Choledochal cyst

Abstract Choledochal cysts (CCs) in children are summarized according to aetiology, classification, treatment, and management issues, including complications and choice of operative approach. The types of CCs (cystic, fusiform, forme fruste) are defined according to their structure and the presence of pancreatico-biliary malunion, and how the type of CCs influences symptoms, signs, and treatment in different age groups of children is discussed. As surgical intervention relies increasingly on minimally invasive techniques, the importance of understanding open complete excision for effective surgical cure is emphasized to allow effective surgical cure according to symptoms, age, and type of CCs. Customizing of the Roux limb and the role of intraoperative endoscopy for clearing ducts of debris are presented as practical manoeuvres for improving post-operative outcome. Limitations of endoscopic treatment and the impact of concurrent intrahepatic bile duct dilatation are current controversies, as is how to manage an asymptomatic CC patient, because the classic indication for surgical intervention is symptomatic relief. Given the known risk of malignant transformation, there is a treatment dilemma with respect to asymptomatic patients.