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, which are common biliary atresia symptoms.

Other symptoms may include:

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

Diagnosis

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

• Abdominal ultrasound
• Blood tests to check total and direct bilirubin levels
• A hepatobiliary iminodiacetic acid (HIDA) scan, also called cholescintigraphy, is used to help determine if the bile ducts and gallbladder are working properly
• Liver biopsy to diagnose biliary atresia, to determine the 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 % of babies with Biliary Atresia. The child required to be supplemented with vitamins and nutrition.

FAQs

1. What are the common symptoms of biliary atresia in infants?

Symptoms include yellowing of the skin and eyes (jaundice), dark urine, pale stools, poor weight gain, and an enlarged liver.

2. How is biliary atresia diagnosed at an early stage?

Doctors use blood tests to check liver function, ultrasound or imaging to look at the bile ducts, and sometimes a liver biopsy to confirm the diagnosis.

3. What treatments are available for biliary atresia?

Treatment may include the Kasai surgery to restore bile flow or, in some cases, a liver transplant if needed.

4. Can surgery help children with biliary atresia?

Yes. Early surgery can improve bile flow, reduce jaundice, and prevent liver damage. The Kasai procedure is most effective when done within the first few months of life.

5. What is the long-term outlook for biliary atresia patients?

With early treatment and regular follow-up, many children can grow well and maintain good liver function. Some may still need a liver transplant later, but overall outcomes are much better with timely care.