Children's Hospital Colorado

Biliary Atresia

What is biliary atresia?

Biliary atresia is a liver disease that starts in infancy and is caused by inflammation and scarring of the bile ducts or "biliary tree." The biliary tree is a series of tubes that drain bile from the liver into the intestines. Improper bile drainage impairs the digestion of fats, which leads to poor growth, vitamin deficiencies and chronic liver damage.

Usually around the time of diagnosis, the large bile duct that exits the liver is blocked due to scar tissue, and the bile cannot flow through. We use a surgical procedure known as the Kasai procedure, or Kasai portoenterostomy, to re-establish bile flow. This early surgical correction to clear the blockage and allow bile to flow into the intestine can sometimes work as a long-term solution, but most will ultimately need a liver transplant.

What are some biliary atresia causes?

Doctors do not currently know the cause of biliary atresia in infants. However, many hospitals across the country, including Children's Hospital Colorado, are conducting research on the cause of biliary atresia.

Who gets biliary atresia?

Biliary atresia occurs in about one out of every 12,000 live births in the United States and is more common in females, premature infants and Asians.

What are biliary atresia symptoms?

Biliary atresia likely starts prior to birth, and symptoms develop in the first three months of life. Most children with biliary atresia are full-term and normal size at birth. Common signs of biliary atresia include:

  • Jaundice (yellowing of the skin and eyes)
  • Dark tea-colored urine
  • Pale clay-colored stool (non-pigmented stool)

What tests are used to reach a biliary atresia diagnosis?

Making an early diagnosis of biliary atresia is important because the long-term outcome depends on the age of the initial surgery. If a newborn older than two weeks displays symptoms of jaundice, we will perform these other tests:

  • Blood test: Our first step is to perform a blood test, which tells us if there is a liver problem.
  • Abdominal ultrasound: Using sound waves to produce images of inside the body, an abdominal ultrasound is a painless way to look at the liver, gallbladder and surrounding organs.
  • Liver biopsy: In this procedure, our surgeon passes a small needle through the skin and between the ribs to reach the liver. We perform the biopsy while your child is under general anesthesia, so the child experiences no pain or discomfort. After removing a very small tissue sample from the liver, a board-certified pediatric pathologist examines it under a microscope.
  • Intraoperative cholangiogram: A special kind of X-ray imaging called the intraoperative cholangiogram is used during surgery. It allows the surgeon to see a live video of the bile ducts in real-time. By injecting a dye in the gallbladder and bile ducts, the surgeon can inspect the biliary tree to see if there's a blockage. If the surgeon confirms biliary atresia, they will perform the Kasai procedure at that time to remove the blockage.

Why choose us to test for biliary atresia?

The Pediatric Liver Center at Children's Colorado is one of the largest in the nation. Here, we have seven board-certified specialists caring for more than 1,500 children with liver diseases. Many of these doctors are also involved in research studies aimed at identifying the cause of biliary atresia and discovering new potential therapies for treatment.

Because we are actively involved in research and identified biliary atresia as one of our focuses, we have an efficient system to quickly and accurately identify the condition. Our multidisciplinary team of specialists is set up to perform bloodwork, radiologic evaluations and liver biopsies so we can rapidly diagnose biliary atresia.

How is biliary atresia treated?

Biliary atresia is initially treated with the Kasai procedure, which is a surgical procedure that provides a way for bile to flow out of the liver into the intestine. In this procedure, the surgeon removes the damaged and scarred bile duct and connects the intestine to the liver to allow the bile to flow. When this operation is successful, the jaundice and pale-colored stools disappear, and the bilirubin levels in the blood return to normal. Bilirubin is an orange-yellow pigment made in the liver to breakdown hemoglobin.

This operation has the best chance for success when it is performed as early as possible. After the operation, we prescribe medications such as ursodiol to aid in bile flow and optimize the child’s growth and health.

The Kasai procedure is not always a permanent solution to the complications associated with biliary atresia. Even with early surgery, many infants still develop liver cirrhosis, which is permanent scarring of the liver. At some point, these children will require liver transplantation. Approximately 50% of children with biliary atresia will require liver transplantation by 2 years of age.

Why choose us for biliary atresia treatment?

Children's Colorado has one of the largest Pediatric Liver Centers in the nation, and our liver specialists are nationally and internationally recognized experts in pediatric liver diseases, including biliary atresia. Our surgeons have been performing Kasai procedures since 1975 and pediatric liver transplants since 1989, both with excellent patient outcomes.

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