In this condition, the main trunk (the common bile duct) of the biliary tree is structurally abnormal, probably from the time of birth. Usually by the age of 2 or 3 years the bile begins to collect in the duct. It forms a sack or cyst which then presses on the bile duct and may prevent bile from reaching the intestine. Bile can back up into the liver and the patient becomes jaundiced (yellow). Occasionally the accumulation of bile becomes infected causing abdominal pain and fever.
The classic symptoms of abdominal mass, pain and jaundice is rarely seen during childhood. Some children may not show symptoms for years. In some patients, the cyst can be felt by the doctor examining the abdomen. Blood studies such as a serum amylase and liver function tests may be drawn during acute episodes of pain. In most patients the diagnosis can be confirmed by using Ultrasound pictures or by injecting a radioactive substance and performing a CT scan which gives an "image" of the abnormal duct.
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 may occur in the newly formed bile duct. If the choledochal cyst is not correctly diagnosed, blockage of bile may result in scarring of the liver (cirrhosis). If left untreated, choledochal cysts may develop into cancer.
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