Liver Transplantation Unit, Hospital Dr Cosme Argerich, Buenos Aires, Argentina.
HPB (Oxford). 2007;9(4):281-4. doi: 10.1080/13651820701329258.
Caroli's disease (CD) is a benign congenital disorder characterized by segmental cystic dilatation of the intrahepatic biliary ducts. Therapeutic strategy includes medical treatment, percutaneous, endoscopic or surgical drainage of the affected bile ducts, liver resection or transplantation. The aim of this study was to analyse the results and long-term follow-up of a consecutive series of patients who underwent surgical treatment for CD.
Between 1995 and 2005, 10 patients were surgically treated for CD. Variables evaluated were: age, gender, clinical presentation, diagnostic procedures, percutaneous and surgical treatments, histopathological analysis and outcome.
The average age of the patients was 45.8 years. Recurrent cholangitis was the main clinical manifestation (70%). In unilateral CD a liver resection was performed in nine patients (left lateral sectionectomy in seven, left hepatectomy in one and right hepatectomy in one). In bilateral disease a cholecystectomy, duct exploration, hepaticojejunostomy and liver biopsy of both lobes were performed. Average follow-up was 60 months. All the patients are alive and free of symptoms without recurrence in the remnant liver.
Liver resection is the preferred therapeutic option for unilateral CD, demonstrating good results in long-term follow-up. In bilateral disease, hepaticojejunostomy could be considered as an alternative or a previous step to liver transplantation, which still remains the ultimate option.
Caroli 病（CD）是一种良性先天性疾病，其特征为肝内胆管节段性囊状扩张。治疗策略包括药物治疗、经皮、内镜或手术引流受累胆管、肝切除术或肝移植。本研究旨在分析连续系列接受手术治疗的 CD 患者的结果和长期随访。
1995 年至 2005 年期间，10 例患者接受了 CD 的手术治疗。评估的变量包括：年龄、性别、临床表现、诊断程序、经皮和手术治疗、组织病理学分析和结果。
患者的平均年龄为 45.8 岁。复发性胆管炎是主要的临床表现（70%）。在单侧 CD 中，9 例患者行肝切除术（7 例行左外侧叶切除术，1 例行左半肝切除术，1 例行右半肝切除术）。在双侧疾病中，行胆囊切除术、胆管探查、胆肠吻合术和双侧肝活检。平均随访时间为 60 个月。所有患者均存活且无残留肝脏复发症状。
肝切除术是单侧 CD 的首选治疗方法，长期随访结果良好。在双侧疾病中，胆肠吻合术可作为肝移植的替代或前期步骤，肝移植仍然是最终的选择。
