LIVER-TRANSPLANTATION FOR ACUTE BUDD-CHIARI SYNDROME COMPLICATED BY PORTAL AND INFERIOR VENA-CAVA THROMBOSIS

The prognosis of patients suffering from Budd-Chiari syndrome is poor without treatment. Both medical and surgical treatments have some bene fit. Surgical treatment consists either of portosystemic shunt surgery or liver transplantation. It is still debatable which patients might benefit from either of these procedures. We report a patient with Budd -Chiari syndrome with extensive thrombotic involvement of both systemi c and portal venous systems. The Budd-Chiari syndrome appeared acutely following a spontaneous abortion in a 33-year-old woman who was 4 wee ks pregnant. The course was fulminant with acute liver failure. Thromb olytic therapy failed to resolve the inferior vena cava thrombosis. Be cause of the extensive and progressive involvement of both portal and caval systems, an emergent caval and portal vein thrombectomy followed by liver transplantation was performed. This was followed by sustaine d anticoagulant therapy. The patient was discharged 3 weeks after tran splantation and has remained asymptomatic since February 1991. We conc luded that orthotopic liver transplantation, with portal and inferior vena cava thrombectomy, can be performed in patients with complete acu te hepatocellular necrosis resulting from massive thrombosis of hepati c veins, abdominal vena cava, and portal vein.