Biliary atresia, extrahepatic is an uncommon gastrointestinal illness marked by the damage or absence of all or part of the bile duct outside the liver (extrahepatic bile duct). The bile duct makes a connection to the liver towards the gallbladder, which then connects to the small intestine.

Bile is a liquid generated by the liver responsible for transporting waste products from the liver and facilitating fat and vitamin absorption by the intestines. Because bile ducts are lacking or disrupted in biliary atresia, bile accumulates abnormally in the liver. Affected infants’ skin and the whites of their eyes turn yellowish (jaundice), and they develop liver scars (fibrosis). Additional abnormalities, such as heart problems and intestine, spleen, and renal anomalies, may be present in rare cases. Furthermore, there is no known cause for biliary atresia.


Biliary atresia, extrahepatic symptoms commonly emerge between the ages of two and six weeks. Typical symptoms include:

  • Jaundice
  • Pale stools
  • Dark urine
  • Swollen stomach
  • Abnormal enlargement of the liver

Additional symptoms may appear between the ages of six and ten weeks. Some signs are:

  • Increase blood pressure
  • Poor weight gain

Intrahepatic bile ducts (bile ducts inside the liver) are also affected. If neglected, biliary atresia can lead to liver scarring (cirrhosis) and liver failure (hepatic failure).


Biliary atresia can only be diagnosed by direct testing of the bile ducts. These examinations include the following:

Viruses can be detected using blood testing, which reveals elevated levels of liver enzymes, bilirubin, and gamma-glutamyl transpeptidase.


Although there is no cure for biliary atresia, extrahepatic, prompt diagnosis, and surgical intervention enhance short- and long-term results in most patients. Special attention to nutritional needs and food is required for children with this disease. Formulas, supplements, and dietary restrictions may be required for affected newborns. 

The following therapies are also necessary:

  • Antibiotics. This could be used to treat bile duct infections (cholangitis).
  • Surgery. Surgery is required to eliminate the obstruction and enable bile to keep flowing into the bile ducts and small intestine.
  • Genetic Counseling. People with biliary atresia, extrahepatic, and their relatives may benefit from this. The only other option is supportive care therapy.

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