Choledochal Cysts are a congenital abnormality that usually presents in childhood. They are cysts that grow on or around the bile duct and causes abnormal enlargement. Different types of chledochal cysts can be found depending on the location within the bile duct.

Adult presentation is rare and usually exhibits one or more severe complications. Frequently, adults with choledochal cysts complain of vague pain in the upper middle region or right upper quadrant and can develop jaundice or cholangitis. The most common symptom in adults is abdominal pain. A classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass are symptoms found in only 10 percent to 20 percent of patients.

Adults with cysts are especially predisposed to the development of cholangiocarcinoma, with the occurrence increasing from 0.7% in the first decade of life to >14% after the age of 20 years. Because of the risk of malignant change in choledochal cysts, the ideal treatment is surgical removal.