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What Is Biliary Atresia?

Biliary atresia is a rare and serious liver disease that affects newborn babies. In this condition, the bile ducts, which are the tubes that carry bile from the liver to the gallbladder, are blocked or missing. Bile is a liquid that helps the body break down fat. When the bile is blocked, it builds up in the liver and causes damage.

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What Is Biliary Atresia?

Biliary atresia is a rare and serious liver disease that affects newborn babies. In this condition, the bile ducts, which are the tubes that carry bile from the liver to the gallbladder, are blocked or missing. Bile is a liquid that helps the body break down fat. When the bile is blocked, it builds up in the liver and causes damage.

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What Causes Biliary Atresia?

Doctors do not know the exact cause of biliary atresia. It is not a hereditary disease, which means it is not passed down from parents. It is also not contagious and is not caused by anything the mother did during pregnancy. It is a rare problem that happens very early in life, but it is not clear why.

What Are the Symptoms?

The most common symptom is jaundice, which is a yellow color of the skin and the whites of the eyes. Many newborns have mild jaundice that goes away. Jaundice from biliary atresia is different. It starts after 2 weeks of age and gets worse. Other main signs are very pale, clay-colored stools (poop) and dark-colored urine.

How Is Biliary Atresia Diagnosed?

A doctor will do blood tests to check the baby's liver. They will also do an ultrasound scan of the baby's belly. A special scan, called a HIDA scan, may be used to see if bile is flowing from the liver. The only way to be 100% sure is with surgery. A surgeon will do a test called a cholangiogram to look at the bile ducts.

How Is Biliary Atresia Treated?

This condition must be treated with surgery. The main surgery is called the "Kasai procedure." A surgeon removes the blocked bile ducts and attaches a part of the baby's own intestine directly to the liver. This creates a new path for the bile to drain out. This surgery is most successful if it is done in the first 3 months of the baby's life.

FAQs on Biliary Atresia

Is the Kasai procedure a cure?

No. The Kasai procedure is a treatment, not a cure. It allows the baby to grow and stay healthy for many years, but the liver damage may continue. Many children who have this surgery will still need a liver transplant later in life.

Did I do something to cause this?

No. Biliary atresia is not caused by anything you did or did not do during your pregnancy. It is a rare condition that doctors are still researching.

Is my baby in pain?

The condition itself is not usually painful for the baby. They may be fussy or irritable, but they are not in constant pain.

When to See Your Doctor

You must see a doctor immediately if your baby is still jaundiced (has yellow skin or eyes) after 2 weeks of age. You should also call your doctor right away if your baby's stools are ever pale, white, or a light clay color. Early diagnosis is the most important factor in a good outcome.