Biliary Atresia—Infant

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 leave the liver, it builds up and damages it.

Biliary atresia is a rare, life-threatening condition.

Causes

The cause of biliary atresia is not clear. For some infants, the ducts do not form properly and are closed off. The ducts may also swell and make a blockage. 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
• Being around 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 health issue.

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. A baby may have:

• Jaundice—yellowing of the skin and eyes
• Dark urine (pee)
• Gray or white stools—these are caused by lack of bile
• Swollen belly
• Poor weight gain and growth
• Irritability
• Bleeding due to not getting enough vitamins

Baby with Jaundice.

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Diagnosis

The doctor will ask about the infant’s symptoms and health history. A physical exam will be done. Caregivers may be asked to take the infant to see a doctor who focuses on liver problems.

The diagnosis is based on tests such as:

• Blood tests
• Ultrasound—to look at the 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 get the bile flowing 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—this is a life-saving surgery to help bile flow

A liver transplant will be needed in the first few years of life if the Kasai procedure does not work.

Sometimes the liver gets badly damaged even if the Kasai procedure works. This is called cirrhosis. A liver transplant may be needed later in life if the infant has cirrhosis.

Prevention

Biliary atresia cannot be prevented.