Budd-Chiari Syndrome: Aetiologi, Diagnosis, and Management

Abstract

Budd-Chiari Syndrome (BCS) is known as a disease caused by the presence of outflow obstruction of the hepatic vein and is commonly not considered as a diagnosis. Such obstruction may happen in all hepatic vein flow points, starting from small veins to the inferior vena cava opening in the right atrium, regardless of the aetiology. This syndrome occurs in approximately 0.001% population and has various aetiologies, including congenital and acquired prothrombotic conditions, myeloproliferative disease, and oral contraceptives use. Advancement in imaging enables most BCS to be diagnosed based on non-invasive imaging tests. Colour Doppler ultrasonography, which has sensitivity and specificity rate of 85-95%, is an appropriate technique for early investigation of Budd-Chiari syndrome. Colour Doppler ultrasonography and MSCT with contrast have a significancy rate of 85-91.3% with a p value > 0.05 between both modalities in diagnosing BCS. Management of BCS starts with the non-invasive to highly invasive procedures, consisting of medical or recanalization therapy to prevent necrosis.