Biliary atresia

Definition of Biliary atresia

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.

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.

Signs and tests

The health care provider will perform a physical exam, which includes feeling the patient’s belly area. The doctor may feel an enlarged liver.

Treatment

An operation called the Kasai procedure is done to connect the liver to the small intestine, going around the abnormal ducts. It is most successful if done before the baby is 8 weeks old. However, a liver transplant may still be needed.

Expectations (prognosis)

Early surgery will improve the survival of more than a third of babies with this condition. The long-term benefit of liver transplant is not yet known, but is expected to improve survival.

Review

Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 11/2/2009

Bile produced in the liver

ADAM Medical Encyclopedia

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