儿童活体肝移植42例临床报告

目的 总结成人活体部分供肝儿童肝移植的临床疗效和经验.方法 42例儿童患者,年龄80 d至14岁.小于1岁者28例;体重3.08～45 kg,小于10kg者27例.移植前有不同程度的黄疸、腹水、营养不良和肝功能严重损害.其中父母供肝36例,祖母供肝4例.舅父和表兄供肝各1例.供肝类型包括:左外叶31例.Ⅱ段肝组织1例,左半肝8例.右半肝2例.对供肝的肝静脉、肝动脉和受者的肝动脉、肝静脉、门静脉进行成形.以便吻合;供肝动脉较短者,以供者大隐静脉搭桥.免疫抑制方案:采用环孢素A(CsA)+糖皮质激素21例,CsA+吗替麦考酚酯(MMF)+糖皮质激素8例,他克莫司(Tac)+糖皮质激素7例,Tac+MMF+糖皮质激素6例.术后随访时问2～43个月.结果 移植物与受者质量比为0.91%～5.71%,移植物与受者标准肝体积比为40.7%～137.1%.术后早期32例(76.2%,32/42)出现并发症,死亡5例,其中4例死于血管并发症;随访期9例出现并发症,死亡4例,其中3例死于血管并发症;意外死亡2例.其余31例(73.8%,31/42)健康存活.结论 成人活体部分供肝儿童肝移植是治疗儿童终末期肝病的有效方法,术后血管并发症是主要的死亡原因.预防和治疗血管并发症能明显提高手术成功率.

Adult-to-children living donor liver transplantation: a report of 42 cases

Objective To present our clinical outcomes of adult-to-children living donor liver transplantation(A-CLDLT) and summarize our experience. Methods The clinical data, preoperative assessment, surgical strategies and complications of 42 adult donors and children recipients who underwent A-CLDLT from April 2006 to December 2009 in Children's Hospital of Chongqing Medical University were retrospectively analyzed. These 42 recipients (21 boys and 21 girls) aged from 80 days to 14 years whose body weight at the time of operation was 3. 08 to 45. 00 kg. In all the children recipients, 28 cases suffered biliary atresia, 6 Wilson' s diseases, 4 glycogen storage diseases, 3 cavernous transformation of the portal vein, and 1 fulminant hepatitis. All recipients were followed up from 2 to 43 months. Results The living donors were 24 mothers, 12 fathers, 4 grandmothers, 1 elder brother and 1 uncle with ABO compatible with the children recipients. Thirty-one left lateral lobes, Ⅰ segment Ⅱ, 8 left lobes without middle hepatic vein (MHV) and 2 right lobes without MHV were obtained. Average hospital length was 9. 8 days for the donor group without any complications except one donor suffered from postoperative bile leakage. Postoperative immunosuppression included prednisone, Tac, cyclosporine and mycophenolate mofetil Thirty-six postoperative complications occurred in 32 recipients (76. 2 %,), and 5 died from postoperative complications including 4 postoperative vascular complications and 1 Aspergillus pulmonary infection. The perioperative mortality rate of recipients was 11.9 % (5/42). During follow-up term, 9 suffered from complications and 4 died including 1 case of hemophagocytic syndrome and 3 cases of stricture of hepatic vein. In addition, 2 recipients died of accidental asphyxia and food poisoning during follow-up term. The longest survival time was 43 months. At present, 31 recipients were survival (73. 8 %). Conclusion A-CLDLT has become an effective method to children with end-stage liver disease. The postoperative vascular complications are the predominant cause of death. Prevention and therapy of the vascular complications are extremely helpful to raise the survival rate of A-CLDLT.