TREATMENT OF THE BUDD-CHIARI SYNDROME WITH ORTHOTOPIC LIVER-TRANSPLANTATION AND LONG-TERM ANTICOAGULATION

The Budd-Chiari syndrome (BCS) with hepatic vein occlusion is a rare d isorder that can effectively be treated with orthotopic liver transpla ntation. In this retrospective analysis we report on 7 patients who re ceived 9 liver grafts for terminal BCS. One patient died after 4 month s due to cytomegalovirus-pneumonia. The actuarial survival rate is 85. 7% compared to more than 90% in all other 376 patients transplanted be tween September 1988 and April 1993 at our institution. Anticoagulatio n management consisted of early postoperative intravenous heparin and continuation with dicoumarin. One patient with thrombocytosis received hydroxyurea. Under this regimen one postoperative hemorrhage requirin g laparotomy was observed. Discontinuation of oral anticoagulation due to gastrointestinal bleeding caused hepatic artery and portal vein th rombosis in 1 patient who had to be retransplanted. One recurrence req uiring retransplantation as well was due to an insufficient dicoumarin intake. In conclusion, terminal BCS represents a good indication for orthotopic liver transplantation with a closely-monitored anticoagulat ion to avoid such adverse side effects as thrombosis and hemorrhage.