Learn more about biliary atresia and treatments for this condition

Biliary atresia is a rare disorder of the liver and bile ducts that affects babies shortly after birth.

To meet some of the children on the liver transplant waiting list and to learn more about becoming a live donor, visit UPMC.com/BeADonor.

In babies with biliary atresia, bile ducts swell and eventually become blocked. Bile ducts are small pathways that carry fluid from the liver to the small intestine. These fluids help the digestion process by breaking down fats and absorbing vitamins.

Blocked bile ducts can lead to bile becoming trapped within the liver. Over time, this causes serious damage, eventually leading to conditions such as cirrhosis, or scarring of the liver.

While biliary atresia is extremely rare, the cause is unknown. Left untreated, the condition can be life threatening.

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Biliary Atresia Signs and Symptoms

The symptoms of biliary atresia typically appear within the first few weeks after birth. The most noticeable sign is jaundice when a baby’s skin and eyes look yellow. Other symptoms include:

  • Dark yellow or brown urine
  • Gray or white stools
  • Swollen belly
  • Enlarged liver or spleen

Kasai procedure

The most common treatment for babies with biliary atresia is the Kasai procedure. During this operation, surgeons remove the damaged bile ducts outside the liver. Then they re-establish bile flow from the liver to the small intestine by joining the two together. If performed before the baby is three months old, the Kasai procedure may be completely or partially successful in about 80 percent of cases.

Liver transplant

When the Kasai procedure is not successful, a liver transplant is the only hope for a cure.

During a liver transplant, the failing liver is removed and replaced with a healthy liver. With almost 17,000 people on the liver transplant waiting list, and fewer than 5,000 livers available, the wait for a transplant can take months or years.

Babies with biliary atresia can receive a liver transplant from a living donor. A living-donor liver transplant is a life-saving procedure that gives hope to children on the liver transplant waiting list.

Read more about how a living-donor liver transplant for biliary atresia gave one UPMC Children’s patient a second chance.

How You Can Help

By becoming a living donor for a child with biliary atresia, you will help to reduce the transplant waiting list, and you will give that child a second chance at life.

As a living liver donor, a piece of your healthy liver is removed and transplanted into a baby with an unhealthy liver. These transplants are possible because of the liver’s unique ability to regenerate, or regrow.

About Transplant Services

For more than four decades, UPMC Transplant Services has been a leader in organ transplantation. Our clinicians have performed more than 20,000 organ transplant procedures, making UPMC one of the foremost organ transplant centers in the world. We are home to some of the world’s foremost transplant experts and take on some of the most challenging cases. Through research, we have developed new therapies that provide our patients better outcomes — so organ recipients can enjoy better health with fewer restrictions. Above all, we are committed to providing compassionate, complete care that can change – and save – our patients’ lives. Visit our website to find a provider near you.