Knoop M, Lemmens H P, Bechstein W O, Blumhardt G, Schattenfroh N, Keck H, Neuhaus P
Department of Surgery, University Hospital Rudolf Virchow, Free University of Berlin, Germany.
Clin Transplant. 1994 Feb;8(1):67-72.
The Budd-Chiari syndrome (BCS) with hepatic vein occlusion is a rare disorder that can effectively be treated with orthotopic liver transplantation. In this retrospective analysis we report on 7 patients who received 9 liver grafts for terminal BCS. One patient died after 4 months due to cytomegalovirus-pneumonia. The actuarial survival rate is 85.7% compared to more than 90% in all other 376 patients transplanted between September 1988 and April 1993 at our institution. Anticoagulation management consisted of early postoperative intravenous heparin and continuation with dicoumarin. One patient with thrombocytosis received hydroxyurea. Under this regimen one postoperative hemorrhage requiring laparotomy was observed. Discontinuation of oral anticoagulation due to gastrointestinal bleeding caused hepatic artery and portal vein thrombosis in 1 patient who had to be retransplanted. One recurrence requiring 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 anticoagulation to avoid such adverse side effects as thrombosis and hemorrhage.
布加综合征（BCS）伴肝静脉闭塞是一种罕见疾病，原位肝移植可有效治疗该疾病。在这项回顾性分析中，我们报告了7例因终末期BCS接受9次肝移植的患者。1例患者在术后4个月因巨细胞病毒肺炎死亡。与1988年9月至1993年4月间在我们机构接受移植的其他376例患者中超过90%的患者相比，实际生存率为85.7%。抗凝管理包括术后早期静脉注射肝素，并持续使用双香豆素。1例血小板增多症患者接受了羟基脲治疗。在此方案下，观察到1例术后出血需要开腹手术。1例患者因胃肠道出血停用口服抗凝药后发生肝动脉和门静脉血栓形成，不得不再次移植。另1例需要再次移植的复发是由于双香豆素摄入不足。总之，终末期BCS是原位肝移植的良好适应证，需密切监测抗凝情况以避免血栓形成和出血等不良副作用。
