Biliary atresia is a blockage in the small tubes (ducts) that carry bile from the liver to the small intestine. Bile is a fluid that helps the body break down the fat in food. Blockage of the ducts can damage the liver. If this disease is not treated, the liver will stop working.
This is a rare, life-threatening condition that can affect babies soon after birth.
What is the cause?
The cause of biliary atresia is not known. Some possible causes may be:
The bile ducts do not form properly before birth.
A viral infection or problem with the body’s immune system after birth damages the ducts.
What are the symptoms?
Symptoms may include:
Yellowing of the skin and eyes (jaundice) after 2 weeks of age that does not go away by 4 weeks of age
Pale, clay-colored bowel movements
How is it diagnosed?
Biliary atresia is usually diagnosed during the first 2 months of life. Tests may include:
Blood and urine tests
Tests of bowel movement
An ultrasound, which uses sound waves to show pictures of the liver and bile ducts
Nuclear scan, which uses a tiny amount of radioactive chemical injected into a vein to show how well bile flows from the liver to the small intestine
Liver biopsy, which is the removal of a small sample of tissue for testing
How is it treated?
Biliary atresia can be treated with 2 types of surgery:
Kasai procedure: The damaged ducts are removed and replaced with a loop of intestine so the bile can flow from the liver into the intestine.
Liver transplant: The baby is given a liver from another baby who has died or part of a liver from an adult.
Even after a successful Kasai procedure, most babies with biliary atresia slowly develop scarring of the liver (cirrhosis) and need a liver transplant by adulthood.
Your childâ€™s healthcare provider may recommend changes in your babyâ€™s diet or vitamin supplements.
How can I take care of my child?
Follow your childâ€™s healthcare provider’s instructions. Ask your provider:
How and when you will hear your childâ€™s test results
How long it will take for your child to recover
How to take care of your child at home
What symptoms or problems you should watch for and what to do if your child has them
Make sure you know when your child should come back for a checkup.
Developed by RelayHealth.
Pediatric Advisor 2015.1 published by RelayHealth. Last modified: 2013-04-12 Last reviewed: 2014-04-25
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
â€œTests for liver, gallbladder, and biliary disorders.â€ www. merckmanuals.com/home/liver_and_gallbladder_disorders/diagnosis_of_liver_gallbladder_dand_biliary_disorders/tests_for_liver_gallbladder_and_biliary_disorders.html.
Nelson Textbook of Pediatrics, 19th ed. Robert M. Kliegman, MD, Bonita M.D. Stanton, MD, Joseph St. Geme, Nina Schor, MD, PhD and Richard E. Behrman, MD. Elsevier. 2011.