Budd-Chiari syndrome. Historical and clinical review with an analysis of surgical corrective procedures.

Seventeen patients who fulfilled the criteria for the Budd-Chiari syndrome (centrilobular congestion and necrosis, a defined postsinusoidal block, and rapid onset of ascites) have been analyzed in terms of cause, prognosis, and treatment. Causal factors included caval web or tumor, hepatic tumor, a hypocoagulable state, myeloproliferative disease, and veno-occlusive disease. Location of the outflow block was suprahepatic (vena cava or major hepatic veins) in 13 patients and intrahepatic in 4. In five patients, a side-to-side portacaval shunt was effective in dissipating ascites with restoration to a normal lifestyle, as were transatrial fracture of a caval web (one patient) and resection of a huge cystadenoma of the liver (one patient). A peritoneal shunt provided effective palliation in three patients.