Treatment is dependent on the type of bile duct cyst. Because of a 20-fold increased risk of cholangiocarcinoma in Type I cysts, surgical resection and Roux-en-Y hepaticojejunostomy anastomosis is the treatment. Cholecystectomy is also performed at this time. The treatment of Type II cysts is excision of the diverticulum. Type III is associated with a low risk of malignancy, so excision is not necessary. Endoscopic sphincterotomy may be performed if the patient develops occasional jaundice or cholangitis.

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