Biliary Atresia

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KEY POINTS

• Biliary atresia is a rare, life-threatening condition that can affect babies soon after birth. It means that there is a blockage in the small tubes going from the liver to the small intestine. Blockage of the ducts can damage the liver.
• Biliary atresia is treated with surgery, and usually a liver transplant.
• Follow your child’s healthcare provider's instructions. Ask how to take care of your child at home.

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What is biliary atresia?

Biliary atresia is a blockage in the small tubes (ducts) that carry bile from the liver to the small intestine. Bile is a fluid that helps the body break down the fat in food. Blockage of the ducts can damage the liver. If this disease is not treated, the liver will stop working.

This is a rare, life-threatening condition that can affect babies soon after birth.

What is the cause?

The cause of biliary atresia is not known. 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 damages the ducts.

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?

Biliary atresia is usually diagnosed during the first 2 months of life. Tests may include:

• Blood and urine tests
• Tests of bowel movement
• An ultrasound, which uses sound waves to show pictures of the liver and bile ducts
• Nuclear scan, which uses a tiny amount of radioactive chemical injected into a vein to show how well bile flows from the liver to the small intestine
• Liver biopsy, which is the removal of a small sample of tissue for testing

How is it treated?

Biliary atresia can be treated with 2 types of surgery:

• Kasai procedure: The damaged ducts are removed and replaced with a loop of intestine so the bile can flow from the liver into the intestine.
• Liver transplant: The baby is given a liver from another baby who has died or part of a liver from an adult.

Even after a successful Kasai procedure, most babies with biliary atresia slowly develop scarring of the liver (cirrhosis) and need a liver transplant by adulthood.

Your child’s healthcare provider may recommend changes in your baby’s diet or vitamin supplements.

How can I take care of my child?

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

• How and when you will get 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

Make sure you know when your child should come back for a checkup. Keep all appointments for provider visits or tests.