Search
biliary atresia
Classifiaction:
- type 1:
- obliteration of the common bile duct
- proximal bile ducts are patent
- type 2:
- atresia of the hepatic duct, with cystic structures found in the porta hepatis
- type 3: (>90% of patients)
- atresia of the right & left hepatic ducts to the level of the porta hepatis
Epidemiology:
- 1 per 10,000-15,000 live births
- most common surgically treatable cause of cholestasis encountered during the newborn period
- highest in Asian populations, black:white ratio 2:1
Pathology:
- obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction to bile flow
Laboratory:
- serum bilirubin (total and direct):
- conjugated hyperbilirubinemia
- hepatobiliary function tests
- serum alkaline phosphatase
- serum 5' nucleotidase
- serum gamma-glutamyl transpeptidase
- serum ALT, serum AST
- serum bile acids
- serum alpha1-antitrypsin with Pi typing:
- alpha1-antitrypsin deficiency is the most common inherited liver disease that presents with neonatal cholestasis
- sweat chloride:
- biliary tract involvement is a well-recognized complication of cystic fibrosis
Special laboratory:
- cholangiography
Differential diagnosis:
- intrahepatic biliary hypoplasia
- Alagille syndrome
- hemochromatosis
- Caroli disease
- Herpes simplex
- cholestasis
- lipid storage disorders
- cystic fibrosis
- rubella
- cytomegalovirus
- syphilis
- galactosemia
- toxoplasmosis
Complications:
- biliary cirrhosis, hepatocellular carcinoma
Management:
- surgical correction
General
atresia (atretic, atresic, imperforate)
biliary disease
References
- Schwarz SM
eMedicine: Biliary Atresia
http://emedicine.medscape.com/article/927029-overview
- MedlinePlus: Biliary atresia
http://www.nlm.nih.gov/medlineplus/ency/article/001145.htm