Two-step procedure in Budd-Chiari syndrome with severe intrahepatic vena cava stenosis: vena cava stenting and portocaval shunt

Budd-Chiari syndrome is characterized by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Venous decompressive shunt surgery and liver transplantation represent efficient surgical treatments of Budd-Chiari syndrome. In the case presented here, severe intrahepatic compression of the inferior vena cava (IVC) was caused by the hypertrophic caudate lobe. A mere portocaval shunt was not feasible because of a large pressure gradient across the intrahepatic stenosis. A two-step procedure with preoperative radiological dilation and stenting of the intrahepatic IVC followed by a portocaval shunt was successfully performed. Consequently, liver transplantation and its subsequent immunosuppression could be avoided.