Biliary tract malformations

Abstract

The biliary tree extends from the canals of Hering at the margin of the most peripheral portal tracts to the ampulla of Vater. Malformations occur at every level of this structure. Phenotypic features dominate present understanding of these malformations and of the disorders with which they are associated. Classifications of disease will likely shift from a phenotypic basis to a genotypic basis as genes implicated in biliary tree development and function are identified. Involvement of such genes in biliary tree disorders now considered inflammatory, such as extrahepatic biliary atresia, awaits study. The concept of "feeble cholangiocytes" postnatally susceptible to the effects of "toxic bile" is presented.