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 progression to liver failure within the first two years of life.

Causes

The condition is congenital, which means it is present from birth.

Symptoms

Newborns with this condition may appear normal at birth. However, jaundice (yellowing of the skin) develops by the second or third week of life. The infant may gain weight normally for the first month, but then will lose weight, become irritable and have worsening jaundice.

Other symptoms may include:

• Enlarged liver
• Enlarged spleen
• Dark Urine
• Pale or clay-colored stools
• Slow or no weight gain
• Slow growth

Exams and Tests

If your pediatrician suspects biliary atresia, he or she will refer you to a pediatric gastroenterologist. You will likely be seen by a pediatric GI specialist who specializes in liver diseases.

The doctor will perform a physical exam, which includes feeling the patient's belly area. The doctor may feel an enlarged liver or spleen. He or she will order several tests to diagnose biliary atresia and rule out other possible problems.

Abdominal Ultrasound

Looks at liver and bile ducts; real-time images show blood flow.

Blood Tests

Checks levels of bilirubin in the liver and elsewhere. Bilirubin is a bile pigment. Elevated levels of bilirubin cause jaundice.

Hepatobiliary Iminodiacetic Acid (HIDA) Scan

This radiology imaging test helps determine if the bile ducts and gallbladder are working properly. A small amount of 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 doesn't 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 is done to determine the severity of cirrhosis or to rule out other causes of jaundice. Your child will receive sedation medications or a general anesthetic before the biopsy. These medications will prevent your child from feeling pain or anxiety or remembering the procedure. An area on the right side of your child's abdomen or mid-upper abdomen will be cleansed with an antibacterial soap. The biopsy site will then be numbed with an injection of lidocaine (numbing medicine). The pediatric gastroenterologist 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. The doctor will apply a small pressure dressing (bandage) to the site.

After the biopsy your child will stay in or return to his/her room for four hours, when a repeat blood count is checked. (Children less than one year are observed overnight.) He or she should lie on the right side or back, depending on the biopsy site, for two hours. Being in bed will reduce the chance of bleeding after the biopsy. Once your child is awake and alert, he/she will be allowed to drink fluids. If fluids are tolerated, a regular diet can be restarted. For the remainder of the day, your child will need to participate in quiet activity. The results of the liver biopsy are usually available two weeks after the procedure.

Treatment

An operation called the Kasai procedure is done to connect the liver to the small intestine, going around the abnormal ducts. It is most successful if done before the baby is eight weeks old. Even with a successful surgery, many children continue to have scarring of the liver and will eventually experience liver failure and require a liver transplant sometime before adulthood.

Research

The Pediatric Gastroenterology/Hepatology/Nutrition division participates in a multi-center research study investigating biliary atresia. Talk to your child’s doctor if you would like to know more about the study or if you have questions about your child participating.

For More Information

American Liver Foundation - liverfoundation.org

Biliary Atresia Research Consortium - barcnetwork.org

North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition - NASPGHAN.org

The Childhood Liver Disease and Education Network - childrennetwork.org