Imaging features of ductal plate malformations in adults

• Published in: Clinical radiology 2011-11, Vol.66 (11), p.1086-1093
• Main Authors: Venkatanarasimha, N, Thomas, R, Armstrong, E.M, Shirley, J.F, Fox, B.M, Jackson, S.A
• Format: Article
• Language: English
• Subjects: Adult; Bile Duct Diseases - congenital; Bile Duct Diseases - diagnosis; Bile Duct Diseases - diagnostic imaging; Bile Duct Diseases - pathology; Bile Duct Neoplasms - diagnosis; Bile Duct Neoplasms - diagnostic imaging; Bile Duct Neoplasms - pathology; Bile Ducts, Intrahepatic - abnormalities; Bile Ducts, Intrahepatic - diagnostic imaging; Biological and medical sciences; Caroli Disease - diagnosis; Diagnosis, Differential; Gastroenterology. Liver. Pancreas. Abdomen; Hamartoma - diagnosis; Hamartoma - diagnostic imaging; Hamartoma - pathology; Humans; Liver Cirrhosis - congenital; Liver Cirrhosis - diagnosis; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Malformations; Medical sciences; Middle Aged; Polycystic Kidney, Autosomal Recessive - diagnosis; Radiography; Radiology; Ultrasonography
• ISSN: 0009-9260; 1365-229X
• DOI: 10.1016/j.crad.2011.05.008

Ductal plate malformations, also known as fibrocystic liver diseases, are a group of congenital disorders resulting from abnormal embryogenesis of the biliary ductal system. The abnormalities include choledochal cyst, Caroli’s disease and Caroli’s syndrome, adult autosomal dominant polycystic liver disease, and biliary hamartoma. The hepatic lesions can be associated with renal anomalies such as autosomal recessive polycystic kidney disease (ARPKD), medullary sponge kidney, and nephronophthisis. A clear knowledge of the embryology and pathogenesis of the ductal plate is central to the understanding of the characteristic imaging appearances of these complex disorders. Accurate diagnosis of ductal plate malformations is important to direct appropriate clinical management and prevent misdiagnosis.