Department of Radiology, University Hospitals Gasthuisberg, Herestraat 49, Leuven, Belgium.
Eur J Gastroenterol Hepatol. 2011 Jul;23(7):578-85. doi: 10.1097/MEG.0b013e3283470fcd.
Caroli disease is a rare congenital disorder characterized by segmental, nonobstructive dilatation of intrahepatic bile ducts. The term Caroli syndrome is used for the association of Caroli disease with congenital hepatic fibrosis.
To provide an overview of the clinical presentation and imaging features of Caroli disease and syndrome, with an emphasis on magnetic resonance imaging.
Retrospective analysis of medical records on eight patients in whom a histologic diagnosis of Caroli disease or syndrome had been made.
Presenting signs and symptoms were (hepato)splenomegaly, hematemesis and/or melena, cholangitis, jaundice, and recurrent fever. The central dot sign, defined in the literature as a dot or bundle of strong contrast enhancement within dilated intrahepatic ducts, was found in seven cases on various imaging modalities. A 'dot-like structure' was found in one case in which only unenhanced studies were available. There was a tendency toward a right hepatic-lobe predominance.
There is an overlap between the imaging features of Caroli disease and Caroli syndrome. Our findings support earlier reports that the central dot sign is highly specific for the disease, and that it can be reliably detected by current imaging techniques.
Caroli 病是一种罕见的先天性疾病，其特征为肝内胆管节段性、非阻塞性扩张。Caroli 综合征是指 Caroli 病与先天性肝纤维化相关联的一种疾病。
本文旨在综述 Caroli 病和综合征的临床表现和影像学特征，重点介绍磁共振成像。
对 8 例经组织学诊断为 Caroli 病或综合征的患者的病历进行回顾性分析。
主要表现为肝脾肿大、呕血和/或黑便、胆管炎、黄疸和反复发热。在各种影像学检查中，7 例发现中央点征，该征在文献中定义为扩张的肝内胆管内的点状或束状强对比增强。1 例仅行非增强检查的患者发现了“点状结构”。存在右肝叶优势的倾向。
Caroli 病和 Caroli 综合征的影像学特征存在重叠。我们的研究结果支持了早期的报告，即中央点征对该疾病具有高度特异性，并且可以通过当前的成像技术可靠地检测到。
