Biliary atresia is a blockage in the tubes (ducts) that carry a liquid called bile from the liver to the gallbladder.
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Biliary atresia occurs when the bile ducts inside or outside the liver are abnormally narrow, blocked, or absent. The bile ducts carry a digestive fluid from liver to small bowel to break down fats and to filter out waste from the body.
The cause of the disease is not clear. It may be due to:
Viral infection after birth
Exposure to toxic substances
Multiple genetic factors
Some medicines such as carbamazepine
It more commonly affects people of East Asian and African-American descent.
The bile ducts help remove waste from the liver and carry salts that help the small intestine break down (digest) fat.
In babies with biliary atresia, bile flow from the liver to the gallbladder is blocked. This can lead to liver damage and cirrhosis of the liver, which can be deadly.
Symptoms usually start to occur between 2 to 8 weeks. Jaundice (a yellow color to the skin and mucus membranes) develops slowly 2 to 3 weeks after birth. The infant may gain weight normally for the first month. After that point, the baby will lose weight and become irritable, and will have worsening jaundice.
Berlin SC. Diagnostic imaging of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 38.
Cazares J, Ure B, Yamataka A. Biliary atresia. In: Holcomb GW, Murphy JP, St. Peter SD, eds. Holcomb and Ashcraft's Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier; 2020:chap 43.
Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC. Cholestasis. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 383.
O'Hara SM. The pediatric liver and spleen. In: Rumack CM, Levine D, eds. Diagnostic Ultrasound. 5th ed. Philadelphia, PA: Elsevier; 2018:chap 51.
Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.