Biliary atresia is a blockage in the tubes (ducts) that carry a liquid called bile from the liver to the gallbladder. The condition is congenital, which means it is present from birth.
Causes, incidence, and risk factors:
Biliary atresia occurs when the bile ducts inside or outside the liver do not develop normally. It is not known why the biliary system fails to develop normally.
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 is deadly if not treated.
Symptoms:
Newborns with this condition may appear normal at birth. However, jaundice (a yellow color to the skin and mucous membranes) develops by the second or third week of life. The infant may gain weight normally for the first month, but then will lose weight and become irritable, and have worsening jaundice.
The health care provider will perform a physical exam, which includes feeling the patients belly area. The doctor may feel an enlarged liver.
Tests to diagnose biliary atresia include:
Abdominal x-ray
Abdominal ultrasound
Blood tests to check total and direct bilirubin levels
Hepatobiliary iminodiacetic acid (HIDA) scan, also called cholescintigraphy, to help determine if the bile ducts and gallbladder are working properly
Liver biopsy to determine severity of cirrhosis or to rule out other causes of jaundice
X-ray of the bile ducts (cholangiogram)
Review Date: 10/8/2007
Reviewed By: Deirdre O’Reilly, MD, MPH, Neonatologist, Division of Newborn Medicine, Children’s Hospital Boston and Instructor in Pediatrics, Harvard Medical School, Boston, Massachusetts. Review Provided by VeriMed Healthcare Network.
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Neonatology, Pediatric Surgery 
