By: Pamela L. Valentino, MD, MSc, FAAP & Sanjiv Harpavat, MD, PhD, FAAP
Biliary atresia is a serious liver disease that affects infants. About 1 in every 15,000 infants have the disease. We do not know why some infants have biliary atresia while others do not.
Bile is a liquid made by the liver to help digest food. Normally, tubes called bile ducts carry bile from the liver to the intestines. In babies with biliary atresia, the bile ducts are blocked. The bile cannot pass, so it backs up in the liver and causes damage. Learn how biliary atresia affects babies, and why
early diagnosis and treatment is key.
What problems does biliary atresia cause?
Biliary atresia can cause jaundice, commonly visible as yellowing of the eyes and skin, and pale stools. This is because bile contains a yellow pigment called "conjugated bilirubin." When the bile ducts are normal, the yellow pigment flows to the intestines and gives our stools color. However, when bile ducts are blocked, the yellow pigment backs up. The eyes and skin become jaundiced, and the stools become pale.
Biliary atresia also causes liver injury. It happens because bile is a strong chemical that digests the fats we eat. When bile cannot leave the liver, it starts digesting the liver and causes liver damage over time.
What are some signs & symptoms of biliary atresia?
Most infants with biliary atresia are born full-term and appear healthy. By 4-6 weeks of age, the jaundice and pale stools develop. In addition, their urine may appear dark.
What tests are done to diagnose biliary atresia?
If you have any concerns, your baby should be seen by their pediatrician as soon as possible. The first test the pediatrician will perform is a blood test for conjugated bilirubin (this is also called a "direct bilirubin" test). If the test for conjugated bilirubin is normal, your baby does not have biliary atresia.
If the test for conjugated bilirubin is high, more testing for biliary atresia is needed. This includes an ultrasound of the abdomen, as well as blood and imaging tests to look for other possibilities. Ultimately, more invasive tests may be needed, such as a liver biopsy and/or surgery to better see the bile ducts.
What can be done to make my child better?
If you are concerned, the most important thing is to see a physician quickly. The physician will check for a conjugated bilirubin level in the blood and, if needed, refer to a gastroenterologist or liver specialist quickly.
Infants with biliary atresia are treated with the "Kasai portoenterostomy." This surgery connects the liver to the intestines to create a drain for bile to flow from the liver into the intestines.
Remember
Regular visits with your child's pediatrician can help diagnose biliary atresia early and prevent related complications.
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About Dr. Valentino
Pamela L. Valentino, MD, MSc, FAAP, a member of the American Academy of Pediatrics (AAP) Section on Gastroenterology, Hepatology and Nutrition, is the clinical center director of Hepatology and medical director of liver transplant at Seattle Children’s Hospital. Dr. Valentino is also an Associate Professor at the University of Washington in the Department of Pediatrics. It is Dr. Valentino’s mission to help children with liver diseases and liver transplants lead health full lives. She focuses her clinical work and research efforts on bile duct diseases, bile duct complications following liver transplant and Wilson disease.
About Dr. Harpavat
Sanjiv Harpavat, MD, PhD, FAAP, is lead author of the AAP clinical report, "Identifying Infants with Biliary Atresia by 2-4 Weeks of Life: Guidance for the Primary Care Provider" and Chair of the AAP Section on Gastroenterology, Hepatology and Nutrition.
Editor's note: The authors would like to acknowledge David T. Schindel, MD, FAAP, and Humberto Lugo-Vicente, MD, for writing a previous version of this article.