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:
- A viral or bacterial infection after birth
- Problem with the immune system
- Exposure to toxic substances
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:
- Dark urine
- Gray or white stools—caused by lack of bile
- Swollen abdomen
- Poor weight gain and growth
- Bleeding due to vitamin deficiencies
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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:
- Blood test—to detect high levels of a certain type of bilirubin or liver damage
- Ultrasound —to look at ducts for enlargement or reason for blockage
- Hepatobiliary scintigraphy —to look at bile flow in the ducts
Your child’s liver may need to be tested and/or viewed. This can be done with:
- Diagnostic surgery (intraoperative cholangiogram) to view the liver and ducts—repairs will be made during this surgery if blockage is found
- Liver biopsy —liver tissue is taken and examined under a microscope to confirm diagnosis
Your child will have special dietary needs.
- They may need extra calories to grow.
- The lack of bile will prevent your child's body from properly using fat from foods and certain vitamins.
- Extra vitamins A, D, E, and K will be needed.
- Your child may also need a special type of fat in the diet.
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:
- A transplant will be needed in the first few years of life if the Kasai procedure is not successful.
- A transplant may be needed later in life if liver damage, called cirrhosis, continues even after successful Kasai procedure by adulthood.
Since healthy transplanted liver tissue will grow there are a number of options:
- An infant can accept a portion of a liver from an adult donor that has died. The liver will eventually grow into a full size liver.
- A portion of the liver from a live donor can be donated. The liver tissue in both the donor and infant/child will regrow to full size.
There are no current guidelines to prevent biliary atresia.
American Liver Foundation
National Institute of Diabetes and Digestive and Kidney Diseases
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