Biliary atresia is a rare liver disease that occurs in infants. It is often found shortly after birth. The disorder affects tubes in the liver called bile ducts. If not treated with surgery, it can be fatal.
Bile is a fluid made by the liver. Normally the bile ducts take bile to the small intestine. Bile helps with digestion and also carries waste products. When a child has biliary atresia, the bile ducts in the liver are blocked. Bile becomes backed up in the liver. This damages the liver. Many vital body functions also are affected.
There are two types of biliary atresia:
Biliary atresia must be treated with surgery. It is the most common cause of liver transplants in children in the U.S. It is more common in girls than in boys.
Experts don’t know what causes biliary atresia. It is not passed down from parent to child, so it is not an inherited disease.
Researchers are looking at possible causes, such as:
Babies with biliary atresia usually seem healthy at birth. Most often, symptoms start to occur between 2 weeks and 2 months of life. Symptoms may include:
Symptoms of biliary atresia may look like other liver conditions or health problems. Always see your child's healthcare provider for a diagnosis.
Your child’s healthcare provider will give your child an exam and take a health history. The provider may also do several tests. These may include the following.
Without surgery, biliary atresia can be fatal. Two types of surgery are used to treat this condition. Your child's healthcare provider will discuss which surgery would be a good choice for your child.
This surgery connects the bile drainage from the liver directly to the intestinal tract. It goes around (bypasses) the blocked bile ducts. This surgery can allow a child to grow and be in fairly good health for a few years. At some point, bile will back up into the liver. This causes liver damage. A liver transplant may also be needed over time.
A liver transplant removes the damaged liver and replaces it with a new liver from a donor. The new liver can be one of the following:
After a liver transplant, the new liver begins working and the child's health often gets better quickly.
After a transplant, a child must take medicines to help prevent the body’s immune system from attacking, or rejecting, the new liver. These medicines are called antirejection medicines. Rejection is part of the immune system’s normal way of fighting against viruses, tumors, and other foreign substances. Antirejection medicines weaken the immune system’s response.
It’s important to have regular contact with your child’s healthcare provider and members of the transplant team after a liver transplant.
Before your child has surgery, nutrition may be a problem. With biliary atresia, not enough bile reaches the intestine to help digest fats in the diet. Liver damage can cause a lack of protein and a lack of vitamins. Children with liver disease need more calories than a normal child because they have a faster metabolism.
Your child’s healthcare provider may recommend that your child see a pediatric nutritionist. The nutritionist can advise you on your child's diet. A nutritional plan may include a balanced diet and added vitamins, as directed by your child’s healthcare provider.
Some children with liver disease become too sick to eat normally. If this is the case, your child’s provider may also recommend that you give high-calorie liquid feedings. These feedings are given through a tube called an NG, or nasogastric tube. The tube is guided into your child’s nose, down the esophagus, and into the stomach. A high-calorie liquid can be given through the tube to add to your child's diet if your child can only eat small amounts of food. Or it can replace meals if your child is too sick to eat.
After surgery, your child's digestion may go back to normal. Or you may still need to give your child extra vitamins or adjust your child's diet. Talk with your child's healthcare provider for recommendations.
Many factors affect how well children with biliary atresia do over time. These factors include:
After a liver transplant, your child's health will typically improve. But your child must follow a strict medical routine. This includes taking:
It is important to work closely with your child's healthcare providers.
Call your child’s healthcare provider right away if you see that your child has any of the following:
Also call your child’s healthcare provider to ask what symptoms you should look for if your child:
Tips to help you get the most from a visit to your child’s healthcare provider: