Management of Budd-Chiari syndrome.

KEY CONCEPTS: 1. Medical therapy alone is rarely sufficient for long-term management of patients with hepatic vein thrombosis. 2. Enthusiasm for intravascular stents (transjugular intrahepatic portosystemic shunt [TIPS] or vena caval stents) for the management of Budd-Chiari syndrome must be tempered by the limited interval of expected utility, the likelihood of stent occlusion/revisions, and the potential complications that stent migration would impose upon a subsequent liver transplant. 3. Both decompressive shunts and liver transplantation provide excellent long-term survival for patients with the Budd-Chiari syndrome. The determination of which surgical procedure is most appropriate is aided by assessment of the etiology of hepatic vein thrombosis, hepatic reserve, liver histology, and splanchnic venous anatomy. 4. Progressive hepatic damage may develop in patients with Budd-Chiari syndrome who have patent surgical shunts or TIPS. Lifelong follow-up and tracking of hepatic function are indicated. Some patients with shunts will require salvage with liver transplantation. 5. Long-term anticoagulation should be considered after transplantation, even in patients who do not have an identifiable coagulation disorder. Copyright 2006 AASLD.