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Biliary atresia

What is Biliary Atresia?

Biliary atresia is a blockage in the tubes (ducts) that carry bile from the liver to the gallbladder. This congenital condition 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. 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, 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. 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
  • Enlarged spleen
  • Floating stools
  • Foul-smelling stools
  • Pale or clay-colored stools
  • Slow or no weight gain
  • Slow growth

Diagnosis

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

  • Abdominal x-ray
  • 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 determine severity of cirrhosis or to rule out other causes of jaundice
  • X-ray of the bile ducts (cholangiogram)

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 8 weeks old. However, a liver transplant may still be needed. The North American Society of Pediatric Gastroenterology, Hepatology and Nutrition has more information on treatment and procedures for biliary atresia.

Biliary atresia is treated in our Division of Pediatric Gastroenterology and Nutrition and its Pediatric Liver Center. Please visit the CHILDREN Network page to see ongoing studies related to biliary atresia.

Related Information:

External Links:

National Library of Medicine 

North American Society of Pediatric Gastroenterology, Hepatology and Nutrition 


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