50. Clinical profile and etiology of Budd-chairi: experience from our centre

Abstract

Background and Aims: Budd–chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the level of the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium. The clinical features and etiology of Budd–Chiari syndrome (BCS) vary from region to region. The aim was to study its presentation at our Centre. Methods: 30 consecutive cases of BCS was evaluated during the period from June 2017 till December 2017 at Madras Medical College, Chennai. The diagnosis was made on the basis of MR venography and Doppler ultrasonography. Results: The study population was made up of 16 females (mean age, 32.4 years) and 14 males (mean age, 46.5 years). Nine patients presented with acute disease, while 21 patients had subacute presentation. Abdominal pain, jaundice, upper gastrointestinal bleed and tender hepatomegaly was the commonest presentation. Ascites and pedal edema was seen only in 33% of the cases. BCS was caused by hepatic venous obstruction in 63%, inferior vena cava in 6% and combined obstruction in 31%. Doppler ultrasound accurately detected the site of bock in 24 of 30 patients (80%). Aetiologically, five had APLA syndrome, five secondary to cirrhosis, four had protein C and protein S deficiency, two were due to pregnancy and eleven (37%) were idiopathic. Conclusions: Hepatic venous outflow obstruction is a common problem; abdominal pain, tender hepatomegaly and UGI bleed was the common presentation, ascites was seen only in 1/3rd of the cases showing a changing spectrum compared to various studies. Subacute presentation is more frequent and females are at an increased risk. Idiopathic and inherited/acquired hypercoagulable states were the most common etiology. The authors have none to declare.