Definition

Biliary atresia is a blockage of bile ducts in infants. The bile ducts are tubes that let a fluid called bile pass from the liver to the gallbladder. Bile is made in the liver, passes to the gallbladder for storage, then moves to the intestine for use.

Bile breaks down the fat in food so it can be absorbed by the body. It also carries waste and toxins out of the body. If bile cannot pass out of the liver, the bile builds up and damages the liver. Biliary atresia is a life-threatening condition.

Causes

The true cause of biliary atresia is not completely clear. For some, the ducts do not develop properly and the tubes are closed off. A blockage may also develop caused by swelling of the ducts before or after birth. Swelling may be caused by:

• A viral or bacterial infection after birth
• Problem with the immune system
• Exposure to toxic substances

It is not believed that biliary atresia is caused by a genetic issue that is passed on from parents.

Risk Factors

Biliary atresia is more common in female infants, though it can occur in males. It is also more common in premature infants and children of Asian or African American descent.

Symptoms

Jaundice is the yellowing of the skin and whites of the eyes caused by elevated bilirubin. Elevated bilirubin is caused by a backup of bile in the liver. It may be present soon after birth. Most other symptoms will be present in the first 2-4 weeks of life. A few symptoms may not occur for 3 months or longer. Symptoms may include:

• Dark urine
• Gray or white stools—caused by lack of bile
• Swollen abdomen
• Poor weight gain and growth
• Irritability
• Bleeding due to vitamin deficiencies

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Diagnosis

You will be asked about your child’s symptoms and medical history. A physical exam will be done. Other conditions can cause similar symptoms. An infant with signs of liver problems will be referred to a specialist.

A diagnosis of biliary atresia will be made based on the results of multiple tests including:

• Blood test—to detect high levels of a certain type of bilirubin or liver damage
• Ultrasound —to look at ducts for enlargement or reason for blockage
• Hepatobiliary scintigraphy —to look at bile flow in the ducts

Your child’s liver may need to be tested and/or viewed. This can be done with:

• Diagnostic surgery (intraoperative cholangiogram) to view the liver and ducts—repairs will be made during this surgery if blockage is found
• Liver biopsy —liver tissue is taken and examined under a microscope to confirm diagnosis

Treatment

Prevention

There are no current guidelines to prevent biliary atresia.