Budd-Chiari syndrome.

A 16-year-old Ethiopian male patient presented with 18 months history of anasarca, anemia & hepatomegaly associated with hypoprothrombinemia, and diagnosed to have Budd-Chiari syndrome (BCS). The patient responded markedly to medical therapy with diuretics, anticoagulation and salt restriction. The clinical features and treatment of BCS is briefly reviewed and discussed.

BACKGROUND

BCS is a rare disorder resulting from obstruction to the outflow of blood from the liver. It results from occlusion or partial occlusion of one, two, or all three of the major hepatic veins and/or occlusion or partial occlusion of the inferior vena cava (IVC). The clinical diagnosis is difficult and Radiology plays a critical role for diagnosis and classification. Type I is occlusion of the IVC with/without hepatic veins, type II is occlusion of major hepatic veins with/without IVC, and type III is occlusion of small centrilobar veins.