Liver Transplantation for Congenital Biliary Dilatation: A Single-Center Experience
Liver Transplantation for Congenital Biliary Dilatation: A Single-Center Experience
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DOI：
10.1159/000319373
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发表时间：
2010-01-01
影响因子：
2.7
通讯作者：
Uemoto, Shinji
中科院分区：
文献类型：
--
作者：
Hori, Tomohide;Oike, Fumitaka;Uemoto, Shinji
背景：先天性胆管扩张症是一种罕见的疾病。虽然难治性胆管炎的可能性和/或恶性肿瘤的出现使肝胆手术合法化，但反复的胆管炎和胆道梗阻即使在多次手术后也会导致继发性肝硬变。肝移植治疗先天性胆管扩张症尚无明确指南。患者：共有1101名肝移植受者参加了这项研究。回顾分析11例先天性胆管扩张症患者的临床资料，其中Caroli病5例。结果：11例患者中，9例在肝移植前进行了首次手术，2例Caroli病患者在首次手术时接受了肝移植，效果良好。所有患者都有由肝硬变引起的顽固性症状，在年龄=F2的患者中生长延迟相当严重)。一名ABO血型不合的患者死亡。1例Caroli病合并肝内癌患者在肝移植后存活11.8年，无任何复发。结论：先天性胆管扩张伴顽固性症状和继发于肝功能衰竭的并发症的患者适合于肝移植。我们认为，肝移植是治疗先天性胆管扩张症的一种有效的治疗方法，并充分考虑了临床病程、生长迟缓、影像表现和组织病理学分析等诸多伴随因素。版权所有(C)2010 S.Karger AG，巴塞尔
Background: 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. Patients: 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. Results: 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 = 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. Conclusions: 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. Copyright (C) 2010 S. Karger AG, Basel