Biliary atresia

The liver makes bile which helps to digest fat. Bile ducts are tubes that drain bile from the liver to the intestine. Biliary atresia is a disease in newborns where bile ducts get scarred and cannot drain the bile. Then the bile gets trapped in the liver and causes scarring (cirrhosis).

What are the symptoms of biliary atresia?

Most babies with biliary atresia appear healthy after birth. Many signs and symptoms of biliary atresia can be seen in other causes of liver inflammation. These include yellow eyes, yellow skin (jaundice), dark urine, enlarged liver or spleen, and poor appetite. In addition, babies with biliary atresia usually have very pale stool.

How is biliary atresia diagnosed in children?

Blood tests often show high bilirubin and liver enzymes. A liver ultrasound sometimes shows a very small or missing gallbladder. A diagnosis is confirmed by a combination of tests including a liver biopsy and cholangiogram. A liver biopsy  |  En Español uses a needle to take a small sample of liver to look at under a microscope. A cholangiogram is done with a needle to inject dye (contrast) into the bile ducts to see if they are scarred.

What is the treatment for biliary atresia?

The only treatment for biliary atresia is an operation called the Kasai procedure. This surgery connects the liver to the intestine to drain the bile. It is best to do this by 2 months of age. If the Kasai procedure does not work, the child will eventually need a liver transplant. Biliary atresia can cause poor growth, vitamin deficiencies, itching, fluid in the belly (ascites), and bleeding. Other treatment is based on each child's needs such as special formula and vitamins depending on what problems they may have.

What is the long-term outlook of biliary atresia?

Without any treatment, all babies with biliary atresia develop liver failure and die by 2-3 years of age. With the Kasai procedure, half of the children will still need a liver transplant by 2 years of age. By adulthood, 80% of people will need a liver transplant.