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Definition Return to top
Biliary atresia is an obstruction of the bile ducts caused by their failure to develop normally in the fetus. This is a congenital condition (present at birth).Causes, incidence, and risk factors Return to top
Biliary atresia is caused by the abnormal development of the bile ducts inside or outside the liver. The purpose of the biliary system is to remove waste products from the liver, and to carry bile salts necessary for fat digestion to the small intestine.
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, if not treated, will eventually be fatal.
Newborns with this condition may appear normal at birth but jaundice develops by the 2nd or 3rd week of life. The infant may gain weight normally for the 1st month, then weight loss and irritability develop accompanied by increasing levels of jaundice. It is not known why the biliary system fails to develop normally.
Symptoms Return to top
Signs and tests Return to top
During a physical examination, the doctor may detect an enlarged liver.
Tests that reveal biliary atresia include:
Treatment Return to top
An operation called the Kasai procedure is done to connect the liver to the small intestine, bypassing the malformed ducts. It is most successful if performed before the baby is 10 to 12 weeks old. However, a liver transplant may still ultimately be required.
Expectations (prognosis) Return to top
Early surgical intervention will improve the survival of more than one-third of those affected by the condition. The long-term benefit of liver transplant is not yet known, but is expected to improve survival.
Complications Return to top
Calling your health care provider Return to top
Call your health care provider if your child appears jaundiced, or if other symptoms suggestive of this disorder develop.
Update Date: 1/12/2003 Updated by: Andrew J. Muir, M.D., M.H.S., Division of Gastroenterology, Duke University Medical Center, Durham, NC. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |