Liver and intrahepatic bile ducts - nontumor
Developmental anomalies / cysts
Extrahepatic 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: Extrahepatic biliary atresia[TI] full text[sb] liver

Cite this page: Arora, K. Extrahepatic biliary atresia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverextrahepaticbiliaryatresia.html. Accessed November 23rd, 2017.
Definition / general
  • Most common cause of pathologic infant jaundice; common reason for pediatric liver transplantation
  • Stenotic or atretic portions of extrahepatic biliary tree cause chronic extrahepatic large duct obstruction
  • Histologically resembles choledochal cyst or other causes of large duct obstruction
  • Note: since biopsies have 7% false positive rate, radiographic studies are mandatory
  • Divided into fetal / embryonic and perinatal / acquired forms (eMedicine: Biliary Atresia Imaging [Accessed 26 October 2017])
  • Fetal / embryonic form: 10 - 35% of cases; symptoms start shortly after birth; 10 - 20% have congenital anomalies
  • Perinatal form: 65 - 90%; become jaundiced several weeks after birth; no associated congenital anomalies; characterized by a progressive inflammatory lesion, which suggests a role for infectious or toxic agents causing bile duct obliteration
Pathogenesis
  • Possible immune mechanism; distinctive portal tract inflammatory infiltrate involving CD4(+) Th1 cell mediated immunity
Treatment
  • Hepatoportenterotomy (Kasai procedure), liver transplant
  • To assess likelihood of success of surgical correction, surgeon may biopsy porta hepatis, pathologist indicates size and number of bile ducts present; also indicates amount of fibrosis and inflammation
Gross images

Images hosted on other servers:

Extrahepatic biliary atresia

Microscopic (histologic) description
  • Lobular cholestasis, portal neutrophilic infiltrate, bile ductular proliferation (peaks at 200 days), with elongated and angulated ductules and occasional bile plugs
  • Variable vacuoles and lymphocytes
  • May have focal giant cell transformation (periportal, not extensive), ductopenia (occurs rapidly at 400 days) and focal extramedullary hematopoiesis
  • Late: fibrosis or cirrhosis
Microscopic (histologic) images

Images hosted on other servers:

Extrahepatic biliary atresia

Positive stains
Molecular / cytogenetics description
  • 10% have mutations in Jagged1 gene (associated with Alagille syndrome)
Differential diagnosis