Miller W J, Sechtin A G, Campbell W L, Pieters P C
Department of Radiology, Medical College of Virginia, Virginia Commonwealth University, Richmond 23298-0001, USA.
AJR Am J Roentgenol. 1995 Aug;165(2):333-7. doi: 10.2214/ajr.165.2.7618550.
In 1958 Jacques Caroli described communicating cavernous ectasia of the biliary tree as an uncommon cause of chronic, often life-threatening hepatobiliary disease. The disease now most often referred to as Caroli's disease is a rare condition characterized by nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts. In the so-called pure form, dilatation is classically segmental and saccular and is associated with stone formation and recurrent bacterial cholangitis. In the form associated with congenital hepatic fibrosis, bile duct dilatation usually is less prominent; portal hypertension and eventual liver failure typically develop as a result of the hepatic fibrosis. Caroli's disease usually is manifested in childhood and is thought to be congenital and probably inherited. Associated conditions include renal cystic disease, choledochal cysts, and cholangiocarcinoma. This pictorial essay illustrates the broad spectrum of imaging findings in Caroli's disease.
1958年，雅克·卡罗利将胆管交通性海绵状扩张描述为慢性、常危及生命的肝胆疾病的一种罕见病因。现在最常被称为卡罗利病的这种疾病是一种罕见病症，其特征是肝内胆管出现非阻塞性囊状或梭形扩张。在所谓的单纯型中，扩张典型地呈节段性和囊状，并伴有结石形成和复发性细菌性胆管炎。在与先天性肝纤维化相关的类型中，胆管扩张通常不那么明显；门静脉高压和最终的肝功能衰竭通常是肝纤维化的结果。卡罗利病通常在儿童期出现，被认为是先天性的，可能具有遗传性。相关病症包括肾囊性疾病、胆总管囊肿和胆管癌。这篇影像学文章阐述了卡罗利病广泛的影像学表现。
