Biliary Atresia: Causes, Symptoms, and Treatment
Medically reviewed by Achuta Uppu, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Biliary Atresia?
A pediatric gastroenterologist or hepatologist, a doctor who treats children's liver problems, and a pediatric surgeon treat biliary atresia. Treatment is surgery called the Kasai procedure, which works best when done early. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is biliary atresia?
Biliary atresia is a rare problem in newborn infants. The bile ducts are the tubes that carry bile from the liver to the gut. Bile is a fluid that helps digest food. In biliary atresia, these tubes are scarred and blocked. Bile cannot flow into the intestine, so it builds up in the liver and damages it. Over time this can cause cirrhosis, which is scarring of the liver, and liver failure.
It affects about 1 out of every 12,000 infants in the United States. It only happens in newborn infants. It is slightly more common in female infants and in infants with Asian or African American heritage.
Symptoms
The first sign is jaundice, yellowing of the skin and the whites of the eyes. Many newborns are jaundiced in the first 2 weeks of life. A baby whose jaundice lasts longer than 2 weeks should be checked for biliary atresia. Other signs include:
- Pale yellow, gray, or white stools.
- Poor weight gain and growth.
- Swelling of the belly from fluid buildup.
Causes and who gets it
Doctors do not know what causes biliary atresia. It is not an inherited disease, so it does not pass from parent to child. Research points to several possible factors:
- Infections with certain viruses.
- Contact with harmful chemicals.
- Problems with the immune system.
- A problem with liver and bile duct development in the womb.
- Certain gene changes that raise the chance of the condition.
How doctors diagnose it
Doctors check any newborn whose jaundice lasts longer than 2 weeks. Bile contains a reddish-yellow substance called bilirubin. Pale stools are another sign doctors look for. They happen because bilirubin is not reaching the intestines and passing out in the stool.
Treatment
The usual first treatment is surgery called the Kasai procedure. It works best when done early. The surgeon removes the damaged bile ducts outside the liver. Then the surgeon uses a loop of the infant's own small intestine to replace the damaged bile ducts. If the surgery is successful, bile flows from the liver to the small intestine. The Kasai procedure does not cure biliary atresia. After a successful surgery, most infants no longer have jaundice and have a lower risk of problems from advancing liver disease. Within 3 months of the surgery, doctors have an idea of whether it worked. Even after the Kasai procedure, children may have poor nutrition and growth problems, such as failure to thrive.
A common problem after surgery is a liver infection called cholangitis. Doctors treat it with antibiotics. If the surgery does not work, or the liver keeps getting damaged, the child needs a liver transplant. A liver transplant replaces the sick liver with a healthy liver from a donor. Even with treatment, about half of children with biliary atresia will need a liver transplant by age 2. With treatment, most infants with biliary atresia survive to adulthood.
When to see a doctor
Call a doctor right away if your newborn has jaundice that lasts longer than 2 weeks or has pale, gray, or white stools. Early treatment with the Kasai procedure may slow or, in some cases, prevent cirrhosis and liver failure. Call 911 or your local emergency number right away for a baby who has trouble breathing, has blue or gray skin, is very sleepy or hard to wake, has a seizure, or has bleeding that will not stop.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Early treatment with a surgery called the Kasai procedure may slow or, in some cases, prevent the development of cirrhosis and liver failure.
Common questions
Is biliary atresia inherited?
No. It does not pass from parent to child. Doctors still do not know what causes it.
What is the Kasai procedure?
It is surgery that removes the damaged bile ducts outside the liver and uses a loop of the small intestine to replace them. If it works, bile flows from the liver to the small intestine. It is usually the first treatment.
Will my child need a liver transplant?
Many children do. Even with treatment, about half of children with biliary atresia will need a liver transplant by age 2. In some cases, after a successful procedure, children never need a liver transplant.
Can biliary atresia be prevented?
Doctors do not know what causes it, so they do not know how to prevent it. Finding it early and doing the Kasai procedure early can slow liver damage.
Sources
- National Institute of Diabetes and Digestive and Kidney Diseases - Definition and Facts of Biliary Atresia
- National Institute of Diabetes and Digestive and Kidney Diseases - Symptoms and Causes of Biliary Atresia
- National Institute of Diabetes and Digestive and Kidney Diseases - Treatment for Biliary Atresia
- MedlinePlus - Newborn jaundice
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
