Campbell D A, Rolles K, Jamieson N, O'Grady J, Wight D, Williams R, Calne R
Department of Surgery, Addenbrooke's Hospital, Cambridge.
Surg Gynecol Obstet. 1988 Jun;166(6):511-8.
Conventional medical and surgical management of Budd-Chiari syndrome is often unsuccessful. In this communication, we report the results of 19 hepatic transplants done for 17 patients suffering from Budd-Chiari syndrome. The first patient who had a transplant did not receive anticoagulant therapy during the postoperative period, and recurrent thrombosis of the hepatic veins in the newly transplanted liver rapidly developed. Sixteen patients who subsequently underwent transplantation were managed using a philosophy of early postoperative anticoagulant treatment when feasible. Using this approach, the cumulative proportion of patients surviving at one and three years was 88 per cent, and no recurrent hepatic vein thrombosis occurred during a mean follow-up period of 28.2 months. Forty-four per cent of the patients who were deliberately given anticoagulant medication experienced a hemorrhagic complication in the postoperative period, but there were no associated deaths. In spite of anticoagulant therapy, 31 per cent of the patients on anticoagulantion medication experienced a thrombotic complication that did not involve the hepatic veins; one early and one late death resulted, and a third patient required urgent retransplantation. Despite the difficulties in patient management, a carefully selected population of patients with hepatic failure secondary to Budd-Chiari syndrome appear to be well served by hepatic transplantation with early postoperative and long term anticoagulant therapy.
布加综合征的传统药物及手术治疗往往效果不佳。在本报告中，我们汇报了为17例布加综合征患者实施的19例肝移植手术结果。首例接受移植的患者在术后未接受抗凝治疗，新移植肝脏的肝静脉迅速出现复发性血栓形成。随后接受移植的16例患者在可行的情况下采用术后早期抗凝治疗策略。采用这种方法，患者1年和3年的累积生存率为88%，在平均28.2个月的随访期内未出现肝静脉复发性血栓形成。44%接受抗凝药物治疗的患者在术后出现出血并发症，但均未导致死亡。尽管进行了抗凝治疗，31%接受抗凝药物治疗的患者出现了不涉及肝静脉的血栓形成并发症；导致1例早期死亡和1例晚期死亡，另有1例患者需要紧急再次移植。尽管患者管理存在困难，但对于精心挑选的继发于布加综合征的肝衰竭患者，肝移植联合术后早期及长期抗凝治疗似乎效果良好。
