Doctronic

Understanding Biliary Atresia: A Rare Liver Disease in Infants

Benjamin Seth Martinez | MD

Medically reviewed by Benjamin Seth Martinez | MD, Statpearls - Director of Clinical Content on May 6th, 2023.

Key Takeaways

  • This condition affects 1 in 12,000-15,000 newborns and occurs when bile ducts outside the liver become inflamed and scarred during fetal development, completely blocking bile flow.
  • Jaundice persisting beyond 2-3 weeks of life is the primary red flag, accompanied by clay-colored stools, dark urine, and an enlarged, firm abdomen from liver swelling.
  • The Kasai procedure (hepatoportoenterostomy) must be performed before 90 days of age for optimal results—success rates drop significantly from 80% to less than 20% after this window.
  • Without treatment, progressive liver cirrhosis develops within the first year of life, making early liver transplantation necessary by age 2-3 in most cases.
  • Children require fat-soluble vitamin supplements (A, D, E, K), special formulas with medium-chain triglycerides, and close monitoring for portal hypertension and growth failure throughout childhood.

Biliary atresia is a rare liver disease that affects only infants. It occurs when the bile ducts, which are tubes that carry a digestive fluid called bile from the liver to the small intestine, become swollen and blocked. This blockage prevents bile from reaching the intestine, causing it to build up in the liver and leading to liver damage.

Causes of Biliary Atresia

The exact cause of biliary atresia is not known, but doctors believe several factors may trigger the disease, including:

  • Genetic changes

  • Immune system problems

  • Developmental issues in the womb

  • Exposure to toxic substances

  • Viral or bacterial infections after birth

Biliary atresia is not hereditary, and babies cannot catch it from someone else. Premature girls and babies of Asian or African-American descent are at a higher risk of developing the disease.

Symptoms of Biliary Atresia

The most common symptom of biliary atresia is jaundice, which causes the baby's skin and the whites of their eyes to appear yellow. While jaundice is common in newborns, it usually goes away within 2 to 3 weeks. In babies with biliary atresia, jaundice persists longer.

Other symptoms may include:

  • Swollen abdomen

  • Gray or white stools

  • Dark urine

  • Frequent nosebleeds

  • Intense itching

Diagnosing Biliary Atresia

To diagnose biliary atresia, doctors may perform several tests, including:

  • Blood tests to check for high levels of bilirubin

  • X-rays to look for an enlarged liver and spleen

  • Ultrasound to create detailed images of the baby's organs

  • Liver scans to show if and where the flow of bile is blocked

  • Liver biopsy to examine a small sample of liver tissue under a microscope

  • Diagnostic surgery to directly view the liver and bile ducts

Understanding Biliary Atresia: A Rare Liver Disease in Infants

Treatment Options for Biliary Atresia

The most common treatment for biliary atresia is the Kasai procedure, which involves replacing the blocked bile ducts with a portion of the baby's intestine. This allows bile to drain from the liver into the intestine. The Kasai procedure has an 80% success rate when performed before the baby is 3 months old.

If the blocked bile ducts are inside the liver, medication may help remove the excess bile, and vitamin A, D, and E supplements may be prescribed. However, in most cases, a liver transplant will be necessary.

Outlook for Babies with Biliary Atresia

Babies who undergo a successful Kasai procedure may recover and lead a full, active life. However, most children with biliary atresia will require specialized medical care throughout their lives and may eventually need a liver transplant.

For more information on biliary atresia, visit:

The Bottom Line

Early recognition and surgical intervention before 3 months of age dramatically improves outcomes, though most children will eventually require liver transplantation. Any newborn with jaundice lasting beyond 3 weeks needs immediate evaluation to rule out this condition. If you notice persistent yellowing of your infant's skin or unusual stool colors, Doctronic can help connect you with pediatric specialists quickly.

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