Extrahepatic bile ducts
Nontumor
Extrahepatic biliary atresia

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 7 February 2018, last major update September 2012

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Extrahepatic biliary atresia[TI] full text[sb]

Cite this page: Gulwani, H. Extrahepatic biliary atresia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/gallbladderextrahepaticbiliaryatresia.html. Accessed June 18th, 2018.
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

Images hosted on other servers:

Various images

Positive stains