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Pediatr Radiol. 2009 Apr;39(4):317-27; quiz 420-1. doi: 10.1007/s00247-008-1070-z. Epub 2008 Dec 16.

Fibropolycystic liver disease in children.

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  • 1Kansas City University of Medicine & Biosciences, Kansas City, MO, USA.


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.

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