Budd-Chiari syndrome: Our experience of 71 patients

Background: Hepatic venous outflow obstruction (Budd-Chiari syndrome) is fr equently encountered as a cause of portal hypertension at our centre. Methods and Results: We studied the clinical presentation, therapeutic moda lities and outcome of 71 patients with hepatic venous outflow obstruction b etween 1992 and 1997. Twenty-seven patients presented with acute disease, w hile 44 had chronic presentation. Abdominal pain, distension, jaundice and upper gastrointestinal bleeding were the commonest presenting symptoms. The majority of patients had distended veins, hepatomegaly, splenomegaly, asci tes and ankle oedema. The diagnosis was made on the basis of inferior vena cavography/functional hepatography and pulsed Doppler ultrasonography and/o r liver biopsy in 39 patients and pulsed Doppler ultrasonography and/or liv er biopsy in 32 patients. Pulsed Doppler ultrasonography accurately detecte d the site of the block in 31 of 39 patients (79.4%). The obstruction was i n the hepatic vein in 20 patients, in the inferior vena cava in 10, and in both in 41 patients. Aetiologically, four had pregnancy-related disease, fo ur tumour-related, three hypercoagulable states, 18 inferior vena cava memb ranes and 42 were idiopathic. Of 30 patients in whom liver biopsy was carri ed out, eight had centrizonal congestion and necrosis, 13 had mixed feature s and nine had established cirrhosis. Seven patients underwent a shunt oper ation and surgical membranotomy was carried out in one. Three patients (4.2 %) died in the hospital. Conclusions: Hepatic venous outflow obstruction is a common problem; patien ts present with abdominal pain, distension, jaundice, distended veins, asci tes and ankle oedema. Chronic presentation is more frequent. Pulsed Doppler ultrasound, venography and liver biopsy are very helpful in diagnosis. (C) 2000 Blackwell Science Asia Pty Ltd.