Biliary atresia

Definition

Biliary atresia is a blockage in the tubes (ducts) that carry a liquid called bile from the liver to the gallbladder. The condition is type of birth defect (congenital condition).

Causes

Biliary atresia occurs when the bile ducts inside or outside the liver do not develop normally. It is not known why the biliary system fails to develop normally.

The bile ducts help remove waste from the liver and carry salts that help the small intestine break down (digest) fat.

In babies with biliary atresia, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver

Newborns with this condition may appear normal at birth. However, jaundice develops by the second or third week of life. The infant may gain weight normally for the first month, but then will lose weight and become irritable, and have worsening jaundice.

Symptoms

• Jaundice, a yellow color of the skin and eyes
  • May be seen at birth or develop over several months
  • May get worse (progress) or come and go (intermittent)
• Slow or no weight gain
• Slow growth in a child 0 - 5 years old
• Irritability
• Dark urine
• Splenomegaly (enlarged spleen)
• Pale or clay-colored
• Floating stools
• Foul-smelling stools

Exams and Tests

The health care provider will perform a physical exam, which includes feeling the patients belly area. The doctor may feel an enlarged liver.

Tests to diagnose biliary atresia include:

• Blood bilirubin level -- usually increased
• Abdominal x-ray
• Abdominal ultrasound
• Hepatobiliary iminodiacetic acid (HIDA) scan, also called cholescintigraphy -- helps tell if the bile ducts and gallbladder are working properly
• Cholangiogram, an x-ray of the bile ducts
• Liver biopsy to determine degree of cirrhosis or to rule out other causes of jaundice

Treatment

An operation called the Kasai procedure is done to connect the liver to the small intestine, going around the abnormal ducts. It is most successful if done before the baby is 10 - 12 weeks old. However, a liver transplant may still be needed.

Outlook (Prognosis)

Early surgery will improve the survival of more than a third of babies with this condition. The long-term benefit of liver transplant is not yet known, but is expected to improve survival.

Possible Complications

• Liver failure
• Infection
• Surgical complications
• Failure of the Kasai procedure
• Irreversible cirrhosis

When to Contact a Medical Professional

Call your health care provider if your child appears jaundiced, or if other symptoms suggestive of this disorder develop.