Fibropolycystic liver disease in children |
| |
Authors: | Myka Call Veigel Julia Prescott-Focht Michael G. Rodriguez Reza Zinati Lei Shao Charlotte A. W. Moore Lisa H. Lowe |
| |
Affiliation: | (1) Kansas City University of Medicine &; Biosciences, Kansas City, MO, USA;(2) Department of Radiology, University of Missouri-Kansas City, St. Luke’s Hospital, 4401 Wornall Road, Kansas City, MO 64111, USA;(3) University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA;(4) Department of Pathology, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA;(5) Department of Radiology, University of Missouri-Kansas City, 2401 Gillham Road, Kansas City, MO 64108, USA;(6) Department of Radiology, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA |
| |
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. |
| |
Keywords: | Pediatric Biliary Cyst Choledochal Congenital Embryology |
本文献已被 SpringerLink 等数据库收录! |
|