Liver transplantation for Caroli's disease-report of seven cases from a single center / 中华器官移植杂志

ABSTRACT

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.