Pediatric Radiology

, 39:317

Fibropolycystic liver disease in children

  • Myka Call Veigel
  • Julia Prescott-Focht
  • Michael G. Rodriguez
  • Reza Zinati
  • Lei Shao
  • Charlotte A. W. Moore
  • Lisa H. Lowe
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

Authors and Affiliations

  • Myka Call Veigel
    • 1
    • 2
  • Julia Prescott-Focht
    • 2
  • Michael G. Rodriguez
    • 3
  • Reza Zinati
    • 2
  • Lei Shao
    • 4
  • Charlotte A. W. Moore
    • 5
    • 6
  • Lisa H. Lowe
    • 5
    • 6
  1. 1.Kansas City University of Medicine & BiosciencesKansas CityUSA
  2. 2.Department of RadiologyUniversity of Missouri-Kansas City, St. Luke’s HospitalKansas CityUSA
  3. 3.University of Missouri-Kansas City School of MedicineKansas CityUSA
  4. 4.Department of PathologyChildren’s Mercy Hospitals and ClinicsKansas CityUSA
  5. 5.Department of RadiologyUniversity of Missouri-Kansas CityKansas CityUSA
  6. 6.Department of RadiologyChildren’s Mercy Hospitals and ClinicsKansas CityUSA