Radiology Unit, Department of Diagnostic and Therapeutic Services, IRCCS ISMETT (Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Via Tricomi 5, 90127, Palermo, Italy.
Department of Medical Imaging, Mater Dei Hospital, Msida, MSD 2090, Malta.
Abdom Radiol (NY). 2019 Jun;44(6):2156-2171. doi: 10.1007/s00261-019-01966-9.
Fibropolycystic liver diseases, also known as ductal plate malformations, are a group of associated congenital disorders resulting from abnormal development of the biliary ductal system. These disorders include congenital hepatic fibrosis, biliary hamartomas, polycystic liver disease, choledochal cysts and Caroli disease. Recently, it has been thought to include biliary atresia in this group of diseases, because ductal plate malformations could be implicated in the pathogenesis of this disease. Concomitant associated renal anomalies can also be present, such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney and nephronophthisis. These disorders can be clinically silent or can cause abnormalities such as cholangitis, portal hypertension, gastrointestinal bleeding and infections. The different types of ductal plate malformations show typical findings at magnetic resonance (MR) imaging. A clear knowledge of the embryology and pathogenesis of the ductal plate plays a pivotal role to understand the characteristic imaging appearances of these complex diseases. Awareness of these MR imaging findings is central to the detecting and differentiating between various fibropolycystic liver diseases and is important to direct appropriate clinical management and prevent misdiagnosis.
纤维多囊性肝病，也称为胆管板畸形，是一组与先天性胆管系统发育异常相关的先天性疾病。这些疾病包括先天性肝纤维化、胆管错构瘤、多囊肝病、胆总管囊肿和 Caroli 病。最近，人们认为胆道闭锁也属于这组疾病，因为胆管板畸形可能与该病的发病机制有关。同时也可能存在相关的肾脏异常，如常染色体隐性多囊肾病 (ARPKD)、海绵肾和肾单位肾痨。这些疾病可能没有临床症状，也可能导致胆管炎、门静脉高压、胃肠道出血和感染等异常。不同类型的胆管板畸形在磁共振成像 (MR) 上有典型表现。清楚了解胆管板的胚胎发生和发病机制对于理解这些复杂疾病的特征性影像学表现至关重要。认识到这些 MR 成像表现对于检测和区分各种纤维多囊性肝病非常重要，有助于指导适当的临床管理和防止误诊。
