Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy

Fetal Pediatr Pathol. 2019 Dec;38(6):477-483. doi: 10.1080/15513815.2019.1627621. Epub 2019 Jun 17.

Abstract

Introduction: Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy. Methods: We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia. Conclusion: The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.

Keywords: Choledochal cyst; atretic choledochal cyst; biliary atresia; cystic biliary atresia; neonatal cholestasis; obstructive cholangiopathy.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Insufficiency / diagnosis
  • Adrenal Insufficiency / pathology*
  • Aged
  • Biliary Atresia / diagnosis
  • Biliary Atresia / pathology*
  • Choledochal Cyst / diagnosis
  • Choledochal Cyst / pathology*
  • Diagnosis, Differential
  • Female
  • Fetal Growth Retardation / diagnosis
  • Fetal Growth Retardation / pathology*
  • Gallbladder / pathology*
  • Humans
  • Liver / pathology
  • Osteochondrodysplasias / diagnosis
  • Osteochondrodysplasias / pathology*
  • Urogenital Abnormalities / diagnosis
  • Urogenital Abnormalities / pathology*

Supplementary concepts

  • Intrauterine Growth Retardation, Metaphyseal Dysplasia, Adrenal Hypoplasia Congenita, And Genital Anomalies