Liver & intrahepatic bile ducts
Developmental anomalies / cysts
Extrahepatic biliary atresia
Author: Komal Arora, M.D.
Topic Completed: 1 April 2012
Minor changes: 16 December 2019
Copyright: 2002-2019, PathologyOutlines.com, Inc.
PubMed Search: Extrahepatic biliary atresia[TI] full text[sb] liver
Table of Contents
Definition / general | Pathogenesis | Treatment | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Arora K. Extrahepatic biliary atresia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverextrahepaticbiliaryatresia.html. Accessed September 21st, 2020.
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
- CD56 (biliary epithelium stains strongly in > 65% of portal tracts, Am J Surg Pathol 2003;27:1454)
Molecular / cytogenetics description
- 10% have mutations in Jagged1 gene (associated with Alagille syndrome)
Differential diagnosis
- Alpha-1-antitrypsin deficiency: PAS diastase visualizes AAT deposits in periportal hepatocytes
- Congenital cytomegalovirus infection
- Also neonatal hepatitis, total parental nutrition, obstruction by a choledochal cyst, arteriohepatic dysplasia, familial progressive intrahepatic cholestasis and alteration of the bile acid metabolism
Additional references
