Biliary Atresia

Biliary atresia is a condition in which the bile ducts do not develop before birth. The purpose of the bile ducts is to drain waste products from the liver and to carry bile salts – which are necessary for fat digestion – to the small intestine. Without bile ducts, this can’t happen. This condition can lead to cirrhosis of the liver and liver damage if not treated. No one knows why bile ducts fail to develop normally.

Newborns with this condition appear normal and then develop jaundice, or yellowing of the skin and eyes, around two to three weeks of age. The infant may gain weight normally for the first month of life, and then weight loss and irritability develop with increased levels of jaundice. Stool may be clay colored and foul smelling.

In an examination, the physician may note an enlarged liver and spleen when he or she feels the child’s abdomen. He or she may order a blood test, which would show increased bilirubin in the blood, an X-ray showing an enlarged liver or spleen or an ultrasound showing the same thing. Sometimes a cholangiogram is done; this is a procedure in which dye is injected into the veins to see if blood is getting to the ducts. A liver biopsy also may be done to assess the level of damage, if any, to the liver.

An operation called the Kasai procedure is done to correct this condition. This connects the liver to the small intestine so that bile can drain. It bypasses the malformed ducts. The procedure is most successful before the baby is eight weeks old. Usually, it is only a temporary solution, and a liver transplant is eventually needed.

Early surgery improves the survival chances by more than one third.

For an appointment with a Washington University pediatric surgeon, call (314) 454-6022, Monday-Friday, 8 a.m.-5 p.m.