What is Biliary Atresia?

Biliary atresia is a rare disease of the bile ducts that affects only infants. Bile ducts are pathways that carry a digestive fluid called bile from the liver to the small intestine. Once there, it breaks down fats and absorbs vitamins. It then filters waste out of the body.

With biliary atresia, these ducts swell and become blocked. Bile is trapped in the liver, where it starts to destroy cells. Over time, the liver can be scarred -- a condition called cirrhosis. Once this happens, it can't filter out toxins the way it should.

Some babies get it in the womb. But most often, symptoms appear between 2 and 4 weeks after birth.

Causes

Doctors believe a number of things may trigger biliary atresia, including:

• Change in a gene
• Problem with the immune system
• Problem with the way the liver or bile ducts develop in the womb
• Toxic substances
• Viral or bacterial infection after birth

It isn't passed down from one family member to another, and babies can't catch it from someone else.

Girls who are born prematurely are most at risk. So are Asian and African- American babies.

Symptoms

If your baby has biliary atresia, one of the first things you'll notice is that their skin and the whites of their eyes look yellow. This is called jaundice. Jaundice is very common among infants, especially in those born before 38 weeks, but it usually goes away in 2 to 3 weeks. Jaundice caused by biliary atresia lasts longer than that.

Their abdomen also may swell, they'll have gray or white stools, and their pee will be dark. This happens because their liver can't process bilirubin -- a reddish-brown substance that's made when red blood cells break down. It's what gives poop its brown color.

Some babies also might have frequent nosebleeds or intense itching.

Diagnosis

Many liver conditions have the same symptoms as biliary atresia. To make sure they find the right cause, your child's doctor may test their blood for high levels of bilirubin. They may also do some or all of the following:

• X-rays: A small amount of radiation creates a picture that's recorded on film or a computer. This checks for an enlarged liver and spleen.
• Ultrasound: High- frequency sound waves show detailed images of their organs.
• Liver scans: Special X-rays use chemicals to create an image of their liver and bile ducts. This can show if and where the flow of bile is blocked.
• Liver biopsy: Their doctor will take a small sample of tissue so it can be looked at under a microscope. This can show if it's likely they have biliary atresia and help rule out other liver problems such as hepatitis.
• Diagnostic surgery: They'll be given medicine to make them sleep, and their doctor will make a small cut in their stomach area so they can look at their liver and bile ducts.

Treatment

The most common treatment is the Kasai procedure. It's done if the blocked bile ducts are outside the baby's liver. During the operation, your baby's surgeon will replace the blocked bile ducts with part of their intestine. This lets bile drain from their liver through the new "duct" and into their intestine.

If it's done before your baby is 3 months old, the surgery has about an 80% success rate. If it's not successful, babies usually need a liver transplant within 1 to 2 years.

If the blocked bile ducts are inside the liver, medication may help get rid of bile, and vitamin A, D, and E supplements may be prescribed. But a liver transplant probably will be needed.

Outlook

If a baby has a successful Kasai procedure, they may recover and have a full, active life. But in most cases, they'll need specialized medical care for the rest of their life. Eventually, they may also need a liver transplant.

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