Surgical management of patients with the Budd-Chiari syndrome

Twelve patients with the Budd-Chiari syndrome were managed surgically at The Johns Hopkins Hospital over a 10-year period. Ten patients were female and 2 were male. Nine were white, 2 were black, and 1 was Asian. Three patients had polycythemia vera, one had paroxysmal nocturnal hemoglobinuria, and one was taking estrogens. The diagnosis was confirmed in all 12 patients by liver biopsy and angiography. Five patients had a patent inferior vena cava and underwent a mesocaval shunt. There were 2 hospital deaths in this group. Ascites redeveloped in 1 patient and was controlled with a Le Veen shunt. The remaining 2 patients did well, are ascites free, and have angiographically demonstrated patent shunts. Histological findings in these 2 patients showed the condition of the liver to have improved markedly. Seven of the 12 patients had occlusion of the inferior vena cava due to thrombosis (6) or compression by the enlarged liver (1), and underwent mesoatrial shunts. There were 2 hospital deaths in this group. One late death occurred from liver failure following thrombosis of the entire mesenteric venous system. Ascites redeveloped in another patient and was controlled with a Le Veen shunt. The remaining 3 patients did well and were demonstrated angiographically to have patent shunts. Two of the 3 patients redeveloped ascites at 4 and 10 months postoperatively and required a second mesoatrial shunt. They are now ascites free, with angiographically demonstrated patent shunts 10 months and 12 months, respectively, following their second operation. The follow-up for the 6 patients still alive ranges from 15 to 74 months.