以食道靜脈曲張出血為表現的Caroli's症候群：一病例報告

Caroli's Syndrome Presented with Esophageal Variceal Bleeding: A Case Report

Caroli's disease是一個不常見的膽道疾病，易被誤診為多囊性肝臟疾病。其中的一個變異型為Caroli's syndrome，其除了膽管擴張以外還合併有先天性的肝臟纖維化，可表現有門脈高壓及其相關的併發症。若只侵犯到局部的肝葉時可以做部分肝切除來治療。但以長期的預後來看仍以肝臟移植為首選。我們報告了一位36歲女性，此次因食道靜脈瘤破裂出血而住院，經由我們一連串的檢查，最後診斷為Caroli's syndrome。

Caroli's disease is an uncommon biliary tract disease. It is easily misdiagnosed as polycystic liver disease. A common variant is Caroli's syndrome, in which bile duct dilatation is associated with congenital hepatic fibrosis. It usually presents as portal hypertension and its complications. Hepatic resection has been suggested for patients with monolobular or segmental involvements. Liver transplantation is the long-term treatment of choice. We report a 36 year-old female who was admitted for esophageal variceal bleeding. Imaging studies leading to the diagnosis of Caroli's syndrome are presented.