Biliary Atresia: A Rare Pediatric Liver Disease

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.**

Biliary atresia is the most common cause of chronic liver disease in neonates. It occurs once in every 10,000 to 20,000 births. Asian populations most frequently affected; African Americans are affected approximately twice as much as Caucasians.***

The cause of biliary atresia is not known. Some researchers and physicians believe that babies are born with biliary atresia, implying the problem with the bile ducts occurred during pregnancy while the liver was developing.

Others believe that the disease begins after birth, and may be caused by exposure to infections or exposures to toxic substances. Biliary atresia does not seem to be linked to medications the mother took, illnesses the mother had, or anything else the mother did during her pregnancy. Currently, there is not a genetic link known for biliary atresia. The disease is unlikely to occur more than once in a family.***

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:

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  • Dark urine

  • Enlarged spleen

  • Floating stools

  • Foul-smelling stools

  • Pale or clay-colored stools

  • Slow or no weight gain

  • Slow growth

General Stool Color Chart

Diagnosis

Tests to diagnose BA will include at least one of the following. Each center may have a preferred diagnostic method therefore it is best to discuss what works best for your child with your medical team. 
 

  • 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)**

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An infant undergoing a HIDA scan

Treatments:

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.*

For more information on the Kasai, please visit UPMC's Website.

A dissected liver after removal 

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The damaged liver after removal from an infant boy

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sources: *UPMC, ** Johns Hopkins, ***Lurie Children's Hospital Of Chicago