A 27-year experience with surgical treatment of Budd-Chiari syndrome

Objective To determine the effects of surgical portal decompression in Budd -Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver f unction, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. Summary Background Data Experiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on th e liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. Methods From 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to -side portacaval shunt (SSPCS); the second had occlusion involving the infe rior vena cava (IVC) treated by a portal decompressive procedure that bypas sed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation nd were referred too late for treatment by portal d ecompression, required orthotopic liver transplantation. Results In the 32 patients with BCS resulting from hepatic vein occlusion a lone, SSPCS had a surgical death rate of 3%, and 94% of the patients were a live 3.5 to 27 years after surgery. All 31 survivors remained free of ascit es and almost all had normal liver function, No patient with a patent shunt had encephalopathy. The SSPCS remained patent in ail but one patient. Live r biopsies showed no evidence of congestion or necrosis, and 48% of the bio psies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thr ombosis. All patients survived surgery, but Rye subsequently developed thro mbosis of the synthetic graft and died. Because of the poor results, mesoat rial shunt was abandoned. Instead, a high-flow combination shunt was introd uced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patient s who underwent combined SSPCS and GAS, and the shunts functioned effective ly during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for ortho topic liver transplantation. Three patients died of liver failure while awa iting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively. Conclusions SSPCS in BCS with hepatic vein occlusion alone results in rever sal of liver damage, correction of hemodynamic disturbances, prolonged surv ival, and good quality of life when performed early in the course of BCS. S imilarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has be en discontinued in the authors' program because of an unacceptable incidenc e of graft thrombosis and death. In patients with advanced cirrhosis from l ong-standing, untreated BCS, orthotopic liver transplantation is the only h ope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression.