肝移植治疗Caroli病七例报道

目的 探讨肝移植治疗Caroli病的临床效果.方法 回顾性分析1999年9月至2008年2月期间7例弥漫型Caroli病患者行肝移植治疗后的临床资料.对其临床特征、手术方式、并发症类型以及随访情况进行分析.结果 7例患者术前均表现为复发性胆管炎症状并最终确诊为弥散型Caroli病.其中男性3例,女性4例;年龄10～31岁,平均16岁.6例患者肝移植术前接受保守治疗,1例曾行胆囊切除、T型管引流术.4例患者行劈离式肝移植,2例行全肝移植,1例行亲属活体供肝肝移植.其中2例患者术中采用静脉-静脉转流术.手术平均耗时9.1 h.术后并发症包括:肺部感染3例、急性排斥反应2例、胸腔积液2例、肝断面胆汁漏1例.经抗感染、调整免疫抑制方案及用量、胸腔穿刺抽吸及经皮穿刺引流等对症保守治疗后症状得到控制.1例肝移植受者术后19 d死于急性肾功能衰竭合并多器官功能衰竭,其余6例受者均存活.随访发现,存活的6例受者健康状况良好,其中最长存活已达7年.结论 应用肝移植治疗弥散型Caroli病具有良好的临床效果.

Liver transplantation for Caroli's disease-report of seven cases from a single center

Objective To investigate the clinical effects of liver transplantation including living related liver transplantation for Caroli's disease (CD). Methods Seven consecutive patients with diffused type of Caroli's disease had undergone liver transplantation (LT) from September 1999 to February 2007 in our single center. The clinical characteristics and survival of these patients were retrospectively reviewed. Results All 7 patients were diagnosed as Caroli's disease with diffused type which manifested recurrent cholangitis in clinical symptoms. Among them, 4 were female and 3 male.The mean age was 16 years old (ranging from 10 to 31 years old). Six patients were subjected to conservative therapy and only one patient had previously undergone cholecystectomy and T tube drainage before transplantation. In types of surgery, 4 patients accepted split liver transplantation with right liver lobe, two got whole liver transplantation and only one underwent living related liver transplantation. In two patients venovenous bypass was done during the operation. The mean duration of surgery was 9. 1 h. Post-transplant complications included pulmonary infection (3 cases), acute rejection (2 cases), pleural effusion (2 cases) and biliary leakage in the split section of donor liver (1 case). One patient died within 19 days caused by acute renal failure and multiple organs dysfunction.The rest six patients are alive without any signs of recurrence of protopathy and the longest survival time is 7 years. Conclusion Liver transplantation is a valuable treatment to Caroli's disease with diffused type. Due to the organ shortage, living related liver transplantation may own identical effects on LT.