Biliary Atresia Clinical Care Program

About Biliary Atresia

Biliary atresia is a serious liver disorder that occurs before or shortly after birth when a baby's bile ducts (the tubes that carry bile from the liver) become blocked. The body needs bile to aid digestion and carry wastes from the liver out of the body. When blocked ducts prevent bile from being excreted, the liver becomes damaged.

Causes and forms

The cause of biliary atresia is a mystery. Two forms of biliary atresia are generally recognized, although more may exist.

Perinatal biliary atresia

The most common is the perinatal form which accounts for 65-90 percent of all cases. These children typically appear healthy at birth. As a newborn, these infants pass normal-colored stool and are of average weight. Between 4-8 weeks of age however, jaundice develops.

Embryonic/fetal biliary atresia

The less-common embryonic or fetal form occurs in 10-35 percent of biliary atresia cases. These children are jaundiced as newborns and frequently there are associated birth defects such as abnormalities of the heart, major blood vessels, spleen and intestines.

Neither form is thought to be inherited. Many theories about the causes of biliary atresia have been proposed, but none have been proven.

For more information

Learn more about the liver, diagnosis of biliary atresia, treatment options and the long-term outlook for children with the condition. A glossary of terms and frequently asked questions about biliary atresia are also available.

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