Internal Medicine

0521779407-B02 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:52

Budd-Chiari Syndrome 259

Budd-Chiari Syndrome................................

MINDIE H. NGUYEN, MD

history & physical

History

■membranous occlusion of the hepatic vein:

➣congenital or acquired as a post-thrombotic event

➣most common cause worldwide

■underlying thrombotic diathesis:

➣myeloproliferative disorder: most common cause in U.S.

➣other hematological disorder: antiphospholipid syndrome,

lupus anticoagulant, paroxysmal nocturnal hemoglobinuria,

protein C or S or antithrombin III deficiency

➣miscellaneous: pregnancy or high-dose estrogen use, chronic

infections (eg, aspergillosis, amebic abcess), tumors (eg, HCC,

renal cell carcinoma), chronic inflammatory disease (eg, IBD,

Behcet’s disease), trauma

■idiopathic: 30% of cases

Signs & Symptoms

■classic triad: hepatomegaly, ascites, abdominal pain

■presentation: acute, subacute, or chronic

tests

Basic Studies

■blood (CBC, INR, LFT’s): abnormal but nonspecific

■ascitic fluid: high protein concentration (>2.0 g/dL); low white blood

cell (< 500); serum-ascites albumin gradient usually >1.1

Imaging

■ultrasound: sensitivity and specificity: 85–90%

■CT: less sensitive and specific than color Doppler ultrasound

■MRI: sensitivity and specificity: 90%

■hepatic venography (gold standard):

➣thrombus within hepatic veins, “spider-web” pattern of collater-

als, or inability to cannulate the hepatic veins

Liver Biopsy

■valuable in evaluating the extent of fibrosis and guiding therapy

■pathologic findings:

➣necrotic and pale centrilobular areas