Internal Medicine

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

260 Budd-Chiari Syndrome

➣high-grade venous congestion

➣heterogenous involvement of liver is occasionally problematic;

caudate lobe usually spared

differential diagnosis

■right-sided congestive heart failure

■constrictive pericarditis

■metastatic and infiltrative disease involving the liver

■granulomatous liver disease

management

What to Do First

■assess severity of portal hypertension and liver disease

■identify and evaluate for potential predisposing conditions

General Measures

■provides only short-term, symptomatic benefit

■diuretics: effective for ascites

■anticoagulation: to prevent recurrent thrombosis

specific therapy

■TIPS:

➣successful as a bridge to transplantation for acute/fulminant BCS

➣successful for long-term treatment for portal hypertension

➣many patients required repeated stent revisions

■angioplasty, stent placement in hepatic veins and/or inferior vena

cava

■transcardiac membranotomy for membranous obstruction

■surgical shunts: patency rate: 65–95%

■liver transplantation: for fulminant course or end-stage cirrhosis

follow-up

■monitor for signs of portal hypertension: ascites, varices, ence-

phalopathy

■monitor liver synthetic dysfunction and signs of progressive liver

disease

■monitor shunt patency

■removal or control of predisposing risk factors

complications and prognosis

Complications

■TIPS: shunt stenosis or occlusion; hepatic encephalopathy