Biliary atresia is a blockage in the tubes (ducts) that carry a liquid called bile from the liver to the gallbladder.
Jaundice newborns - biliary atresia; Newborn jaundice - biliary atresia; Extrahepatic ductopenia; Progressive obliterative cholangiopathy
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:
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.
Other symptoms may include:
An operation called the Kasai procedure is done to connect the liver to the small intestine. The abnormal ducts are bypassed. The surgery is more successful if done before the baby is 8 weeks old.
Liver transplant may still be needed before 20 years of age in most of the cases.
Early surgery will improve the survival of more than one third of babies with this condition. The long-term benefit of a liver transplant is not yet known, but it is expected to improve survival.
Complications may include:
Call your provider if your child appears jaundiced, or if other symptoms of biliary atresia develop.
Published Date: February 24, 2022
Published By: 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.
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