Biliary atresia

With early treatment, many children with biliary atresia live long and active lives. About half of babies who have the Kasai procedure before 2 months of age will have enough bile flow to grow and stay healthy for a number of years. Very rarely, children with biliary atresia live their entire lives without needing a liver transplant.

If a liver transplant is needed, the outlook remains very good. In the U.S., more than 90% of children are alive five years after transplant. And long-term survival after liver transplant in general is excellent, with an 80% survival rate at 30 years after transplant.

What affects your child's long-term prognosis

Several important factors influence how well a child with biliary atresia will do over time:

• Timing of Kasai surgery. The procedure works best when done before 60 days of age. Earlier surgery gives the best chance of restoring bile flow and protecting the liver.
• Success of Kasai surgery. For about half of all children, Kasai surgery restores enough bile flow to improve jaundice and support typical growth and development for a period of time. Some children may live for many years with their own livers after a successful Kasai surgery. The success of Kasai surgery depends on the expertise of the surgeon and medical center.
• Complications. Even when the surgery is successful, some children may develop complications over time, such as liver scarring, infection in the bile ducts or increased pressure in the liver's blood vessels, called portal hypertension. These complications can affect growth, nutrition and overall liver function.
• Need for liver transplant. A transplant is needed if Kasai surgery doesn't work well or the liver becomes too damaged. Fortunately, survival after transplant is very high, especially when it's done at a center that specializes in pediatric liver care.
• Ongoing medical care. Children with biliary atresia need lifelong monitoring to track liver health, manage nutrition and detect complications early. Care from a team that includes liver specialists, surgeons and dietitians helps give children the best chance to grow and thrive.