Wambura Casmir, Sharma Munish, Surani Salim
Internal Medicine, The Aga Khan University, Dar es Salaam, TZA.
Internal Medicine, Corpus Christi Medical Center, Corpus Christi, USA.
Cureus. 2020 Jan 15;12(1):e6661. doi: 10.7759/cureus.6661.
Caroli disease and Caroli syndrome are two rare congenital diseases of the intrahepatic bile ducts. Caroli syndrome is characterized by the saccular dilatation of intrahepatic bile ducts associated with congenital hepatic fibrosis. It is rarely diagnosed in childhood. We hereby describe a case of Caroli syndrome in a young Tanzanian female who had abdominal pain and distension since childhood. Her history suggested the presence of portal hypertension possibly from congenital hepatic fibrosis. The diagnosis was reached based on ultrasound, computed tomography (CT) scan of the abdomen, and magnetic resonance cholangiopancreatography (MRCP).
卡罗里病和卡罗里综合征是两种罕见的肝内胆管先天性疾病。卡罗里综合征的特征是肝内胆管囊状扩张并伴有先天性肝纤维化。它在儿童期很少被诊断出来。我们在此描述一例年轻坦桑尼亚女性的卡罗里综合征病例，该患者自童年起就有腹痛和腹胀症状。她的病史提示可能存在由先天性肝纤维化引起的门静脉高压。诊断是基于腹部超声、计算机断层扫描（CT）以及磁共振胰胆管造影（MRCP）做出的。
