Fibropolycystic Liver Disease in Children
Document Type
Article
Publication Title
Pediatric Radiology
Abstract
Fibropolycystic liver diseases are a group of associated congenital disorders that present most often in childhood. These disorders include congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic liver disease, choledochal cysts and Caroli disease. We present a discussion and illustrations of the embryology, genetics, anatomy, pathology, imaging approach and key imaging features that distinguish fibropolycystic liver disease in children. The pathogenesis of these disorders is believed to be abnormal development of the embryonic ductal plates, which ultimately form the liver and biliary systems. An understanding of the abnormal embryogenesis helps to explain the characteristic imaging features of these disorders.
DOI
10.1007/s00247-008-1070-z
Publication Date
4-2009
ISSN
1432-1998
Recommended Citation
Veigel MC, Prescott-Focht J, Rodriguez MG, Zinati R, Shao L, Moore CA, Lowe LH. Fibropolycystic Liver Disease in Children. Pediatric Radiology. 2009; 39(4). doi: 10.1007/s00247-008-1070-z.
