Portal Tract A portal tract in an infant with nonsyndromic paucity of intrahepatic bile ducts shows arteries and veins but no bile ducts. The hepatocytes are compact and clustered.
Centrilobular Region The centrilobular region in nonsyndromic bile duct paucity shows cholestasis and giant cell transformation of hepatocytes , nonspecific features of many neonatal cholestatic disorders.
Portal Tract Another portal tract in an infant with nonsyndromic paucity of intrahepatic bile ducts shows arteries and veins but no bile ducts. The hepatocytes are compact and clustered.
Higher Magnification Higher magnification of the centrilobular region in nonsyndromic bile duct paucity shows cholestasis and giant cell transformation of hepatocytes, nonspecific features of many neonatal cholestatic disorders.
TERMINOLOGY
Synonyms
• Hypoplasia of intrahepatic bile ducts
• Intrahepatic biliary atresia
Definitions
• Heterogeneous group of disorders that cause bile duct paucity or ductopenia in patients without congenital abnormalities indicative of Alagille syndrome
Ductopenia defined as significant decrease in number of interlobular bile ducts
– Ratio of number of interlobular bile ducts to number of portal tracts is < 0.4, with normal between 0.9 and 1.8
– Alternatively, ductopenia is diagnosed when > 50% of portal tracts lack bile ducts; normally, 80-100% of portal tracts contain ducts