Biliary Atresia

What Is Biliary Atresia?

Biliary atresia is when a newborn has blocked tubes in the liver. These tubes, called bile ducts, carry bile from the liver to the small intestine. If they’re blocked, bile builds up in the liver and damages it.

Babies with biliary atresia (BILL-ee-air-ee ah-TREE-zhah) need surgery and may need a liver transplant later in life. Children who have a liver transplant generally do well.

What Happens in Biliary Atresia?

Bile is a yellow-green fluid that the liver makes. Bile ducts carry bile to the gallbladder, where it’s stored until needed. Bile ducts also carry bile from the gallbladder to the small intestine, where it helps break down fat in food.

When a baby has biliary atresia, bile can’t flow out of the liver into the small intestine because inflammation (swelling and irritation) and scarring block the bile ducts. Doctors aren’t sure what causes the inflammation, but it might be due to:

• infections
• toxins
• immune problems
• problems with how the bile ducts formed

The buildup of bile damages the liver, which can lead to scarring (cirrhosis) and liver failure.

A child can’t survive without a working liver.

What Are the Signs & Symptoms of Biliary Atresia?

Most healthy newborns have jaundice (yellow-tinted skin and eyes) in the first week of life. But in babies with biliary atresia, it lasts longer because the bile ducts begin closing up.

A baby with biliary atresia also may have:

• dark urine (pee)
• pale poop
• an enlarged liver

How Is Biliary Atresia Diagnosed?

If a baby’s jaundice doesn’t improve by 2 weeks of age, doctors might suspect biliary atresia.

Other liver conditions that cause liver inflammation (like infections and genetic conditions) cause the same symptoms as biliary atresia. So doctors do tests to diagnose the problem, such as:

• blood tests, including tests to check bilirubin levels and liver enzymes
• abdominal ultrasound
• liver scan to check bile flow
• liver biopsy

How Is Biliary Atresia Treated?

Biliary atresia needs quick treatment by liver specialists and surgeons. Babies with blocked bile ducts need a surgery called the Kasai procedure. This lets bile drain from the liver directly into the small intestine. At the start of the surgery, the doctor will do a cholangiogram to see if bile ducts are blocked. If they’re not blocked, the baby won’t need the procedure.

Jaundice usually gets better after the Kasai procedure.

Liver Transplant

Most babies with biliary atresia will need a liver transplant later in childhood or before they become adults. A baby whose jaundice doesn’t improve over the weeks and months after Kasai surgery will need one sooner.

Signs that a child needs a transplant include:

• poor growth
• worsening liver failure 
• frequent liver infections
• high blood pressure in the veins draining the liver

What Else Should I Know?

Babies with biliary atresia have problems digesting fats and absorbing some vitamins. They also have problems absorbing enough calories and nutrients, even after Kasai procedure. Your doctor may recommend working with a dietitian. Some children may need:

• concentrated formula or fortified breast milk
• extra calorie supplements
• vitamin supplements
• nasogastric (NG) tube feeding

How Can Parents Help?

Caring for a baby with biliary atresia can feel overwhelming for families. Reach out to your care team for help when needed. They are ready to support you and your child.

You also can find support and education online at:

• Childhood Liver Disease Research Network
• American Liver Foundation
• Children’s Liver Disease Foundation