Hirvikoski T, Nordenström A, Wedell A, Ritzén M, Lajic S. J Clin Endocrinol Metab. 2012 Mar 30

DOI: 10.1210/jc.2012-1222

Abstract 

Background:Prenatal deficit of androgens or androgen action results in atypical genitalia in individuals with XY disorders of sex development (XY,DSD). XY,DSD include mainly disorders of gonadal development and testosterone synthesis and action. Previously, most XY,DSD individuals were assigned to the female sex. Constructive genital surgery allowing heterosexual intercourse, gonadectomy, and hormone therapy for feminization were often performed. However, outcome studies are scarce.Objective:Our objective was evaluation of satisfaction with genital surgery and sexual life in adults with XY,DSD.

Design and Methods:We evaluated 57 individuals with XY,DSD from the German multicenter clinical evaluation study with a condition-specific questionnaire. The individuals were divided into subgroups reflecting the absence/presence of partial androgen effect or genital constructive surgery.

Results:Dissatisfaction with function of the surgical result (47.1%) and clitoral arousal (47.4%) was high in XY,DSD partially androgenized females after feminization surgery. Dissatisfaction with overall sex life (37.5%) and sexual anxieties (44.2%) were substantial in all XY,DSD individuals. Problems with desire (70.6%), arousal (52.9%), and dyspareunia (56.3%) were significant in XY,DSD complete females. 46,XY partially androgenized females reported significantly more often partners of female (9.1%) or both sexes (18.2%) and dyspareunia (56.5%) compared with controls. Individuals with complete androgen insensitivity syndrome stated significant problems with desire (81.8%), arousal (63.6%), and dyspareunia (70%).

Conclusions:Care should be improved in XY,DSD patients. Constructive genital surgery should be minimized and performed mainly in adolescence or adulthood with the patients’ consent. Individuals with DSD and their families should be informed with sensibility about the condition. Multidisciplinary care with psychological and nonprofessional support (parents, peers, and patients’ support groups) is mandatory from child to adulthood.

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Summary 

This study examined the adult sexual function and satisfaction of 57 patients affected with XY disorders of sex development (XY,DSD). Long-term follow-up studies of the psychosocial or psychosexual adaptation of persons receiving medical and surgical care many years earlier are filled with uncertainty with regard to etiologic interpretations. In this study, Köhler et al. report that a large proportion of this cohort of German patients expressed dissatisfaction with overall treatment, and dissatisfaction with both the cosmetic and functional results of genital surgery. Reported sexual problems were high, and this was more pronounced in the group having received surgery compared with those who had not. Patient attributions regarding those aspects of care contributing to poor outcomes notwithstanding, limited details of early treatment and related experiences leave etiologic explanations speculative. No influence of patient age or year of surgery on satisfaction with cosmetic or functional results of surgery was found, thereby bringing into question the general proposition that improved surgical techniques in more recent years will necessarily deliver higher patient satisfaction; might there be other factors in these patients’ backgrounds or experiences which are equally or more likely to influence complex constructs such as ‘satisfaction’ than surgical results alone?

A high proportion of patients with XY,DSD with partial masculinization who were raised as girls reported a homosexual or bisexual orientation (i.e. attraction to women or women and men). Although the investigators attributed this outcome to the influence of early androgen exposure, this interpretation is necessarily speculative because no other predictors of non-heterosexual orientation were simultaneously tested. The accompanying finding that XY women with complete absence of prenatal androgen exposure were, by and large, heterosexual does not exclude the role for important social experiences in shaping the expression of sexual orientation.

The authors conclude that, while early genital surgery might have been the cause of some sexual problems, other factors, such as “feeling different due to DSD, especially fears and shame of having an abnormal appearance of the genitalia, gonadectomy, infertility, lifelong sex hormone substitution, lack of androgen action, and secrets about the condition and lack of professional and nonprofessional psychological support” may have been influential in the case of negative outcomes.

What is urgently needed are studies that simultaneously incorporate both biological and social-environmental variables in statistical models predicting multi-faceted psychosocial/sexual outcomes. International efforts at operationalizing the principles of clinical management articulated in the DSD Consensus Statement {1}, together with the formation of patient registries, will hopefully yield data sets that better reflect both the biologic and social factors impinging on these patients and their families.

References 

1. Consensus statement on management of intersex disorders. International Consensus Conference on Intersex.
Lee PA, Houk CP, Ahmed SF, Hughes IA, International Consensus Conference on Intersex organized by the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology Pediatrics. 2006 08 01; 118(2): e488-500
PMID: 16882788 DOI: 10.1542/peds.2006-0738

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