Choledochal cyst disease is uncommon. The presentation of the disease is being seen more commonly in the adult population than in the pediatric population, particularly in the West, making this a diagnosis a general surgeon should consider when evaluating a patient with biliary disease. The diagnosis of this disease has been greatly facilitated by improving technologies, particularly MRCP and interventional techniques of PTC. The consequences of not treating choledochal cysts can result in malignant transformation. When possible, complete surgical excision of the cyst is recommended and should be performed as early as possible to prevent complications and the progression of liver fibrosis . Long-term follow-up is required for surveillance for late complications and for cancer, particularly in type IV and V choledochal cysts where complete excision is not possible.
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