In the United States and in most western European societies, female rearing was most frequently recommended to parents whose infant had ambiguous genitalia. When the decision to raise the child as a female was made, surgery was usually undertaken to remove any ambiguity of the genitalia and to feminize the external appearance. This was done with the hope of a good psychosocial outcome.
Minto et al undertook a study involving individuals with several intersex conditions which included ambiguous genitalia, and who were living as adult females. Individuals were recruited from the Androgen Insensitivity Syndrome Support Group, the Adrenal Hyperplasia Network and the Intersex Clinic at University College in London Hospital.
Questionnaires were distributed and individuals could respond anonymously or identify themselves, in which case, their records would be examined with their permission. The self-administered questionnaires included the Golombok-Rust inventory of sexual satisfaction (GRISS) for women. Of the 39 patients included in this study, 11 had no clitoral surgery and 28 had had clitoral surgery. Almost all individuals who had undergone gonadectomy were taking hormone replacement therapy. Historical trends were noted in that most individuals seen before 1979 had undergone clitorectomy, while those operated on since 1980 usually underwent nerve-sparing clitoral reduction surgery. Many individuals also had vaginal reconstructive surgery.
The authors did multiple types of analysis of the data; however, the bottom line is that of the 39 participants, 13 individuals had never been sexually active and the 28 sexually active individuals had below normal scores in terms of sexual function. A low score on sensuality was evident in the clitoral surgery group when compared to the non-surgical group. Both groups had difficulty with orgasm, which is relatively rare in a sexually healthy population. Of the 28 who had clitoral surgery, 18 found it impossible to have orgasm, compared with none among those who had not had clitoral surgery.
It was difficult to determine exactly why most of the study individuals were having difficulty with sexual function because only a questionnaire was used to obtain the data. There did not appear to be a difference among those patients recruited from the clinic versus those in support groups.
It would appear that genital surgery at a young age did not lead to satisfactory gender identity and sexual activity. However, it is not clear what the most appropriate approach should be. The authors encourage debate about the ethical issues, the development of reliable information, support of research in this area and how important it is to share this information with parents and patients who are considering clitoral surgery.
Minto CL et al. Lancet 2003;361:1252-1257.
First Editor’s Comment: The outcomes of the management of intersex are not perfect. This study following up on previously treated individuals suggests that clitorectomy does not lead to sexual satisfaction, however, neither does clitoral reduction. Clearly, more research and discussion are needed in this area.
Judith G. Hall, OC, MD
Second Editor’s Comment: As the authors acknowledge, interpretation of their study is hampered by the small number of study subjects and the possibility that those electing to participate were among the more dissatisfied patients contacted initially. Quite interesting are the data that indicate that clitoromegaly itself is associated with sexual dysfunction. In addition to the concept that clitoral recession will permit the child to more readily accept her female sex assignment, the procedure is performed to ease parental acceptance of their newborn child. Those who have dealt on a personal and daily basis with parents of children with ambiguous genitalia know the need to assure and reassure parents is a paramount goal which is difficult to attain. Early clitoral recession by a skilled surgeon is most often recommended by this writer in those neonates with more severe degrees of genital ambiguity.
Because of widespread neonatal screening for CAH, there is an increasing number of females with the most severe form of genital ambiguity known as Prader V or complete incorporation of the urethra into the phallus/clitoris. In the opinion of this writer and many others it is inappropriate to rear these genotypic and potentially fertile girls as males, thus necessitating genital surgery. Since both clitoromegaly and clitoral surgery impede sexual satisfaction, the challenge is to devise a corrective procedure that does not do so.
It would have been of interest to learn whether in those women with ambiguous genitalia who did not undergo clitoral surgery, clitoromegaly during childhood and young adulthood was a matter of significant concern. Counseling girls with ambiguous genitalia, whether operated upon or not, needs to begin in mid-childhood and to be conducted by individuals skilled in the management of this problem, as mentioned by Slijper in an excellent commentary regarding this article, in the same issue of Lancet (2003;361:1236-1237).
Minto’s article also provides further support for the antenatal treatment with glucocorticoids of women bearing female CAH offspring at risk for development of ambiguous genitalia. It will be of great interest to assess the psychosexual development, orientation, and sexuality of these subjects as adult women. With the observations collected to date the impression is that they are normal little girls.
Allen W. Root, MD
Third Editor’s Comment: The topic of intersex management, outcome, and research has received much attention in the past 2-3 years. The reader should be aware of publication of a collection of excellent papers presented in May 2002 at a conference entitled "Genetic and Hormonal Basis of Sexual Differentiation Disorders" (The Endocrinologist 2003;13:175-287) and of a "Summary of a Research Workshop on Intersex" held in sequence with the above conference (to be published in The Endocrinologist). Furthermore an excellent review entitled "Management of Children with Intersex Conditions: Psychological and Methodological Perspectives" by S. Berenbaum was presented in GGH 19:1.
Robert M. Blizzard, MD