Selective surgical therapy of the Budd-Chiari syndrome provides superior survivor rates than conservative medical management.

The Budd-Chiari syndrome is an unusual form of portal hypertension caused by hepatic vein occlusion, which results in centrilobular congestion and necrosis. Its overall mortality rate exceeds 50% at 2 years, and optimal treatment remains controversial. To determine optimal therapy on the basis of clinical and angiographic data, we retrospectively analyzed 30 patients with this disease treated at the University of California, Los Angeles or the University of Southern California School of Medicine between 1955 and 1985. Twelve patients were treated conservatively with diuretics, anticoagulants, paracentesis, and/or peritoneal-venous shunt. Eighteen patients were treated by definitive surgery: side-to-side portocaval shunt (four), mesocaval shunt (four), side-to-side splenorenal shunt (one), liver transplantation (two), transatrial membranotomy (TM) (two), TM followed by inferior vena cava reconstruction (IVCR) (one), TM followed by mesocaval shunt (one) simultaneous IVCR and side-to-side portocaval shunt (one), IVCR (one), and azygousatrial shunt followed 4 years later by a splenopulmonary shunt (one). Overall the surgical group had survival rates superior to the medical group, with a 2-year survival rate of 54% for the surgical group vs. 9% for the medical group (p less than 0.089). On the basis of these data we conclude that surgical therapy is superior to medical therapy for the Budd-Chiari syndrome. However, the operation must be tailored to treat the specific anatomic and clinical abnormality. Guidelines to select the proper surgical procedure are given.