to renal parenchyma) mass lesion splitting / separation of central renal sinus complex infiltrative without bulge of renal contour ± focal caliceal dilatation CT (52% accuracy due to overstaging): sessile filling defect in opacified collecting system thickening + induration of pelvicaliceal wall central solid mass in renal pelvis expanding centrifugally compression of renal sinus fat invasion of renal parenchyma (infiltrating growth pattern) with preservation of renal contour coarse punctate calcific deposits (0.7-6.7%) may mimic urinary calculi variable enhancement of tumor @Ureter Site:lower 1/3 (70%), mid 1/3 (15%), upper 1/3 (15%) IVP: nonfunctioning kidney in advanced tumor (46%) hydronephrosis ± hydroureter (34%) single / multiple ureteral filling defects (19%) irregular narrowing of ureteral lumen Retrograde: "champagne glass" / "goblet sign" = focal expansion of ureter around + distal to mass (probably secondary to to-and-fro peristalsis of mass) "Bergman sign" = "catheter-coiling sign" = coiling of catheter on retrograde catheterization below the mass CT: intraluminal soft-tissue mass eccentric / circumferential thickening of ureteral wall Dx:cytologic analysis of urine (selective lavage, ureteral urine collection, brush biopsy, ureteroscopy DDx:papilloma (benign lesion, fronds lined by normal epithelium) Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : TRANSITIONAL CELL CARCINOMA

Bladder TCC Incidence:5% of all new malignant neoplasms; most common tumor of genitourinary tract; 2% of all cancer deaths in United States Staging T 1=A=lesions involving mucosa + submucosa T 2=B1=invasion of superficial muscle layer T 3a=B2=invasion of deep muscular wall T 3b=C=invasion of perivesical fat T 4a=D1=extension to perivesical organs (seminal vesicles, prostate, rectum) T4b=invasion of pelvic / abdominal wall D2=distant metastases Staging accuracy:50% clinically; 32-80% for CT; 73% for MRI Overstaging due to:edema following endoscopy /endoscopic resection, fibrosis from radiation therapy Histo:80% low-stage superficial papillary neoplasm, (multifocal in 1/3), becoming invasive in 10-20%; 20% invasive (almost always solitary) Site:lateral wall of bladder, bladder diverticulum (in 0.8-10.8%) METACHRONOUS TCC OF BLADDER (a)in 23-40% of primary renal TCC after 15-48 months (b)in 20-50% of primary ureteral TCC after 10-24 months IVP (70% accuracy rate): irregular filling defect with broad base and fronds (DDx: rectal gas marginated by Simpsons white line) F gross / microscopic hematuria "sterile" pyuria frequency, urgency, dysuria history of previous clinical TB (25%) @EXTRARENAL SIGNS ON ABDOMINAL PLAIN FILM osseous / paraspinous changes of TB (discitis + psoas abscess) calcified granulomas in liver, spleen, lymph nodes, adrenals @RENAL MANIFESTATION Renal TB in 4-8% of patients with pulmonary TB! Radiographic evidence of pulmonary TB in 10 mm large unilocular fluid collection (severely dilated collecting system) DDx:multicystic dysplastic kidney, perinephric urinoma ADDITIONAL TESTS: (1)Diuresis excretory urography (Whitfield): accurate in 85% (2) Diuresis renography (Iodine-131-iodohippurate sodium / Tc-99m-DTPA) (3)Pressure flow urodynamic study (Whitaker) Rx:early surgical correction may be needed to preserve renal function Notes:


URETHRAL DIVERTICULUM Age:26-74 years; 6 x more common in black women

urinary incontinence (9-32-70%)

Congenital Urethral Diverticulum Acquired Urethral Diverticulum Notes:

asymptomatic (3-20%)

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : URETHRAL DIVERTICULUM

Acquired Urethral Diverticulum Prevalence:0.6-6%; M0.7 in symptomatic kidney absent ureteral jet on affected side (may be present with partially obstructing calculus) direct visualization of prevesical calculus by transabdominal, transrectal, transvaginal US CT (97% sensitive, 96% specific, 97% accurate): calculus within ureter (PATHOGNOMONIC) DDx:phlebolith all stone compositions readily detectable ureteric rim sign (77%) = ureteric edema surrounding impacted small ureteric calculus DDx:gonadal vein ureterovesical junction edema stranding of perinephric / periureteric fat perinephric fluid collection renal enlargement Cx:xanthogranulomatous pyelonephritis Rx:(1)hydration (within 3 hours after meal, during strenuous physical activity, at bedtime) maintaining urine output of 2-3 l/day (2)diet: restrict amounts of protein, sodium, calcium (3)drugs: thiazide diuretics (lowers urinary calcium), allopurinol (lowers urate + oxalate excretion) Prognosis: (1)Spontaneous passage of ureteral calculi in 93% Most stones 5.0increase of >1.5 mm

Dx:documentation of venous reflux Rx:(1)Ivanissevitch procedure = surgery (2)Transcatheter spermatic vein occlusion Notes:


VESICOURETERIC REFLUX A.CONGENITAL REFLUX = PRIMARY REFLUX =incompetence of ureterovesical junction due to abnormal tunneling of distal ureter through bladder wall Prevalence:in 9-10% of normal Caucasian babies; in 1.4% of school girls; in 30% of children with a first episode of UTI short submucosal ureteral tunnel (normally has a length/width ratio of 4:1) large laterally located ureteral orifice Location:uni- / bilateral (frequently involves lower pole ureter in total ureteral duplication) renal scars in 22-50% Prognosis:disappears in 80% Cx:reflux atrophy / nephropathy in 22-50%; end-stage renal disease in 5-15% of adults B.ACQUIRED REFLUX = SECONDARY REFLUX 1.Paraureteric diverticulum = Hutch diverticulum 2.Duplication with ureterocele 3.Cystitis (in 29-50%) 4.Urethral obstruction (urethral valves) 5.Neurogenic bladder 6.Absence of abdominal musculature (prune belly syndrome) Cx:renal scarring with UTI (30-60%) GRADES OF REFLUX (VCUG): GradeI: reflux into distal ureters GradeII: reflux into collecting system (without caliceal dilatation / blunting) GradeIII: all of the above + mild dilatation of pelvis and calices GradeIV: all of the above + moderate dilatation (clubbing of calices) GradeV: all of the above + severe tortuosity of ureter Prognosis: (a)grade I-III resolve with maturation of the ureterovesical junction (b)grade IV-V require surgery to avoid renal scarring + renal impairment + hypertension Renal scarring:>20% chance for grade III-V reflux; 2-3% chance for grade I-II reflux Radionuclide cystography: Lower radiation dose to gonads than fluoroscopic cystography (5 mrad)! Evaluation of bladder volume at reflux, volume of refluxed urine, residual urine volume, ureteral reflux drainage time (a)indirect: IV injection of Tc-99m DTPA (b)direct: instillation of 1 mCi Tc-99m pertechnetate (more sensitive for reflux during filling phase, which occurs in 20%) US: intermittent hydroureteronephrosis = variable size of collecting system redundant mucosa causing apparent thickening of renal pelvic wall large thin-walled bladder midline-to-orifice distance >7-9 mm has high probability of vesicoureteric reflux Notes:


WILMS TUMOR =NEPHROBLASTOMA Most common malignant abdominal neoplasm in children 1-8 years old (10%)! 3rd most common malignancy in childhood (after leukemia + brain tumors; neuroblastoma more common in infancy)! 3rd most common of all renal masses in childhood (after hydronephrosis + multicystic dysplastic kidney)! Incidence:1:10,000 livebirths; 450 cases/year in USA; familial in 1-2%; multifocal in 10%; bilateral in 4.4-9% Age:peak age at 2.5-3 years (range of 3 months to 8 years); rare during first year; 50% before 3 years, 75% before 5 years; 90% before 8 years; rare in adults; M:F = 1:1 Histo:arises from undifferentiated metanephric blastema as nephroblastomatosis, recapitulates the developing embryonic kidney (a) aggregates of small blastemal cells (b) neoplastic nodules (c) elongated mesenchymal cells Multilocular cystic nephroma, mesoblastic nephroma, nephroblastomatosis are related to the more favorable types of Wilms tumor! In 14% associated with: (1)Sporadic aniridia (= severe hypoplasia of iris) (2)Hemihypertrophy: total / segmental / crossed (2.5%); Ipsilateral or contralateral kidney affected Increased incidence of all embryonal tumors (adrenal cortical neoplasms, hepatoblastoma) (3)Beckwith-Wiedemann syndrome = EMG-syndrome (exomphalos, macroglossia, gigantism) + hepatomegaly, hyperglycemia from islet cell hyperplasia (4)Genitourinary disorders (4.4%): (a)Drash syndrome (pseudohermaphroditism, glomerulonephritis, nephrotic syndrome) (b)Renal anomalies (horseshoe kidney, duplex / solitary / fused kidney) (c)Genital anomalies (cryptorchidism, hypospadia, ambiguous genitalia) Stage: Itumor limited to kidney IIlocal extension into perirenal tissue / renal vessels outside kidney / lymph nodes IIInot totally resectable (peritoneal implants, other than paraaortic nodes involved, invasion of vital structures) IVhematogenous metastases (lung, liver, bone [rare], brain) Vbilateral renal involvement at diagnosis (5-10%) palpable abdominal mass (90%) hypertension (47-90%) abdominal pain (25%) fever (15%) gross hematuria (7-15%) microscopic hematuria (15-20%) large tumor (average size 12 cm) sharply marginated with compressed renal tissue = pseudocapsule partially cystic = focal hemorrhage and necrosis (71%) curvilinear / phlebolithic calcifications in 5% on plain film, in 15% on CT (DDx: regular stippled calcifications in neuroblastoma) distorted "clobbered" calices tumor may invade IVC / right atrium (4-10%) tumor may cross midline hypervascular tumor: enlarged tortuous vessels, coarse neovascularity; small arterial aneurysms, vascular lakes parasitization of vascular supply US: fairly evenly echogenic mass ± irregular anechoic areas due to central necrosis + hemorrhage MR: hypointense on T1WI, variable on T2WI NUC: nonfunctioning kidney (10%) hypo- / iso- / hyperperfusion on radionuclide angiogram absent tracer accumulation on delayed static images displacement of kidney + distortion of collecting system Prognosis:90% survival rate depending on pathologic pattern, age at time of diagnosis, extent of disease VARIANT:Cystic partially differentiated nephroblastoma =combination of MLCN + Wilms tumor elements Incidence: ?; M < F polypoid masses within locules Notes:

multiple noncommunicating locules


WOLMAN DISEASE =PRIMARY FAMILIAL XANTHOMATOSIS = rare autosomal recessive lipidosis with accumulation of cholesterol esters and triglycerides in visceral foam cells + various tissues (liver, spleen, lymph nodes, adrenal cortex, small bowel) Etiology:deficiency of lysosomal acid esterase / acid lipase malabsorption in neonatal period: failure to thrive, diarrhea, steatorrhea, vomiting delayed growth, diminished muscle mass, abdominal distention hepatosplenomegaly extensive bilateral punctate calcifications (calcification of fatty-acid soaps) throughout enlarged adrenals (maintaining their normal triangular shape) is DIAGNOSTIC enlarged fat-containing lymph nodes small bowel wall thickening (due to infiltration of mucosa of small bowel by lipid-filled histiocytes impairing absorption) generalized osteoporosis CT & MR:attenuation + signal intensities consistent with deposition of lipids Dx:assay of leukocytes / cultured skin fibroblasts Prognosis:death occurs within first 6 months of life Notes:


ZELLWEGER SYNDROME = CEREBROHEPATORENAL SYNDROME autosomal recessive muscular hypotonia hepatomegaly + jaundice craniofacial dysmorphism retardation brain dysgenesis (lissencephaly, macrogyria, polymicrogyria) renal cortical cysts Prognosis: death in early infancy

Notes:

seizures, mental

Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : GENERAL OBSTETRICS

Level I Obstetric Ultrasound Indication:MS-AFP >2.5 multiples of mean (MoM) between 14 and 18 weeks MALimited scope of examination to identify frequent causes of MS-AFP elevation in 20-50% of pregnancies: 1.Gestational age >2 weeks more advanced than estimated clinically (18%)2.Multiple gestations (10%)3.Unsuspected fetal demise (5%)4.Obvious fetal NTD / abdominal wall defectOutcome:no cause identified in 50-80%Recommendation if level I ultrasound is unrevealing: (1)amniocentesis for AF-AFP (with normal results in >90%)(2)level II obstetric ultrasound (skipping amniocentesis)

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Level II Obstetric Ultrasound Indication:AF-AFP >2 MoMAccuracy:identification of abnormal fetuses in 99%Examination targeted for: 1.Open neural tube defect:anencephaly, encephalocele, open spina bifida, amniotic band syndrome resulting in open neural tube defect2.Closed neural axis anomaly:hydrocephalus, Dandy-Walker malformation3.Abdominal wall defect:gastroschisis, omphalocele, gastropleuroschisis from amniotic band syndrome4.Upper GI obstruction:esophageal atresia ± tracheoesophageal fistula, duodenal obstruction5.Cystic hygroma6.Teratoma:sacrococcygeal, lingual, retropharyngeal7.Renal anomalies:obstructive uropathy, renal agenesis, multicystic dysplastic kidney, congenital Finnish nephrosis Risk of fetal chromosomal anomaly is only 0.6-1.1% with normal level II sonogram!

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First Trimester Bleeding =VAGINAL BLEEDING IN FIRST TRIMESTERFrequency:15-25% of all pregnancies, of which 50% terminate in abortionA.INTRAUTERINE CONCEPTUS IDENTIFIED1.Blighted ovum / blighted twin2.Threatened abortion3.Implantation bleed4.Early fetal death5.Gestational trophoblastic disease6.Subchorionic hemorrhageB.NORMAL ENDOMETRIAL CAVITY(a)with b-HCG level >1,800 mlU/mL1.Recent spontaneous abortion2.Ectopic pregnancy(b)with b-HCG level 2.5 MoM / equivalent to the 5th percentile; 4.5 MoM for multiple gestationsPower of detection at >2.5 MoM cutoff: 98% of gastroschisis 90% of anencephalic fetuses 75-80% of open spinal defects 70% of omphaloceles Incidence:2-5% screen-positive rate (in 16% normal MS-AFP on retesting); 6-15% of fetuses have some type of major congenital defect; in 1.3 per 1,000 tests fetal anomaly detected The higher the AFP elevation the higher the probability of fetal anomalies 20-38% of women with unexplained high MS-AFP (ie, in absence of fetal abnormality) suffer adverse pregnancy outcomes (premature birth, preeclampsia, 2-4 x IUGR, 10 x perinatal mortality, 10 x placental abruption)!(b)Elevation in AMNIOTIC FLUID (AF-AFP)=defined as >2 MoMIncidence:10 MoM + negative amniotic fluid acetylcholinesterase) 7.OligohydramniosF.PLACENTAL LESION1.Infarct2.Chorioangioma3.Peri- and intraplacental hematoma resulting in fetomaternal hemorrhage4.Placental lakes, intervillous thrombosisG.LOW BIRTH WEIGHTH.Normal pregnancy + MATERNAL DISORDER1.Hepatitis2.HepatomaI.Fetal-maternal blood mixing: collection of MS-AFP samples after amniocentesis mnemonic:"GEM MINER CO"*H0*Gastroschisis Esophageal atresia Multiple gestations Mole Incorrect menstrual dates Neural tube defects Error (laboratory) Renal disease in fetus (autosomal recessive polycystic kidney disease, renal dysplasia, obstructive uropathy, congenital Finnish nephrosis) Chorioangioma Omphalocele Low Alpha-fetoprotein =MS-AFP 34 weeks

Polyhydramnios Oligohydramnios Intrauterine Membrane In Pregnancy Notes:

Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : AMNIOTIC FLUID VOLUME

Polyhydramnios =amniotic fluid volume >1500-2000 cm3 at termIncidence:1.1-2-3.5% fetus does not fill the AP diameter of uterus single largest pocket devoid of fetal parts / cord >8 cm in vertical direction AFI >20-24 cmPrognosis:64% perinatal mortality with severe polyhydramniosEtiology: A.IDIOPATHIC (60%)associated with macrosomia in 19-37% Suggested cause: (1)increased renal vascular flow(2)bulk flow of water across surface of fetus + umbilical cord + placenta + membranesB.MATERNAL CAUSES (20%)1.Diabetes (5%)2.Isoimmunization (Rh incompatibility)3.Placental tumors: chorioangiomaC.FETAL ANOMALIES (20-63%)(a)gastrointestinal anomalies (6-16%)impairment of fetal swallowing (esophageal atresia in 3%); high intestinal atresias / obstruction of duodenum / proximal small bowel (1.2-1.8%), omphalocele, meconium peritonitis (b)nonimmune hydrops (16%)(c)neural tube defects (9-16%)anencephaly, hydranencephaly, holoprosencephaly, myelomeningocele, ventriculomegaly, agenesis of corpus callosum, encephalocele, microcephaly (d)chest anomalies (12%)diaphragmatic hernia, cystic adenomatoid malformation, tracheal atresia, mediastinal teratoma, primary pulmonary hypoplasia, extralobar sequestration, congenital chylothorax (e)skeletal dysplasias (11%)dwarfism (thanatophoric dysplasia, achondroplasia), kyphoscoliosis, platyspondyly (f)chromosomal abnormalities (9%)trisomy 21, 18, 13 (g)cardiac anomalies (5%)VSD, truncus arteriosus, ectopia cordis, septal rhabdomyoma, arrhythmia (h)genitourinary malformationsCause:? hormonally mediated polyuriaunilateral UPJ obstruction, unilateral multicystic dysplastic kidney, mesoblastic nephroma (i)miscellaneous (8%)cystic hygroma, facial tumors, cleft lip / palate, teratoma, amniotic band syndrome, congenital pancreatic cyst In polyhydramnios efforts to detect fetal anomalies should be directed at SGA fetuses! mnemonic:"TARDI"Twins Anomalies, fetal Rh incompatibility Diabetes Idiopathic

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : AMNIOTIC FLUID VOLUME

Oligohydramnios =amniotic fluid volume 10 mmPrognosis:21% survival rate; 50% with intellectual impairment; 80% with isolated mild ventriculomegaly (atrial width >10 and 12 months of age

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : FETAL CNS ANOMALIES

Cystic Intracranial Lesion mnemonic:"CHAP VAN"Choroid plexus cyst Hydrocephalus, Holoprosencephaly, Hydranencephaly Agenesis of corpus callosum + cystic dilatation of 3rd ventricle Porencephaly Vein of Galen aneurysm Arachnoid cyst Neoplasm (cystic teratoma)

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : FETAL CNS ANOMALIES

Abnormal Cisterna Magna Normal size between 15 and 25 weeks MA: >2 to 3 mm during 9-13 weeks MA(b)>6 mm during 14-21 weeks MA The smallest measurement should be used!Image plane:axial plane (slightly craniad to that of the BPD measurement) that includes cavum septi pellucidi, cerebellar hemisphere and cisterna magnaIncidence:among the most common anomaly in 1st trimester + early 2nd trimesterCauses: A.NORMAL VARIANT (0.06%)B.CHROMOSOMAL DISORDERStrisomy 21 (in 45-80%), Turner syndrome (45 X0), Noonan syndrome, trisomy 18, XXX syndrome, XYY syndrome, XXXX syndrome, XXXXY syndrome, 18p-syndrome, 13q-syndrome 30-40% of fetuses with Down syndrome have nuchal skin thickening!C.NONCHROMOSOMAL DISORDERS1.Multiple pterygium syndrome = Escobar syndrome2.Klippel-Feil syndrome (fusion of cervical vertebrae, CHD, deafness (30%), cleft palate3.Zellweger syndrome = cerebrohepatorenal syndrome (large forehead, flat facies, macrogyria, hepatomegaly, cystic kidney disease, contractures of extremities)4.Robert syndrome5.Cumming syndrome larger lymphangiomas with radiating septations are usually found with trisomy 18 nuchal fullness >3 mm during 1st trimester is seen in trisomy 21 / 18 / 13 (30-50% PPV) often reverting to normal by 16-18 weeks septations within nuchal translucency carries a 20- to 200-fold risk for chromosomal anomalies compared with normal Sensitivity:2-44-75% for detection of trisomy 21Specificity:99% for detection of trisomy 21Positive screen:1.2-3% in general population (exceeding 0.5% risk of amniocentesis)False positives:1-2-8.5%OB-management:thorough sonographic evaluation at 18-20 weeks MADDx:chorioamnionic separation

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : FETAL NECK ANOMALIES

Macroglossia 1.Beckwith-Wiedemann syndrome2.Down syndrome3.Hypothyroidism4.Mental retardation

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Micrognathia 1.Pierre-Robin syndrome2.Treacher-Collins syndrome3.Goldenhar syndrome (hemifacial microsomia)4.Seckel syndrome (bird-headed dwarfism)5.Multiple pterygium syndrome6.Pena-Shokeir syndrome7.Beckwith-Wiedemann syndrome8.Arthrogryposis9.Skeletal dysplasias10.Trisomy 13, 18, 9 (abnormal karyotype in 25%)Prognosis:20% survival

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Maxillary Hypoplasia 1.Down syndrome2.Drugs (alcohol, dilantin, valproate)3.Apert / Crouzon syndrome4.Achondroplasia5.Cleft lip/palate

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : FETAL CHEST ANOMALIES

Pulmonary Hypoplasia Path:absolute decrease in lung volume / weight for gestational ageCause: 1.Prolonged oligohydramnios (20-25%)2.Skeletal dysplasia (small thorax)3.Intrathoracic mass (lung compression)4.Large hydrothorax (lung compression)5.Neurologic condition (reduced breathing activity)6.Chromosomal abnormality7.CHD with R-sided cardiac obstructing lesion thoracic circumference (TC) 0.80 (75% sensitive, 80-90% specific); not applicable for intrathoracic masses

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Intrathoracic Mass in order of frequency: 1.Diaphragmatic hernia / eventration2.Cystic adenomatoid malformation3.Bronchopulmonary sequestration4.Bronchogenic cyst with bronchial compression5.Bronchial atresia

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Chest Mass Unilateral Chest Mass 1.Congenital diaphragmatic hernia2.Cystic adenomatoid malformation3.Bronchopulmonary sequestration4.Bronchogenic cyst5.Unilateral bronchial atresia / stenosis Bilateral Chest Masses 1.Laryngeal / tracheal atresia2.Bilateral cystic adenomatoid malformation3.Bilateral congenital diaphragmatic herniae Mediastinal Mass 1.Goiter2.Cystic hygroma3.Pericardial teratoma4.Neuroblastoma Cystic Chest Mass 1.Bronchogenic cyst2.Enteric cyst3.Neurenteric cyst4.Cystic adenomatoid malformation (Type I)5.Congenital diaphragmatic hernia6.Pericardial cyst7.Mediastinal meningocele Complex Chest Mass 1.Congenital diaphragmatic hernia2.Cystic adenomatoid malformation (Type I, II, III)3.Pulmonary sequestration4.Complex enteric cyst5.Pericardial teratoma Solid Chest Mass 1.Congenital diaphragmatic hernia (bowel ± liver)2.Cystic adenomatoid malformation type III3.Pulmonary sequestration4.Obstructed lung from bronchial atresia, laryngeal atresia, bronchogenic cyst5.Bronchopulmonary foregut malformation6.Pericardial tumor7.Heterotopic brain tissue Regressing Fetal Chest Mass 1.Cystic adenomatoid malformation2.Bronchopulmonary sequestration

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Chest Wall Mass 1.Hemangioma2.Cystic hygroma3.Teratoma4.Hamartoma5.Thoracic myelomeningocele

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Pleural Effusion 1.Primary idiopathic chylothorax (most common)2.Hydrops fetalis (multiple causes)3.Chromosome anomaly: trisomy 21, 45 XO (mostly)4.Pulmonary lymphangiectasia / cystic hygroma5.Lung mass: cystic adenomatoid malformation, bronchopulmonary sequestration, congenital diaphragmatic hernia, chest wall hamartoma (uncommon)6.Pulmonary vein atresia7.Idiopathic

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders

FETAL CARDIAC ANOMALIES Incidence:1:125 births = 0.8% of population; most common of all congenital malformations (40%) 90% occur as isolated multifactorial traits with a recurrence risk of 2-4% 10% are associated with multiple birth defects responsible for 50% of childhood deaths from congenital malformationsAntenatal sonographic diagnosis to prompt cardiac evaluation: A.ABNORMALITIES IN CARDIAC POSITIONB. CNS1.Hydrocephalus2.Microcephaly3.Agenesis of corpus callosum4.Encephalocele (Meckel-Gruber syndrome)C.GASTROINTESTINAL1.Esophageal atresia2.Duodenal atresia3.Situs abnormalities4.Diaphragmatic herniaD.VENTRAL WALL DEFECT1.Omphalocele2.Ectopia cordisE. RENAL1.Bilateral renal agenesis2.Dysplastic kidneysF.TWINS1.Conjoined twins

Prenatal Risk Factors For Congenital Heart Disease In Utero Detection Of Cardiac Anomalies Structural Cardiac Abnormalities & Fetal Hydrops Fetal Echocardiographic Views Notes:

Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : FETAL CARDIAC ANOMALIES

Prenatal Risk Factors For Congenital Heart Disease A.FETAL RISK FACTORS1.Symmetric IUGR2.Arrhythmias(a)fixed bradycardia (50%)(b)tachycardia (low risk)(c)irregular: PACs, PVCs (low risk)3.Abnormal fetal karyotype (CHD in Down syndrome in 40%; in Trisomy 18 / 13 in >90%; in Turner syndrome in 35%)4.Extracardiac somatic anomalies by US: omphaloceles (20%), duodenal atresia, hydrocephaly, spina bifida, VACTERL5.Nonimmune hydrops (30-35%)6.Oligo- / polyhydramnios B.MATERNAL RISK FACTORS1.Maternal heart disease (10%)2.Insulin-dependent diabetes mellitus (4-5%)3.Phenylketonuria (15% if maternal phenylalanine >15%)4.Collagen vascular disease: SLE5.Viral infection: rubella6.Drugs(a)phenytoin (in 2% PS, AS, coarctation, PDA)(b)trimethadione (in 20% transposition, tetralogy, hypoplastic left heart)(c)sex hormones (in 3%)(d)lithium (7%): Ebstein anomaly, tricuspid atresia(e)alcohol (25% of fetal alcohol syndrome): VSD, ASD(f)retinoic acid = isotretinoin (?15%)7.Paternal CHD (risk uncertain) C.MENDELIAN SYNDROMES1.Tuberous sclerosis2.Ellis-van Creveld syndrome3.Noonan syndrome D.FAMILIAL RISK FACTORS FOR RECURRENCE OF HEART DISEASE-overall incidence: 6-8:1,000 livebirths-affected sibling: 1-4% (risk doubled)-affected parent: 2.5-4% In 50% of neonates with CHD there is no identifiable risk factor!Poor prognostic features: (1)Intrauterine cardiac failure (hydrops)(2)Severe trisomy (18, 13)(3)Hypoplastic left heart + endocardial fibroelastosis(4)Delivery in center without pediatric cardiology

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In Utero Detection Of Cardiac Anomalies A.ABNORMAL HEART POSITION1.Diaphragmatic hernia2..Lung anomaly3.Pleural effusion4.Cardiac defectB.CHAMBER ENLARGEMENTRA:LA:1.Tricuspid regurgitation1.Mitral stenosis2.Tricuspid valve dysplasia2.Aortic stenosis3.Ebstein anomalyRV:LV:1.Coarctation1.Aortic stenosis2.Normal in 3rd trimester2.CardiomyopathyC.ABNORMAL FOUR-CHAMBER VIEW1.Septal rhabdomyoma2.Endocardial cushion defect3.Ventricular septal defect4.Ebstein anomaly5.Single ventricleD.VENTRICULAR DISPROPORTION1.Hypoplastic right / left ventricle2.Hypoplastic aortic arch3.Aortic / subaortic stenosis4.Coarctation of aorta5.Ostium primum defectE. INCREASED AORTIC ROOT DIMENSION1.Tetralogy of Fallot2.Truncus arteriosus3.Hypoplastic left ventricle with transpositionF.DECREASED AORTIC ROOT DIMENSION1.Coarctation of aorta2.Hypoplastic left ventricle 26-80% of serious cardiac anomalies can be detected on four-chamber view! Increased sensitivity >20 weeks + by including outflow views!

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Structural Cardiac Abnormalities & Fetal Hydrops 1.Atrioventricular septal defect + complete heart block2.Hypoplastic left heart3.Critical aortic stenosis4.Cardiac tumor5.Ectopia cordis6.Dilated cardiomyopathy7.Ebstein anomaly8.Pulmonary atresia

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Fetal Echocardiographic Views A.FOUR-CHAMBER VIEW1.Position of heart within thorax2.Number of cardiac chambers3.Ventricular proportion4.Integrity of atrial + ventricular septa5.Position + size + excursion of AV valvesB.PARASTERNAL LONG-AXIS VIEW1.Continuity between ventricular septum + anterior aortic wall2.Caliber of aortic outflow tract3.Excursion of aortic valve leafletsC.SHORT-AXIS VIEW OF OUTFLOW TRACTS1.Spatial relationship between aorta + pulmonary artery2.Caliber of aortic + pulmonary outflow tractsD.AORTIC ARCH VIEW

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FETAL GASTROINTESTINAL ANOMALIES 1.Esophageal atresia ± TE fistula2.Duodenal atresia3.Meconium peritonitis4.Hirschsprung disease5.Choledochal cyst6.Mesenteric cyst

Abdominal Wall Defect Nonvisualization Of Fetal Stomach Double Bubble Sign Dilated Bowel In Fetus Bowel Obstruction In Fetus Hyperechoic Fetal Bowel Intraabdominal Calcifications In Fetus Cystic Mass In Fetal Abdomen Fetal Ascites Notes:

Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : FETAL GASTROINTESTINAL ANOMALIES

Abdominal Wall Defect Prevalence:1:2,000 pregnancies1.Gastroschisis2.Omphalocele:-upper abdominal wall defect3.Ectopia cordis4.Pentalogy of Cantrell-midabdominal wall defect: classic omphalocele-lower abdominal wall defect5.Bladder exstrophy6.Cloacal exstrophy7.Amniotic band syndrome8.Limb-body wall complex

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Nonvisualization Of Fetal Stomach Fetal swallowing begins at 11 weeks MAIncidence:2% (stomach is visualized in almost all normal fetuses by 14 weeks + in all normal fetuses by 19 weeks)1.Physiologic gastric emptying / intermittent swallowing (repeat scan after 30 minutes)2.Decreased amniotic fluid volume3.CNS abnormalities that impair swallowing4.GI tract abnormalities:(a)congenital diaphragmatic hernia(b)esophageal atresia ± TE fistula Nonvisualization of fetal stomach and polyhydramnios in 33% fetuses with esophageal atresia after 24 weeks MA!5.Cleft palate

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Double Bubble Sign =fluid filled stomach + proximal duodenum A persistently fluid-filled duodenum is always abnormal!1.Duodenal atresia (usually not seen 7 mm in diameter increased peristalsis polyhydramnios (if obstruction above level of mid jejunum; exceptions are esophageal atresia + TE fistula) due to fetal inability to cycle amniotic fluid through gutCx:Meconium peritonitis (50%)DDx:(1)Other cystic masses: duodenal atresia, hydronephrosis, ovarian cyst, mesenteric cyst(2)Chronic chloride diarrhea

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Hyperechoic Fetal Bowel Definition:bowel echogenicity > boneIncidence:0.2-0.6% of 2nd trimester fetusesCause:(?) "constipation" in utero due to decreased swallowing, hypoperistalsis, bowel obstruction + increased fluid absorption1.Normal small bowel variant (especially 5 mm at 15-20 weeks, >8 mm at 20-30 weeks, >10 mm at >30 weeks

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : GYNECOLOGY

Precocious Puberty =early onset of puberty

premature thelarche / adrenarche / mensesIsolated Premature Adrenarche =pubic hair development due to action of

adrenal androgens increased levels of adrenal androgens prepubertal uterus + ovaries (0.1-1 cm3 ) Isolated Premature Thelarche =breast enlargementmay occur without endocrine abnormalities prepubertal uterus + ovaries Pseudoprecocious Puberty =PSEUDOSEXUAL PRECOCITY = incomplete precocious puberty=pubertal changes occurring independently of the action of pituitary gonadotropins, ie, early development of secondary sex characteristics without ovulationCause:ovarian tumor (eg, granulosa theca-cell tumor, thecoma, choriocarcinoma), ovarian cyst, estrogen-producing adrenal tumor, hypothyroidism, neurofibromatosis, estrogen ingestion low gonadotropin levels after LHRH stimulation increased estradiol levels prepubertal uterus + ovaries asymmetric ovarian enlargement (one ovary 2.4-7 cm3 ) with macrocysts (>9 mm) True Precocious Puberty = TRUE ISOSEXUAL PRECOCITY = complete precocious puberty=early development of gonads + secondary sex characteristics with ovulation before 8 years of ageCause: (1)Idiopathic activation of hypothalamic-pituitary-gonadal axis (80%)(2)Lesion of pituitary gland / hypothalamus increased levels of estrogen increased gonadotropin levels after LHRH stimulation advanced bone age adult-sized ovaries (1.2-12 cm3 ) dominance of corpus over cervix length

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Amenorrhea Primary Amenorrhea =failure to menstruate by 16 years of ageCause: A.FEMALE ANATOMIC ANOMALIESB.CONGENITAL DISORDERS OF SEXUAL DIFFERENTIATION(a)pure gonadal dysgenesis bilateral dysfunctional / streak gonads(b)mixed gonadal dysgenesis testis + streak gonadRisk:in 25% development of dysgerminoma / gonadoblastoma in dysgenetic gonads with Y chromosomeC.OVARIAN FAILURE / DYSFUNCTIOND.HYPOTHALAMIC / PITUITARY CAUSES absent / streak gonads + infantile uterus:1.Hypogonadotropic hypogonadism(a)hypothalamic dysfunction: hypothalamic tumor, Kallmann disease (= lack of pulsatile GnRH release), systemic illness, constitutional growth delay, extreme physical / psychological / nutritional stress (cystic fibrosis, sickle cell disease, Crohn disease)(b)pituitary dysfunction: disruption of pituitary stalk from child abuse, head trauma2.Hypergonadotropic hypogonadism=ovarian tissue fails to respond to endogenous gonadotropins(a)abnormal karyotype: Turner syndrome, XY gonadal dysgenesis(b)radiation, chemotherapy, autoimmune disease absent uterus:1.Testicular feminization = male intersex = male pseudohermaphroditism (end-organ insensitivity to testosterone)2.Müllerian dysgenesis (= Mayer-Rokitansky-Küster-Hauser syndrome) normal fallopian tubes + ovariesassociated with: unilateral renal abnormality (50%), skeletal abnormality (12%) small infantile uterus:1.Androgen-producing virilizing tumors of adolescent ovary (usually Sertoli-Leydig cell tumor) unilateral adnexal mass2.Turner syndrome3.In utero exposure to diethylstilbestrol normal uterus + unilateral ovarian tumor:1.Estrogen-producing with disruption of menstrual cycle: granulosa cell tumor, thecoma hydrometrocolpos:1.Vaginal membrane / septum bilateral ovarian enlargement:1.Polycystic ovary syndrome (= Stein-Leventhal syndrome): most common cause of secondary amenorrhea

Secondary Amenorrhea 1.Pregnancy: most common cause in girls >9 years of age2.Polycystic ovary syndrome3.Asherman syndrome4.All causes of primary amenorrhea

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Calcifications Of Female Genital Tract A.UTERUS1.Uterine fibroid2.Arcuate arteriesB.OVARIES1.Dermoid cyst (50%)2.Papillary cystadenoma (psammomatous bodies)3. Cystadenocarcinoma4. Hemangiopericytoma5. Gonadoblastoma6. Chronic ovarian torsion7. Pseudomyxoma peritoneiC.FALLOPIAN TUBES1.Tuberculous salpingitisD.PLACENTAE.LITHOPEDION

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Free Fluid In Cul-de-sac 1.Follicular rupture2.Ovulation3.Ectopic pregnancy4.S/P culdocentesis5.Ovarian neoplasm6.Pelvic inflammatory disease

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : PELVIC MASS

Frequency Of Pelvic Masses 1.Benign adnexal cyst34%2.Leiomyoma14%3.Cancers14%4.Dermoid13%5.Endometriosis10%6.Pelvic inflammatory disease 8%

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Cystic Pelvic Masses A.CYSTIC ADNEXAL MASSB.EXTRAADNEXAL CYSTIC MASS1.Peritoneal inclusion cyst2.Mesenteric cyst3.Lymphocele4.Bladder diverticulum5.Ectopic gestation6.Fluid-distended bowel7.Loculated pelvic abscess: appendiceal, diverticular, postoperative

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Complex Pelvic Mass mnemonic:"CHEETAH"Cystadenoma / cystadenocarcinoma Hemorrhagic cyst Endometrioma Ectopic pregnancy Teratoma (dermoid) Abscess (from adjacent appendicitis, etc.) Hematoma in pelvis

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Solid Pelvic Masses 1.Pedunculated myoma (most common)2.Fibroma3.Adenofibroma4.Thecoma5.Brenner tumor

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Extrauterine Pelvic Masses 1.Solid adnexal mass2.Metastatic disease3.Lymphoma4.Pelvic kidney5.Rectosigmoid carcinoma6.Prostate carcinoma7.Benign prostatic enlargement8.Bladder carcinoma9.Retroperitoneal tumor10.Intraperitoneal fat11.Vascular mass / malformation12.Hematoma13.Bowel

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : ADNEXA

Adnexal Masses A.CYSTIC1.Physiologic ovarian cyst:-Graafian follicle:at midcycle 3 mm increased echogenicity within a cystAge:13% of neoplasms malignant in premenopause; 45% of neoplasms malignant in postmenopauseCx:(1)Torsion (in 10-20%)(2)Rupture (rare)(3)Infection Classification: A.TUMORS OF SURFACE EPITHELIUM (60%)85-95% of all ovarian cancers (although majority of epithelial tumors are benign) 1.Serous ovarian tumor2.Mucinous ovarian tumor3.Endometrioid tumor4.Cystadenofibroma5.Clear cell adenocarcinoma6. Brenner tumor7.Undifferentiated carcinomaB.GERM CELL TUMORS (30%)40% of germ cell tumors are malignant (a)benign1.Dermoid cyst = mature teratoma (most common)(b)malignantaccount for 75% of ovarian cancers seen in 1st-2nd decade of life; 50% fibrous component"tumor of low malignant potential":borderline malignant Solid Ovarian Tumor 1.Fibroma2.Thecoma3.Granulosa cell tumor4.Sertoli-Leydig cell tumor5.Brenner tumor6.Sarcoma7.Dysgerminoma8.Endodermal sinus tumor9.Teratoma10.Metastasis11.Endometrioma12.Massive ovarian edema

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : ADNEXA

Ovarian Cyst Image Signature Of Ovarian Cysts A.SIMPLE CYST=sharply defined wall; NO internal septations / mural nodulesUS:

pulsatility index >1.0 / RI >0.4

(unreliable!)MR: isointense to urine on T1WI + T2WIB.COMPLEX CYST=does not satisfy criteria for hemorrhagic cysts / endometrioma internal septations / mural nodules / internal echoes mixed signal intensity, hyperintense on T2WIC.HEMORRHAGIC CYSTUS: echogenic mass whirled pattern of mixed echogenicity "ground-glass" pattern = diffuse low-level echoes "fishnet weave" pattern = fine interdigitating septations NO color Doppler signalsMR: intermediate / high intensity on T1WI hyperintense with distinct central area of hypointensity on T2WI Management Of Ovarian Cyst A.PREMENOPAUSAL1.Unilocular cyst 6 cmRx:surgeryN.B.:All follow-up scans should take place in the immediate postmenstrual period, when follicular cysts should not be present! B.POSTMENOPAUSAL1.Unilocular nonseptated thin-walled cyst 0.7 (resistive index 3 cm / cyst with low RI 18% of complex cysts are malignant!Rx:CA-125 determination + surgical exploration Screening of 1300 symptomatic women:-in 2.5% abnormalities on US-in 1.9% benign ovarian tumors-in 0.15% ovarian cancers

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Home : OBSTETRICS AND GYNECOLOGY : Differential diagnosis of obstetic and gynecologic disorders : UTERUS

Postmenopausal Bleeding 1. Endometrial atrophy (most commonly)

thin atrophic endometrium is prone to superficial ulceration in 75% endometrial thickness

15 mm heterogeneous endometrium irregular poorly defined endometrial-myometrial interface

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Thickened Irregular Endometrium Normal endometrial thickness: 5-6 mm Types: (a)glandular-cystic hyperplasia (more common)Histo:dilated glands lined by tall columnar / cuboidal epithelium small cysts within evenly echogenic endometriumPrognosis:NO premalignant condition(b)adenomatous hyperplasia endometrium with irregular hypoechoic areasPrognosis:precursor of endometrial cancer3.Endometritis4.Primary carcinoma of the endometriumLocation:predominantly in uterine fundus; 24% in isthmic portion) irregular heterogeneous endometrium mean endometrial thickness of 18.2 mm5.Tamoxifen-related endometrial changes=nonsteroidal antiestrogen may act as partial estrogen agonist with proliferative effects on endometrium6.Metastatic carcinoma:ovary, cervix, fallopian tube, leukemia 7.Hydatidiform mole echogenic mass with irregular sonolucent areas8.Incomplete abortion9.Submucosal leiomyoma

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Fluid Collection Within Endometrial Canal Types:blood, mucus, purulent materialA.PREMENOPAUSAL1.Congenital obstructive lesion: imperforate hymen, vaginal septum, vaginal / cervical atresia2.Acquired obstructive lesion: cervical stenosis (following instrumentation / radiation), cervical carcinoma3.Spontaneous hematometra in bleeding disorders4.Pregnancy: intrauterine, ectopic, incomplete abortionB.POSTMENOPAUSAL1.Cervical stenosis2.Pyometrium3.Polyps4.Endometrial / cervical / ovarian cancer

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Endometrial Cysts 1.Endometrial cystic atrophyHisto:cystically dilated atrophic glands lined by single layer of flattened / low cuboidal epithelium very thin endometrium of 27 mm (b)on transvaginal scan100% visualization if gestational sac >12 mm Transvaginal scan not necessary if on transabdominal scan gestational sac >27 mm without evidence of embryo! Predictive of miscarriage (in 94%): "first-trimester oligohydramnios" (misnomer: not diminished size of amnionic cavity but rather chorionic cavity) =mean sac diameter - CRL < 5 mm (with a live embryo at 5.5-9.0 weeks) Notes:

Home : OBSTETRICS AND GYNECOLOGY : Anatomy and physiology of female reproductive system : ANATOMY OF GESTATION

Yolk Sac =rounded sonolucent structure (outside amniotic cavity) within chorionic sac (= extracoelomic cavity) connected to umbilicus via a narrow stalk; formed by proliferation of endodermal cells at around 4 weeks MA; part of yolk sac is incorporated into fetal gut; the rest persists as a sac connected to the fetus by the vitelline ductFunction: (a)transfer of nutrients from trophoblast to embryo prior to functioning placental circulatio(b)early formation of blood vessels + blood precursors on sac wall(c)formation of primitive gut(d)source of primordial germ cells Mean size: 1.0 mm by 4.7 weeks MA; 2.0 mm by 5.6 weeks MA; 3.0 mm by 7.1 weeks MA; 4.0 (2.2-5.3) mm by 10 weeks MA; disappears around 12 weeks MA Earliest visualization: at 4-5 weeks MA as one of the "double blebs" on endovaginal scan; in 65% with GS size of >8 mm Visualization excludes the possibility of an ectopic / anembryonic pregnancy! Failed pregnancy: Abnormal pregnancy outcome (using endovaginal technique) generally if (a)yolk sac absent with GS diameter of >20 mm (100% specificity + 100% PPV)(b)yolk sac diameter >5.6 mm at 18 mm Cardiac Activity Heart begins to contract at a CRL of 1.5-3 mm = 6th week MA Earliest visualization (on endovaginal scan): (a)in 65% of embryos with a CRL of 2-4.9 mm(b)in 100% at > 5 mm CRL = 6.2 weeksFailed pregnancy: nonvisualization of cardiac activity with CRL of 2-12 mm means embryonic demise in 94%! Spontaneous pregnancy loss at 38 weeks GA Notes:

Home : OBSTETRICS AND GYNECOLOGY : Anatomy and physiology of female reproductive system : FETAL MENSURATION

CNS Ventricles width of 3rd ventricle:2 months of age have a malignant tumor with a 50-90% probabilityCx:(1)dystocia in 6-13%(2)massive intratumoral hemorrhage(3)fetal death in utero / stillbirthRx:1.Complete tumor resection + coccygectomy + reconstruction of pelvic floor: up to 37% recurrence rate, esp. without coccygectomy2.Multiagent chemotherapy (in malignancy) with long-term survival rate of 50%DDx:1.Myelomeningocele (superior to sacrococcygeal region, not septated, axial bone changes)2.Rectal duplication, anterior meningocele (purely cystic)3.Hemangioma, lymphangioma, lipomeningocele, lipoma, epidermal cyst, chordoma, sarcoma, ependymoma, neuroblastoma Notes:

Home : CENTRAL NERVOUS SYSTEM : Skull and spine disorders

SPLIT NOTOCHORD SYNDROME =spectrum of anomalies with persistent connection between gut + dorsal ectodermEtiology:failure of complete separation of ectoderm from endoderm with subsequent splitting of notochord and mesoderm around the adhesion about 3rd week of gestation fistula / isolated diverticula / duplication / cyst / fibrous cord / sinus along the tractTypes: 1.Dorsal enteric fistula = fistula between intestinal cavity + dorsal midline skin traversing prevertebral soft tissue, vertebral body, spinal canal, posterior elements of spine bowel ostium / exposed pad of mucous membrane in dorsal midline in newborn opening passes meconium + feces dorsal bowel hernia into a skin- / membrane-covered dorsal sac after passing through a combined anterior + posterior spina bifida2.Dorsal enteric sinus=blind remnant of posterior part of tract with midline opening to dorsal external skin surface3.Dorsal enteric enterogenous cyst=prevertebral / postvertebral / intraspinal enteric-lined cyst derived from intermediate part of tractIntraspinal enteric cyst Age at presentation:20-40 years intermittent local / radicular pain worsened by elevation of intraspinal pressureLocation:intraspinal in lower cervical / upper thoracic region enlarged spinal canal at site of cyst hemivertebrae, segmentation defect, partial fusion, scoliosis in region of cyst4.Dorsal enteric diverticulum=tubular / spherical diverticulum arising from dorsal mesenteric border of bowel as a persistent portion of tract between gut + vertebral column5.Dorsal enteric cyst=involution of portion of diverticulum near gut mass in abdomen / mediastinum (due to bowel rotation) Notes:

Home : CENTRAL NERVOUS SYSTEM : Skull and spine disorders : SPONDYLOLISTHESIS

Isthmic Spondylolisthesis = open-arch type Cause:usually bilateral spondylolysis=separation of anterior part (vertebral body, pedicles, transverse processes, superior articular facet) from posterior part (inferior facet, laminae, dorsal spinous process)Age:often 1.25 Notes:

Home : CENTRAL NERVOUS SYSTEM : Skull and spine disorders : SPONDYLOLISTHESIS

Degenerative Spondylolisthesis = closed-arch type =PSEUDOSPONDYLOLISTHESISCause:degenerative / inflammatory joint disease (eg, rheumatoid arthritis)Pathophysiology:excess motion of facet jointsAge:usually >60 years commonly symptomatic narrowing of spinal canal hypertrophy of facet joints ratio of maximum anteroposterior diameter of spinal canal at any level divided by diameter at L1 50% at age 45;>90% by age 60 spastic gait disorder neck painLocation:C4-5, C5-6, C6-7 (greater normal cervical motion at these levels)Sequelae:(a)direct compression of spinal cord(b)neural foraminal stenosis(c)ischemia due to vascular compromise(d)repeated trauma from normal flexion / extensionDDx of myelopathy: rheumatoid arthritis, congenital anomalies of craniocervical junction, intradural extramedullary tumor, spine metastases, cervical spinal cord tumor, arteriovenous malformation, amyotrophic lateral sclerosis, multiple sclerosis, neurosyphilis

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SYRINGOHYDROMYELIA =SYRINGOMYELIA = SYRINX (used in a general manner reflecting difficulty in classification)=longitudinally oriented CSF-filled cavities + gliosis within spinal cord frequently involving both parenchyma + central canalAge:primarily childhood / early adult life loss of sensation to pain + temperature (interruption of spinothalamic tracts) trophic changes [skin lesions; Charcot joints in 25% (shoulder, elbow, wrist)] muscle weakness (anterior horn cell involvement) spasticity, hyperreflexia (upper motor neuron involvement) abnormal plantar reflexes (pyramidal tract involvement)Location:predominantly lower end of cervical cord; extension into brainstem (= syringobulbia)CT: distinct area of decreased attenuation within spinal cord (100%) swollen / normal-sized / atrophic cord no contrast enhancement flattened vertebral border (rare) with increased transverse diameter of cord change in shape + size of cord with change in position (rare) filling of syringohydromyelia with intrathecal contrast(a)early filling via direct communication with subarachnoid space(b)late filling after 4-8 hours (80-90%) secondary to permeation of contrast materialMyelography: enlarged cord (DDx: intramedullary tumor) "collapsing cord sign" = collapsing of cord with gas myelography as fluid content moves caudad in the erect position (rare)MR: cystic area of low signal intensity on T1WI, increased intensity on T2WI presence of CSF flow-void (= low signal on T2WI) within cavity from pulsations beaded cavity from multiple incomplete septations cord enlargement Hydromyelia Syringomyelia Reactive Cyst Notes:

Home : CENTRAL NERVOUS SYSTEM : Skull and spine disorders : SYRINGOHYDROMYELIA

Hydromyelia =PRIMARY SYRINGOHYDROMYELIA =CONGENITAL SYRINGOHYDROMYELIA=dilatation of persistent central canal of spinal cord (in 70-80% obliterated) which communicates with 4th ventricle (= communicating syringomyelia)Histo:lined by ependymal tissue Associated with: (1)Chiari malformation in 20-70% metameric haustrations within syrinx on sagittal T1WI(2)Spinal dysraphism(3)Myelocele(4)Dandy-Walker syndrome(5)Diastematomyelia(6)Scoliosis in 48-87%(7)Klippel-Feil syndrome (8)Spinal segmentation defects(9)Tethered cord (in up to 25%)

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Home : CENTRAL NERVOUS SYSTEM : Skull and spine disorders : SYRINGOHYDROMYELIA

Reactive Cyst =POSTTRAUMATIC SPINAL CORD CYST= CSF-filled cyst adjacent to level of trauma; usually single (75%) cord injury (not related to severity of original injury)Rx:shunting leads to clinical improvement Notes:

late deterioration in patients with spinal


TETHERED CORD =TIGHT FILUM TERMINALE SYNDROME = LOW CONUS MEDULLARIS =abnormally short + thickened filum terminale with low position of conus medullarisEtiology:failure of ascent of conus (normal location of tip of conus medullaris: L 4/5 at 16 weeks of gestation, L 2/3 at birth, L1/2 >3 months of age)Pathophysiology:mechanical + metabolic + vascular insults with stretching of cordAge at presentation:5-15 years (in years of growth spurt); M:F = 2:3Associated with:lipoma in 29-78%, diastematomyelia, imperforate anus dorsal nevus, dermal sinus tract, hair patch (50%) bowel + bladder dysfunction in childhood spastic gait with muscle stiffness lower extremity weakness + muscle atrophy asymmetric hyporeflexia + fasciculations orthopedic anomalies: scoliosis, pes cavus, tight Achilles tendon hypalgesia, dysesthesia paraplegia, paraparesis radiculopathy (adults) hyperactive deep tendon reflexes extensor plantar responses anal / perineal pain (in adults) back pain (particularly with exertion) lumbar spina bifida occulta with interpedicular widening scoliosis (20%) diameter of filum terminale >2 mm at L5-S1 level (55%), small fibrolipoma within thickened filum (23%), small filar cyst (3%), spinal cord ending in a small lipoma (13%) posteriorly located tethered conus medullaris + filum terminale (supine views) conus medullaris below level of L2 by age 12 (86%) abnormal lateral course of nerve roots (>15° angle relative to spinal cord) widened triangular thecal sac tented posteriorly (thecal sac pulled posteriorly by filum)MR: prolonged T1 relaxation in center of spinal cord on T1WI in 25% (? myelomalacia / mild hydromyelia)Rx:decompressive laminectomy / partial removal of lipoma ± freeing of cord Notes:


TERATOMA OF SPINE =neoplasm containing tissue belonging to all 3 germinal layers at sites where these tissues do not normally occur Incidence:0.15% (excluding sacrococcygeal teratoma)Age:all ages; M:F = 1:1Path:solid, thin- / thick-walled partially / wholly cystic with clear / milky / dark cyst fluid, uni- / multilocular, presence of bone / cartilageLocation:intra- / extramedullary complete block at myelography syringomyelia above level of tumor spinal canal may be focally widened Notes:

Home : CENTRAL NERVOUS SYSTEM : Differential diagnosis of skull and spine disorders

MANDIBLE & MAXILLA

Mandibular Hypoplasia = Micrognathia Destruction Of Temporomandibular Joint Radiolucent Lesion Of Mandible Tooth Mass Notes:


SKULL

Sutural Abnormalities Wormian Bones Increased Skull Thickness Abnormally Thin Skull Osteolytic Lesion Of Skull Lytic Area In Bone Flap Button Sequestrum Absent Greater Sphenoid Wing Absence Of Innominate Line Widened Superior Orbital Fissure Tumors Of The Central Skull Base Notes:


CRANIOVERTEBRAL JUNCTION

Craniovertebral Junction Anomaly Platybasia Notes:


ATLAS AND AXIS

Atlas Anomalies Axis Anomalies Atlantoaxial Subluxation Notes:


VERTEBRAL BODY

Small Vertebral Body Enlarged Vertebral Body Enlarged Vertebral Foramen Cervical Spine Fusion Vertebral Border Abnormality Bony Projections From Vertebra Vertebral Endplate Abnormality Bullet-shaped Vertebral Body Bone-within-bone Vertebra Ivory Vertebra Notes:


TUMORS OF VERTEBRA

Expansile Lesion Of Vertebrae Bone Tumors Favoring Vertebral Bodies Primary Tumor Of Posterior Elements Notes:


INTERVERTEBRAL DISK

Vacuum Phenomenon In Intervertebral Disk Space Intervertebral Disk Calcification Intervertebral Disk Ossification Schmorl Node Notes:


SPINAL CORD

Intramedullary Lesion Intradural Extramedullary Mass Epidural Extramedullary Lesion Tumors Of Nerve Roots And Nerve Sheaths Cord Lesions Cord Atrophy Delayed Uptake Of Water-Soluble Contrast In Cord lesion Extra-arachnoid Myelography Notes:


SACRUM

Destructive Sacral Lesion Notes:

Home : NUCLEAR MEDICINE : Gallium scintigraphy : GALLIUM-67 CITRATE

Indications A.InfectionGallium has been largely replaced with WBC imaging but can be used in chronic infection 1.Inflamed / infarcted bowel (eg, Crohn disease) DDx:normal bowel excretions (must be cleared by enema; bowel pathology shows persistent activity)2. Diffuse lung uptake sarcoidosis, diffuse infections (TB, CMV, PCP), lymphangitic metastases, pneumoconioses (asbestosis, silicosis), diffuse interstitial fibrosis (UIP), drug-induced pneumonitis (bleomycin, cyclophosphamide, busulfan), acute radiation pneumonitis, recent lymphangiographic contrast 3.Lymph node involvementsarcoidosis, TB, MAI, Hodgkin disease DDx:NOT seen in Kaposi sarcoma, a useful distinction in AIDS patients with hilar nodesB.TumorNeoplastic uptake is variable; prominent uptake is usually seen in: 1.Non-Hodgkin lymphoma (especially Burkitt)2.Hodgkin disease3.Hepatoma4.MelanomaUseful in: -detection of tumor recurrence-DDx of focal cold liver lesions on Tc-99m sulfur colloid scan No Ga-67 Uptake most benign neoplasms; hemangioma; cirrhosis; cystic disease of the breast, liver, thyroid; reactive lymphadenopathy; inactive granulomatous disease

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Home : CENTRAL NERVOUS SYSTEM : Differential diagnosis of brain disorders

BRAIN ATROPHY

Cerebral Atrophy Cerebellar Atrophy Notes:


EXTRA-AXIAL LESIONS

Extra-axial Tumor Leptomeningeal Disease Pericerebral Fluid Collection In Childhood Notes:


VENTRICLES

Ventriculomegaly Colpocephaly Intraventricular tumor Notes:


PERIVENTRICULAR REGION

Periventricular Hypodensity Enhancing Ventricular Margins Periventricular Calcifications In A Child Periventricular T2WI-hyperintense Lesions Notes:


HYPODENSE BRAIN LESIONS

Diffusely Swollen Hemispheres Edema Of Brain Brain Herniation Cholesterol-containing CNS Lesions Cyst With A Mural Nodule Midline Cyst Posterior Fossa Cystic Malformation Suprasellar Low-density Lesion With Hydrocephalus Mesencephalic Low-density Lesion Intracranial Pneumocephalus Notes:


HYPERDENSE INTRACRANIAL LESIONS

Intracranial Calcifications Increased Density Of Falx Intraparenchymal Hemorrhage Dense Cerebral Mass Dense Lesion Near Foramen Of Monro Notes:


BRAIN MASSES

Classification Of Primary CNS Tumors Incidence Of Brain Tumors CNS Tumors Presenting At Birth CNS Tumors In Pediatric Age Group Multifocal CNS Tumors CNS Tumors Metastasizing Outside CNS Calcified Intracranial Mass Avascular Mass Of Brain Jugular Foramen Mass Dumbbell Mass Spanning Petrous Apex Posterior Fossa Tumor In Adult Cystic Mass In Cerebellar Hemisphere Cerebellopontine Angle Tumor Lesion Expanding Cavernous Sinus Notes:


ENHANCING BRAIN LESIONS

Gyral Enhancement Ring-enhancing Lesion Of Brain Dense And Enhancing Lesions Multifocal Enhancing Lesions Innumerable Small Enhancing Cerebral Nodules Enhancing Lesion In Internal Auditory Canal Notes:


VASCULAR DISEASE

Classification Of Vascular CNS Anomalies Occlusive Vascular Disease Displacement Of Vessels Notes:


BASAL GANGLIA

Bilateral Basal Ganglia Lesions In Childhood Low-attenuation Lesion In Basal Ganglia Basal Ganglia Calcification Linear Echogenic Foci In Thalamus + Basal Ganglia Notes:

Home : NUCLEAR MEDICINE : Water-soluble contrast media : ADVERSE CONTRAST REACTIONS

RESPIRATORY DISTRESS wheezing (inconsequential) bronchoconstriction (life-threatening) laryngeal edema (life-threatening)A.MILD 50 mg diphenhydramine 0.3 mL epinephrine (1:1000) SQmay repeat after 15 min up to 1 mL supplemental oxygen at 2-3 L/minif persistent, metaproterenol / terbutaline in metered-dose inhalerB.SEVERE(add to the above) 250 mg aminophylline IV over 15-30 min with careCx:hypotension, cardiac arrhythmia 200-400 mg hydrocortisone IVif unsuccessful, may require intubation if anxiety exacerbates bronchospasm, sedation with 5-10 mg Demerol IV Notes:


SELLA

Destruction Of Sella J-shaped Sella Enlarged Sella Intrasellar Mass Hypointense Lesion Of Sella Parasellar Mass Suprasellar Mass Enhancing Supra- and Intrasellar Mass Perisellar Vascular Lesion Notes:


PINEAL GLAND

Classification Of Pineal Gland Tumors Intensely Enhancing Mass In Pineal Region Notes:

Home : CENTRAL NERVOUS SYSTEM : Anatomy of brain

EMBRYOLOGY

Neurulation Brain Growth Neuronal Migration Notes:


CEREBRAL VESSELS

Common Carotid Artery External Carotid Artery Branches Internal Carotid Artery Carotid Siphon Anterior Cerebral Artery (ACA) Middle Cerebral Artery Posterior Cerebral Artery Arterial Anastomoses Of The Brain Cerebral Veins Notes:


CEREBELLAR VESSELS

Vertebral Artery Anterior Inferior Cerebellar Artery Posterior Inferior Cerebellar Artery Superior Cerebellar Artery Notes:

Home : CENTRAL NERVOUS SYSTEM : Brain disorders

ABSCESS OF BRAIN

Pyogenic Abscess Granulomatous Abscess Notes:


CHIARI MALFORMATION

Chiari I Malformation (adulthood) Chiari II Malformation (childhood) Chiari III Malformation Chiari IV Malformation Notes:


EMPYEMA OF BRAIN

Subdural Empyema Epidural Empyema Notes:

Home : NUCLEAR MEDICINE : Renal and adrenal scintigraphy : RENAL AGENTS

Tc-99m DTPA =Tc-99m diethylenetriamine pentaacetic acid=agent of choice for assessment of(1)Perfusion(2)Glomerular filtration = relative GFR(3)Obstructive uropathy(4)Vesicoureteral reflux Pharmacokinetics: chelating agent; 5-10% bound to plasma protein; extracted with 20% efficiency on each pass through kidney (= filtration fraction); excreted exclusively by glomerular filtration (similar to inulin) without reabsorption / tubular excretion / metabolism Time-activity behavior: -abdominal aorta (15-20 seconds)-kidneys + spleen (17-24 seconds); liver appears later because of portal venous supply-renal cortical activity (2-4 minutes): mean transit time of 3.0 ± 0.5 minutes; static images of cortex taken at 3-5 minutes-renal pelvic activity (3-5 minutes): peak at 10 minutes; asymmetric clearance of renal pelvis in 50%; accelerated by furosemide Biologic half-life:20 minutesDose:10-20 mCiRadiation dose:0.85 rads/mCi for renal cortex; 0.6 rads/mCi for kidney; 0.5 rads/mCi for bladder; 0.15 rads/mCi for gonads; 0.15 rads/mCi for whole body Adjunct: Lasix administration (20-40 mg IV) 20 minutes into exam allows assessment of renal pelvic clearance with accuracy equal to Whitaker test (DDx of obstructed from dilated but nonobstructed pelvicalyceal system)

Notes:


GANGLION CELL TUMOR

Gangliocytoma Ganglioglioma Notes:

Home : NUCLEAR MEDICINE : Brain scintigraphy : RADIONUCLIDE CISTERNOGRAPHY

Hydrocephalus A.Normal-pressure hydrocephalus reversal of normal CSF flow dynamic = tracer moves from basal cisterns into 4th, 3rd, and lateral ventricles loss of w signB.Obstructive hydrocephalus delay (up to 48 hours) for tracer to surround convexities + reach arachnoid villi positive w sign Notes:


NEONATAL INTRACRANIAL HEMORRHAGE

Germinal Matrix Bleed Choroid Plexus Hemorrhage Intracerebellar Hemorrhage Intraventricular Hemorrhage Periventricular Leukoencephalopathy Notes:

Home : NUCLEAR MEDICINE : Bone scintigraphy : BONE MARROW AGENTS

Soft-tissue Uptake A.Physiologic 1.Breast2.Kidney: accentuated uptake with dehydration, antineoplastic drugs, gentamicin3.Bowel: surgical diversion of urinary tract B.Faulty preparation with radiochemical impurity(a)free pertechnetate (TcO4-)Cause:introduction of air into the reaction vial activity in mouth (saliva), salivary glands, thyroid, stomach (mucus-producing cells), GI tract (direct secretion + intestinal transport from gastric juices), choroid plexus(b)Tc-99m MDP colloidCause:excess aluminum ions in generator eluate / patient ingestion of antacids; hydrolysis of stannous chloride to stannous hydroxide, excess hydrolized technetium diffuse activity in liver + spleen C.Neoplastic conditions(a)Benign tumor1.Tumoral calcinosis2.Myositis ossificans(b)Primary malignant neoplasm1.Extraskeletal osteosarcoma / soft-tissue sarcoma: bone forming2.Neuroblastoma (35-74%): calcifying tumor3.Breast carcinoma4.Meningioma5.Bronchogenic carcinoma (rare)6.Pericardial tumor(c)Metastases with extraosseous activity1.to liver: mucinous carcinoma of colon, breast carcinoma, lung cancer, osteosarcomamnemonic:"LE COMBO"Lung cancer Esophageal carcinoma Colon carcinoma Oat cell carcinoma Melanoma Breast carcinoma Osteogenic sarcoma 2.to lung: 20-40% of osteosarcoma metastatic to lung demonstrate Tc-99m MDP uptake3.Malignant pleural effusion, ascites, pericardial effusion D. Inflammation1.Inflammatory process (abscess, pyogenic / fungal infection):(a)adsorption onto calcium deposits(b)binding to denatured proteins, iron deposits, immature collagen(c)hyperemia2.Crystalline arthropathy (eg, gout)3.Dermatomyositis, scleroderma4.Radiation: eg, radiation pneumonitis5.Necrotizing enterocolitis6.Diffuse pericarditis7.Bursitis8.Pneumonia E.Trauma1.Healing soft-tissue wounds2.Rhabdomyolysis: crush injury, surgical trauma, electrical burns, frostbite, severe exercise, alcohol abuse3.Intramuscular injection sites:especially Imferon (= iron dextran) injections with resultant chemisorption; meperidine4.Ischemic bowel infarction (late uptake)5.Hematoma: soft tissue, subdural6.Heterotopic ossification7.Myocardial contusion, defibrillation, unstable angina pectoris8.Lymphedema F.Metabolic1.Hypercalcemia (eg, hyperparathyroidism):(a)uptake enhanced by alkaline environment in stomach (gastric mucosa), lung (alveolar walls), kidneys (renal tubules)(b)uptake with severe disease in myocardium, spleen, diaphragm, thyroid, skeletal muscle2.Diffuse interstitial pulmonary calcifications: hyperparathyroidism, mitral stenosis3.Amyloid deposits G.Ischemia with dystrophic soft-tissue calcifications=necrosis with dystrophic calcification@Spleen: infarct (sickle cell anemia in 50%), microcalcification secondary to lymphoma, thalassemia major, hemosiderosis, glucose-6-phosphate-dehydrogenase deficiency@Liver: massive hepatic necrosis@Heart: transmural myocardial infarction, valvular calcification, amyloid deposition@Muscle: traumatic / ischemic skeletal muscle injury@Brain: cerebral infarction (damage of blood-brain barrier)@Kidney: nephrocalcinosis@Vessels: calcified wall, calcified thrombus Abnormal Uptake Within Kidneys 1.Effect of chemotherapeutic drugs:bleomycin, cyclophosphamide, doxorubicin, mitomycin C, 6-mercaptopurine2.S/P radiation therapy3.Metastatic calcification4.Pyelonephritis5.Acute tubular necrosis6.Iron overload7.Multiple myeloma8.Renal vein thrombosis9.Ureteral obstruction Abnormal Uptake Within Breast 1.Breast carcinoma2.Prosthesis3.Drug-induced Abnormal Uptake In Ascitic, Pleural, Pericardial Effusion 1.Uremic renal disease2.Infection3.Malignant effusion

Notes:

Home : ORBIT : Differential diagnosis of orbital disorders

ORBIT

Spectrum Of Orbital Disorders Intraconal Lesion Extraconal Lesion Orbital Mass In Childhood Mass In Superolateral Quadrant Of Orbit Extraocular Muscle Enlargement Notes:


GLOBE

Spectrum Of Ocular Disorders Microphthalmia Macrophthalmia Ocular Lesion Vitreous Hemorrhage Dense Vitreous In Pediatric Age Group Retinal Detachment Choroidal Detachment Leukokoria Notes:


OPTIC NERVE

Optic Nerve Enlargement Notes:


LACRIMAL GLAND

Lacrimal Gland Lesion Lacrimal Gland Enlargement Notes:

Home : ORBIT : Anatomy of orbit

ORBITAL CONNECTIONS

Superior Orbital Fissure Inferior Orbital Fissure Optic Canal Notes:

Home : ORBIT : Orbital disorders

ENDOPHTHALMITIS

Infectious Endophthalmitis Sclerosing Endophthalmitis Notes:

Home : EARS, NOSE, AND THROAT : Differential diagnosis of ear, nose, and throat disorders

EAR

Hearing Deficit Pulsatile Tinnitus ± Vascular Tympanic Membrane Temporal Bone Sclerosis External Ear Masses Middle Ear Masses Inner Ear Masses Notes:


SINUSES

Opacification Of Maxillary Sinus Paranasal Sinus Masses Granulomatous Lesions Of Sinuses Hyperdense Sinus Secretions Opacified Sinus & Expansion / Destruction Notes:


NOSE

Nasal Vault Masses Mass In Nasopharynx Notes:


PHARYNX

Parapharyngeal Space Mass Pharyngeal Mucosal Space Mass Masticator Space Mass Carotid Space Mass Retropharyngeal Space Mass Prevertebral Space Mass Notes:


AIRWAYS

Inspiratory Stridor In Children Airway Obstruction In Children Tracheal Tumor Notes:


LARYNX

Vocal Cord Paralysis Epiglottic Enlargement Aryepiglottic Cyst Notes:


NECK

Solid Neck Masses In Childhood Lymph Node Enlargement Of Neck Congenital Cystic Lesions Of Neck Branchial Fistula Air-containing Masses Of Neck Notes:


PAROTID GLAND

Parotid Gland Enlargement Multiple Lesions Of Parotid Gland Notes:


THYROID

Congenital Dyshormonogenesis Hyperthyroidism Decreased / No Uptake Of Radiotracer Increased Uptake Of Radiotracer Prominent Pyramidal Lobe Thyroid Calcifications Cystic Areas In Thyroid Thyroid Nodule Discordant Thyroid Nodule Hot Thyroid Nodule Cold Thyroid Nodule Notes:

Home : NUCLEAR MEDICINE : Thyroid and parathyroid scintigraphy : THYROID SCINTIGRAPHY

Tc-99m Pertechnetate Physical decay:10 mCi Tc-99m decays to 2.7 x 10-7 mCi Tc-99Physical half-life:2 x 105 yearsBiologic half-life:6 hoursDecay:by photon emission of 140 keV Quality control: (1)1 mm in diameter(b)90% of surface perfusion defects that are larger than 2 x 2 cm(c)26% of emboli that partially occlude a vessel A history of prior PE decreases probability of acute embolism because

small V/Q mismatches never resolve!Therapeutic implications: (a)high probability scan:treat for PE(b)indeterminate scan:pulmonary angiogram(c)low probability

scan:consider other diagnosis, unless clinical suspicion very high PIOPED (Prospective Investigation of Pulmonary Embolism Diagnosis) study results: Probability of PEin angiogrampositive in high13%88%intermediate39%33%low34%16%normal14%9%Indications for pulmonary angiography: 1.Embolectomy is a therapeutic option2.Indeterminate V/Q scan with high clinical suspicion + risky anticoagulation therapy3.Specific diagnosis necessary for proper management (vasculitis, drug induced, lung cancer with predominant vascular involvement) Overall accuracy: 68% for perfusion scan only, 84% for ventilation-perfusion scan 100% sensitivity in detection of PE is due to the occurrence of multiple emboli (usually >6-8), at least one of which causes a perfusion defect! A normal perfusion scan virtually excludes PE! In an individual 90% "stripe sign" = rim of preserved peripheral activity to a perfusion defect usually indicates(a)nonembolic cause(b)old / resolving pulmonary embolism Correlation with CXR: CXR categorynondiagnostic V/Q scan no acute abnormality12%linear atelectasis12%pulmonary edema12%pleural effusion36%parenchymal consolidation82% focal lung opacity + not ventilated + not perfused = "indeterminate scan"Cause:pneumonia, pulmonary embolism with infarction, segmental atelectasis perfusion defect larger than CXR opacity= high probability for PE perfusion defect substantially smaller than CXR opacity = low probability for PE perfusion defect of comparable size= intermediate probability focal lung opacity (not changed >1 week) + not ventilated + not perfused = low probability for PE When there is lung opacity, evaluate well-aerated areas for perfusion defects! COPD does not diminish usefulness of V/Q scan, but does increase likelihood of an indeterminate result! 75% of patients with pulmonary edema + without pulmonary embolism have a normal perfusion scan! Influence of clinical estimate: V/Q scanClinical probabilityPE present high-probability>80%96%low-probability100) hypotension (systolic blood pressure 5-7%)Probably abnormal10-12 hours post infarction -requires presence of residual collateral blood flow-30-40% maximum accumulation in hypoxic cells with a 60-70% reduction in blood flow (greater levels of occlusion reduce uptake)Uptake post infarction: -earliest uptake by 6-12-24 hours;-peak uptake by 48-72 hours;-persistent uptake seen up to 5-7 days with return to normal by 10-14 days Sensitivity:90% for transmural infarction, 40-50% for subendocardial (nontransmural) infarctionSpecificity:as low as 64% Dose:15-20 mCi IV (minimal count requirement of 500,000/view)Imaging:at 3-6 hours (60% absorbed by skeleton within 3 hours) Indications: 1.Lost enzyme pattern = patient admitted 24-48 hours after infarction2.Equivocal ECG + atypical angina:(a)left ventricular bundle branch block(b)left ventricular hypertrophy(c)impossibility to perform stress test(d)patient on digitalis3.ST depression without symptoms4.Equivocal enzyme pattern + equivocal symptoms5.S/P cardiac surgery (perioperative infarction in 10%, enzymes routinely elevated, ECG always abnormal), requires preoperative baseline study as 40% are preoperatively abnormal6.For detection of right ventricular infarction NOT HELPFUL: 1.In differentiating multiple- from single-vessel disease2.Typical angina3.Normal ECG stress test + NO symptoms Scan interpretation: [Grade 2+ and above are positive] Grade 0no activityGrade 1+faint uptakeGrade 2+slightly less than sternum, equal to ribsGrade 3+equal to sternumGrade 4+greater than sternum "doughnut" pattern = central cold defect (necrosis in large infarct) usually in cases of large anterior + anterolateral wall infarctions uptake in inferior wall extending behind sternum (anterior projection) suggests RV infarction SPECT imaging improves sensitivity (eliminates rib overlap) diffuse uptake can be seen in angina, cardiomyopathy, subendocardial infarct, pericarditis and normal blood pool (normal blood pool can be eliminated with delayed imaging) FALSE POSITIVES (10%) A.Cardiac causes1.Recent injury: myocardial contusion, resuscitation, cardioversion, radiation injury, adriamycin cardiotoxicity, myocarditis, acute pericarditis2.Previous injury: left ventricular aneurysm, mural thrombus, unstable angina, previous infarct with persistent uptake3.Calcified heart valves / coronaries (rare) / chronic pericarditis4.Cardiomyopathy: eg, amyloidosisB.Extracardiac causes:1.Soft-tissue uptake: breast tumor / inflammation, chest wall injury, paddle burns from cardioversion, surgical drain, lung tumor2.Osseous: calcified costal cartilage (most common), lesions in rib / sternum3.Increased blood pool activity secondary to renal dysfunction / poor labeling technique (improvement on delayed images) mnemonic:"SCUBA"Subendocardial infarction (extensive) Cardiomyopathy / myocarditis Unstable angina Blood pool activity Amyloidosis FALSE NEGATIVES (5%) Myocardial metastasis PERSISTENTLY POSITIVE SCAN (>2 weeks) =ongoing myocardial necrosis indicating poor prognosis, may continue on to cardiac aneurysm, repeat infarction, cardiac death-in 77% of persistent / unstable angina pectoris-in 41% of compensated congestive heart failure-in 51% of ECG evidence of ventricular dyssynergy Prognosis:the larger the area, the worse the mortality + morbidity Notes:

Home : HEART AND GREAT VESSELS : Cardiovascular disorders

ANOMALOUS PULMONARY VENOUS RETURN

Total Anomalous Pulmonary Venous Return Partial Anomalous Pulmonary Venous Return = PAPVR Notes:


CARDIOMYOPATHY

Congestive Cardiomyopathy Hypertrophic Cardiomyopathy Restrictive Cardiomyopathy Notes:

Home : NUCLEAR MEDICINE : Water-soluble contrast media

VENOGRAPHY (1)Foot / calf discomfort or pressure or burning(a)~24% with 60% HOCM(b)~5% with 40% HOCM / 300 mg I/mL LOCM The addition of 10-40 mg lidocaine/50 mL of contrast media decreases patient discomfort!(2)Postphlebography deep vein thrombosis(a)26-48% with 60% HOCM(b)0-9% with dilute HOCM / LOCM Infusion of 150-200 mL of 5% dextrose in water / 5% dextrose in 0.45% saline / heparinized saline through injection site immediately after examination reduces likelihood of DVT! Notes:

Home : NUCLEAR MEDICINE : Statistics : STATISTICS

Disease Prevalence =proportion of diseased subjects to total population=(TP + FN) / (TP + TN + FP + FN) = D+ / total Sensitivity + specificity are independent of prevalence Affects predictive values + accuracy of a test result Example: Test A: 90% sensitivity + 90% specificityGOLD STANDARD Tnormalabnormalsubtotal Enormal 90 10 100 Sabnormal10 90 100 T subtotal100100200 NPV = 90% PPV = 90% Test B: prevalence of 10%, 90% sensitivity + specificityGOLD STANDARD Tnormalabnormalsubtotal Enormal 1622 164 Sabnormal18 18 36 T

subtotal18020 200 NPV = 99% PPV = 50% Test C: prevalence of 90%, 90% sensitivity + specificityGOLD STANDARD Tnormalabnormalsubtotal Enormal 18 18 36 Sabnormal2 162164 T

subtotal20 180200 NPV = 50% PPV = 99%

Notes:


TRANSPOSITION OF GREAT ARTERIES

Complete Transposition oOf Great Arteries Corrected Transposition Of Great Arteries Notes:

Home : LIVER, BILE DUCTS, PANCREAS, AND SPLEEN : Differential diagnosis of hepatic, biliary, pancreatic, and splenic disorders

LIVER

Diffuse Hepatic Enlargement Increased Liver Attenuation Generalized Increase In Liver Echogenicity Primary Benign Liver Tumor Primary Malignant Liver Tumor Focal Liver Lesion Solitary Echogenic Liver Mass Bulls-eye Lesions Of Liver Cystic Liver Lesion Vascular "Scar" Tumor Of Liver Low-density Mass In Porta Hepatis Low-density Hepatic Mass With Enhancement Fat-containing Liver Mass Hepatic Calcification Portal Venous Gas Hyperperfusion Abnormalities Of Liver Dampening Of Hepatic Vein Doppler Waveform Aberrant Hepatic Artery Notes:


GALLBLADDER

Nonvisualization Of Gallbladder On OCG Nonvisualization Of Gallbladder On US High-density Bile Displaced Gallbladder Alteration In Gallbladder Size Diffuse Gallbladder Wall Thickening Focal Gallbladder Wall Thickening Mobile Intraluminal Mass In Gallbladder Comet-tail Artifact In Liver And Gallbladder Echogenic Fat In Hepatoduodenal Ligament Notes:


BILE DUCTS

Gas In Biliary Tree Obstructive Jaundice In Adult Neonatal Obstructive Jaundice Large Nonobstructed CBD Filling Defect In Bile Ducts Bile Duct Narrowing Papillary Stenosis Periampullary Tumor Double-duct Sign Congenital Biliary Cysts Notes:


PANCREAS

Congenital Pancreatic Anomalies Pancreatic Calcification Fatty Replacement & Atrophy Of Pancreas Pancreatic Mass Pancreatic Neoplasm Hypervascular Pancreatic Tumors Pancreatic Cyst Hyperamylasemia Notes:


SPLEEN

Nonvisualization Of Spleen Small Spleen Splenomegaly Splenic Lesion Solid Splenic Lesion Cystic Splenic Lesion Increased Splenic Density Splenic Calcification Iron Accumulation In Spleen Hyperechoic Splenic Spots Notes:

Home : LIVER, BILE DUCTS, PANCREAS, AND SPLEEN : Anatomy of liver, bile ducts, and pancreas

LIVER

Portal Venous Anatomy Functional Segmental Liver Anatomy Hepatic Arterial Anatomy (Michels classification) Hepatic Fissures Size Of Liver Normal Hemodynamics Parameter Of Liver Liver Tests Notes:


BILE DUCTS

Normal Size Of Bile Ducts Bile Duct Variants Pancreaticobiliary Junction Variants Notes:


Congenital Gallbladder Anomalies

Agenesis Of Gallbladder Hypoplastic Gallbladder Septations Of Gallbladder Gallbladder Ectopia Notes:


PANCREAS

Pancreatic Development & Anatomy Notes:

Home : LIVER, BILE DUCTS, PANCREAS, AND SPLEEN : Disorders of liver, biliary tract, pancreas, and spleen

CHOLANGIOCARCINOMA

Intrahepatic Cholangiocarcinoma Extrahepatic Cholangiocarcinoma Notes:


CHOLANGITIS

Acute Cholangitis AIDS Cholangitis Primary Sclerosing Cholangitis Recurrent Pyogenic Cholangitis Secondary Sclerosing Cholangitis Notes:


CHOLECYSTITIS

Acute Cholecystitis Acute Acalculous Cholecystitis Chronic Cholecystitis Emphysematous Cholecystitis Xanthogranulomatous Cholecystitis Notes:


ECHINOCOCCAL DISEASE

Echinococcus Granulosus Echinococcus Multilocularis Notes:


HEPATIC HEMANGIOMA

Cavernous hemangioma of liver Infantile Hemangioendothelioma Of Liver Notes:


HEPATITIS

Acute Hepatitis Chronic Hepatitis Notes:

Home : NUCLEAR MEDICINE : Liver and gastrointestinal tract scintigraphy : GASTROINTESTINAL SCINTIGRAPHY

Gastrointestinal Bleeding Detection depends on: (1)Rate of hemorrhage (> 0.05 mL/min); NUC more sensitive than angiogram(2)Continuous versus intermittent bleeding (most GI hemorrhages are intermittent)(3)Site of hemorrhage(4)Characteristics of radionuclide agentANGIOGRAPHY: detection requires a bleeding rate of approximately 0.5 mL/min; 63% sensitivity for upper GI bleed; 39% sensitivity for lower GI bleed Tc-99m Sulfur Colloid Indication:bleeding must be active at time of tracer administration; length of active imaging can be increased by fractionating dose-Disappearance half-life of 2.5-3.5 minutes (rapidly cleared from blood by RES + low background activity)-Active bleeding sites detected with rates as low as 0.05-0.1 mL/min-Not useful for upper GI bleeding (interference from high activity in liver + spleen) or bleeding near hepatic / splenic flexureDose:10 mCi (370 MBq) Imaging: every image should be for 500,000-1,000,000 counts with oblique + lateral images as necessary (a)every 5 seconds for 1 minute ("flow study"= radionuclide angiogram) (b)60-second images at 2, 5, 10, 15, 20, 30, 40, 60 minutes; study terminated if no abnormality up to 30 minutes(c)delayed images at 2, 4, 6, 12 hours extravasation of tracer seen in active bleedingSpecificity:almost 100% (rare false-positives due to ectopic RES tissue)False positives:transplanted kidney, ectopic splenic tissue, modified marrow uptake, male genitalia, arterial graft, aortic aneurysm Tc-99m-labeled RBCs (In Vivtro Labeling Preferred) Indications:acute / intermittent bleeding (0.35 mL/min)-Remains in vascular system for prolonged period-Liver + spleen activity are low allowing detection of upper GI tract hemorrhage-Low target-to-background ratio (high activity in great vessels, liver, spleen, kidneys, stomach, colon; probably related to free pertechnetate fraction) Dose:10-20 mCiImaging: (a)every 2 seconds for 64 seconds(b)static images for 500,000-1,000,000 counts at 2, 5, and every consecutive 5 minutes up to 30 minutes + every 10 minutes until 90 minutes(c)delayed images at 2, 4, 6, 12 hours up to 36 hours Localization of bleeding site: may be difficult secondary to rapid transit time (reduced bowel motility with 1 mg glucagon IV) or too widely spaced time intervals; overall 83% correlation with angiography increase in tracer accumulation over time in abnormal location bleeding site conforms to bowel anatomy change in appearance with time consistent with bowel peristalsis Sensitivity: in 83-93% correctly identified bleeding site (50-85% within 1st hour, may become positive in 33% only after 12-24 hours); collection as small as 5 mL may be detected; superior to sulfur colloid -50% sensitivity for blood loss 90% sensitivity for blood loss >500 mL/24 hoursFalse positives (5%): physiologic uptake in stomach + intestine, renal pelvis uptake, hepatic hemangioma, varices, inflammation, isolated vascular process (AVM, venous / arterial graft) False negatives: 9% for bleeding of 80%enhanced by -fasting for 3-6 hours to reduce gastric secretions passing through bowel-nasogastric tube suction to remove gastric secretions-premedication with pentagastrin (6 µg/kg SC 15 minutes before study) to stimulate gastric secretion of pertechnetate-premedication with cimetidine (300 mg qid x 48 hours) to reduce release of pertechnetate from mucosa-voiding just prior to injection False positives: Barrett esophagus, duodenal ulcer, ulcerative colitis, Crohn disease, enteric duplication, small bowel, hemangioma, AV malformation, aneurysm, volvulus, intussusception, urinary obstruction, uterine blush False negatives: ulcerated epithelium

Notes:


ABNORMAL INTRA-ABDOMINAL AIR

Abnormal Air Collection Pneumoperitoneum Pseudopneumoperitoneum Pneumoretroperitoneum Pneumatosis Intestinalis Soap-bubble Appearance In Abdomen Of Neonate Notes:


ABDOMINAL CALCIFICATIONS & OPACITIES

Opaque Material In Bowel Diffuse Abdominal Calcifications Focal Alimentary Tract Calcifications Abdominal Wall Calcifications Abdominal Vascular Calcifications Notes:


ABNORMAL INTRA-ABDOMINAL FLUID

Ascites Fluid Collections Intra-abdominal Cyst In Childhood Notes:


ESOPHAGUS

Esophageal Contractions Abnormal Esophageal Peristalsis Diffuse Esophageal Dilatation Air Esophagogram Abnormal Esophageal Folds Esophageal Inflammation Esophageal Ulceration Double-barrel Esophagus Esophageal Diverticulum Tracheobronchoesophageal Fistula Long Smooth Esophageal Narrowing Focal Esophageal Narrowing Esophageal Filling Defect Esophageal Mucosal Nodules / Plaques Extrinsic Esophageal Impression Notes:


STOMACH

Widened Retrogastric Space Gastric Pneumatosis Gastric Atony Narrowing Of Stomach Intramural-extramucosal Lesions Of Stomach Gastric Filling Defects Filling Defect Of Gastric Remnant Thickened Gastric Folds Gastric Ulcer Bulls-eye Lesions Complications Of Postoperative Stomach Lesions Involving Stomach And Duodenum Notes:


DUODENUM

Extrinsic Pressure Effect On Duodenum Thickened Duodenal Folds Duodenal Filling Defect Duodenal Tumor Enlargement Of Papilla Of Vater Duodenal Narrowing Dilated Duodenum Postbulbar Ulceration Notes:


SMALL BOWEL

Small Bowel Diverticula Small Bowel Ulcer Separation Of Bowel Loops Normal Small Bowel Folds & Diarrhea Dilated Small Bowel & Normal Folds Abnormal Small Bowel Folds Atrophy Of Folds Ribbonlike Bowel Delayed Small Bowel Transit Multiple Stenotic Lesions Of Small Bowel Small Bowel Filling Defects Small Bowel Tumors Notes:


CECUM

Ileocecal Valve Abnormalities Coned Cecum Cecal Filling Defect Notes:


COLON

Colon Cutoff Sign Colonic Thumbprinting Colonic Urticaria Pattern Colonic Ulcers Multiple Bulls-eye Lesions Of Bowel Wall Double-tracking Of Colon Colonic Narrowing Colonic Filling Defects Colonic Polyp Notes:


RECTUM and ANUS

Rectal Narrowing Enlarged Presacral Space Lesions Of Ischiorectal Fossa Notes:


PERITONEUM

Peritoneal Mass Notes:


MESENTERY & OMENTUM

Omental Mass Mesenteric Mass Mesenteric / Omental Cysts Umbilical Tumor Notes:


ABDOMINAL LYMPADENOPATHY

Regional Patterns Of Lymphadenopathy Enlarged Lymph Node With Low-density Center Notes:


ENTEROPATHY

Protein-losing Enteropathy Malabsorption Notes:

Home : GASTROINTESTINAL TRACT : Anatomy and function of gastrointestinal tract

GASTROINTESTINAL HORMONES

Cholecystokinin Gastrin Glucagon Secretin Notes:


ESOPHAGUS

Lower Esophageal Anatomy Muscular Rings Of Esophagus Notes:


STOMACH

Gastric Cells Effect Of Bilateral Vagotomy Pylorus Notes:


SMALL BOWEL

Duodenal Segments Small Bowel Folds Normal Bowel Caliber Small Bowel Peristalsis Notes:


INTESTINAL FUNCTION

Intestinal Gas Intestinal Fluid Defecography / Evacuation Proctography Notes:


PERITONEUM

Peritoneal Spaces Notes:


BLUNT ABDOMINAL TRAUMA CT is imaging method of choice for evaluation of stable patients Hemoperitoneum Hypovolemia Blunt Trauma To Spleen Blunt Trauma To Liver (20%) Blunt Trauma To Gallbladder (2%) Blunt Trauma To GI Tract (5%) Blunt Trauma To Pancreas (3%) Blunt Trauma To Kidney Blunt Trauma To Ureteropelvic junction (rare) Blunt Trauma To Bladder Notes:


ESOPHAGITIS

Acute Esophagitis Candida Esophagitis Caustic Esophagitis Chronic Esophagitis Cytomegalovirus Esophagitis Drug-induced Esophagitis Herpes Esophagitis Human Immunodeficiency Virus Esophagitis Reflux Esophagitis Viral esophagitis Notes:

Home : NUCLEAR MEDICINE : Liver and gastrointestinal tract scintigraphy : GASTROINTESTINAL SCINTIGRAPHY

Gastric Emptying Dose:0.5-1 mCi(a)Tc-99m sulfur colloid cooked with egg white / liver pâté as solid food(b)In-111 DTPA for simultaneous measurement of liquid phaseImaging:1-minute anterior abdominal images obtained at 0, 10, 30, 60, 90 minutes in erect position if dual-head camera available; anterior and posterior imaging performed with geometric mean activity calculatedPharmacokinetics: 79% tracer activity in stomach for solid phase at 10 minutes; 65% at 30 minutes; 33% at 60 minutes; 10% at 90 minutes Normal result:50% of activity in stomach at time zero; should empty by 60 ± 30 minutes acutely delayed emptying in stress (pain, cold), drugs (morphine, anticholinergics, levo-dopa, nicotine, b-adrenergic antagonists), postoperative ileus, acute viral gastroenteritis, hyperglycemia, hypokalemia chronically delayed gastric emptying in gastric outlet obstruction, postvagotomy, gastric ulcer, chronic idiopathic intestinal pseudoobstruction, GE reflux, progressive systemic sclerosis, dermatomyositis, spinal cord injury, myotonia dystrophica, familial dysautonomia, anorexia nervosa, hypothyroidism, diabetes mellitus, amyloidosis, uremia abnormally rapid gastric emptying in gastric surgery, ZE syndrome, duodenal ulcer disease, malabsorption (pancreatic exocrine insufficiency / celiac sprue) Notes:


GASTRIC ULCER

Benign Gastric Ulcer Malignant Gastric Ulcer Notes:


GASTRITIS

Corrosive Gastritis Emphysematous Gastritis Erosive Gastritis Phlegmonous Gastritis Notes:


HERNIA

External Hernia Internal Hernia Hiatal Hernia Umbilical Hernia Notes:


LEIOMYOSARCOMA

Leiomyosarcoma Of Small Bowel Leiomyosarcoma Of Stomach Carney Syndrome Notes:


KIDNEY

Absent Renal Outline On Plain Film Nonvisualized Kidney On Excretory Urography Unilateral Large Smooth Kidney Bilateral Large Kidneys Bilateral Small Kidneys Unilateral Small Kidney Increased Echogenicity Of Renal Cortex Hyperechoic Renal Pyramids In Children Iron Accumulation In Kidney Depression Of Renal Margins Enlargement Of Iliopsoas Compartment Notes:

Home : NUCLEAR MEDICINE : Water-soluble contrast media

EXCRETORY UROGRAPHY Clearance:>99% of contrast material eliminated through kidney (30 hoursDose:5-10 mCiRadiation dose:0.014 rads/mCi for gonads; 0.015 rads/mCi for whole body

Notes:


POLYCYSTIC KIDNEY DISEASE

Autosomal Dominant Polycystic Kidney Disease Autosomal Recessive Polycystic Kidney Disease Notes:


RENAL CYST

Simple Cortical Renal Cyst Atypical / Complicated Renal Cyst Renal Sinus Cyst Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : URACHAL ANOMALIES

Alternating Sinus =cystic dilatation of urachus periodically emptying into bladder / umbilicus Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : URACHAL ANOMALIES

Urachal Diverticulum (3%) =urachus communicates only with bladder dome Notes:


URETERAL DUPLICATION =RENAL DUPLICATION Complete Duplication Incomplete / Partial Duplication Notes:

Home : NUCLEAR MEDICINE : Thyroid and parathyroid scintigraphy : THYROID SCINTIGRAPHY

Iodine-131 Indication:thyroid uptake study, thyroid imaging, treatment of hyperthyroidism, treatment of functioning thyroid cancer, imaging of functioning metastasesProduction:by fission decayPhysical half-life:8.05 days (allows storing for long periods) Decay:principal gamma energy of 364 keV (82% abundance) + significant beta decay fraction of a mean energy of 192 keV (92% abundance)Dose:30-50 µCi (1.2 rad/µCi = 50 rad for thyroid) Radiation dose: (90% from beta decay, 10% from gamma radiation) 0.6 mrad/mCi for whole body; 1.2 mrad/µCi for thyroid (critical organ) Pharmacokinetics:identical to I-123Disadvantages: (a)Too energetic for gamma camera, well suited for rectilinear scanner with limited resolution(b)High radiation dose prohibits use for diagnostic purposes(c)Ectopic thyroid tissue just as well detectable with I-123 or Tc-99m pertechnetate

Notes:

Home : UROGENITAL TRACT : Renal, adrenal, ureteral, vesical, and scrotal disorders : URETHRAL DIVERTICULUM

Congenital Urethral Diverticulum Cause:ectopic cloacal epithelium; M>F Notes:


GENERAL OBSTETRICS

Level I Obstetric Ultrasound Level II Obstetric Ultrasound First Trimester Bleeding Positive &b;-HCG Without IUP Dilated Cervix Uterus Large For Dates Empty Gestational Sac Alpha-fetoprotein Use Of Karyotyping Notes:


PLACENTA

Abnormal Placental Size Vascular Spaces Of The Placenta Placental Tumor Unbalanced Intertwin Transfusion Notes:


UMBILICAL CORD

Abnormal Cord Attachment Umbilical Cord Lesions Notes:


FETAL CHEST ANOMALIES

Pulmonary Hypoplasia Intrathoracic Mass Chest Mass Chest Wall Mass Pleural Effusion Notes:


GYNECOLOGY

Precocious Puberty Amenorrhea Calcifications Of Female Genital Tract Free Fluid In Cul-de-sac Notes:


PELVIC MASS

Frequency Of Pelvic Masses Cystic Pelvic Masses Complex Pelvic Mass Solid Pelvic Masses Extrauterine Pelvic Masses Notes:


ADNEXA

Adnexal Masses Ovarian Tumors Ovarian Cyst Notes:


UTERUS

Postmenopausal Bleeding Thickened Irregular Endometrium Fluid Collection Within Endometrial Canal Endometrial Cysts Diffuse Uterine Enlargement Uterine Masses Fundic Depression On HSG Notes:


VAGINA

Vaginal Cyst Vaginal Fistula Vaginal & Paravaginal Neoplasm Notes:

Home : OBSTETRICS AND GYNECOLOGY : Anatomy and physiology of female reproductive system

ANATOMY OF GESTATION

Choriodecidua Gestational Sac Yolk Sac Embryo Amnionic Membrane Umbilical Cord Placental Grading Uteroplacental Circulation Notes:


ASSESSMENT OF FETAL WELL-BEING

Amniotic Fluid Index Biophysical Profile (Platt and Manning) = BPP Stress Tests Notes:


INVASIVE FETAL ASSESSMENT

Amniocentesis Chorionic villus sampling (CVS) Cordocentesis Notes:


UTERUS Uterine Size Uterine Zonal Anatomy (on T2WI) Endometrium Pelvic Spaces Cervical Length Pelvic Ligaments Notes:

Home : OBSTETRICS AND GYNECOLOGY : Obstetric and gynecologic disorders

EMBRYONIC DEMISE Incidence: 20-71% loss rate of one twin

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