Pediatric and Developmental Pathology

, Volume 8, Issue 6, pp 725–731

Does ”Cystic” Biliary Atresia Represent a Distinct Clinical and Etiological Subgroup? A Series of Three Cases

  • Vera De Matos
  • Jessi Erlichman
  • Pierre A. Russo
  • Barbara A. Haber
Article

DOI: 10.1007/s10024-005-0018-7

Cite this article as:
De Matos, V., Erlichman, J., Russo, P.A. et al. Pediatr Dev Pathol (2005) 8: 725. doi:10.1007/s10024-005-0018-7

Abstract

We describe a unique series of 3 cases of biliary atresia (BA) associated with a choledochal cyst. All 3 children presented with jaundice at birth and had no other abnormalities. Although these children had a fetal form of BA, their presentation and outcome differed from those of biliary atresia splenic malformation syndrome (BASM), the well-described form of early onset BA. Unlike those with BASM, these children had no other associated malformations, had a normal birth weight, and did not yet require a liver transplant. We believe that the present series of patients and their associated pathology may represent a distinct phenotype with a common, prenatally acquired etiology that is different from other fetal forms of BA, such as BASM, and from patients who present with the classic perinatal form of BA.

Keywords

biliary atresia choledochal cyst neonatal cholestasis 

Copyright information

© Society for Pediatric Pathology 2005

Authors and Affiliations

  • Vera De Matos
    • 1
  • Jessi Erlichman
    • 1
  • Pierre A. Russo
    • 1
  • Barbara A. Haber
    • 1
  1. 1.Divisions of Gastroenterology and Nutrition and Anatomical PathologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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