Budd-Chiari Syndrome: An Uncommon Cause of Chronic Liver Disease that Cannot Be Missed

Abstract

Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.

Keywords: Angioplasty; Anticoagulation; Hepatic venous outflow tract obstruction; Portal shunt; Splanchnic thrombosis; Thrombophilia; Transjugular intrahepatic portosystemic shunt; Transplantation.