Gallbladder & extrahepatic bile ducts

Extrahepatic bile duct nonneoplastic

Extrahepatic biliary atresia



Last author update: 1 September 2012
Last staff update: 22 June 2022

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PubMed Search: Extrahepatic biliary atresia

Hanni Gulwani, M.B.B.S.
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Cite this page: Gulwani H. Extrahepatic biliary atresia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/gallbladderextrahepaticbiliaryatresia.html. Accessed July 5th, 2022.
Definition / general
  • Acquired sclerosing inflammatory disorder that replaces bile ducts by thread-like 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
Laboratory
  • Persistent conjugated hyperbilirubinemia
Treatment
  • 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
Microscopic (histologic) description
  • Early:
    • 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
  • Late:
    • Ductopenia
Microscopic (histologic) images

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Ballooning and feathery degeneration

Large areas of bile lake formation

Formation of hepatic nodules

Extrahepatic biliary obstruction

Positive stains
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