Biliary Atresia: What Is It?

Do you know what is the most common reason for liver transplantation in children? It’s a rare condition called biliary atresia that only affects babies, occurring in approximately 1 in 10,000 to 15,000 births per year in the United States. Four hundred to 600 new cases are diagnosed in the United States each year. Girls are slightly more at risk, as are babies of Asian or African-American heritage.

Biliary atresia refers to injury and blockage of the bile ducts, the network of tubes that drain bile from the liver into the intestine. Infants with this condition develop jaundice and cirrhosis (scarring of the liver) in the first few months of life. Surgery to restore bile flow is the first step in therapy. Later, the child may need a liver transplant.

About 10% to 20% of infants with biliary atresia also have abnormalities in other organs, including the heart and spleen.

While the precise cause of biliary atresia remains unknown, it is well-understood that prompt diagnosis and timely surgical care is a key factor in achieving the best outcomes for these infants.

Why is biliary atresia such a serious condition?

A properly functioning liver is vital. The liver is the second largest organ in the body, and it has three major functions. The first is detoxification. Like a refinery, the liver eliminates toxins that we ingest and the byproducts of metabolism. Next, the liver is an important factory for production of useful substances like carbohydrates, specialized proteins and the bile that helps us with digestion. Lastly, the liver acts as storage for essential vitamins and glycogen, a source of energy we use to get through the day. Because biliary atresia blocks the bile ducts and results in liver injury and scarring, it hinders these important functions from happening.

Symptoms of biliary atresia

Most babies with biliary atresia appear healthy at birth. Symptoms usually appear between 2 and 6 weeks of age, and may include:

• Yellowing of the skin and whites of the eyes
• Stools appearing pale gray
• Dark urine
• Sometimes, intense itching

Treatment for biliary atresia

At Ochsner Hospital for Children, we are working to improve outcomes for children with biliary atresia. We’ve developed three key initiatives to ensure that children with biliary atresia in Louisiana get earlier diagnoses, earlier surgery and the best shot at long-term health.

• Universal newborn screening: A simple blood test in the first few days of life may provide an early clue to biliary atresia. Babies born at Ochsner Baptist and Ochsner Kenner now are routinely screened, allowing definitive diagnostic testing to occur weeks earlier.
• Rapid diagnostic pathway: We’ve developed a multidisciplinary team and fast-track diagnostic pathway that can diagnose (or rule out) biliary atresia in less than 48 hours.
• Cutting-edge clinical research trials: Ochsner Hospital for Children is pleased to be part of a multicenter trial of a new medication for infants with biliary atresia who have undergone initial surgical care. This exciting project seeks to keep these patients’ livers healthier longer, delaying or avoiding the need for liver transplantation.

If a child needs a liver transplant, each family can expect comprehensive care with your child’s best interests at the heart of all we do. Every patient starts their journey by being matched with a registered nurse coordinator who will guide them through every step of the process. Transplantation is truly a team effort; our experts in hepatology, transplantation and surgery are supported by a team of registered dietitians, social workers, pediatric psychologists and palliative care specialists. We build each child’s team to suit his/her own needs so that nothing important is left to chance.