Satisfaction With Genital Surgery and Sexual Life of Adults With XY Disorders of Sex Development

Abstract

Disorders of sex development (DSD) are rare disorders defined as discrepancy of chromosomal, gonadal, or anatomic sex. Prenatal lack of androgens or partial androgenization results in atypical development of the genitalia in individuals with XY DSD. In the past, most XY DSD individuals with ambiguous genitalia were assigned to the female sex and often underwent early feminizing treatment, including constructive genital surgery to allow heterosexual intercourse, gonadectomy, and hormone therapy. Few outcome studies of the effect of early feminizing genital surgery on sexual satisfaction and performance among adults with XY DSD have been performed. There is increasing evidence, primarily from DSD patients, that questions the benefit of early feminization treatment. The aim of this study was to evaluate satisfaction with reconstructive genital surgery and sexual life among adults with XY DSD. A total of 57 individuals with XY DSD enrolled in the German multicenter clinical evaluation study of DSD were interviewed using a condition-specific questionnaire assessing standardized measures of satisfaction with treatment. The sexual life and sexual problems evaluated included the following: satisfaction with overall sex life, present partnership, previous sexual partners, decreased sexual desire, problems with sexual arousal, and pain during intercourse (dyspareunia). The individuals were divided into 3 subgroups: (1) XY DSD complete females (XY DSD-CF) or individuals with complete androgen insensitivity syndrome, raised as females; (2) XY DSD partially androgenized females (XY DSD-PF), raised as females; and (3) XY DSD partially androgenized males, assigned to the male gender. Some comparisons were done with a control group of normal individuals surveyed about sexual satisfaction. There was substantial dissatisfaction with overall treatment among individuals with XY DSD-PF (43.3%) and XY DSD-CF (56.2%). XY DSD-PF individuals who had feminization surgery expressed high dissatisfaction, with the functional result (47.1%) and clitoral arousal (47.4%) compared with controls. Individuals with XY DSD-PF reported a substantially higher proportion of having female sexual partners (9.1%) or partners of both sexes (18.2%) and dyspareunia (56.5%) compared with female controls. XY DSD-CF individuals had decreased sexual desire (70.6%), arousal (52.9%), and dyspareunia (56.3%) compared with controls. Most individuals with complete androgen insensitivity syndrome reported significant sexual problems compared with controls; there was decreased desire (81.8%), arousal (63.6%), and dyspareunia (70%) (all P < 0.05). These findings indicate that use of early feminizing surgery in XY DSD individuals should be avoided or minimized, and that surgical correction should be performed primarily in puberty or adulthood with the parents and patients consent. The decision of sex of rearing might not have been appropriate in some cases, and the choice of gender of rearing can be postponed in some families. Information about the disorder should be provided to DSD-affected individuals and their families in an age-appropriate and sensible manner. Patients and their families should be followed from childhood to adolescence and adulthood, and should receive multidisciplinary care with psychological support and input from other patients and families with DSD.