Sexual function in women with complete androgen insensitivity syndrome.

OBJECTIVE: To investigate sexual function in women with complete androgen insensitivity syndrome (CAIS) and to investigate the prevalence of factors that might contribute to sexual difficulties. DESIGN: Cross sectional survey and clinical examination. SETTING: Tertiary hospital multidisciplinary intersex clinic and an international peer support group for CAIS. PATIENT(S): Sixty-six adult women with CAIS. INTERVENTION(S): Self-completed survey of sexual function, genital normality perceptions, and compliance and satisfaction with vaginal hypoplasia treatments. Hospital case notes review, and genital examination for prevalence of vaginal and clitoral hypoplasia. MAIN OUTCOME MEASURE(S): Golombok-Rust Inventory of Sexual Satisfaction (GRISS) scores of study participants were compared against the scores of the test validation population (as control). In physical examination participants, anatomical dimensions were assessed against published normal values for clitoral and vaginal sizes. RESULT(S): We found that 90% of women with CAIS in this study had sexual difficulties when compared with the general female population, most commonly sexual infrequency and vaginal penetration difficulty; 77% perceived their vagina as small, but on genital examination only 35% had vaginal hypoplasia. CONCLUSION(S): Androgen deficiency leads to sexual problems. Vaginal hypoplasia and negative psychological adaptation to living with an intersex condition are likely to have contributed to the high rates of sexual problems found in this study. Treatments for vaginal hypoplasia need to be evaluated with outcome studies of long-term sexual function, quality of life, and satisfaction. Clinical services for the management of intersex conditions need to be multidisciplinary and aim to optimize the patient's physical and psychological health.

Meeting between experts: evaluation of the first UK forum for lay and professional experts in intersex.

At present the clinical management of intersex is in turmoil. The policy of non-disclosure of diagnosis is widespread and cosmetic genital surgery is routinely performed on infants throughout the world. Some clinicians feel such practices are in the interest of the intersex child and the family, but some intersex adults are calling for a moratorium on sex assignment genital surgery. These widely opposing views have led to distrust between groups. One way to begin to address these critical issues is to facilitate dialogue with equal input from clinicians and intersex people and families. Clinicians are experts by training, but patients and families are experts through lived experiences. Our paper reports the rationale, process and outcome of the first UK forum bringing together these different experts to address some of the most complex issues in clinical services. In communicating our experiences, we hope that it will provide a useful reference point for those seeking similar service-user/provider collaboration in other areas of medicine.

Female genital appearance: "normality" unfolds.

OBJECTIVE: To describe variations in genital dimensions of normal women. DESIGN: Observational cross-sectional study. SETTING: Elizabeth Garrett Anderson Hospital, University College Hospital NHS Trust, London, UK. POPULATION: Fifty premenopausal women having gynaecological procedures not involving the external genitalia under general anaesthetic. METHODS: A cross sectional study using digital photography and measurements of the external genitalia. MAIN OUTCOME MEASURES: Clitoral size, labial length and width, colour and rugosity, vaginal length, distance from clitoris to urethral orifice, distance from posterior fourchette to anterior anal margin. RESULTS: A wide range of values were noted for each measurement. There was no statistically significant association with age, parity, ethnicity, hormonal use or history of sexual activity. CONCLUSION: Women vary widely in genital dimensions. This information should be made available to women when considering surgical procedures on the genitals, decisions for which must be carefully considered between surgeon and woman.

XY females: revisiting the diagnosis.

OBJECTIVES: To investigate the accuracy of assigned diagnosis in XY female intersex conditions. DESIGN: Cross sectional hospital case notes review. SETTING: Tertiary hospital multidisciplinary intersex clinic. SAMPLE: Forty-six adult intersex women with a complete or mosaic XY karyotype. METHODS: All clinical features and investigation results were reviewed and a diagnosis was assigned. This was compared to the original diagnosis assigned. MAIN OUTCOME MEASURES: Data collected included presentation, all investigations, subsequent clinical course and all treatments (medical and surgical). These data were employed to assign an up-to-date intersex diagnosis, which was compared with the recorded diagnosis in the hospital case notes. Diagnoses were then rated according to level of accuracy. RESULTS: The 47.8% patients had an accurate diagnosis, 32.6% of diagnoses were inaccurate and currently under review, 13% had a wrong diagnosis and 6.5% remain with an unknown aetiology for their XY intersex condition. CONCLUSIONS: Diagnostic accuracy is assumed to be high when evaluating published work on these conditions; however, this study shows 52.1% of patients have unknown, inaccurate or wrong diagnoses. Assigning the wrong diagnosis may be harmful, for example, if it leads to irreversible virilising changes or development of a gonadal malignancy, and for all cases excludes accurate condition management and genetic counselling for both the patient and their immediate family.

The effect of clitoral surgery on sexual outcome in individuals who have intersex conditions with ambiguous genitalia: a cross-sectional study.

BACKGROUND: The effects on sexual function of surgical removal of parts of the clitoris are unknown. For infants with intersex conditions and ambiguous genitalia being raised female, this surgery is often undertaken in early childhood. Our aim was to assess the effects of surgery on sexual outcome in this population. METHOD: We did a cross-sectional study to which we recruited 39 adults who had intersex conditions with ambiguous genitalia who were living as female from clinical (n=15) and peer-support (n=24) settings. We obtained data by use of a postal questionnaire, incorporating a validated sexual function assessment inventory. We also obtained hospital notes of 36 respondents who did not want to remain anonymous, and did genital examinations of 19 women. We assessed sexual problems in relation to surgical history and compared the results for our population to those of a healthy control group. FINDINGS: Of the 39 individuals enrolled, 28 had been sexually active and all had sexual difficulties. The 18 women who had undergone clitoral surgery had higher rates of non-sensuality (78%) and of inability to achieve orgasm (39%) than did the ten who had not had surgery (20% [p=0.002] and 0% [p=0.03], respectively). INTERPRETATION: Sexual function could be compromised by clitoral surgery. Debate on the ethics of the use of this surgery in children should be promoted and further multicentre research is needed to ensure representative samples and comprehensive outcome assessment. Meanwhile, parents and patients who consent to clitoral surgery should be fully informed of the potential risks to sexual function.