Liver & intrahepatic bile ducts
Developmental anomalies / cysts
Intrahepatic biliary atresia
Author: Komal Arora, M.D.
Last author update: 1 April 2012
Last staff update: 28 January 2025 (update in progress)
Copyright: 2002-2025, PathologyOutlines.com, Inc.
PubMed Search: Intrahepatic biliary atresia
Table of Contents
Definition / general | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Arora K. Intrahepatic biliary atresia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/liverintrahepaticbiliaryatresia.html. Accessed April 2nd, 2025.
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 versus 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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Liver biopsy
