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Intersex is a broadly defined term that usually denotes the presence of discordance of the biological aspects of sex: at least some aspect of the genitalia, internal organs, gonadal tissue, or chromosomes is more typical of the other sex, or incompletely differentiated. When the external genitalia appear to be "in between", they are described as ambiguous.[6]
In approximately 1 in 5,000 infants there is enough variation in the appearance of the external genitalia to give rise to hesitation about appropriate assignment by the physician involved. Typical examples would be an unusually prominent clitoris in an otherwise apparently normal girl, or complete cryptorchidism or mild hypospadias in an otherwise apparently normal boy. In most of these cases, a sex is tentatively assigned and the parents told that tests will be performed to quickly confirm the apparent sex. Typical tests in this situation might include a pelvic ultrasound to detect a uterus, a testosterone or 17-hydroxyprogesterone level, and/or a karyotype. In some of these cases a pediatric endocrinologist is consulted to confirm the tentative sex assignment. The expected assignment is usually confirmed within hours to a couple of days in these cases.
In a much smaller proportion of cases, the process of assignment is more complex, and involves both determining what the biological levels of sex may be as well as choosing the best sex assignment. Approximately 1 in 20,000 infants is born with enough ambiguity that assignment becomes a more drawn-out process of multiple tests and intensive education of the parents about sexual differentiation. In some of these cases, it is clear that the child will face major discordances or abnormalities of anatomy or function as he or she grows up, and deciding upon the sex of assignment is a matter of weighing the advantages and disadvantages of either assignment.