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Biliary Atresia

What is Biliary Atresia?

Biliary atresia occurs when the bile ducts, responsible for carrying bile from the liver to the gallbladder, do not develop properly. This blockage prevents the normal flow of bile, leading to liver damage and potentially life-threatening cirrhosis if left untreated.  While the exact cause of biliary atresia is still unknown, researchers believe it may either originate during pregnancy or shortly after birth. There is no evidence suggesting that maternal medication, illnesses, or lifestyle choices contribute to the development of this condition.​​ It's important to note that biliary atresia is not hereditary and typically does not recur within families. It is most commonly found in Asian populations, although African Americans are affected at higher rates than Caucasians. As a parent of a child with biliary atresia, early diagnosis and appropriate treatment are crucial. Consult with your healthcare provider to discuss available options and develop a comprehensive care plan for your child's well-being. Remember, you are not alone in this journey. There are medical professionals and support networks ready to assist you every step of the way. Stay strong, stay informed, and together, we can navigate through this challenging time.

Biliary Atresia: A Rare Pediatric Liver Disease
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Symptoms

Biliary atresia is a condition that may not show immediate signs in newborns. However, by the second or third week of life, jaundice may develop. In the first month, the baby may gain weight normally, but thereafter, weight loss, irritability, and worsening jaundice may occur.

 

Additional symptoms may include:

  • Dark urine

  • Enlarged spleen

  • Floating stools

  • Foul-smelling stools

  • Pale or clay-colored stools

  • Slow or no weight gain

  • Slow growth

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Stool Color Chart (Perinatal Services BC)

Image of an infant undergoing a HIDA scan.

Image of an infant undergoing a HIDA scan.

Diagnosis:

As a parent who has just received a diagnosis of biliary atresia for your child, it's important to understand the tests used to confirm this condition. Each medical center may have its own preferred diagnostic method, so it's advisable to discuss with your medical team what would work best for your child. ​

  • Blood tests: Measure bilirubin levels in the blood to determine jaundice severity and liver function.

  • Abdominal ultrasound ​

  • Liver biopsy: Analyze a small sample of liver tissue to assess cirrhosis severity or rule out other causes of jaundice

    Other tests may include: 

  • Hepatobiliary iminodiacetic acid (HIDA) scan: Evaluate bile duct and gallbladder function to diagnose biliary atresia.

  • Cholangiogram: Use x-rays and contrast dye to visualize bile ducts for detecting obstructions or abnormalities.

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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. 

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Damaged liver after removal from an infant.

Dissected liver after removal.

Sources

Biliary Atresia. (2019, September 19). Johns Hopkins Medicine. Retrieved October 23, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-diseases/biliary-atresia

Conditions - Biliary Atresia. (n.d.). Lurie Children's. Retrieved October 23, 2023, from https://www.luriechildrens.org/en/specialties-conditions/biliary-atresia/

Biliary Atresia. (n.d.). Perinatal Services BC. Retrieved October 23, 2023, from http://www.perinatalservicesbc.ca/health-professionals/professional-resources/screening/biliary-atresia

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