Biliary atresia is a rare condition in newborn children in which the common bile duct between the liver and the small intestine is blocked or absent. If unrecognised, the condition leads to liver failure but not (as one might think) to kernicterus. It has no known cause, and the only effective treatment is by surgery.

Symptoms and diagnosis

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Initially, the symptoms are indistinguishable from neonatal jaundice, a common phenomenon. Prolonged jaundice that is resistant to phototherapy and/or exchange transfusions should prompt a search for secondary causes. By this time, liver enzymes are generally measured, and these tend to be grossly deranged, hyperbilirubinaemia is conjugated and therefore does not lead to kernicterus. Ultrasound investigation or other forms of imaging can confirm the diagnosis.

Biliary atresia is a rare disorder. About one in 15,000 to 20,000 babies do not have complete bile ducts. There is no known genetic link. Biliary atresia seems to affect girls more than boys. Within the same family, it is common for only one child in a pair of twins or only one child within the same family to have it. Asians and African-Americans are affected more frequently than Caucasians. There does not appear to be any link to medications taken during pregnancy.

Pathophysiology

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There is no known cause of biliary atresia, although it may be associated with a number of rare syndromes, such as malrotation of the intestine.

As the biliary tract cannot transport bile to the intestine, bile is retained in the liver and results in damage and the ultimate destruction of that organ.

Treatment

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If the intrahepatic biliary tree is unaffected, surgical reconstruction of the extrahepatic biliary tract is possible. This surgery is called a Kasai portoenterostomy.

If the atresia is complete, only liver transplantation is a therapeutic option.

External links

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• Intro. to pediatric blood tests for liver function
• Biliary Atresia, Liver Diseases and Treatments, Cincinnati Children's Hospital Medical Center
• Biliary Atresia Research Consortium (U.S.)
• Children's Liver Disease Foundation (U.K.)
• European Biliary Atresia Registry
• Imagine Bright Futures - Musings and research on biliary atresia (the leading cause of pediatric liver transplants).

Research links

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• Choledochal cyst associated with extrahepatic bile duct atresia
• Understanding How Inflammation Causes Biliary Atresia, Jorge Bezerra, MD, Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center

Support groups

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• LIVER FAMILIES: An international online support group for families whose lives have been touched by pediatric liver disease and transplant.

Gastroenterology | Hepatology | Pediatrics | Congenital disorders | Rare diseases

Gallengangatresie | La atresia de vías biliares | Atresie des voies biliaires | sappitieatresia | Galgangatresie | Gallegangsatresi | Gallvägsatresi