Biliary Atresia

Biliary atresia is a condition in infants in which the bile ducts outside and inside the liver are scarred and blocked. Bile can’t flow into the intestine, so bile builds up in the liver and damages it. The damage leads to scarring, loss of liver tissue and function, and cirrhosis.

Typically, the first sign of biliary atresia is yellowing of the skin and whites of the eyes, called jaundice, which results from the buildup of bile in the body. Infants with biliary atresia typically develop jaundice by 3 to 6 weeks of age. Experts don’t know what causes biliary atresia.

To diagnose biliary atresia, doctors will ask about the infant’s medical and family history, perform a physical exam, and order a series of tests. If test results suggest that an infant is likely to have biliary atresia, the next step is surgery to confirm the diagnosis.

Doctors treat biliary atresia with a surgery called the Kasai procedure and eventually, in most cases, a liver transplant. Thanks to advances in treatment, more than 80 to 90 percent of infants with biliary atresia survive to adulthood.

Children with biliary atresia may have reduced bile flow to the small intestine and liver damage, which may lead to malnutrition. To make sure infants and children with biliary atresia get enough nutrients and calories, doctors may recommend a special eating plan and supplements.

The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other components of the National Institutes of Health (NIH) conduct and support research into many diseases and conditions.