Learning About Biliary Atresia

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 scarred or blocked. 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 small intestine to help digest fat. But when the bile ducts are blocked, 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 in the first months of a baby's life.

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-colored 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 and scans of the liver and belly. The baby may need a liver biopsy. In a biopsy, the doctor takes a small piece of the liver to check for the disease.

How is biliary atresia treated?

Doctors usually first try a Kasai surgery. In this surgery, the bile ducts are removed. Then part of the small intestine is connected to the liver to replace the bile ducts. The small intestine drains the bile out of the liver.

If the Kasai surgery doesn't work, the child will probably need a liver transplant.

Follow-up care is a key part of your child's treatment and safety. Be sure to make and go to all appointments, and contact your doctor if your child is having problems. It's also a good idea to know your child's test results and keep a list of the medicines your child takes.