For Patients For Caregivers For Medical Professionals

HELPLINE 1-800-465-4837
Mon-Fri 9am – 7pm

Biliary Atresia

Biliary atresia is a disease of the bile ducts that affects only infants. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the small intestine, where it helps digest fats.

In biliary atresia, the bile ducts become inflamed and blocked soon after birth. This causes bile to remain in the liver, where it starts to destroy liver cells rapidly and cause cirrhosis, or scarring of the liver.

Healthy Liver

HEALTHY

FIBROSIS

CIRRHOSIS

CANCER

The Healthy Liver

A healthy liver has the amazing ability to grow back, or regenerate when damaged.

Fibrosis

When treated successfully at this stage, there’s a chance your liver can heal itself.

Liver Cancer

Cirrhosis and hepatitis B are leading risk factors for primary liver cancer.

Liver Failure

Liver failure is a life-threatening condition that demands urgent medical care.

There are many different types of liver disease. But no matter what type you have, the damage to your liver is likely to progress in a similar way.

Whether your liver is infected with a virus, injured by chemicals, or under attack from your own immune system, the basic danger is the same – that your liver will become so damaged that it can no longer work to keep you alive.

Cirrhosis, liver cancer, and liver failure are serious conditions that can threaten your life. Once you have reached these stages of liver disease, your treatment options may be very limited.

That’s why it’s important to catch liver disease early, in the inflammation and fibrosis stages. If you are treated successfully at these stages, your liver may have a chance to heal itself and recover.

Talk to your doctor about liver disease. Find out if you are at risk or if you should undergo any tests or vaccinations.


What causes biliary atresia?

The cause of this disease is not known. In some infants, the condition is most likely congenital, meaning present from birth. About one in 10 babies with biliary atresia have other congenital defects. Some research indicates that an early viral infection may be linked to biliary atresia.

Scientists do know that biliary atresia is not hereditary; parents do not pass it on to their child. It is also not contagious, and it is not preventable. It is also not caused by anything an expectant mother did or did not do.


What are the symptoms of biliary atresia?

Symptoms of biliary atresia usually appear between two and six weeks after birth. The baby will appear jaundiced, with yellowing of the skin and whites of the eyes. The liver may harden and the abdomen may become swollen. Stools appear pale grey and the urine may appear dark. Some babies may develop intense itching.


How is biliary atresia diagnosed?

Since other conditions lead to symptoms similar to those of biliary atresia, doctors must perform many tests before a conclusive diagnosis can be made. These tests may include blood and liver tests, an ultrasound examination, X-rays and a liver biopsy, in which a small amount of liver tissue is removed with a needle for examination in a laboratory.


How is biliary atresia treated?

Unfortunately, there is no cure for biliary atresia. The only treatment is a surgical procedure in which the blocked bile ducts outside the liver are replaced with a length of the baby’s own intestine, which acts as a new duct. This surgery is called the Kasai procedure after Dr. Morio Kasai, the Japanese surgeon who developed it.

The aim of the Kasai procedure is to allow drainage of bile from the liver into the intestine through the new duct. The operation is completely or partially successful about 80 percent of the time if performed early (before 3 months of age). In babies who respond well, jaundice and other symptoms usually disappear after several weeks.

In cases where the Kasai procedure does not work, the problem often lies in the fact that blocked bile ducts are “intrahepatic,” or inside the liver, as well as extrahepatic, or outside the liver. No procedure, except for liver transplant, has been developed to replace blocked intrahepatic ducts.

The Kasai procedure is most successful in babies younger than 3-months-old, so early diagnosis is important.

If the Kasai procedure is not successful, the only other option is a liver transplant. However, a suitable donor organ must be found quickly, before damage to the liver from the backed-up bile becomes deadly.


What happens after surgery?

The aim of treatment after surgery is to encourage normal growth and development. If bile flow is good, the child is given a regular diet. If tests show that bile flow is reduced, a low-fat diet and vitamin supplements will be required, since the absorption of fats and vitamins is impaired.


What happens after surgery?

The aim of treatment after surgery is to encourage normal growth and development. If bile flow is good, the child is given a regular diet. If tests show that bile flow is reduced, a low-fat diet and vitamin supplements will be required, since the absorption of fats and vitamins is impaired.


What happens to children with biliary atresia?

Without successful treatment, few children with biliary atresia live beyond age two. In some cases, where the Kasai procedure is completely successful, the child may recover and live a normal life. However, in most cases, even when surgery is successful, patients will suffer gradual damage to the liver. These children will need specialized medical care throughout their lives, and many will ultimately need a liver transplant.

  • Have you ever treated any other patients with biliary atresia before?
  • Have blood tests be performed to look at my bilirubin levels?
  • What is the status of my child’s liver?
  • Does my child have liver damage?
  • Will my child need a liver transplant?
  • (If transplant is an option) Would it be best to pursue a living donation (from a friend or family member) or would we to wait for a deceased donor?
  • What transplant hospitals are there in my state/UNOS region?
  • Will the Kasai Procedure be needed to be done?
  • Has the provider doing this procedure done this on many other patients?
  • Is there any medication that can help with the portal hypertension?
  • Is there any medication that can help with the pruritis?

Support Groups Near You

Visit the American Liver Foundation support group page here to find the closest support group to you.


Online Support Groups

Visit the American Liver Foundation online support group at Inspire…

Biliary Atresia Support Group on Facebook
Visit the American Liver Foundation Biliary Atresia support group on Facebook. For more details, click here…

Aiden

Aiden

Biliary Atresia
Annistyn K.

Annistyn K.

Biliary Atresia
Anoura  A.

Anoura A.

Biliary Atresia
Danielle

Danielle

Biliary Atresia
Domenic F.

Domenic F.

Biliary Atresia
Elise's Story

Elise’s Story

Biliary Atresia
Gavin S.

Gavin S.

Biliary Atresia
Hannah S.

Hannah S.

Biliary Atresia
Jax S.

Jax S.

Biliary Atresia
Jeffrey D.

Jeffrey D.

Biliary Atresia
Josh D.

Josh D.

Biliary Atresia
Kristen H.

Kristen H.

Biliary Atresia
Matt R.

Matt R.

Biliary Atresia
Meiyee Chen

Meiyee Chen

Biliary Atresia
Millie T.

Millie T.

Biliary Atresia
Natalie Williams

Natalie Williams

Biliary Atresia
Nick W.

Nick W.

Biliary Atresia
Noel R.

Noel R.

Biliary Atresia
Tran T.

Tran T.

Biliary Atresia
Xander

Xander

Biliary Atresia

 

Read Annistyn's Story
A Patient's Biliary Atresia Story

Suggested Reading


Share this page
FacebooktwitterlinkedinmailFacebooktwitterlinkedinmail

Visit Us On FacebookVisit Us On TwitterVisit Us On LinkedinVisit Us On YoutubeVisit Us On Instagram