Liver and intrahepatic bile ducts - nontumor
Developmental anomalies / cysts
Intrahepatic biliary atresia

Author: Komal Arora, M.D. (see Authors page)

Revised: 26 October 2017, last major update April 2012

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PubMed Search: Intrahepatic biliary atresia[TIAB]

Cite this page: Arora, K. Intrahepatic biliary atresia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverintrahepaticbiliaryatresia.html. Accessed December 11th, 2017.
Definition / general
  • Also called paucity of intrahepatic bile ducts in neonates
  • Occurs in syndromic form (Alagille syndrome) or nonsyndromic form (Eur J Hum Genet 2012;20:251)
  • In nonsyndromic form, bile duct loss is present from birth
  • May actually represent cystic fibrosis, alpha-1-antitrypsin deficiency, trihydroxycoprostanic acidemia
  • Bile duct recovery depends on etiology
Microscopic (histologic) description
  • Loss of interlobular bile ducts (0.5 ducts/portal tract vs. normal 0.9 - 1.8 ducts/portal tract)
  • Count only bile ducts accompanying hepatic arterioles in center of portal tracts, keratin staining may be helpful
  • Usually minimal ductular proliferation
  • No / minimal fibrosis, no / minimal inflammation
Microscopic (histologic) images

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Various images

Alagille syndrome with bile duct paucity