Biliary Atresia

Biliary atresia is a rare disease affecting the bile ducts that lead from the liver to the intestine causing them to be damaged preventing bile from leaving the liver.

your quick guide to this page

what is biliary atresia?: Learn what defines biliary atresia and what its general consequences are.
fast facts: Get a quick overview of biliary atresia through statistics and bite-sized facts.
symptoms: Learn about the symptoms of biliary atresia, how to recognize them, and what to do if you experience them.
lifestyle impacts: Find out how biliary atresia impacts your lifestyle.
what can I do?: Discover management and treatment strategies for biliary atresia.
additional resources: Find useful links, education, articles, recipes and more that can help with biliary atresia.

what is biliary atresia?

Biliary atresia is a relatively rare disease that begins in early infancy and affects about one in every 10,000 to 20,000 infants. In biliary atresia, the bile duct that leads from the liver to the intestine becomes damaged preventing bile from leaving the liver.

In the early stages the bile duct outside the liver is mainly affected, but in later stages, bile ducts inside the liver are also damaged. This can lead to a build-up of bile in the liver which can be harmful. Unless bile flow can be established, liver function is gradually lost and affected children rarely survive beyond two years of age.

What is bile?

Bile is a yellow-green fluid made in the liver and stored in the gallbladder. It then flows through the common bile duct to the small intestine, where it helps with digestion and absorption of dietary fats and fat-soluble vitamins. Bile is also necessary for removing waste products and toxins from the body.

lifestyle impacts

Biliary atresia is a serious liver disease which can lead to severe liver damage and death if left untreated. Infants will require surgery as a treatment followed by special diets high in calories and fat-soluble vitamins (A, D, E, K) to reduce deficiencies. If infants are malnourished, they may experience development delays and slower growth.

what can I do?

prevention

The cause of biliary atresia is unknown. Scientists do know this condition is not inherited and some research suggests it may be the result of a viral infection early in life. Therefore prevention methods for biliary atresia are unknown.

diagnosis

Since symptoms of biliary atresia are similar to those of other liver diseases, doctors must perform many tests to have a definitive diagnosis. These tests can include:

Blood tests: Elevated liver enzymes or bilirubin levels can indicate liver dysfunction.
Ultrasound examination: An imaging test can reveal abnormalities in the liver or bile ducts.
Liver biopsy: A small sample of liver tissue may be taken to assess the extent of damage or scarring.

treatment

The main goal in treating biliary atresia is to restore bile flow from the liver and prevent further damage to the liver cells. Treatment options include:

1. Kasai Procedure (Hepatoportoenterostomy)

The Kasai procedure is the most common surgical treatment for biliary atresia. It is a surgical procedure called the Kasai procedure that replaces an infants external bile duct with a piece of their intestine. The Kasai procedure’s goal is to allow bile from the liver to drain into the intestine through the new duct. This procedure is successful about 80% of the time if performed before 3 months old.

What happens after surgery?

After the Kasai procedure, the infant should experience normal development and growth. Additional testing is often required to determine the amount of bile flow from the liver to the digestive tract. If tests show that the bile flow is normal, the child can partake in a regular balanced diet. If the tests show there is a decrease in bile flow, a low-fat diet with vitamin supplementation may be necessary since absorption of fat and fat-soluble vitamins may be reduced.

2. Liver Transplantation

In cases where the Kasai procedure does not repair liver function successfully, or if the liver becomes severely damaged, a liver transplant may be necessary. This involves replacing the diseased liver with a healthy donor liver. Liver transplantation has a high success rate and can dramatically improve the quality of life for affected children.

3. Medications and Supportive Care

In addition to surgery, infants with biliary atresia may require medications to manage symptoms and improve liver function. Nutritional support is also important, as affected children may need extra vitamins, minerals, and fats to ensure proper growth and development.

additional resources

Here are a few questions to ask your doctor or medical team:

What tests can be done to understand my child’s liver condition?
What is the status of my child’s liver? Do they have liver damage?
Will my child need to undergo the Kasai Procedure?
Will my child need a liver transplant?