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Liver and intrahepatic bile ducts-nontumor

Developmental anomalies/cysts

Extrahepatic biliary atresia

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 23 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● 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)
● 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
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● 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
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Extrahepatic biliary atresia

Micro description
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● 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

Micro images
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Extrahepatic biliary atresia

Positive stains
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● CD56 (biliary epithelium stains strongly in >65% of portal tracts, Am J Surg Pathol 2003;27:1454)

Molecular description
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● 10% have mutations in Jagged 1 gene (associated with Alagille’s syndrome)

Differential diagnosis
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● 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
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● Pediatr Dev Pathol 2004;7:109, Pediatr Res 2004;56:79

End of Liver and intrahepatic bile ducts-nontumor > Developmental anomalies/cysts > Extrahepatic biliary atresia

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