Management dilemmas with choledochal cysts

Context: Choledochal cysts are rare and of unknown cause. Their presentation is protean, with the classical triad of pain, jaundice, and mass rarely seen. The potential complications are serious, including pancreatitis, cholangitis, and cholangiocarcinoma. Objective: To present the current experience and evidence relating to all aspects of choledochal cysts to derive appropriate management recommendations. Methods: Review of relevant literature in the English language indexed on MEDLINE. Results: The elaboration of the classification of choledochal cysts. We describe the modes of presentation and optimal investigation and summarize the current theories on etiology and malignant transformation. The results of different management strategies are presented. Conclusions: Choledochal cysts are often detected during the investigation of nonspecific symptoms, or even incidentally detected. Magnetic resonance imaging is the best imaging modality for the diagnosis and characterization of these cysts. Complete excision and hepaticojejenostomy is the management of choice.