亲属活体供肝移植治疗儿童终末期肝病六例

目的 探讨亲属活体供肝移植(LDLT)治疗儿童终末期肝病的效果,并总结治疗经验.方法 2005年9月至2007年1月对6例终末期肝病患儿进行了LDLT.6例患者中,原发病为肝内外胆管弥漫性囊性扩张症Ⅳ型伴肝硬化1例、肝豆状核变性2例、门静脉海绵样变性3例;供者为患儿的母亲3例、父亲2例及舅父1例,分别切取供者的右半肝1例和左半肝5例作为供肝;在切除受者全部病肝和保留下腔静脉后,对受者施行部分供肝的原位肝移植.术后依据供、受者的临床表现、血液学指标和影像学检查,对活体供肝移植的治疗效果进行评价.结果 术后对供、受者随访了6～21个月.6例供者均健康存活,未发生并发症.6例受者中,1例于术后第4天死于门静脉血栓形成,1例于术后5个月时死于肝内静脉血栓形成,其余4例均长期健康存活.结论 亲属活体供肝移植是治疗儿童终末期肝病的有效方法.术前进行仔细的供、受者选择和完善的影像学检查,术中应用精确的手术技术,术后给予严格的管理是儿童LDLT成功的关键.

Living donor liver transplantation for children with end-stage liver diseases:a report of 6 cases

Objective To evaluate the efficacy of living donor liver transplantation(LDLT)in the treatment of children with end-stage liver diseases and summarize the experience of LDLT for children. Methods LDLT was performed on 6 children with end-stage liver diseases in our hospital from September 2005 to January 2007. There were 2 cases of Wilson's disease associated with liver cirrhosis,one case of Caroli's disease with liver cirrhosis and 3 cases of portal vein cavernous transformation with severe hypertension. Their age ranged from 7 to 14 years,5 children received the left lobe grafts from their mother or father,and 1 children received the right lobe graft from her mother's brother.The evaluation of the outcome was made by clinical features,1aboratory and imaging examinations.Results Six donors in LDLT resumed to their pre-transplantation live soon after the operation without occurrence of any complication. Four of the 6 children survived for 6-20 months now,1 died of portal vein thrombosis on the 4th day after the operation,and 1 died of intrahepatic vein thrombosis after 5 months. Conclusions LDLT may be an effective option to treat children with end-stage liver diseases.Accurate preoperative imaging determination,good perioperative ICU care and precise surgical skills are critical for success of LDLT in children.