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Biliary Atresia

Overview

Biliary atresia is a liver-related condition in infants. It causes the bile ducts (tubes located inside and outside the liver) to become blocked. These tubes carry bile (a fluid that helps digestion) into the intestine. When these ducts become blocked, bile builds up in the liver and causes damage. Over time, biliary atresia can lead to decreased liver function and cirrhosis (severe liver scarring).

Symptoms of Biliary Atresia

By 3 to 6 weeks of age, babies with biliary atresia typically develop jaundice (a yellow tint to their skin and eye whites). The buildup of bile in the liver causes jaundice and generally is the only sign or symptom of biliary atresia.

When to see a doctor

If you notice your baby’s skin or eye whites becoming yellow in color, you should take them to see their doctor right away.

Causes of Biliary Atresia

The cause of biliary atresia is unknown, but it may be due to bile ducts that do not form properly during a baby’s development.

Who’s at risk

Female babies may have an increased risk for biliary atresia compared to male babies. Other risk factors include:

  • Asian or African American heritage

  • Maternal smoking during pregnancy

  • Preterm birth (before 36 weeks of gestation)

  • Poor maternal nutrition during pregnancy

Babies born with biliary atresia may also have other health problems that affect the heart, spleen, blood vessels or intestine. 

Diagnosis of Biliary Atresia

Your child’s pediatrician will begin checking for biliary atresia by completing a physical exam. They will ask questions about your child’s personal and family medical history. From there, they may order one or more of the following tests to confirm biliary atresia:

  • Blood test. The doctor may check a sample of your child’s blood for bilirubin (an orange-yellow pigment) and signs of liver disease.

  • Ultrasound. This imaging test uses sound waves to create detailed images of your child’s liver.

If biliary atresia is suspected, certain specialists may be involved, including gastroenterology and pediatric surgery. These teams may then perform one or both of the following more invasive tests:

  • Liver biopsy. The doctor may remove a small amount of tissue from your child’s liver (a biopsy) to check for signs of disease or damage

  • Cholangiogram. This test is typically done in the operating room by the surgeon by injecting dye into the bile ducts.

Treatments for Biliary Atresia

If your child has biliary atresia, the first treatment step is usually a surgery known as the Kasai procedure. During this procedure, your child’s surgeon removes their damaged bile ducts and replaces them with a loop of your child’s own small intestine. Sometimes, this procedure is enough to treat biliary atresia. In other cases, children with the condition will eventually need a liver transplant. During a liver transplant, the surgeon removes your child’s diseased liver and replaces it with a healthy donor liver.

Home remedies

Your child may need to make certain dietary adjustments prior to having the Kasai procedure or in the months following, including:

  • Eating a special formula, or adding supplements to pumped breast milk (for infants)

  • Having a feeding tube or intravenous (IV) nutrition supplement

  • Having a special eating plan

  • Taking special supplements

Contact Us

If you suspect that your child may have Biliary Atresia, make an appointment with an Orlando Health pediatrician today so your child can start on the road to improved health.