Biliary Atresia | Symptoms & Causes

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Why is biliary atresia a concern?

There are two main reasons why biliary atresia is a problem:

1. It makes it harder for bile to get to your child’s small intestines. Without enough bile in the small intestines, two problems happen:

  • It’s harder for your child’s body to digest fat. This is a concern because:
    • Fat is a major sources of calories (energy) for children.
    • Some vitamins, such as A, D, E and K, need to bond with fat, or else they can’t be absorbed.
    • Fat plays a vital role in maintaining healthy skin and hair, insulating body organs against shock, maintaining body temperature and promoting healthy cell function.
    • Not only is it harder for your child’s body to get the energy she needs, living with biliary atresia – or any chronic condition – means that she needs more energy to begin with, putting her at even more risk of malnutrition.
  • It’s harder for your child’s body to get rid of certain toxins that need bile to help them exit the body.

2. It causes liver damage. This may happen in two ways:

  • As the biliary atresia progresses, inflammation and scarring damage the liver more and more, eventually resulting in cirrhosis of the liver.
  • If bile can’t leave the liver, it accumulates. This can result in a buildup of toxins that causes even more liver damage and raises your child’s cholesterol level.

Nutrition and biliary atresia

Children with biliary atresia have special nutritional needs. Our registered pediatric dieticians can make recommendations including:

  • feeding with special formulas that contain a kind of fat that’s more easily digestible
  • adding specific vitamins to your child's diet, as directed by your child's physician
  • adding MCT (medium-chain triglyceride) oil for extra calories to the diet. MCTs are more easily digested without bile than other types of fats. MCT oil can be added to foods and liquids that your child eats.

If your baby can’t take in the amount of calories needed to maintain a normal growth rate, we may recommend liquid feedings. These are given through a tube called a nasogastric tube (NG) that is guided into the nose, down the esophagus and into her stomach.

What causes biliary atresia? 

We know that biliary atresia is caused by inflammation and scarring of the bile ducts, but what causes the inflammation and scarring is still unknown.

Biliary atresia does not seem to be linked to anything the mother did during pregnancy, such as any medications she might have taken.

There are two types of biliary atresia:

1. embryonal biliary atresia

  • probably present at birth
  • signs and symptoms appear earlier
  • often associated with other malformations, including:
    • intestinal malrotation (the intestine is not twisted in the usual position within the abdomen)
    • polysplenia (multiple spleens)
    • abnormal veins
    • congenital heart defects
2. perinatal biliary atresia
  • 85 percent of biliary atresia cases are thought to develop after birth, within the first weeks of life.
  • Some researchers believe this type may be caused by exposure to infections or toxic substances.

What are the symptoms of biliary atresia?

Infants with biliary atresia may be born with jaundice, but usually appear healthy at birth. Most often, you or your child’s pediatrician will notice signs within the first two weeks to two months of your child’s life. These may include:

  • jaundice
  • dark urine
  • light-colored stools

Signs that may appear later include:

  • distended (swollen) abdomen
  • weight loss

These are later signs, not usually in the first months of life.

Long-term outlook

While there’s no cure for the condition, there are ways for your child to have a longer and healthier life. The first step is surgery, called the Kasai portoenterostomy, in which the blocked bile duct is bypassed using a piece of your child’s intestine. Afterwards, special formulas and vitamin preparations, as well as other medications, can help your child grow and help to preserve her liver function.

Nonetheless, many children who have Kasai portoenterostomies early in life will need a liver transplant at some point.

Current national statistics confirm that about 85 percent of children who have liver transplants survive the first year, and most children who survive the first year go on to live into adulthood.


Q: My baby was born jaundiced. Should I be concerned?

A: Not necessarily – many babies have jaundice (yellow discoloration of the skin and whites of the eyes) in the first week or two of life. This is a different kind of jaundice from biliary atresia, and does not mean there is a liver problem.

But if the jaundice goes away and then returns, or lasts longer than 2 weeks, that's often a sign that something is wrong, and you should talk to your child’s pediatrician.

Q: What’s the long-term outlook if my child has biliary atresia?

A: Unfortunately, there are no medications that can unblock your child’s bile ducts or encourage new ducts to grow. Early surgery (Kasai portoenterostomy) and good medical care can help your child to live longer and have a better quality of life. If your child’s liver starts to fail, a liver transplant can be life saving.

Up to 66 percent of children with biliary atresia, even those who undergo the Kasai portoenterostomy, will eventually need to have a liver transplant.

Q: Can biliary atresia be detected before birth?

A: No, there is no prenatal test that can detect biliary atresia.

Q: If my child has biliary atresia, does that mean that I’m more likely to have another child with the condition?

A: No, researchers believe that biliary atresia isn’t genetic, and the condition doesn’t recur in families with any frequency. Even in identical twins, one infant may have biliary atresia and the other may not.

Q: What’s the difference between bile and bilirubin?

A: Bile is a golden yellow fluid made by your child’s liver. It’s stored in the gallbladder and passed through the common bile duct into the intestine, where it helps digest fat.

Bilirubin is a yellowish pigment that is found in bile. It is a chemical formed by breakdown of red blood cells.

Q: What causes jaundice?

A: Jaundice occurs when there is too much bilirubin in your child’s blood (hyperbilirubinemia). Because bilirubin is yellow, it causes a yellowing of the baby's skin and tissues.

Not all yellowing of the skin is jaundice, however. Some babies eat a lot of orange vegetables, such as carrots, and their skin may appear yellow or orange. In that case, the whites of the child’s eyes remain white, and blood tests show that the child’s bilirubin levels are normal.

Q: Why would there be too much bilirubin in my child’s blood?

A: The problem starts as your child’s old red blood cells are broken down. As this occurs, hemoglobin (the protein molecule in red blood cells) is changed into bilirubin and, in a healthy system, is removed by the liver. If the liver can’t remove the bilirubin, it gets backed up in the blood.


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