Epidemiology, presentation, diagnosis, and outcomes of choledochal cysts in adults in an urban environment

Background Choledochal cysts (CDC) are rare congenital cystic lesions of the biliary tract. In North America the incidence of CDC is estimated as 1/150,000; it is not clear that the disease pattern in North America is similar to that in Asia. Methods Retrospective chart review. Statistical analysis was under taken using Fisher’s exact test. Results Presentation, epidemiology, diagnosis, and outcome were evaluated in 51 patients with CDC. Malignant transformation was identified in 4 patients presenting uniformly with jaundice (P = .027). Type 4a cysts (54.9%) were the most common cyst identified. Four (14%) type IVa and two (13%) type I cysts developed postoperative stricture. No patient developed cholangiocarcinoma after complete resection of their cyst. Conclusions Types I and IVa cysts can be treated similarly with excellent outcome. However, our observation of a high proportion of type 4a cysts may represent a specific North American pattern of this disease requiring a re-evaluation of the classification system.