小儿肝移植13例经验回顾

Six years experience of pediatric liver transplantation 13 cases reports

目的总结小儿肝脏移植13例的经验及教训。方法从2001年9月至2007年8月对13例终末期肝病患儿实施了肝移植术，其中先天性胆道闭锁7例，先天性肝纤维化3例，肝豆状核变性1例，Caroli病1例，肝脏血管内皮肉瘤1例，年龄5个月至12岁。供体及受体在门诊进行3～67个月随访并收集数据，对受体存活期与手术方式、术者经验、患儿年龄及原发病、家庭经济状况、父母教育背景等因素的关系进行分析。结果本组13例患儿全部顺利完成肝移植手术。不同手术方式的结果：亲体肝移植4例，供体随访6～59个月。均无并发症，健康生活；4例受体，1例于手术后15个月死于急性坏死性肠炎，另外3例无并发症健康生活，其中1例手术后25个月免疫耐受。尸体肝移植9例，目前存活4例，已死亡4例；另外1例手术后14个月因慢性排斥反应行二次肝脏移植手术。不同手术时间的结果：2001年1月至2003年10月间手术6例，已死亡5例，2年生存率为33．3％；2004年4月至2007年4月间开展手术7例，已死亡1例，存活6例，2年生存率为57．1％。结论肝移植是治疗小儿终末期肝病的有效方法，随着经验的积累，手术并发症和病死率有大幅度降低的趋势。医生和护理人员的经验，患儿的病情，家庭的经济实力，家庭主要成员的心理承受力、挽救孩子生命的动机、坚定程度和毅力，医护人员和家长的密切配合是影响肝移植预后的因素。

Objective To present our experience of pediatric liver transplantation（LT） in 13 children. Methods From November 2001 to July 2007, 13 patients with end-stage liver diseases were performed LT. Among them, 7 were biliary atresia, 3 were congenital hepatic fibrosis, 1 was Caroli＇s disease, 1 was Wilson＇s disease, and the other one was hepatohemoangioendotheliosarcoma. Patients＇ age ranged from 5 months to 12 years. Four of 13 children underwent parental living donor liver transplantation （LDLT）, among whom 1 was right lobe transplantation and 3 were left lateral lobe transplantation. Seven recipients underwent reduced size LT, whose grafts were the left lobe of 4 cases and left lateral lobe of 3 cases. The other 2 children underwent split LT, and the grafts were segmental graft （segment Ⅱ） and right posterior segment graft. The liver was totally removed in 11 recipients, and the whole liver and retrohaptic inferior vena cave were removed in the other 2 recipients. Results Grafts＇ weight of recipients ranged from 260 g tO 565 g（1.7%-3%BW）. LDLT donors and all recipients had been followed up for up to 6 years after operation. All of the four LDLT donors recovered soon after the operation without any complication. Three of the four LDLT recipients have survived till now without any complications, the other 1 was died of acute necrotizing enterocolitis. One of the nine patients underwent cadaver LT was re-transplanted for chronic rejection on the 14th month after operation, 5 of them have survived till now. From January of 2001 to October of 2003, 6 LTs were performed, 5 recipients died, and 2 years survival rate was 33.3%. From April of 2004 to April of 2007, 7 LTs were performed, only 1 recipient died, and 2 years survival rate was 57. 1%. Conclusions Liver transplantation is an effective treatment for the children with end-stage liver disease. The indication should be controlled strictly. Patients condition, family finance and mental stress should also be taken into consideration. The mortality and complication will decrease significantly withsurgeon＇s experience increase.