Biliary Atresia

Condition Description

Biliary atresia (BIL-ee-ayr-ee ah-TREE-zee-uh) is the congenital absence or closure of the ducts that drain bile from the liver. One of the functions of the liver is to produce bile (made up of cholesterol, bile salts, and waste products) that drains from the liver into the small intestine where it helps digest food. Absence of the bile drainage system (called the hepatic system) or its disappearance is called biliary atresia.

With biliary atresia, a progressive inflammatory process begins in the liver soon after birth, typically first affecting the ducts outside the liver. Swelling and scarring of the drainage system traps the bile within the liver and this back-up then damages the liver cells very rapidly, resulting in liver cirrhosis (scarring and decreased function). Biliary atresia only affects newborns and is not hereditary, contagious, or preventable. A possible cause may be a virus that sets up an autoimmune reaction where the body's own defenses begin to damage its own cells.

Babies will appear normal at birth but develop jaundice (skin and sclera of eyes turn yellow), dark urine, and light-colored stools in the first week of life. Every baby jaundiced after 1 month of age should be evaluated for biliary atresia by testing the baby's blood. As the baby's bilirubin increases due to lack of drainage, the abdomen becomes very firm and enlarged due to the increasing liver size. As this occurs, the baby will become irritable and lose weight (although fluid buildup may mask this). This rare condition occurs in 1 of every 15,000 births, slightly more often in females. It is more common in Asian and black children. About 300 babies are born each year with biliary atresia in the US. 

Using data from Minnesota births between 20146-2020, we found 15 babies were born with biliary atresia, resulting in a rate of 0.5 babies per 10,000 births. Annually, about 3 babies are born with biliary atresia.

Parental education and support are essential, and local, regional, and national organizations may be very helpful.

Condition specific organizations

• American Liver Foundation
• Children's Liver Disease Foundation
• Medline Plus - biliary atresia
• National Organization for Rare Disorders (NORD) Rare Disease Database - Biliary Atresia