Therapeutic experiences with Budd-Chiari syndrome: report of 100 cases

One-hundred cases of Budd-Chiari syndrome were treated from December 1982 to March 1988. There were 62 males and 38 females. Their ages ranged from 15 to 62 years averaged 32.6 years Seventy-six cases had intractable ascites, 56 had esophageal varices and 22 had upper gastrointestinal bleeding. There were 37 cases with membranous obstruction, 57 with occlusion of the inferior vena cava above the confluence of the hepatic veins and 6 with occlusion of hepatic veins. Eighty-one cases (81%) were operated upon. Operative mortality was 8.6% (7/81). Follow-up from 2 to 63 months revealed that 60 operated cases (74.1%) had good results, while 11 of 19 (58%) patients treated conservatively died within two months after admission. It is concluded that the operative procedure must be tailored to the etiology and pathology. The mesoatrial shunt is the operation of choice for occlusion of the retrohepatic IVC and hepatic veins, the cava-atrial shunt for occlusion of the IVC and patent hepatic veins, membranotomy for IVC web, and the meso-caval shunt for intrahepatic venous occlusion. For those who also have superior vena cava compression syndrome, an innominate-atrial shunt should be added.