Kasper H-U, Stippel D L, Töx U, Drebber U, Dienes H P
Institut für Pathologie, Clemenshospital Münster, Düesbergweg 128, 48153, Münster.
Pathologe. 2006 Jul;27(4):300-4. doi: 10.1007/s00292-006-0840-3.
Caroli's disease is a liver disease with segmental cystic dilatation of the intrahepatic bile ducts. It belongs to the group of congenital ductal plate malformations. With an incidence of only 0.05% of all liver cases in the Liver Registry of the University of Cologne, it is a very rare disorder. Caroli's disease is usually combined with cholangitis and bile duct stones. Control of these infections and maintenance of biliary drainage are the main therapeutic aims. The development of intra epithelial neoplasia and invasive carcinoma are rare complications. We report a case of Caroli's disease with the development of cholangiocarcinoma and review the literature.
卡罗里病是一种肝内胆管呈节段性囊状扩张的肝脏疾病。它属于先天性胆管板畸形组。在科隆大学肝脏登记处所有肝脏病例中，其发病率仅为0.05%，是一种非常罕见的疾病。卡罗里病通常合并胆管炎和胆管结石。控制这些感染和维持胆汁引流是主要的治疗目标。上皮内瘤变和浸润性癌的发生是罕见的并发症。我们报告一例并发胆管癌的卡罗里病病例并复习文献。
