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Extrahepatic bile ducts

Non tumor

Extrahepatic biliary atresia

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 15 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Acquired sclerosing inflammatory disorder that replaces bile ducts by threadlike cord embedded in fibrous tissue of porta hepatis
● Leads progressively to loss of intrahepatic ducts and biliary cirrhosis
● Most frequent extrahepatic cause of neonatal cholestasis, causes 1/3 of all neonatal cholestasis
● 1 per 10,000 live births worldwide, 70% girls, usually from uncomplicated pregnancies

Clinical features

● May be due to significantly shorter cilia with abnormal orientation (Mod Pathol 2012;25:751)
● Associated with cardiovascular defects and polysplenia (10-25%), small gallbladder
● Indication for 50% of pediatric liver transplants
● Most common cause of childhood death from liver disease


● Persistent conjugated hyperbilirubinemia


● Kasai procedure (portoenterostomy) before 10-12 weeks may be more helpful if hilar bile ductal structures are patent with lumina 1-4 mm or greater
● Frozen section useful to determine if bile ducts in hilum are present and what their caliber is
● Liver transplantation may be curative

Gross description

● Total or focal complete fibrous obliteration of major hepatic duct lumina or common bile duct

Micro description

● Obstructive changes with ductular proliferation, variable portal edema, lobular cholestasis
● Neutrophils common (highlighted by CAP37, Pathol Res Pract 2010;206:314)
● Variable multinucleated giant hepatocytes

● Ductopenia

Micro images

Various images

Positive stains

● CD56 (may be helpful to differentiate from other causes, Am J Surg Pathol 2003;27:1454)

End of Extrahepatic bile ducts > Non tumor > Extrahepatic biliary atresia

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