What is Biliary Atresia?

Biliary atresia is a blockage in the tubes (ducts) that carry bile from the liver to the gallbladder. It is not known why the biliary system fails to develop normally.  This can lead to liver damage and cirrhosis of the liver, which is deadly if not treated.

Symptoms

Newborns with this condition may appear normal at birth. However, jaundice develops by the second or third week of life or Persistence of Neonatal Jaundice beyond 2 weeks in term and 3 weeks in preterm neonate. The infant may gain weight normally for the first month, but then will lose weight and become irritable, and have worsening jaundice. 

Other symptoms may include:

• Dark urine
• Pale or clay-colored stools
• Enlarged spleen
• Slow or no weight gain
• Slow growth

Diagnosis

Physical exam is performed to feel for an enlarged liver. Other tests include:

• Abdominal ultrasound
• Blood tests to check total and direct bilirubin levels
• Hepatobiliary iminodiacetic acid (HIDA) scan, also called cholescintigraphy, to help determine if the bile ducts and gallbladder are working properly
• Liver biopsy to diagnose biliary atresia, to determine severity of cirrhosis or to rule out other causes of jaundice
• X-ray of the bile ducts (Operative Cholangiogram)

Treatment

An operation called the Kasai Porto Enterostomy procedure is done to connect the liver to the small intestine. It is most successful if done before the baby is 8 weeks old. However, a liver transplant may still be needed in 30 to 60 % babies with Biliary Atresia. Child required to be supplemented with vitamins and nutrition.