Budd-Chiari Syndrome: With Emphasis on Surgical Management

Abstract

Budd-Chiari syndrome (BCS) is a rare and often fatal disorder characterized by hepatic venous outflow obstruction. The occlusion is most often caused by thrombosis of the major hepatic veins and occasionally involves the inferior vena cava (IVC). This syndrome is associated with a variety of intraabdominal as well as systemic disorders. This report presents 2 cases of BCS in which both patients had polycythemia vera and 1 had a mildly decreased level of antithrombin III. A side-to-side portacaval shunt was performed in 1 patient and a mesoatrial shunt in the other owing to concomitant obstruction of the IVC. Dramatic improvement in the status of the liver was demonstrated by a follow-up biopsy in the latter patient.