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Liver Disorders

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Extrahepatic Biliary Atresia (EHBA)

EHBA is a progressive congenital disorder that destroys the external bile duct structure of the liver, impairing normal bile flow (cholestasis). The condition affects one in 10,000 to 20,000 newborns and is the most frequent liver surgical disorder in infants. More common in females, its cause is unknown. Infants with EHBA typically appear healthy at birth, with normal development and weight gain. As EHBA grows worse, growth may be retarded.

While there are no specific diagnostic tests, EHBA in newborns is marked by jaundice lasting beyond two to six weeks, warranting suspicion of a metabolic, anatomic or infectious abnormality. In addition to persistent hyperbilirubinemia (elevated bilirubin in blood), clinical presentation includes greenish-hued skin, clay-colored stools and dark urine. Beyond two weeks, total bilirubin levels greater than 15 percent or direct bilirubin levels greater than 2 mg/dl should be evaluated immediately.

Prompt diagnosis, referral and surgical care increase the likelihood for long-term survival. Research shows surgery on or before two months of age re-establishes bile flow in about 90 percent of cases. Liver transplant is an option.