Taylor A C, Palmer K R
Gastroenterology Department, Western General Hospital, Edinburgh, UK.
Eur J Gastroenterol Hepatol. 1998 Feb;10(2):105-8. doi: 10.1097/00042737-199802000-00001.
Caroli's disease is characterized by multifocal segmental dilatation of the intrahepatic bile ducts. It is a rare congenital condition, which appears to be autosomal recessively inherited in most cases. There are two forms of disease, one associated with congenital hepatic fibrosis and a simple form occurring alone. Recent reports suggest that the simple form may be as common as that with congenital hepatic fibrosis. Other conditions, including choledochal cyst and renal cystic disease, are frequently associated. The major clinical feature is recurrent cholangitis, which may be complicated by intrahepatic calculi and hepatic abscess formation. There is good evidence that malignancy complicates Caroli's disease in approximately 7% of cases. The diagnosis rests on demonstrating that the cystic liver lesions are in continuity with the biliary tree. Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree. The treatment depends on the clinical features and the location of the biliary abnormality. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy. In diffuse Caroli's disease, treatment options include conservative or endoscopic therapy, internal biliary bypass procedures and liver transplantation in carefully selected cases.
卡罗利病的特征是肝内胆管多灶性节段性扩张。它是一种罕见的先天性疾病，在大多数情况下似乎是常染色体隐性遗传。该病有两种形式，一种与先天性肝纤维化相关，另一种为单独出现的单纯形式。最近的报告表明，单纯形式可能与伴有先天性肝纤维化的形式一样常见。其他病症，包括胆总管囊肿和肾囊性疾病，也经常与之相关。主要临床特征是复发性胆管炎，可能并发肝内结石和肝脓肿形成。有充分证据表明，约7%的卡罗利病病例会并发恶性肿瘤。诊断取决于证明肝脏囊性病变与胆管树相连。现代成像技术使诊断更容易，且无需对胆管树进行侵入性成像。治疗取决于临床特征和胆道异常的位置。当疾病局限于一个肝叶时，肝切除术可缓解症状，且似乎可消除恶性肿瘤风险。在弥漫性卡罗利病中，治疗选择包括保守或内镜治疗、内胆管搭桥手术以及在精心挑选的病例中进行肝移植。
