TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC S TENT-SHUNT (TIPSS) IN BUDD-CHIARI SYNDROME WITH PORTAL-VEIN THROMBOSIS

History and clinical findings: A 41-year-old woman, known for 10 month to have polycythaemia vera, developed severe right upper abdominal pa in. The abdomen was tense from marked ascites and the liver enlarged b y 18 cm in the mid-clavicular line. Investigations: Serum bilirubin wa s slightly elevated to 2.2 mg/dl, liver synthesis being much reduced ( recalcifying time minimally 23%, albumin minimally 2.8 g/dl. Doppler s onography detected no flow in the right and middle hepatic veins, indi cating Budd-Chiari syndrome. Portal vein flow was diminished. Treatmen t and course: Heparin treatment had to be stopped because of heparin-a ssociated type II thrombocytopenia and hirudin was substituted. Attemp ted lysis with a total of 100 mg r-tPA failed. As the patient's condit ion deteriorated a TIPSS was implanted to provide portal decompression . incomplete portal vein thrombosis was demonstrated and worsened duri ng the procedure until nearly complete occlusion. Local lysis treatmen t for 2 days with urokinase, 50 000-60 000 U/h, and two shunt revision s finally succeeded in completely dissolving the thrombus. Portocaval pressure fell from 32 to 21 mm Hg, and the size and function of the li ver became almost normal and the ascites disappeared. Anticoagulation with a coumarin derivative was started and hydrocarbamide again given for recurrent thrombocytosis. The patient remained largely symptom-fre e one year after TIPSS. Conclusion: This case demonstrates the effecti veness of TIPSS in Budd-Chiari syndrome, even in complicated portal ve in thrombosis.