Biliary Atresia

Biliary atresia is a blockage in the tubes (ducts) that carry bile (a digestive liquid that helps break down fats) from the liver to the gallbladder and small intestine. Babies with biliary atresia are born with underdeveloped or blocked bile ducts. This means that bile from the liver is unable to reach the intestine, where it normally works to help digest food. Without treatment, bile builds up, causing injury to the liver, and develops into liver failure within the first two years of life.

Biliary atresia is a congenital condition, which means it is present from birth.

Newborns with this condition may appear normal. Jaundice (yellowing of the skin and whites of the eyes) develops by the second or third week of life. The infant may gain weight normally for the first few weeks. Other symptoms include:

Enlarged liver
Enlarged spleen
Dark urine
Jaundice (yellow color in the whites of the eyes)
Pale or clay-colored stools
Slow weight gain

Diagnosis of Biliary Atresia

If your child’s symptoms lead the pediatric gastroenterologist to suspect biliary atresia, he or she will perform a physical exam, which includes feeling your child's abdomen (stomach). Additional tests will be performed to help make a diagnosis and rule out other possible problems, including:

Ultrasound. An abdominal ultrasound looks at the liver and bile ducts and produces pictures.
Blood tests. A blood test checks levels of bilirubin. Bilirubin is a bile pigment. Elevated levels of bilirubin cause jaundice. Other blood tests will measure injury to the liver, function of the liver and investigate possible causes.
Nuclear medicine. A hepatobiliary iminodiacetic acid (HIDA) scan is a radiology imaging test that helps determine if the bile ducts and gallbladder are working properly. A small amount of harmless radioactive dye is injected through the child's vein. A pediatric radiologist will watch to see if the dye appears in the small intestine. If so, the ducts are working properly and biliary atresia can be ruled out. If the dye does not appear, then further tests will be done to confirm a diagnosis of biliary atresia. Sometimes, follow-up X-rays are done 24 hours after the HIDA scan, so you may be asked to return the following day.
Liver biopsy. A liver biopsy may be done to determine whether findings of biliary atresia are present or to rule out other causes of jaundice. While your child is sedated, a pediatric radiologist will insert a needle through the skin and into the liver to obtain a very small piece of liver tissue. The sample will be sent to a lab and analyzed.

Treatments

A multidisciplinary approach is used to manage and treat biliary atresia, including surgery and special nutrition. Treatments include:

Kasai procedure. The Kasai procedure is a surgery that connects the liver to the small intestine. This operation bypasses the abnormal ducts. It is most successful if done before your baby is 8 weeks old.
Liver transplant. Even if a Kasai procedure is successful, many children continue to have scarring of the liver and may eventually experience liver failure. If this happens, a liver transplant may be needed sometime before adulthood.

Key Points to Remember

Biliary atresia is a condition that is present from birth and involves a blockage in the bile ducts that connect the liver and the small intestine.
Babies born with biliary atresia may appear and act normal for the first few weeks of life.
Jaundice often develops in the second or third week of life and is often accompanied by pale or clay-colored stools.
Surgery (Kasai operation) is most successful if done before your baby is 8 weeks old.
Cirrhosis and the need for a liver transplant may develop before adulthood.

Support Services & Resources

Visit the websites below to find support groups and services and learn more about biliary atresia.

Riley at IU Health offers a broad range of supportive services to make life better for families who choose us for their children's care.

Learn More About Riley Support Services

GIKids

The North American Society for Pediatric Gastroenterology, Hepatology and Nutrition provides patient outreach and education for children and families living with biliary atresia.

The Childhood Liver Disease Research Network

This website offers information about current research on childhood liver diseases, including biliary atresia.

Biliary Atresia Research

The Gastroenterology, Hepatology & Nutrition Department at Riley at IU Health participates in the Childhood Liver Disease Research Network, a multicenter research network that investigates childhood liver conditions, including biliary atresia. Talk to your child’s doctor if you want to know more about this research or if you have questions about your child participating in one of these studies.

Locations

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