It is estimated that intersex conditions occur
in one per 2,000 live births. In the past, treatment had been based on the
assumption that infants were gender neutral at birth, and that assignment
of sex of rearing in early years which is reasonably compatible with the
appearance of the external genitalia would provide a normal gender
identity and partner orientation in adulthood. Subsequently, it has been
recognized that there is a complex interaction between prenatal and
postnatal factors that lead to the development of gender and sexual
identity.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
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