Recognition and management of Budd-Chiari syndrome. Experience with 143 patients.

Of 143 patients with Budd-Chiari syndrome treated from December 1982 to August 1988, 100 had intractable ascites, 78 had esophageal varices and 39 had upper gastrointestinal bleeding. In these patients, 49 had membranous obstruction, 134 had occlusive lesions of the inferior vena cava (IVC) above the confluence of the hepatic veins including 50 membranous obstructions, and 9 had occlusion of the hepatic veins. According to the location and extent of lesions, we classified this disease into eight categories; the severity of the disease and corresponding operative risk were used to divide the disease into four grades. One hundred and four patients (73%) were operated upon. There was no death in Stage I and II patients, but in Stage III and IV patients the mortality was 9% and 21% respectively. The overall operative mortality was 9.6%. Follow-up for 1 to 66 months revealed that 73% (76/104) had good results except 4 who died from other causes. Among 39 patients receiving conservative treatment, 13 (33.3%) died of late complications. They all belonged to Stage IV patients. We conclude that the earlier the treatment the better the results, and the operative procedure must be tailored to the etiology and underlying pathology.