Relationship and sexual experiences in women with early-onset oestrogen deficiency: Comparison between women with Turner syndrome and premature ovarian insufficiency.

OBJECTIVE: Age at first date and sexual intercourse have been observed to be delayed in women with Turner syndrome (TS), with delayed puberty being the main factor. We sought to assess relationship and sexual experiences comparing women with TS and premature ovarian insufficiency (POI). DESIGN: Cross-sectional observational study. PATIENTS: 302 women with TS and 53 women with karyotypically normal POI (median age 33.0 [15.0-78.4] and 26.3 [17.8-52.3], respectively). MEASUREMENTS: A self-reporting questionnaire was used to collect data on relationship and sexual experiences. RESULTS: Women with TS were older than women with POI (P = .002). Compared to women with POI, a smaller proportion of women with TS had ever had vaginal sexual intercourse (VSI) (40 [78.4%] vs 169 [58.1%], respectively, P = .006) and women with TS exhibited a delay in the median age at first relationship and VSI (POI 19.3 ± 0.4 vs TS 22.2 ± 1.1, P = <.001). Start of oestrogen replacement therapy at ≤ 14 years of age compared with > 14 years did not result in earlier relationship and sexual debut. After adjusting for age and diagnosis, induction of puberty, as opposed to spontaneous puberty, was associated with a delay in the median age at first relationship and VSI and a reduced probability of having VSI (Hazard ratio = 0.44 [95% confidence interval: 0.32-0.60], P = <.001). CONCLUSIONS: Turner syndrome and induction of puberty are associated with a reduced likelihood and a delay in relationship and sexual experiences. Women needing puberty induction and women with TS more than POI have a delayed mean age at first VSI compared to the general population.

'Botched labiaplasty': a content analysis of online advertising for revision labiaplasty.

A new development in female genital cosmetic surgery (FGCS) is the promotion of revision surgery for 'botched labiaplasty'. This content analysis study reviews the quality of information offered on websites specifically advertising revision labiaplasty. Twelve websites were identified through online searches and were examined for the quality of their clinical information. All sites defined botched labiaplasty as unsatisfactory appearance after labiaplasty. Four gave no further details and five listed asymmetry, irregular labial edges or removal of too much or too little tissue. Four websites described primary botched labiaplasty as 'mutilation'. Inadequacy of the primary surgeon was cited as the cause of botched labiaplasty in 11/12. Only two websites mentioned risks of surgery. Good outcomes were not defined and no website provided outcome data although guaranteed satisfaction was implied in two websites. This study highlights the existence and promotion of services for botched labiaplasty using non-specific and emotive descriptions. These findings suggest that unsatisfactory results from consumers' perspectives are far from uncommon. The same women whose expectations have not been met by primary surgery are now being targeted for repeat surgery with online advertising capitalising on their unchanged motivations.Impact StatementWhat is already known on this subject? Female genital cosmetic surgery (FGCS) is mainly advertised online with labiaplasty as the most commonly performed procedure. A market for labiaplasty revision to correct 'botched' primary procedures is developing. Academic literature and advertising materials are inconsistent when defining indications and determinants of success for labiaplasty or revision.What the results of this study add? A content analysis of websites specifically advertising revision labiaplasty describes the emotive and nonspecific terms used online to promote revision labiaplasty.What the implications are of these findings for clinical practice and/or further research? The existence of services for botched labiaplasty suggests dissatisfaction is common. Women whose expectations have not been met by primary surgery are targeted for repeat surgery through online advertising capitalising on their potentially unchanged motivations. This study demonstrates the need for clearer outcome data for labiaplasty and highlights the need for better advertising standards for FGCS promotion.

Sexual function and health-related quality of life in women with classic bladder exstrophy.

OBJECTIVE: To investigate sexual function and quality of life in adolescent and adult women with classic bladder exstrophy (BE). MATERIALS AND METHODS: A two-part observational cross-sectional study with a questionnaire arm and a retrospective case review arm was performed. The study was undertaken in a centre providing a tertiary referral gynaecology and urology service. Outcomes were sexual function and quality-of-life scores. RESULTS: A total of 44 patients with BE were identified from departmental databases and included in the study, of whom 28 (64%) completed postal questionnaires. Sexual function scores and quality-of-life visual analogue scales were significantly poorer compared with normative data. CONCLUSIONS: Bladder exstrophy has a detrimental psychological impact on women. In future, methodical multidisciplinary paediatric follow-up research will help to identify predictors of better and worse adolescent and adult outcomes. Development and evaluation of cost-effective psychological interventions to target specific problems is also warranted.

Will the introduction of non-invasive prenatal testing for Down's syndrome undermine informed choice?

OBJECTIVE: To investigate whether the introduction of non-invasive pre-natal testing for Down's syndrome (DS) has the potential to undermine informed choice. PARTICIPANTS: Three hundred and ninety-three health professionals; 523 pregnant women. METHODS: A cross-sectional questionnaire study across nine maternity units and three conferences in the UK designed to assess opinions regarding test delivery and how information should be communicated to women when offered Down's syndrome screening (DSS) or diagnosis using invasive (IDT) or non-invasive testing (NIPT). RESULTS: Both pregnant women and health professionals in the NIPT and DSS groups were less likely than the IDT group to consider that testing should take place at a return visit or that obtaining written consent was necessary, and more likely to think testing should be carried out routinely. Compared to health professionals, pregnant women expressed a stronger preference for testing to occur on the same day as pre-test counselling (P = 0.000) and for invasive testing to be offered routinely (P = 0.000). They were also more likely to indicate written consent as necessary for DSS (P = 0.000) and NIPT (P < 0.05). CONCLUSIONS: Health professionals and pregnant women view the consenting process differently across antenatal test types. These differences suggest that informed choice may be undermined with the introduction of NIPT for DS into clinical practice. To maintain high standards of care, effective professional training programmes and practice guidelines are needed which prioritize informed consent and take into account the views and needs of service users.

Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence.

OBJECTIVE: Adult women with complete androgen insensitivity syndrome (CAIS) are increasingly likely to defer or decline gonadectomy despite counselling about malignancy risk. The objectives of this study were to review the evidence on the risk of gonadal malignancy in adult women with CAIS and to explore women's reasons for deferring gonadectomy. STUDY DESIGN: A case series and literature review. PATIENTS: Sixteen women with CAIS over the age of 18 years who have elected to defer gonadectomy. RESULTS: Sixty-two relevant papers were identified. Of these, 14 confirmed that tumours had been reported in 98 adults. Taking into account the limitations of combining historic case series, this review estimates a risk of gonadal malignancy of 14% (range 0% and 22%) in adults with CAIS. The most common reasons women offered for deferring gonadectomy included inconvenience of surgery, concern about surgical risk and reluctance to take hormone replacement therapy. CONCLUSIONS: Perceived benefits for retaining gonads in women with CAIS are prompting more women to keep their gonads in situ. An accurate estimate for adult malignancy risk is unavailable, and the risks currently quoted may be falsely reassuring.

Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study.

OBJECTIVE: We sought to examine the reproductive outcomes of 52 women with classical bladder exstrophy. STUDY DESIGN: This was an observational study with cross-sectional and retrospective arms. RESULTS: The average age of the sample was 33 years (range, 17-63). Of those who had tried, 19/38 (66%) had conceived. A total of 57 pregnancies (3 sets of twins) were reported for the 19 patients and resulted in 34/57 live births (56%), 21/57 miscarriages (35%), 1/57 (2%) termination, and 4/57 (7%) stillbirths or neonatal deaths. Four deliveries resulted in major complications including 1 transection of the ureter (4%), 1 fistula formation (4%), and 2 postpartum hemorrhages (8%). There were 2 admissions to intensive care, one for urinary sepsis and another for massive obstetric hemorrhage. CONCLUSION: Fertility is impaired in women with bladder exstrophy. Pregnancy is high risk both for the mother and baby. Delivery should be at a tertiary referral obstetric unit with urology cover. In the majority of cases planned cesarean section is the most appropriate mode of delivery.

Service users and care providers' experiences of tertiary combined fetal medicine clinics.

OBJECTIVE: To explore service users and care providers' experiences of combined fetal medicine and specialist paediatric clinics. METHOD: A brief survey of service users and care providers at combined fetal medicine clinics, which bring together multiple specialists and expertise for the management of pregnancies complicated by fetal cardiac, renal, neurological or surgical abnormalities. RESULTS: Two hundred and sixty-one patients and 22 health professionals participated. More than 85% of women rated the clinic highly, 61% reported that the service had changed how they viewed the abnormality, and 53% reported that they would welcome further visits to the combined clinic. The majority of health professionals reported that combined clinics improved the accuracy of parental counselling and enhanced communication between specialties involved in the management of complicated pregnancies. The clinics are generally regarded as being useful for the training of junior staff. CONCLUSION: A service model that combines fetal medicine and paediatric specialists in a single clinic can efficiently modify parental perspective on fetal anomalies and enhance professional communication and training. Condition-specific information leaflets could further enhance service quality. A larger study involving a socio-demographically stratified sample of service users is needed to provide more authoritative data.

Marital Status and Gender Differences as Key Determinants of COVID-19 Impact on Wellbeing, Job Satisfaction and Resilience in Health Care Workers and Staff Working in Academia in the UK During the First Wave of the Pandemic.

Background: The COVID-19 pandemic is an unprecedented global public health crisis that continues to exert immense pressure on healthcare and related professional staff and services. The impact on staff wellbeing is likely to be influenced by a combination of modifiable and non-modifiable factors. Objectives: The aim of this study is to evaluate the effect of the COVID-19 pandemic on the self-reported wellbeing, resilience, and job satisfaction of National Health Service (NHS) and university staff working in the field of healthcare and medical research. Methods: We conducted a cross sectional survey of NHS and UK university staff throughout the COVID-19 pandemic between May-November 2020. The anonymous and voluntary survey was disseminated through social media platforms, and via e-mail to members of professional and medical bodies. The data was analyzed using descriptive and regression (R) statistics. Results: The enjoyment of work and satisfaction outside of work was significantly negatively impacted by the COVID-19 pandemic for all of staff groups independent of other variables. Furthermore, married women reporting significantly lower wellbeing than married men (P = 0.028). Additionally, the wellbeing of single females was significantly lower than both married women and men (P = 0.017 and P < 0.0001, respectively). Gender differences were also found in satisfaction outside of work, with women reporting higher satisfaction than men before the COVID-19 pandemic (P = 0.0002). Conclusion: Our study confirms that the enjoyment of work and general satisfaction of staff members has been significantly affected by the first wave of the COVID-19 pandemic. Interestingly, being married appears to be a protective factor for wellbeing and resilience but the effect may be reversed for life satisfaction outside work. Our survey highlights the critical need for further research to examine gender differences using a wider range of methods.

Emotional and sexual wellness and quality of life in women with Rokitansky syndrome.

OBJECTIVE: The objective of the study was to investigate health, well-being, and sexual function in women with Rokitansky syndrome. STUDY DESIGN: Fifty-eight women with Rokitansky syndrome completed 4 questionnaires assessing health-related quality of life, emotional distress, and sexual function and attended for a vaginal examination. RESULTS: Participants reported better overall physical health and poorer overall mental health compared with normative data. Anxiety levels were higher, especially for women who had undergone vaginal treatment. Sexual wellness and function scores were poor. Mean vaginal length was 5.4 cm and was greater in women currently sexually active. Vaginal length had a positive correlation with overall sexual satisfaction but was not related to overall quality of life. CONCLUSION: Rokitansky syndrome has a negative impact on emotional and sexual wellness. Relationships between physical and psychological parameters are complex and require further exploration. There is a need for better treatment studies using prospective methodology to assess the effects of surgical and nonsurgical treatments.

Anorectal malformations: what happens in adulthood?

OBJECTIVE: To comprehensively evaluate the outcomes in adults who were born with anorectal malformations (ARMs), to assess the relationships between the major types of ARM and outcomes, and to examine the relationships between bowel and urinary function and sexual well-being, as advances in surgical treatment for ARMs have led to more patients surviving into adult life, and adult data on urinary and bowel function are scant, with even less known about the effect of diagnosis and treatment on sexual and reproductive function. PATIENTS AND METHODS: In all, 74 adult patients with a diagnosis of ARM confirmed by review of paediatric medical records completed a series of validated questionnaires on urinary and bowel function, body esteem, sexual well being, fertility and overall quality of life. RESULTS: There were high levels of urinary and bowel incontinence for both men and women. A high ARM was associated with a greater degree of incontinence. Both men and women scored more poorly than controls on the body esteem, sexual well-being and quality-of-life assessments. Urinary and bowel incontinence was associated with poorer sexual well-being. CONCLUSIONS: Bowel and urinary outcomes in adults appear to be worse than suggested in paediatric reports, with high levels of both urinary and fecal incontinence. Poorer outcomes appeared to affect sexual well-being adversely. To date, attention to issues during transition from childhood to adulthood, particularly concerns about sexual function and well-being, has been conspicuous by its absence. Patient-focused multidisciplinary care during this transition must address these issues, which are central to an acceptable quality of life.

It is a problem and it is not a problem: Dilemmatic talk of the psychological effects of female genital cutting.

This qualitative study aimed to explore the psychological effects of female genital cutting in the United Kingdom within a systemic psychological framework. Semi-structured interviews were carried out with 13 women who had experienced female genital cutting. Four key themes emerged from thematic analysis of the data: (1) wholeness contested, (2) sexuality milestones and female genital cutting awareness, (3) salvaging family relationships and (4) for our own good. Findings highlight the importance of recognising the relational nature of how women perceive the psychological effects of female genital cutting and how these reactions are negotiated in their social sphere.

Clitoral surgery on minors: an interview study with clinical experts of differences of sex development.

OBJECTIVES: Clitoral surgery on minors diagnosed with differences of sex development is increasingly positioned as a violation of human rights. This qualitative study identified how health professionals (HPs) navigate the contentious issues as they offer care to affected families. DESIGN: Qualitative analysis of audio-recorded semistructured interviews with HPs. All of the interviews were transcribed verbatim for theoretical thematic analysis. SETTING: Twelve specialist multidisciplinary care centres for children, adolescents and adults diagnosed with a genetic condition associated with differences of sex development. PARTICIPANTS: Thirty-two medical, surgical, psychological and nursing professionals and clinical scientists in 12 specialist centres in Britain and Sweden formed the interview sample. RESULTS: All interviewees were aware of the controversial nature of clitoral surgery and perceived themselves and their teams as non-interventionist compared with other teams. Data analyses highlighted four strategies that the interviewees used to navigate their complex tasks: (1) engaging with new thinking, (2) holding on to historical assumptions, (3) reducing the burden of dilemmas and (4) being flexible. In response to recent reports and debates that challenge clitoral surgery on minors, HPs had revised some of their opinions. However, they struggled to reconcile their new knowledge with the incumbent norms in favour of intervention as they counsel care users with variable reactions and expectations. The flexible approach taken may reflect compromise, but the interviewees were often trapped by the contradictory values and assumptions. CONCLUSIONS: If the pathology-based vocabularies and narratives about genital diversity could be modified, and normative assumptions are questioned more often, clinicians may be more adept at integrating their new knowledge into a more coherent model of care to address the psychosocial concerns that genital surgery purports to overcome.

Sexual Experience before Treatment for Vaginal Agenesis: A Retrospective Review of 137 Women.

STUDY OBJECTIVE: To summarize the self-reported sexual experiences of women with vaginal agenesis before treatment and discuss the clinical implications. DESIGN: A retrospective review of pretreatment baseline sexuality data and medical records of women with vaginal agenesis seeking vaginal construction. SETTING: A specialist multidisciplinary center for women with genital differences associated with diverse sex development. PARTICIPANTS: One hundred thirty-seven women with untreated vaginal agenesis associated with Mayer-Rokitansky-Küster-Hauser Syndrome and complete androgen insensitivity syndrome aged 15 to 41 years (mean age, 20 years). INTERVENTIONS: Gynecological examination and completion of questionnaires. MAIN OUTCOME MEASURES: (1) Sexual Experiences Questionnaire; (2) Multidimensional Sexuality Questionnaire; (3) Vaginal Self-Perceptions; and (4) vaginal length. RESULTS: A sizable proportion of women reported having had sexually intimate experiences before any medical intervention on the vagina. Vaginal length, which ranged from dimple to 7 cm and averaged 2.7 cm for the cohort, was unrelated to the range of sexual experiences. Most women perceived their vagina as being too small, but less than half believed that a sexual partner would notice this. Two-thirds of the cohort subsequently completed the dilation program, which was not predicted by pretreatment vaginal length or sexual experience. CONCLUSION: Contrary to the assumption that a vagina of certain dimensions is a prerequisite for women to "have sex," many women with Mayer-Rokitansky-Küster-Hauser syndrome and complete androgen insensitivity syndrome reported having experienced genital and nongenital sexual activities with no medical interventions. It is recommended that treatment providers affirm women's capacity for sexual intimacy, relationships, and enjoyment before they introduce the topic of vaginal construction as a non-urgent choice.

Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia.

PURPOSE: Female sex assignment followed by cosmetic genitoplasty to feminize the genitalia in infancy remains standard practice in the clinical management of ambiguous genitalia. The effects of surgery on genital sensitivity have never been objectively evaluated. To our knowledge the current study is the first to evaluate genital sensitivity and sexual function in women with congenital adrenal hyperplasia. MATERIALS AND METHODS: A total of 28 women with congenital adrenal hyperplasia and 10 normal controls were recruited. Details of prior genital surgery were obtained from medical records. Sensitivity thresholds for the clitoris and upper vagina were measured using a GenitoSensory Analyzer (Medoc, Ramat, Israel). Sexual function was assessed using a standardized measure. RESULTS: Of 28 women with congenital adrenal hyperplasia 24 had undergone feminizing genital surgery. In women who underwent surgery there was significant impairment to sensitivity in the clitoris compared to controls. No difference was observed for the sensitivity threshold in the upper vagina, where surgery had not been done in any of the women. Data on the 4 women with congenital adrenal hyperplasia who had not undergone surgery were similar to those in controls. Sexual function difficulties were more severe in women who underwent surgery, especially vaginal penetration difficulties and intercourse frequency. Linear relationships were observed for impairment to sensitivity and severity of sexual difficulties. CONCLUSIONS: Genital sensitivity is impaired in areas where feminizing genital surgery had been done and impairment to sensitivity are linearly related to difficulties in sexual function. The new information may help inform clinicians and parents making difficult decisions about genital surgery for infants with ambiguous genitalia.

Vaginal Construction and Treatment Providers' Experiences: A Qualitative Analysis.

STUDY OBJECTIVE: To investigate specialist clinicians' experiences of treating vaginal agenesis. DESIGN: Semi-structured interviews. SETTING: Twelve hospitals in Britain and Sweden. PARTICIPANTS: Thirty-two health professionals connected to multidisciplinary teams (MDTs) including medical specialists and psychologists. INTERVENTIONS AND MAIN OUTCOME MEASURES: Theoretical thematic analysis of recorded verbatim data. RESULTS: The gynecologist and psychologist interviewees share certain observations including the importance of psychological readiness for and appropriate timing of treatment. Three overlapping themes are identified in our theoretical analysis of the MDT clinicians' talk: (1) the stigma of vaginal agenesis and the pressure to be "normal" can lead patients to minimize the time, effort, physical discomfort, and emotional cost inherent in treatment. (2) Under pressure, treatment might be presented to patients with insufficient attention to the potential psychological effect of the language used. Furthermore, the opportunity to question what is "normal" in sex is generally not taken up. It can be challenging to help the women to transcend their medicalized experiences to come to experiencing their bodies as sexual and enjoyable. (3) The reality of treatment demands, which are not always adequately processed before treatment, can lead to discontinuation and even disengagement with services. CONCLUSION: The MDT clinicians in this study emphasized the importance of psychological input in vaginal construction. However, such input should proactively question social norms about how women's genitalia should appear and function. Furthermore, rather than steering patients (back) to treatment, the entire MDT could more explicitly question social norms and help the women to do the same. By shifting the definition of success from anatomy to personal agency, the clinical focus is transformed from treatment to women.