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 benefit. 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 systemic and portal venous systems. The Budd-Chiari syndrome appeared acutely following a spontaneous abortion in a 33-year-old woman who was 4 weeks pregnant. The course was fulminant with acute liver failure. Thrombolytic therapy failed to resolve the inferior vena cava thrombosis. Because 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 sustained anticoagulant therapy. The patient was discharged 3 weeks after transplantation and has remained asymptomatic since February 1991. We concluded that orthotopic liver transplantation, with portal and inferior vena cava thrombectomy, can be performed in patients with complete acute hepatocellular necrosis resulting from massive thrombosis of hepatic veins, abdominal vena cava, and portal vein.