Recognition and management of budd-chiari syndrome: Report of one hundred cases

Budd-Chiari syndrome is an unusual form of portal hypertension caused by occlusion of the hepatic venous outflow, and it is often confused with portal hypertension caused by liver cirrhosis. Its prognosis is poor, and optimal therapy remains to be established. This is a report of 100 confirmed cases of this syndrome treated from December 1982 to March 1988 at two vascular centers in China. Sixty-two male and 38 female patients, 15 to 62 years of age (mean age, 32.6 years) were treated. Seventy-six patients had intractable ascites, 56 had esophageal varices, and 22 had upper gastrointestinal bleeding. There were 37 cases of membranous obstruction, 57 cases of occlusion of the inferior vena cava above the confluence of the hepatic veins, and 6 cases of occlusion of the hepatic veins. Eighty-one patients were operated on. Operative mortality rate was 8.6% ( 7 81). Follow-up from 2 to 66 months revealed that 58 of the patients operated on (72%) had good results, whereas 11 of 19 (58%) patients treated nonoperatively died within 2 months after admission. On the basis of these data we conclude that the operative procedure must be tailored to the cause and underlying pathologic characteristics. Mesoatrial shunting is the operation of choice for patients with occlusion of the retrohepatic inferior vena cava and hepatic veins, and inferior vena cava-atrial shunting is the operation of choice for patients with occlusion of the inferior vena cava and patency of the hepatic veins. Membranotomy is used for patients with inferior vena cava webbing, and mesocaval shunting is used for patients with intrahepatic venous occlusion only. For patients who also have superior vena cava compression syndrome, an additional innominate-atrial shunt is recommended. These data also suggest that aggressive attempts at operative therapy may be rewarding.