Biliary atresia is a blockage of bile ducts in infants. The bile ducts are tubes that let a fluid called bile pass from the liver to the gallbladder. Bile is made in the liver, passes to the gallbladder for storage, then moves to the intestine for use.
Bile breaks down the fat in food so it can be absorbed by the body. It also carries waste and toxins out of the body. If bile cannot pass out of the liver, the bile builds up and damages the liver. Biliary atresia is a life-threatening condition.
The true cause of biliary atresia is not completely clear. For some, the ducts do not develop properly and the tubes are closed off. A blockage may also develop caused by swelling of the ducts before or after birth. Swelling may be caused by:
It is not believed that biliary atresia is caused by a genetic issue that is passed on from parents.
Biliary atresia is more common in female infants, though it can occur in males. It is also more common in premature infants and children of Asian or African American descent.
Jaundice is the yellowing of the skin and whites of the eyes caused by elevated bilirubin. Elevated bilirubin is caused by a backup of bile in the liver. It may be present soon after birth. Most other symptoms will be present in the first 2-4 weeks of life. A few symptoms may not occur for 3 months or longer. Symptoms may include:
Baby with Jaundice
Copyright © Nucleus Medical Media, Inc.
You will be asked about your child’s symptoms and medical history. A physical exam will be done. Other conditions can cause similar symptoms. An infant with signs of liver problems will be referred to a specialist.
A diagnosis of biliary atresia will be made based on the results of multiple tests including:
Your child’s liver may need to be tested and/or viewed. This can be done with:
Your child will have special dietary needs.
Follow the diet advised by your child’s doctor to ensure your child gets proper nutrition.
Medications may be needed for itching, swelling, and to increase bile flow.
Surgery must be done to allow bile to flow from the liver to the gallbladder. The surgery does not cure biliary atresia. It should help bile flow to the intestine again and decrease stress on the liver. Without surgery, biliary atresia is fatal.
Hepatoportoenterostomy, also known as the Kasai procedure, is the main surgery. During this procedure the damaged bile ducts will be removed. A section of small intestine will be looped up and attached to the liver. This will allow bile to pass directly from the liver into the intestine.
A liver transplant is the only cure for biliary atresia, but has risks of its own. The severity of the condition will help determine if and when a liver transplant is needed:
Since healthy transplanted liver tissue will grow there are a number of options:
There are no current guidelines to prevent biliary atresia.
American Liver Foundation
National Institute of Diabetes and Digestive and Kidney Diseases
Canadian Liver Foundation
Biliary atresia. American Liver Foundation website. Available at: https://www.liverfoundation.org/for-patients/about-the-liver/diseases-of-the-liver/biliary-atresia#Q4. Updated January 14, 2015. Accessed December 20, 2017.
Biliary atresia. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116791/Biliary-atresia . Updated March 14, 2017. Accessed December 20, 2017.
Biliary atresia. National Institute of Diabetes and Digestive and Kidney Diseases website. Available at: https://www.niddk.nih.gov/health-information/liver-disease/biliary-atresia#2. Accessed December 20, 2017.
Last reviewed November 2018 by
EBSCO Medical Review Board
Michael Woods, MD, FAAP
Last Updated: 12/20/2017