Case Report: Autopsy Findings in Fatal Budd-Chiari Syndrome – A Rare Cause of Sudden Death

Abstract

Abstract: Budd-Chiari Syndrome (BCS) is a rare but life-threatening disorder characterized by the obstruction of hepatic venous outflow, which leads to hepatic congestion, portal hypertension, and potential progression to cirrhosis (Valla, 2009). Predisposing factors include hypercoagulable states, myeloproliferative disorders, and infections (Janssen, Garcia-Pagan, Elias, Mentha, & Valla, 2003). We present an autopsy case of a 24-year-old male, a known case of BCS complicated by Hepatitis C virus (HCV) infection and chronic liver disease, for whom liver transplantation was planned. The patient succumbed during medical management. The medico-legal autopsy revealed gynecomastia, micronodular liver cirrhosis, massive ascites, splenomegaly, esophageal varices, and bilateral pleural effusions. Histopathological examination confirmed cirrhotic architectural disruption. The immediate cause of death was determined to be hepatic failure secondary to BCS. This case underscores the aggressive natural history of BCS, particularly when compounded by concurrent HCV infection, and highlights the critical medico-legal role of autopsy in confirming the diagnosis and elucidating the pathophysiological sequence in rare, fatal presentations.