Successful emergency treatment with a transjugular intrahepatic portosystemic shunt for life-threatening Budd-Chiari syndrome with portal thrombotic obstruction

We report successful treatment of acute severe Budd-Chiari syndrome with po rtal venous thrombosis. The prognosis of patients with this condition is po or, because the therapeutic options are limited. A 38-year-old woman with p olycythemia vera was admitted in a critical condition, and Budd-Chiari synd rome complicated by portal venous thrombosis was diagnosed. Tissue plasmino gen activator and urokinase were infused systemically and were partially ef fective. Transjugular intrahepatic portosystemic shunting to reduce the hig h portal venous pressure was performed successfully and, eventually, her ge neral condition improved. Our experience indicates that emergency transjugu lar intrahepatic portosystemic shunting is an effective therapeutic modalit y for controlling portal hypertension in patients with severe Budd-Chiari s yndrome with portal venous thrombosis.