In this condition, the main trunk (the common bile duct) of the biliary tree is structurally abnormal, probably from the time of birth. Eventually, (usually by age two or three but sometimes not until adolescence or adulthood) the bile accumulates in the duct. It forms a sac or cyst which then presses on the bile duct and prevents bile from reaching the intestine. Bile backs up into the liver and the patient develops jaundice (a yellowing of the skin and eyes). Occasionally, this accumulation of bile becomes infected, causing abdominal pain and fever. In some patients the cyst can be felt by the doctor examining the abdomen.
How is choledochal cyst diagnosed?
In most patients the diagnosis can be confirmed by ultrasound or by injecting a radioactive substance which gives an "image" of the abnormal duct.
How is choledochal cyst treated?
Treatment is surgical. The abnormal bile duct is removed and a piece of intestine is used to replace it. In most cases, surgery permanently corrects the disease. Rarely, infection in the newly formed biliary tree recurs. If the condition is not correctly diagnosed, the blockage of bile may result in scarring in the liver (cirrhosis).
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