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Biliary Atresia

Medically reviewed by Last updated on Jul 4, 2022.

What is biliary atresia?

Biliary atresia is a disease that damages an infant's liver before or shortly after birth. Bile ducts carry bile from your baby's liver to his or her small intestines. Bile helps digest food. When your baby's bile ducts are damaged, bile is trapped in the liver. This can cause cirrhosis (scarring), swelling, and advance to liver failure. The cause of biliary atresia is not known. Biliary atresia can be life-threatening if not treated or if treatment is delayed.

What are the signs and symptoms of biliary atresia?

Your baby may appear normal at birth. Jaundice (yellowing of skin and eyes) appears within the first 90 days of life. Your baby may also have any of the following symptoms:

  • Weight loss or poor growth
  • Discomfort or irritability
  • Foul-smelling, white, or clay-colored bowel movements
  • Dark urine

How is biliary atresia diagnosed?

  • Blood and urine tests will be done to see how your baby's liver is working. These tests will also show if your baby has any viruses that could be causing problems with his or her liver.
  • X-ray or ultrasound pictures will be used to check your baby's liver and bile ducts for blockages or damage. Contrast liquid will be given to help the bile ducts and liver show up better in the pictures. Tell the healthcare provider if your baby has ever had an allergic reaction to contrast liquid.
  • A liver biopsy is a sample of tissue taken from your baby's liver and sent to a lab for tests.

How is biliary atresia treated?

Surgery is done to bypass the damaged ducts and connect the liver to your baby's small intestine. This new duct allows bile to pass from the liver into the intestine. A liver transplant may be needed if the liver is severely damaged and does not work properly. Ask your baby's healthcare provider for more information about liver transplants.

Call your local emergency number (911 in the US) if:

  • Your baby has trouble breathing, or his or her lips and fingernails are turning blue.
  • Your baby has a seizure.

When should I seek immediate care?

  • Your baby has more jaundice (yellow skin or eyes) than before.
  • Your baby is not able to eat or drink, or is urinating less or not at all.
  • Your baby looks very weak or sleeps more than usual.
  • Your baby's vomit has blood in it.

When should I call my baby's doctor?

  • Your baby has a fever.
  • Your baby is irritable and crying more than usual.
  • Your baby's skin is itchy, swollen, or has a rash.
  • You have questions or concerns about your baby's condition or care.

Care Agreement

You have the right to help plan your child's care. Learn about your child's health condition and how it may be treated. Discuss treatment options with your child's healthcare providers to decide what care you want for your child. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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