Biliary Atresia

Jaundice after three weeks of age is often the earliest symptom of biliary atresia. Jaundice is when the skin and whites of the eyes look yellow.

Many newborns have jaundice until two weeks of age. But if your baby has jaundice after two weeks of age, contact their pediatrician. This could be a sign that your baby’s liver ducts are blocked, causing bile to build up. Bile is a fluid the liver makes to help the intestines digest food.

Other symptoms of biliary atresia include:

• White or pale yellow or gray stools
• Dark urine
• Failure to thrive, or not meeting height or weight milestones

Babies develop biliary atresia either in the womb or soon after birth, but we don’t yet know why. Biliary atresia is not an inherited disease, and it does not run in families. Researchers are looking into various causes, including infections, immune system defects, problems during liver and bile duct development in the womb, or a gene mutation.

Biliary atresia is a rare disease. The incidence is one in 10,000 to 12,000. It is more common in Asian and African American infants, and it is more common in boys.

The goal is to diagnose biliary atresia by 45 to 60 days of age. Babies have better outcomes when the condition is caught early. That’s why it’s so important to talk to your child’s pediatrician as soon as you notice symptoms. Your pediatrician will let you know if your child should see a pediatric liver specialist.

Various tests may be used to diagnose biliary atresia and rule out other possible problems, including:

• Blood tests
• Ultrasound of the abdomen
• A liver biopsy, which involves taking a very small piece of your baby’s liver to examine under a microscope

We may also have to conduct a diagnostic procedure called an intraoperative cholangiogram. This involves making a small incision in the abdomen and infusing contrast dye into the gallbladder while watching to see if the dye reaches the large bile ducts outside the liver and then the small intestine. If the dye does not pass through the bile ducts, that confirms that your baby’s liver ducts are obstructed. At that point, the diagnosis of biliary atresia is made, and our surgeon will perform the Kasai procedure during the same operation.

Comer Children’s physicians are working with other U.S. researchers to uncover a noninvasive blood test for diagnosing biliary atresia. This would not only forgo the need for babies to undergo a biopsy or diagnostic surgery, but would also speed up the time it takes to diagnose biliary atresia. Because we are involved in this research, our patients will be among the first in the country to have access to a noninvasive test, once it becomes available.

The Kasai procedure involves removing your baby’s damaged bile ducts, which connect the liver to the small intestine. Then the small intestine will be connected to the liver. This will allow bile to flow directly from the liver into the small intestine.

The operation typically requires a five- to seven-day hospital stay at Comer Children’s, our state-of-the-art pediatric hospital. After discharge from the hospital, your child will need to be monitored in our clinic to ensure the liver is functioning properly.

When successful, the procedure will allow your child to grow and live a normal life for many years; however, the Kasai procedure does not cure all biliary atresia patients. The condition most often continues to damage your child’s liver overtime at variable rates. Of all infants who have had a Kasai procedure, 50% still require liver transplantation before age five

Biliary atresia can cause digestion and nutrition problems in growing children. Babies with biliary atresia will need diet interventions to ensure they grow properly. Our pediatric nutritionists will work closely with you to develop a feeding and nutrition plan specific to your child’s needs. This plan might include:

• A special baby formula if your baby is formula-fed
• Vitamin and mineral supplements that can be added to pumped breast milk, formula and/or food
• A special diet plan that ensures your child is getting enough protein and other nutrients

Some babies with biliary atresia may need to get additional nutrition delivered through a tube in their stomachs or through an intravenous line.

About 80% of children with biliary atresia eventually need a liver transplant before age 20. Our team will see your child on a regular basis to assess liver function and gauge if a liver transplant might be needed in the near future. Our pediatric hepatologist is also a member of our liver transplant team and can help you prepare your child and your family for this operation, should the need arise.