Department of Abdominal Imaging, University of Alabama at Birmingham, Birmingham, AB, USA.
Division of Abdominal Imaging, Department of Radiology, Mayo Clinic, MN, Rochester, USA.
Abdom Radiol (NY). 2022 Jul;47(7):2356-2370. doi: 10.1007/s00261-022-03565-7. Epub 2022 Jun 7.
Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. The most common clinical manifestations of FLDs are portal hypertension, cholestasis, cholangitis, and (in rare cases) cholangiocarcinoma. This article reviews recent updates in the pathophysiology, imaging, and clinical management of FLDs.
纤维多囊性肝病（FLD）是一组罕见的遗传性肝胆疾病，由先天性胆管板畸形（DPM）引起，其原因是胆管细胞初级纤毛上表达的蛋白功能障碍。胆管板的胚胎发育是理解这组疾病的关键。特别是，DPM 可导致不同程度的肝内胆管受累和广泛的胆管疾病，包括先天性肝纤维化、Caroli 病、多囊肝病和 Von Meyenberg 复合体。FLD 的最常见临床表现为门静脉高压、胆汁淤积、胆管炎和（在罕见情况下）胆管癌。本文综述了 FLD 的病理生理学、影像学和临床管理的最新进展。
