In our article1, we limited our recommendation for treatment to those cases similar in nature, i.e., individuals whose only medical situation was that of having a traumatic loss of the penis postnatally. Dr Benjamin asks for advice on management for a much broader spectrum of conditions. He asks for (1) advice for those cases most likely to confront the general practice physician, (2) how this case differs from those in which individuals who are born with normal genitalia request a change of sex, and (3) if the case originally reported is only of statistical interest. We respond to these questions in reverse order.

Cases of a neonate losing his penis due to trauma are exceedingly rare. The particular case reported is notable as it was initially taken as proof for a model of treatment by which cases of ambiguous genitalia came to be managed. From the original reports of the John/Joan case, it was further extrapolated that if a typical unambiguous male could have his gender successfully changed by surgery and sex reassignment, then similarly dealing with instances of hermaphroditism and pseudohermaphroditism, regardless of origin, was appropriate. Our findings dispute this.

The John/Joan case differs fundamentally from other cases in which individuals are born with typical genitalia and request a change of sex. Such individuals are transsexuals. John/Joan was requesting a change back to his original sex. He was not going against his birth genitalia; he longed for them back. Harry Benjamin, who originally coined the term "transsexual," defined it thus:

True transsexuals (XY males and XX females with typical genitalia) feel that they belong to the other sex, they want to be and function as members of the opposite sex, not only to appear as such. For them, their sex organs, the primary (testes) as well as the secondary (penis and others) are disgusting deformities that must be changed by the surgeon's knife.2(p13)

There are no apparent reasons for such beliefs.3

The types of cases of ambiguous genitalia most likely to confront the general physician are those of hypospadic XY males and XX females with congenital adrenal hyperplasia. These individuals often have genitalia not too different from those expected of their genetic sex. In extreme cases, however, the hypospadias is great enough to suggest a female perineum and in cases of congenital adrenal hyperplasia the phallus may be so enlarged and the labia so fused as to suggest a penis and scrotum. However, many, although less common, conditions can lead to ambiguous genitalia.4

Each origin demands its own considerations. Our disagreement with previous practices was that most were painted with the same brush. In our Commentary in this issue of the Archives, we offer specific guidelines for the management of individuals with ambiguous genitalia.5 Our key principle is to have the patient participate as fully as possible, with informed consent, in any decision regarding his or her life.



1. Diamond M, Sigmundson HK. Sex reassignment at birth: long-term review and clinical implications. Arch Pediatr Adolesc Med. 1997;151:298-304.

2. Benjamin H. The Transsexual Phenomenon. New York, NY: Julian Press Inc; 1966.

3. Diamond M. Self-testing: a check on sexual identity and other levels of sexuality. In: Bullough B, Bullough VL, Elias J, eds. Gender Bending. Buffalo, NY: Prometheus Books; 1997:103-125.

4. Diamond M. Sexual identity and sexual orientation in children with traumatized or ambiguous genitalia. J Sex Res. 1997;34:199-211.

5. Diamond M, Sigmundson HK. Management of intersexuality: guidelines for dealing with individuals

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