Biliary Atresia

Biliary atresia is a disease that affects the bile ducts in an infant’s liver. In a healthy liver, bile is made in the liver and drains freely through the bile ducts into the small intestine to aid with digestion. In infants with biliary atresia, the bile ducts become inflamed, narrowed or blocked, preventing the flow of bile into the intestines. This causes bile to build up in the liver, which leads to liver damage and cirrhosis (scarring of the liver). The condition is one of the most common reasons for a liver transplant in children.

At SSM Health Cardinal Glennon Children’s Hospital, our team of experts is passionate about helping children with liver conditions live long, healthy lives and is committed to offering the latest advancements in care. If your child has been diagnosed with biliary atresia, call us today at 314-268-4010 to schedule an appointment to learn how we can help.

Signs & Symptoms of Biliary Atresia

Parents usually notice the signs and symptoms when their infant is between two to six weeks old. The most common symptom parents notice is jaundice, a yellowing of their baby’s skin and eyes. While many newborns have jaundice, jaundice that lasts longer than three weeks may be an early sign of biliary atresia. Other symptoms may include:

• Swollen or distended abdomen
• Pale yellow, gray, or white stools
• Darker urine

There are other diseases that can have similar symptoms, so a complete work-up is needed to diagnose biliary atresia. Tests may include blood work and imaging (such as an ultrasound). A liver biopsy is also often necessary to confirm the diagnosis.

Treating Biliary Atresia

While there is no cure, early treatment of the condition may slow, and in some cases prevent, liver failure. While it can be life-threatening, most babies with biliary atresia will grow to adulthood with proper treatment.

Kasai Procedure

The primary treatment is a surgery called the Kasai procedure. During the surgery, the damaged bile ducts are removed and a piece of the infant’s intestine is used to create a new duct, allowing bile to flow and preventing further buildup of bile within the liver. The Kasai procedure does not cure the condition, but can slow damage to the liver and delay the need for a liver transplant. The procedure is most successful in babies under the age of three months.

Liver Transplant

Unfortunately, the Kasai procedure does not always work and a baby will have signs of advancing liver disease. In this case, the baby will need a liver transplant to survive because the liver can no longer function. During a liver transplant, the diseased liver is replaced with a healthy liver from a donor.

Ongoing Care

Whether the Kasai procedure was successful or child’s liver damage progresses and requires liver transplantation, specialized monitoring is required to ensure liver remains healthy. In addition, children often require a special eating plan to ensure they get the nutrients and calories they need.

While learning your child has biliary atresia is scary, SLUCare Physician Group surgeons at Cardinal Glennon Children’s Hospital are here to support you every step of the way and can help you navigate your child's care. Call us today at 314-268-4010 to schedule an appointment or to request a second opinion.