Magnetic resonance imaging of fibropolycystic liver disease: the spectrum of ductal plate malformations

摘要

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.