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Biliary atresia

What is biliary atresia?

The liver makes a substance called bile that helps with digestion of food. It also helps carry waste out through the liver’s bile ducts. In biliary atresia, some or all of the bile ducts are damaged or missing. This means the bile cannot drain properly from the liver. Symptoms include jaundice, malnutrition due to problems absorbing vitamins and minerals, pale stools, an enlarged spleen and scarring of the liver.

What causes biliary atresia?

Biliary atresia is a congenital condition; a child is born with it. The cause is unknown.

How is it diagnosed?

We take blood tests to check for signs of liver disease. We may obtain an abdominal ultrasound to look at the liver, gallbladder, bile ducts and blood vessels leading to the liver.

A HIDA scan checks the function of the bile ducts using a radioactive fluid that is put into the body. This fluid travels through the bile ducts into the intestines and can show if the bile ducts are missing or blocked.

A liver biopsy can detect signs of liver tissue damage. During this test, we use a small hollow needle to remove a small amount of liver tissue, which we study in a lab. Intraoperative cholangiogram may be needed if we cannot make a diagnosis based on the results of other tests. In this procedure, a surgeon makes an incision near the liver to see a video of the bile ducts, then injects contrast into them. This allows the surgeon to detect problems with the bile ducts.

How we treat Biliary atresia

We treat biliary atresia with the Kasai procedure, during which we remove abnormal bile ducts. We attach the small intestine directly to any working bile ducts, which allows bile to drain into the intestine. It is best for the surgery to be done as soon after diagnosis as possible. Outcomes are better the younger your child is when we perform the surgery. 

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