Divisions of Hepato-Pancreato-Biliary, Transplant and Pediatric Surgery, Department of Surgery, Kyoto University Hospital, Kyoto, Japan.
Dig Surg. 2010;27(6):492-501. doi: 10.1159/000319373. Epub 2010 Nov 10.
Congenital biliary dilatation is a rare disease. Although the possibility of refractory cholangitis and/or the frequency of malignant tumors legitimize hepatobiliary surgery, repeated cholangitis and biliary obstruction result in secondary liver cirrhosis even after polysurgery. There are no definitive guidelines on liver transplantation for congenital biliary dilatation.
A total of 1,101 liver transplantation recipients were enrolled in this study. Eleven patients with congenital biliary dilatation including 5 patients with Caroli's disease were retrospectively analyzed in detail.
Nine of 11 patients underwent initial operations before liver transplantation while 2 patients with Caroli's disease received liver transplantation as initial surgery, with good outcomes. All patients had intractable symptoms caused by liver cirrhosis, and growth delay was considerable in patients aged <20 years. Histopathological analysis of the native liver revealed hepatic fibrosis (≥F2). One patient with ABO incompatibility died. One patient with Caroli's disease accompanied with intrahepatic carcinoma survives 11.8 years after liver transplantation without any recurrences.
Patients with congenital biliary dilatation with refractory symptoms and complications secondary to liver failure are appropriate candidates for liver transplantation. We suggest that liver transplantation is an effective therapeutic option for patients with congenital biliary dilatation with due consideration to many accompanying factors, such as clinical course, growth delay, image findings and histopathological analysis.
先天性胆管扩张症是一种罕见疾病。尽管难治性胆管炎的可能性和恶性肿瘤的发生频率使肝胆手术成为合理选择，但多次胆管炎和胆道阻塞会导致二次肝硬化，即使经过多次手术也是如此。对于先天性胆管扩张症的肝移植，目前尚无明确的指南。
本研究共纳入 1101 例肝移植受者。详细回顾性分析了 11 例先天性胆管扩张症患者（包括 5 例 Caroli 病患者）。
9 例患者在肝移植前接受了初始手术，而 2 例 Caroli 病患者则将肝移植作为初始手术，结果良好。所有患者均有肝硬化引起的难治性症状，年龄<20 岁的患者生长迟缓较为明显。对供肝的组织病理学分析显示存在肝纤维化（≥F2）。1 例 ABO 不合患者死亡。1 例伴有肝内胆管癌的 Caroli 病患者在肝移植后 11.8 年无复发存活。
对于因肝功能衰竭而出现难治性症状和并发症的先天性胆管扩张症患者，肝移植是合适的治疗选择。我们建议，考虑到许多伴随因素，如临床病程、生长迟缓、影像学表现和组织病理学分析等，肝移植是先天性胆管扩张症患者的一种有效治疗选择。
