Pediatric Radiology

, 39:317

Fibropolycystic liver disease in children

Authors

    • Kansas City University of Medicine & Biosciences
    • Department of RadiologyUniversity of Missouri-Kansas City, St. Luke’s Hospital
  • Julia Prescott-Focht
    • Department of RadiologyUniversity of Missouri-Kansas City, St. Luke’s Hospital
  • Michael G. Rodriguez
    • University of Missouri-Kansas City School of Medicine
  • Reza Zinati
    • Department of RadiologyUniversity of Missouri-Kansas City, St. Luke’s Hospital
  • Lei Shao
    • Department of PathologyChildren’s Mercy Hospitals and Clinics
  • Charlotte A. W. Moore
    • Department of RadiologyUniversity of Missouri-Kansas City
    • Department of RadiologyChildren’s Mercy Hospitals and Clinics
  • Lisa H. Lowe
    • Department of RadiologyUniversity of Missouri-Kansas City
    • Department of RadiologyChildren’s Mercy Hospitals and Clinics
Review

DOI: 10.1007/s00247-008-1070-z

Cite this article as:
Veigel, M.C., Prescott-Focht, J., Rodriguez, M.G. et al. Pediatr Radiol (2009) 39: 317. doi:10.1007/s00247-008-1070-z

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

PediatricBiliaryCystCholedochalCongenitalEmbryology

Copyright information

© Springer-Verlag 2008