Transplant What Is Biliary Atresia? By Transplant Services, July 29, 2017 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. 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. 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.