Liver transplantation for Budd-Chiari syndrome: When is it really necessary?

✍ Scribed by Sarwa Darwish Murad; Patrick S. Kamath

• Publisher: John Wiley and Sons
• Year: 2008
• Tongue: English
• Weight: 61 KB
• Volume: 14
• Category: Article
• ISSN: 1527-6465
• DOI: 10.1002/lt.21286

✦ Synopsis

The Budd-Chiari syndrome (BCS) results from obstruction to the hepatic venous outflow tract from the level of the hepatic venules to the entrance of the inferior vena cava into the right atrium. 1 This obstruction causes venous stasis, centrilobular congestion, and hepatocyte necrosis, which leads to centrilobular fibrosis, nodular regenerative hyperplasia, and ultimately cirrhosis of the liver. 2 The principal manifestations of BCS are abdominal pain, ascites, and hepatomegaly with some alteration in the liver biochemical tests. Clinical presentations, however, may range from completely asymptomatic patients, in whom only 1 hepatic vein is obstructed, 3 to patients with liver failure, including, rarely, fulminant failure. The most common cause of hepatic venous outflow obstruction in the West is venous thrombosis due to prothrombotic states, whereas in the Eastern world, membranous obstruction of the inferior vena cava is more prevalent. 4,5 The prothrombotic states may be inherited, such as the factor V Leiden mutation, or acquired, such as myeloproliferative disorders. [6][7][8][9] The inherited conditions associated with most severe thrombophilia, such as antithrombin deficiency and paroxysmal nocturnal hemoglobinuria, are not common causes of BCS. The diagnosis of BCS is usually made by cross-sectional abdominal imaging, either computerized tomography or magnetic resonance imaging being more accurate than ultrasonography. Neither hepatic venography nor liver biopsies are mandatory for diagnosis if the diagnosis is clear on cross-sectional imaging.

Treatment for BCS is usually approached in a step-