TREATMENT OF BUDD-CHIARI SYNDROME WITH PORTOSYSTEMIC SHUNT OR LIVER-TRANSPLANTATION

BACKGROUND: Budd-Chiari syndrome is an uncommon disorder caused by obs truction to hepatic venous outflow, causing varying degrees of hepatic injury depending on the extent, severity, and acuity of the obstructi on. PATIENTS AND METHODS: We reviewed the indications for operative in tervention and the results of treating 32 patients with Budd-Chiari sy ndrome seen at Toronto Hospital between 1958 and 1995. RESULTS: Twenty -one patients underwent portosystemic shunt (PSS) and 7 patients under went liver transplantation (LT) as their initial operative management. Three patients who initially had PSS subsequently required LT. Patien ts with cirrhosis found on biopsy and preservation of hepatocellular f unction were treated with PSS and showed no difference in outcome when compared with patients without cirrhosis (P = 0.35). Patients who wer e treated by PSS with retrohepatic vena caval compression, as shown by high caval gradients had outcomes similar to those for patients with low gradients (P = 0.31). Using the Kaplan-Meier method, 5-year surviv al of PSS patients was 57%. Liver transplantation was used to manage p atients with hepatic decompensation, as well as patients with vena cav al occlusion or failed PSS. The 5-year Kaplan-Meier survival for LT wa s 67%. CONCLUSIONS: Both PSS and LT are effective options in the manag ement of Budd-Chiari syndrome. Portosystemic shunt is the preferred in itial approach even with cirrhosis or retrohepatic caval compression a s long as there is preservation of liver function and a patent vena ca va. Liver transplantation should be used as primary therapy for patien ts with irreversible hepatic decompensation or vena caval occlusion, a nd it can be an effective salvage procedure following failed PSS.