We examined a case of malignant degeneration that occurred as a long-term complication of a choledochal cyst. Analysis of the literature shows the incidence of carcinoma varies with age at the initial appearance of symptoms. The child with a choledochal cyst that appears before 10 years of age carries a minimum risk (0.7%) of subsequent malignant degeneration compared with the patient in the second decade (6.8%) and older (14.3%). Data suggest that carcinoma may be readily overlooked at the time of choledochal cyst exploration. In light of the findings, an age-adjusted strategy for management should be used.
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C. Randle Voyles (1983) studied this question.
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