Paucity of intrahepatic bile ducts

Abstract

Paucity of intrahepatic bile ducts is an uncommon syndrome that products cholestasis in neonatal or toddler age. It is defined as a reduction in the number of interlobular bile ducts using the ratio of the number of portal tracts devoid of bile ducts to the total number of portal tracts. We report the case of a two months old boy admitted in our hospital by prolonged jaundice and very large spontaneous blood tumor in chest wall. We present the differencial diagnosis whith others syndromes whith similar syntomatology and we describe the evolution and the recents advances in treatment.