Definition

Biliary atresia is a blockage of bile ducts in infants. Bile ducts are tubes that pass a fluid called bile from the liver to the gallbladder.

Bile helps with digestion. It also carries waste and toxins out of the body. When bile cannot pass out of the liver, it builds up and damages the liver.

Biliary atresia is a rare, life-threatening condition.

Causes

The cause of biliary atresia is not clear. For some, the ducts do not form properly and are closed off. A blockage may also develop from swelling of the ducts. This may happen before or after birth. The swelling may be due to:

• An infection after birth—due to a virus or bacteria
• A problem with the immune system
• Exposure to toxic substances

Risk Factors

Biliary atresia is more common in female infants, but it also occurs in males. It is also more common in premature infants and children from Asian and Pacific regions.

Changes in certain genes may play a role in this condition.

Symptoms

Most symptoms of biliary atresia appear in the first 2 to 4 weeks of life. A few symptoms may not occur for 3 months or longer. Symptoms may be:

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

Baby with Jaundice Copyright © Nucleus Medical Media, Inc.

Diagnosis

The doctor will ask about the infant’s symptoms and health history. A physical exam will be done. The infant may be sent to a specialist.

The diagnosis is based on tests, such as:

• Blood tests
• Ultrasound—to look at ducts
• Hepatobiliary scintigraphy—to look at bile flow in the ducts

Other tests may be:

• Intraoperative cholangiogram—surgery to view the liver and ducts and repair blockages
• Liver biopsy —a sample of liver tissue is taken and tested

Treatment

The goal of treatment is to restore bile flow and manage symptoms. Options are:

• A special diet with:
  • Extra calories
  • Extra vitamins and minerals
  • A special type of fat
• Medicines to:
  • Reduce itching and swelling
  • Increase bile flow
• The Kasai procedure—life-saving surgery to help bile flow

If the Kasai procedure is not successful, a liver transplant will be needed in the first few years of life.

Even if the Kasai procedure is successful, sometimes the liver gets badly damaged. This is called cirrhosis. In this case, a liver transplant may be needed later in life.

Prevention

There are no known guidelines to prevent biliary atresia.