Children's Hospital Colorado

Biliary Atresia

What is biliary atresia?

Biliary atresia is liver disease of infants caused by inflammation that targets the bile ducts or "biliary tree." The biliary tree is a series of "tubes" that drain bile from the liver into the intestines. Biliary atresia leads to blockage of the large bile duct that exits the liver, resulting in liver damage. Without proper bile drainage, the infant and child has impaired digestion of fats leading to poor growth. In addition, the bile that is "stuck" in the liver damages liver cells, leading to scarring of the liver. Early surgical correction of the blockage can help some infants with biliary atresia, but this serious liver disease often results in the need for liver transplantation.

What causes biliary atresia?

The cause of biliary atresia is not known and is the focus of research nationwide.

Who gets biliary atresia?

Biliary atresia always presents in the first three months of life. In the United States, biliary atresia occurs in one in 10,000 to 15,000 live births. Biliary atresia affects girls slightly more frequently than boys and occurs in all races and ethnicities.

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What are the signs and symptoms of biliary atresia?

Most children who get biliary atresia are full-term and normal size at birth. Common signs of biliary atresia are:

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

How is biliary atresia diagnosed?

It is important to make the diagnosis of biliary atresia early. This is because the long-term outcome depends on the age of initial surgical intervention (known as the Kasai procedure). Therefore, any newborn older than 2 weeks with jaundice should have blood tests to see if there is a liver problem. If so, other tests are likely to be done.

At Children's Hospital Colorado, the other tests performed include:

  • Abdominal ultrasound: a painless way to look at the liver, gallbladder and surrounding organs using sound waves.
  • Liver biopsy: the child is given an anesthetic and a small needle is passed through the skin over the lower part of the ribs on the right side into the liver to take a tiny piece of tissue from the liver to look at under the microscope.
  • Explorative laparotomy: the diagnosis of biliary atresia is confirmed at surgery. The surgeon can directly inspect the biliary tree (drainage system) and inject dye to see if there is a blockage. The surgeon may also take a sample of tissue from the liver.

How can biliary atresia be treated?

Surgery is the only treatment for biliary atresia. The operation is called a hepatoportoenterostomy, or Kasai procedure. Find out what to expect from your surgery at Children's Hospital Colorado.

Kasai procedure at Children's Colorado

During the Kasai procedure, the damaged drainage system is removed and the intestine is connected to the liver with the hope that bile flows again. When this operation is successful, the jaundice disappears and the bilirubin levels (brownish yellow substance found in bile) return to normal.

As bilirubin leaves the body, it gives stool its normal brown color. The operation has the best chance for success when done as early as possible. However, even with early surgery, many infants with biliary atresia still develop liver cirrhosis (permanent scarring of the liver). These children at some point are likely to require liver transplantation. Learn more about the Liver Center at Children's Colorado.

Why choose Children's Colorado for treatment of biliary atresia?

Children's Colorado is one of the only Pediatric Liver Centers in the nation that has seven board-certified transplant hepatologists (liver specialists) that have additional certification as pediatric transplant hepatologists. Many of these doctors are currently involved in research studies aimed at identifying the cause of biliary atresia and discovering new potential therapies for treatment. In addition, Children's Colorado surgeons have been performing pediatric liver transplants for over two decades, with excellent patient outcomes.

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