Managing intersex

For over 40 years doctors have been in the impossible situation of making momentous decisions for intersex children, without well founded scientific principles and with little more to guide them than a personal hunch that they were doing the “right thing for the child.” Despite rapid advances in understanding sexual differentiation and increased accuracy of diagnosis, the clinical management of intersex has changed little. Recently the medical profession has been confronted by the powerfully critical voices of intersex consumer groups (www.cah.org.uk/; www.isna.org/; www.medhelp.org/www/ais). With a serious deficiency of any evidence base, emotive debates on ethics, and clinical concerns over the long term consequences of interventions, it is time to stand back and rethink every aspect of this management.^1–4

Intersex conditions consist of a blending or mix of the internal and external physical features usually classified as male or female—for example, an infant with ambiguous genitalia or a woman with XY chromosomes. Actual prevalence figures are unknown, with population estimates of 0.1% to 2%, though figures can be distorted by varying definitions of intersex.⁵ When intersex is recognised in infancy, doctors decide if the child with an intersex condition is to be raised as a boy or a girl and they recommend surgical and hormonal treatment to reinforce the sex of rearing. Core to this process is a belief in a societal binary two gender system. In the 1950s-70s, John Money gained widespread acclaim for work analysing differentiation of gender identity with intersex subjects.⁶ He stated that to achieve a stable gender identity a child must have unambiguous genitalia and unequivocal parental assurance of the chosen gender. Extrapolated into clinical management, the accepted keys to successful outcome were believed to be an active policy of withholding any details of their condition from the child and early genital surgery, before 18 months of age.⁷ Hence the current intervention of genital surgery has focused on early cosmetic appearance of the genitals rather than later sexual function.

A paternalistic policy of withholding the diagnosis is still practised by some clinicians. No objective work has analysed the widespread effects of such non-disclosure, but the impact on individual patients has been eloquently described.¹,⁸ There are more than just medicolegal reasons for abandoning non-disclosure. Most patients eventually become aware of their diagnosis through a variety of ways—from mortgage applications to television and magazine articles on intersex. Some articulate feelings of anger, distrust, and betrayal directed towards their doctors and families.⁹ Surely if a patient is going to learn the truth whatever happens, it would be more appropriate if they learnt it from their doctor and were given accurate information and appropriate psychological input. Policies of non-disclosure also prohibit access to genetic screening and the important option of peer support groups for shared learning and experiences. Once we accept that there is no place now for non-disclosure we can devote more research to appropriate ways of educating both the family and the patient, and how to tailor psychological support accordingly.

Genital surgery is one of the most controversial interventions in current intersex management. A large proportion of infants with ambiguous genitalia are raised as girls, and surgically feminising the genitalia usually involves a clitoral reduction and a vaginoplasty. In the absence of clinical trials and with minimal objective cohort studies providing data on outcomes on cosmetic, gender, social, or sexual function after this surgery, along with anecdotal evidence of dissatisfaction of adult patients with childhood surgery, both clinicians and parents face huge dilemmas. Current theories of gender development say that both prenatal factors (for example, testosterone) and postnatal factors, including the social environment, are important, and that genital appearance is less relevant.¹⁰ Clinicians, however, remain uneasy about gender development if the genitals remain uncorrected and are concerned over the possible psychological distress from bullying over different genital appearance. Recent work has shown that most children undergoing vaginoplasty will require another operation to permit use of tampons and sexual intercourse.³,⁴ The vagina is non-essential and not even visible in childhood, and most vaginal surgery should be deferred.

Conversely the clitoris is visible in childhood. An erotically important sensory organ, both the clitoris and the clitoral hood are densely innervated.¹¹ Most cosmetic clitoral surgery removes the paired clitoral corpora. The physiology of female orgasm, however, is poorly understood. It is only logical to consider that any surgery to the clitoris, which risks vascular, anatomical, or neurological compromise, could potentially alter sexual response. To date, published studies on outcomes of intersex clitoral surgery contain observer bias and non-objective assessment. None provides evidence for the assertion that adult clitoral sensation and sexual function remain undamaged by clitoral surgery.¹² Indeed it would be expected that people with intersex conditions might suffer an increased incidence of sexual dysfunction owing to the nature of their condition and the many psychological factors that impact on sexual function. Unravelling the complex interplay between surgery and psychology to understand their impact on adult sexual function remains the unconquered challenge. In the meantime, any decision regarding clitoral surgery must be taken with the knowledge of potential damage.

We need to rethink our approach to the management of intersex conditions. We must abandon policies of non-disclosure and manage patients within a multidisciplinary team. Long term follow up studies of adults with intersex conditions are crucial. However, such studies can be done only with the equal involvement of people with these conditions and of peer support groups and the cooperation of all clinicians managing intersex. It is time to create a major intersex research partnership to begin tackling these questions and move forwards towards enlightened and patient empowered care.