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Biliary Atresia

What is biliary atresia?

Biliary atresia is a rare disease that can affect babies soon after birth. This disease destroys the ducts that carry bile from the liver to the small intestine. Bile is a fluid that helps the body digest fats. It is made in the liver and stored in the gallbladder. If the ducts cannot carry bile, the liver gets damaged and stops working. If this disease is not treated, children may not live longer than 2 years.

What is the cause?

The cause of biliary atresia is not known. It is not contagious, so it cannot be passed from one child to another. Some possible causes may be:

  • The bile ducts do not form properly before birth.
  • A viral infection or problem with the body's immune system after birth

What are the symptoms?

Symptoms may include:

  • Yellowing of the skin and eyes (jaundice) after 2 weeks of age that does not go away by 4 weeks of age
  • Dark urine
  • Pale, clay-colored bowel movements
  • Swollen belly

How is it diagnosed?

Several tests may be done to make a diagnosis:

  • Blood and urine tests
  • Tests of bowel movement
  • An ultrasound, which uses sound waves to show pictures of the liver and bile ducts
  • Liver scan, which uses a small amount of a radioactive chemical and a special camera to look at the liver
  • HIDA scan, which uses a tiny amount of radioactive chemical and a scanner to look at the liver and bile ducts
  • Liver biopsy, which is the removal of a small sample of tissue for testing

Biliary atresia is usually diagnosed during the first 2 months of life.

How is it treated?

Treatment is needed to get the bile flowing through the ducts. The most common treatment is an operation called the Kasai procedure. During this surgery, the damaged ducts are removed and replaced with a piece of the baby's intestine. The new ducts allow bile to drain from the liver into the intestine.

Babies usually stay in the hospital for 7 to 10 days after the surgery. The baby will be given antibiotic medicine to lower the risk of infection and other medicine to keep fluid from building up in the belly.

Sometimes the baby will need a liver transplant. Almost all children who have biliary atresia will need to have a liver transplant before they are 20 years old.

How can I take care of my child?

Follow your child’s healthcare provider's instructions. Ask your provider:

  • How and when you will hear your child’s test results
  • How long it will take for your child to recover
  • How to take care of your child at home
  • What symptoms or problems you should watch for and what to do if your child has them
  • If your child needs to take vitamin supplements or have changes in diet

Make sure you know when your child should come back for a checkup.

Developed by RelayHealth.
Pediatric Advisor 2013.2 published by RelayHealth.
Last modified: 2013-03-28
Last reviewed: 2013-02-07
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
© 2013 RelayHealth and/or its affiliates. All rights reserved.
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