What is biliary atresia?

Biliary atresia (say "BILL-ee-air-ee uh-TREE-zhuh") is a liver disease in newborn babies. It is a problem with the bile ducts, which may be blocked or missing. The bile ducts are the tubes that connect the liver and gallbladder to the small intestine.

Normally, the liver produces bile that is stored in the gallbladder and released into the intestine to help digest fat. But when the bile ducts are blocked or missing, the bile stays in the liver and builds up. This causes scarring and harms the liver. The liver isn't able to work well and can fail. If the liver fails, the baby will need a liver transplant.

Experts don't know what causes biliary atresia. It isn't inherited, so it isn't passed down to children.

Biliary atresia is a serious problem. It must be treated right away.

How is biliary atresia diagnosed?

The main symptom of biliary atresia is jaundice. This makes the baby's skin and the whites of the eyes look yellow. The baby may also have dark urine and light-coloured stools.

A number of tests will be done to see if the baby has biliary atresia. The baby's blood, urine, and stool will be tested. The doctor will also do ultrasound tests of the liver and belly. The baby may need a liver biopsy. In a biopsy, the doctor uses a needle to take a small piece of the liver. The piece of the liver is then checked for the disease.

How is biliary atresia treated?

• Doctors usually first try a Kasai procedure. In this surgery, the bile ducts are removed. Then part of the small intestine is moved to replace the bile ducts. The small intestine drains the bile out of the liver.
  • This procedure is best done before the baby is 90 days old.
  • Some children can then lead a normal life without any liver problems.
  • For other children, the procedure works at first, but problems may develop later in childhood. These children may need liver transplants.
• If the Kasai procedure doesn't work, the child will probably need a liver transplant sooner.