Epidemiology

CS occurs rarely, with less than 250 patients reported in the scientific literature. The disease mainly affects young adults. Among the largest case series, the peak ages of onset range from 22 to 32 years of age (17-21); however, CS has been described in children as young as four years old and in elderly patients. Males and females are affected in equal proportions. Most of the reported cases of CS have occurred in Caucasians, but cases have been described infrequently in African Americans.

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