Direct intrahepatic portosystemic shunt in acute primary Budd-Chiari syndrome in a patient with autoimmune hepatitis: case report

Abstract

Budd-Chiari syndrome represents a rare vascular disorder defined by obstruction of hepatic venous outflow which, without timely intervention, may progress to portal hypertension, cirrhosis, and liver failure. Primary Budd-Chiari syndrome refers to intrinsic venous obstruction, which is most commonly associated with underlying prothrombotic states that predispose individuals to venous thrombosis. Interventional procedures are often required as a therapeutic modality when medical therapy alone is insufficient. Transjugular intrahepatic portosystemic shunt (TIPS) is commonly used to reduce portal hypertension in Budd-Chiari syndrome; however, it may not be feasible in the presence of hepatic vein thrombosis where cannulation of the hepatic vein, and thus, shunt creation may not be possible. In such cases, direct intrahepatic portosystemic shunt (DIPS) may provide an alternative route for portal decompression through the creation of a shunt directly between the inferior vena cava and the portal venous system. We describe a 34-year-old female presenting with autoimmune hepatitis and primary Budd-Chiari syndrome who underwent a successful ultrasound-guided direct intrahepatic portocaval shunt creation between the portal vein and the intrahepatic inferior vena cava, resulting in a significant reduction in portal venous pressure gradient and improved portal venous flow. This case highlights the feasibility of DIPS in a tertiary hospital setting and supports its role as an alternative endovascular approach for managing portal hypertension in Budd-Chiari syndrome.