Pediatric biliary atresia

Biliary atresia is a serious liver disease that’s the most common reason for liver transplants in children. Children’s Health℠ is home to the only biliary atresia program in North Texas. Our expert team includes hepatologists (liver doctors) and surgeons who specialize in diagnosing and treating biliary atresia. We offer complete, personalized care to help your child grow up healthy and strong.

What is pediatric biliary atresia?

Biliary atresia is a condition in which a baby’s bile ducts are blocked. These tubes carry bile from the liver to the small intestine. Bile is a liquid that the liver produces. Bile helps digest fats and carries waste from the liver to the intestines and out of the body.

When bile ducts are blocked, bile builds up in the liver. This buildup causes scarring that can lead to liver damage. Without bile for digestion, a baby’s body can’t absorb enough nutrients to grow and thrive. Babies with biliary atresia need treatment early in life to avoid malnutrition and liver failure.

What are the signs and symptoms of pediatric biliary atresia?

Children with biliary atresia usually show signs within a few weeks of birth. Early signs of biliary atresia include:

• Jaundice, a yellow color in the skin and white parts of the eyes

• Light-colored stools (poop)

• Dark-colored urine (pee)

Later, when your baby is about six to 10 weeks old, they may have other signs, such as:

• Irritability (fussiness)

• Failure to thrive (trouble gaining weight)

• Itchy skin

• Swollen belly because of fluid buildup

How is pediatric biliary atresia diagnosed?

Our Biliary atresia Program includes gastroenterologists (specialists in digestive conditions) and hepatologists (specialists in liver conditions). We begin with a physical exam, and we’ll ask about your child’s personal and family medical history.

We’ll also order tests to confirm a diagnosis of biliary atresia and rule out other conditions. Your child may need one or more tests, including:

• Blood tests: We take a small sample of your baby’s blood to test. Blood tests can show how well the liver works and check for the causes of jaundice. A matrix metallopeptidase-7 (MMP-7) blood test measures levels of MMP-7 protein. High MMP-7 levels can be a sign of biliary atresia. Most centers have to send blood samples to outside labs for MMP-7 tests. At Children’s Health, we can do MMP-7 tests in our own lab, which is much faster.

• Ultrasound: This painless imaging test uses sound waves to create images of the inside of the body. We do ultrasounds of your child’s belly to examine the liver and bile ducts.

• Intraoperative cholangiogram: This imaging test uses X-rays with a contrast dye to create images of the bile ducts. We do this test during surgery to view blockages in bile ducts and treat them.

What causes pediatric biliary atresia?

The causes of biliary atresia aren’t fully known. Biliary atresia isn’t an inherited disease.

Certain factors may increase the risk of biliary atresia, including:

• Exposure to harmful chemicals

• Certain gene mutations (changes) that affect the way a baby’s bile ducts develop before birth

How is pediatric biliary atresia treated?

All babies who have biliary atresia need surgery. At Children’s Health, the team at the Biliary atresia Program includes pediatric general surgeons who work closely with gastroenterologists and hepatologists. Our surgeons have advanced training and years of experience in surgical techniques to treat biliary atresia.

Kasai procedure to treat biliary atresia

In this procedure, our surgeons create a new pathway for bile to flow from the liver to the small intestine. We remove damaged parts of the bile ducts and connect the liver directly to the small intestine.

The Kasai procedure allows bile to flow out of the liver, and jaundice and other symptoms will likely go away. The improved bile flow will help the intestines absorb nutrients, so your baby can grow properly.

Liver transplant to treat biliary atresia

The Kasai procedure isn’t a cure, and biliary atresia sometimes causes severe symptoms in infants and young children. Many children eventually need a liver transplant, even after a successful Kasai procedure.

A liver transplant is surgery to remove a diseased liver and replace it with a healthy liver from a donor. Our biliary atresia team works closely with transplant surgeons at Children’s Health to coordinate your child’s care.

Postsurgical care for biliary atresia

After the Kasai procedure or a liver transplant, children typically need:

• Medicines: We’ll prescribe antibiotics to prevent infection and medicines to help with bile flow. Children who’ve had a liver transplant will need medicines to prevent organ rejection.

• Nutritional support: Babies need a special type of formula, and older children need a special eating plan to make sure they’re getting proper nutrition. The registered dietitians on our team will work closely with you to find the right nutrition plan for your child. We’ll prescribe vitamins and other supplements too.

• Regular checkups: Your child will need regular checkups with specialists for the rest of their life. Their care team will check how well their liver is working and how well bile is flowing. They’ll also check for signs of any health problems (complications) that might develop.