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Biliary Atresia, Pediatric

Sina Ogholikhan, MD and Douglas B. Mogul, MD, MPH Reviewed 10/2018
 


BASICS

DESCRIPTION

Biliary atresia (BA) is a congenital disease characterized by fibrosis, obstruction, and obliteration of the biliary system that is universally fatal without intervention. 

EPIDEMIOLOGY

DIAGNOSIS

HISTORY

  • Newborns appear healthy at birth with good growth and development. However, jaundice of the skin and eyes persists beyond the 2-week interval of physiologic jaundice.

  • Acholic stools an...

TREATMENT

SURGERY/OTHER PROCEDURES

  • A hepatoportoenterostomy (Kasai procedure)

    • The Kasai procedure is the only effective therapy for BA other than a liver transplant.

    • The goal of the Kasai procedure is to...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • In conjunction with a GI specialist, obtain routine serum biomarkers including CBC, liver function tests, and GGT to determine progression of disease.

  • Physicians s...

ADDITIONAL READING

  • Caudle SE, Katzenstein JM, Karpen SJ, et al. Language and motor skills are impaired in infants with biliary atresia before transplantation. J Pediatr.  2010;156(6):936–940.e1. ...

CODES

ICD9

  • 751.61 Biliary atresia

  • 576.8 Other specified disorders of biliary tract

  • 789.1 Hepatomegaly

  • 789.2 Splenomegaly

ICD10

  • K83.1 Obstruction of bile duct

  • Q44.2 Atresia of bile ducts

  • R16.0 Hepatomegaly, no...

FAQ

  • Q: How soon should parents contact their primary care provider when they see pale-colored stools?

  • A: The first time they notice these stools are acholic.

  • Q: What should a physician do if he or she i...

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