Biliary Atresia

What is biliary atresia?

Bile is an important fluid produced by your child's liver and stored in the gallbladder. In a healthy system, it flows through a tube called the common bile duct to the small intestine, where it helps to digest food. 

If your child has biliary atresia, the common bile duct is blocked or damaged, so that it's impossible for bile to flow through it, just as it's hard for water to pass through a clogged pipe. This is called cholestasis, or poor bile flow, and quickly leads to malnutrition and liver damage. Biliary atresia symptoms, such jaundice and dark urine, typically occur within the first few weeks of a child's life.

It is fatal if left untreated. This condition:

  • is the most common cause of chronic liver disease in newborns
  • occurs once in every 30,000 births
  • is the most common reason for liver transplants in children
  • affects children of all races and ethnicities, but may be more common in Asians

There are two main reasons why biliary atresia is a problem. First, it makes it harder for bile to get to your child’s small intestines. Without enough bile in the small intestines, it's more difficult for your child's body to absorb fat and harder for the body to get rid of certain toxins that need bile to help them exit the body.

Second, bile accumulation in the liver, causes liver damage. As this condition progresses, inflammation and scarring damage the liver more and more, eventually resulting in cirrhosis of the liver. If bile can’t leave the liver, it accumulates. This can result in a buildup of toxins that causes even more liver damage.

What are the symptoms of biliary atresia?

Infants with biliary atresia may be born with jaundice, but usually appear healthy at birth. Most often, you or your child's pediatrician will notice signs within the first few weeks of your child's life. These may include:

  • jaundice (yellowing of the eyes and skin)
  • dark urine
  • light-colored stools

Signs that may appear later include:

  • distended (swollen) abdomen
  • weight loss

These are later signs, not usually in the first months of life.

What causes biliary atresia?

We know that biliary atresia is caused by inflammation and scarring of the bile ducts, but what causes the inflammation and scarring is still unknown.

There are three types of biliary atresia:

  • biliary atresia without any other malformations: this is the most common type seen in ~85 percent of the babies
  • biliary atresia with at least one major malformation but without laterality ~5 percent
  • biliary atresia with laterality defects ~10 percent, this is what was previously called biliary atresia splenic malformation syndrome.

Meet Melissa and Isabella

The 6- and 2-year-old share just about everything. They share big personalities. They share a love of being lively and loud. And, they also share something else — they were both born with biliary atresia.

Two sisters with biliary atresia sit next to each other in an exam room.
 

How we care for biliary atresia

At Boston Children's Hospital, biliary atresia is treated by a small number of doctors with specific expertise in the Center for Childhood Liver Disease. We're known for our individualized and science-driven approach. We're home to the most extensive research enterprise located in a pediatric hospital in the world, and we partner with a number of top biotech and health care organizations.

Boston Children's is also home to New England's largest pediatric Liver Transplant Program. If your child has biliary atresia, our team will work with you from the beginning to identify the best treatment options for each stage of their condition.