Sexual Self-Concept in Women with Disorders/Differences of Sex Development.

Many women born with disorders or differences of sex development (DSD) report sexual problems, in particular women who have undergone extensive genital reconstruction. Examining cognitions and emotions that hinder or promote sexuality may facilitate understanding these sexual problems and may contribute to the development of specific interventions. In this study, sexual self-concept, body image, and sexual functioning were investigated in relation to genital surgery. To conduct the study, the women's Sexual Self-Concept Scale was translated to Dutch. Evaluation of psychometric properties was conducted in a sample of healthy Belgian and Dutch women participating in an anonymous web-based survey (N = 589, Mdn age, 23 years). The resulting three-factor structure corresponded largely to that of the original version. Compared to control women, women born with a DSD who were included in the Dutch DSD study (N = 99, Mdn age, 26 years) described themselves as being less interested in sex and less sexually active. These women also harbored more negative emotions and cognitions regarding their sexuality and were less satisfied with their external genitalia. In women with a DSD, sexual self-concept was associated with compromised outcomes on sexual functioning and distress. Women who were in a steady relationship, and/or had been sexually active in the past 4 weeks had a more positive sexual self-concept, took a more active role in their sexual relationship, experienced more sexual desire and arousal and less sexual distress than women who were not involved in a partner relationship. Findings in this study indicate that cognitions and emotions related to sexual self-concept play a role in sexual functioning of women with a DSD. A cognitive behavioral counseling approach with focus on coping and exploration of their own sexual needs could prove useful in this group.

Fertility and sexuality issues in congenital lifelong urology patients: male aspects.

PURPOSE: To review existing literature about fertility and sexuality of boys born with complex congenital genitourinary anomalies. METHODS: A Pubmed review was performed in December 2018 to identify the most relevant original manuscripts regarding male complex congenital conditions affecting the urogenital system in male patients including spina bifida (SB), bladder exstrophy-epispadias complex (BEEC) and hypospadias. A comprehensive review was drafted exploring sexual dysfunction from a medical, psychosexual, surgical and reproductive point of view during transition from childhood (or adolescence) to adulthood. RESULTS: About 75% of men with SB have erectile dysfunction (ED) (Gamé et al. in Urology 67(3):566-570, 2006; Diamond et al. in 58(4):434-435, 1986). Most SB patients have impaired sexual development mainly due to diminished self-esteem, dependence on caregivers and lack of privacy (Blum et al. in Pediatrics 88(2):280-285, 1991). Men with BEEC have fewer intimate relationships than women because of the greater difficulties with issues regarding their genitalia and sexual activities (Deans et al. in Am J Obstet Gynecol 206(6):496.e1-496.e6, 2012). However, a good quality of life is achievable with the effective use of coping strategies (Deng et al. in Transl Androl Urol 7:941, 2018; Rikken et al. in BMC Womens Health 18(1):163, 2018; Friedler et al. in Reprod Biomed Online 32(1):54-61, 2016). Chordee occurs in 25% of all hypospadias patients. More severe hypospadias is related to a greater risk for complications. The long-term sexual quality of life (QoL) in men who underwent hypospadias surgery is influenced by a lot of factors. Therefore, an interactive and dynamic biopsychosocial model of sexual QoL was proposed. CONCLUSIONS: The care of patients with congenital urologic conditions becomes a challenge especially in the period of 'transition'. The goal of follow-up is a holistic management viewed from a medical, psychosexual, surgical end reproductive point. All patients should be asked for specific urinary, fecal or sexual concerns.

Development of a decision support tool in pediatric Differences/Disorders of Sex Development.

Decisions about how best to clinically care for young children born with Disorders of Sex Development (DSD) can be challenging because some decisions are irreversible, have lasting physical and mental health effects, and are frequently made before the affected person is able to participate in decision-making. This multi-stage study involved (1) the development of a web-based decision support tool (DST) for parents of infants or young children and the clinicians caring for them; (2) the assessment of communications and decision making between DSD specialists and parents both before and after introduction of the DST; and (3) interviews with a broad range of stakeholders regarding optimizing the DST and integrating it into usual care. Experience over the course of the 3 stages of this research suggests the need for further refinement of the DST to increase acceptability to all stakeholder groups, the necessity to address misperceptions by providers that they are already accomplishing all aspects of SDM in regular care without a DST and misunderstandings by parents that decisions are unnecessary because only a single option is apparent, and to better incorporate the tool into regular clinic workflow.

Sexual quality of life after total phalloplasty in men with penile deficiency: an exploratory study.

PURPOSE: Total phalloplasty is rarely performed today in males with severe penile deficiency, despite its successful use in the transgender population. Can phalloplasty replicate the complexity of penile anatomy and function on the long term? METHODS: Sexual quality of life (QoL) was assessed in 10 men (aged 20-43 years) at least 1 year after phalloplasty in a single institution (80 % radial forearm flap and 20 % anterolateral thigh flap). In all but one, an erectile prosthesis was implanted on average 1 year after phallic reconstruction. Sexual QoL outcomes were compared to those of men with hypospadias repair (n = 73) and control men (n = 50). RESULTS: After phalloplasty (mean 36.9 months, 14-92 months), all men were sexually active (80 % intercourse and 100 % masturbation with orgasm and ejaculation). However, 75 % indicated to be inhibited in seeking sexual contacts, compared to 40 % of hypospadias patients (p < 0.05) and 11 % of controls (p < 0.01). Although 90 % were satisfied with the final surgical result, dissatisfaction with some aspects of genital appearance was present in 50 %. Erogenous neophallus sensitivity was said to be less than previously hoped for. Six men developed urinary complications (urethral stricture and/or fistula), and one man underwent revision of the erectile implant because of dysfunction. Nevertheless, all indicated they would choose again for phalloplasty if necessary. CONCLUSIONS: Total phalloplasty opens new horizons for the treatment of men with penile deficiency, but limitations of the technique should be emphasized prior to surgery. An exploration of patient expectations and continued follow-up including psychological support is important for optimizing psychosexual comfort.

Phalloplasty: A panacea for 46,XY disorder of sex development conditions with penile deficiency?

In cases of severe penile inadequacy, such as in pathological conditions involving penile amputation (e.g. penile cancer), or in 46,XY disorders of sex development with severe undervirilization or maldevelopment of the penis (e.g. idiopathic micropenis, cloacal exstrophy), standard (surgical) penile lengthening techniques do not provide patients with a phallus suitable for sexual intercourse. Genital dissatisfaction can lead to low self-esteem and psychosexual dysfunction. Therefore, phalloplasty, the gold standard in transgender surgery, may provide a possibility to achieve a satisfactory genital appearance and sexual function. Small series have reported cosmetically acceptable and erogenous sensate neophalli with incorporation of a neourethra to allow voiding in a standing position and with enough bulk to allow penile prosthesis insertion for pleasurable intercourse. Although early results seem promising, further publication of series with large numbers and longer follow-up is needed to evaluate to what extent phalloplasty improves physical and sexual outcomes. Complications are of particular concern because of associated scarring and loss of sensitive tissue. Without full preoperative workups assessing patients' expectations and reasons for undergoing surgery, they may still struggle with self/penile image and with psychological barriers for engaging in sexual activity. Recommendations for the psychosocial management of boys and men with penile deficiency are suggested.

An update on surgical and non-surgical treatments for vaginal hypoplasia.

BACKGROUND: In women with vaginal hypoplasia, such as in Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) and in Complete Androgen Insensitivity Syndrome (CAIS), surgical vaginoplasty and non-surgical self-dilation treatments are available to lengthen the vagina and facilitate sexual intercourse, but the best treatment remains controversial. Vaginal dilation has been recommended as a first-line treatment, because of its less invasive character and high success rate. However, the exploration of factors associated with compliance and long-term outcome is incomplete, including whether psychological counselling needs to be embedded in treatment to maximize efficacy. It is not known if failed vaginal dilation therapy jeopardizes further surgical success outcomes, especially because in a number of these procedures ongoing vaginal dilation is required. In addition, if surgery is needed, there is a lack of evidence to inform physicians regarding the optimum surgical technique to use. Also, it is unclear whether maintenance dilation therapy in case of sexual inactivity is crucial to ensure functional success. METHODS: In view of this ongoing debate, we performed a search of all published literature (English language only) restricted to the management of vaginal hypoplasia in patients with MRKH or CAIS from 1898 to March 2013 using Pubmed, Cochrane Library and Web of Science. Of the 6700 articles initially identified, a total of 190 studies are analysed. More specifically, by establishing the risk/efficacy profile (vaginal capacity, complications and long-term durability in terms of sexual function) of the different surgical and non-surgical reconstruction techniques, we evaluate if vaginal dilation proposed as the first-line technique is justified based on the evidence. RESULTS: When anatomical success was defined as a length of ≥7 cm and functional success as coitus, all vaginoplasty techniques yielded significantly higher success rates (>90 versus 75% after vaginal dilation), irrespective of underlying diagnosis or start vaginal length. When functional success was defined as 'satisfaction with sex', including non-genital sex, differences disappeared. Failed dilation therapy does not preclude anatomical (nor functional) success if vaginoplasty afterwards is necessary. Traction vaginoplasty seems to have the highest anatomical (99%) and functional success rates (96%), whereas both split- and full-thickness skin graft procedures and intestinal procedures have the lowest successful outcomes (83-95%). Overall, complication rates were significantly lower within the vaginal dilation groups when compared with the different vaginoplasty techniques. Although no randomized control data exist regarding maintenance dilation, the available evidence suggests that continued dilation is needed to maintain patency in periods of coital inactivity. Despite the expectancy that the probability of further positive outcomes is maximized with psychological counselling, this could not be confirmed. CONCLUSIONS: As the medical literature lacks high-quality comparative outcome studies and prospective, longitudinal studies are scarce, no evidence-based treatment guidelines can be provided. However, because of the physically low complication rate and an overall success chance of 75%, vaginal dilation as first choice treatment seems to be justified. Overall, the laparoscopic Vecchietti procedure, becoming more and more available in specialized centres, is considered an appropriate surgical option in patients who are poorly compliant and failed dilation therapy, or for those who do not want to start with vaginal dilation therapy. Future approaches need to raise a wider range of psychosexually oriented questions, elucidate the relationship between vaginal depth and satisfactory outcomes and gain additional experience concerning the format of acceptable and efficient psychological care.

Vaginal dilation treatment in women with vaginal hypoplasia: a prospective one-year follow-up study.

OBJECTIVE: Vaginal dilation treatment has been shown to be a (cost) effective first-line alternative to surgery in normalizing vaginal length and improving sexual function in women with vaginal hypoplasia. There remains, however, a need for prospective studies, with long-term assessment of multiple outcomes. STUDY DESIGN: This was a prospective, single-centre observational study of 16 women with Mayer-Rokitansky-Küster-Hauser syndrome (n = 12) or 46,XY disorders of sex development (n = 4). All women underwent an outpatient vaginal dilation program supervised by a psychologist and physiotherapist. At baseline (T0), stop of treatment (T1) and 1 year follow-up (T2), semistructured interviews, and validated questionnaires assessed sexual function and distress, self-esteem, vaginal perceptions, and health-related quality of life. Gynecological examinations evaluated vaginal dimensions. RESULTS: Ten women completed the program, 3 are still in the program, and dilation failed in 3 and chose vaginoplasty. Sixty-nine percent reached a normal vaginal length (≥6.5 cm) in 5.8 ± 3.3 months. Seventy percent were sexually active with pleasurable experiences at T1, 57% at T2. The significant decrease in sexual distress at T1 (P < .05) was followed by a nonsignificant increase at T2. Depressive mood symptomatology remained high at T1 and T2, related to loss of bodily integrity and fertility. The majority refused further psychological counseling. CONCLUSION: Vaginal dilation treatment should remain the cornerstone of treatment in women with vaginal hypoplasia. However, the diagnosis remains to have a negative impact on emotional well-being in the long term. The role of psychological intervention as both a primary and adjuvant treatment needs clear evaluation.

Self-assessment of genital anatomy and sexual function within a Belgian, Dutch-speaking female population: a validation study.

INTRODUCTION: Data on self-perceived genital anatomy and sensitivity should be part of the long-term follow-up of genitoplasty procedures. However, no normative data, based on a large sample, exist to date. AIMS: Validation of the Self-Assessment of Genital Anatomy and Sexual Function, Female version (SAGAS-F) questionnaire within a Belgian, Dutch-speaking female population. METHODS: Seven hundred forty-nine women with no history of genital surgery (aged 18-69 years, median 25 years) completed an Internet-based survey of whom 21 women underwent a gynecological examination as to correlate self-reported genital sensitivity assessed in an experimental setting. MAIN OUTCOME MEASURES: The SAGAS-F enables women to rate the sexual pleasure, discomfort, intensity of orgasm, and effort required for achieving orgasm in specified areas around the clitoris and within the vagina, as well as genital appearance. The latter was similarly evaluated by an experienced gynecologist, and women were asked to functionally rate the anatomical areas pointed out with a vaginal swab. RESULTS: Sexual pleasure and orgasm were strongest, and effort to attain orgasm and discomfort was lowest when stimulating the clitoris and sides of the clitoris (P < 0.05). Vaginal sensitivity increased with increasing vaginal depth, but overall orgasmic sensitivity was lower as compared with the clitoris. Functional scores on the SAGAS-F and during gynecological examination corresponded highly on most anatomical areas (P < 0.05). Gynecologist's ratings corresponded highly with the women's ratings for vaginal size (90%) but not for clitoral size (48%). CONCLUSIONS: Replication of the original pilot study results support the validity of the questionnaire. The SAGAS-F discriminates reasonably well between various genital areas in terms of erotic sensitivity. The clitoris itself appeared to be the most sensitive, consistent with maximum nerve density in this area. Surgery to the clitoris could disrupt neurological pathways and compromise erotic sensation and pleasure.

Sexual quality of life after hormonal and surgical treatment, including phalloplasty, in men with micropenis: a review.

INTRODUCTION: The term micropenis encompasses a range of congenital and acquired conditions that result in an abnormally short penis. Small penis size may persist into adulthood, becoming a major cause of dissatisfaction. AIM: To review the literature pertaining to the effects of hormonal and surgical treatment on psychosexual functioning and quality of life (QoL) in individuals with micropenis who were raised male. MAIN OUTCOME MEASURES: Long-term psychosexual and QoL outcomes after hormonal and surgical treatment, including phalloplasty. METHODS: PubMed search for relevant publications (1955-2012) on the role of hormonal and surgical treatment in sexual QoL in adult men with micropenis. RESULTS: Multiple variations in the etiology of micropenis make it difficult to draw firm conclusions that fit all of the patients within this disparate population. However, the literature review supports the conclusions that (i) male gender assignment is preferable for most 46,XY infants with congenital micropenis because of the likelihood of male gender development and genitosexual function; (ii) small penis size persisting into adulthood and dissatisfaction with genital appearance jeopardize sexual QoL; (iii) there is no known intervention, apart from phalloplasty, to guarantee that the penis will become normal in size; (iv) early data suggest that the phalloplasty technique considered the gold standard for gender reassignment in the transgender population can also be transferred to 46,XY patients with micropenis; (v) psychological support should be an integral part of management in order to alleviate the distress and impairment of QoL experienced by these individuals. CONCLUSIONS: Further publication of series with large numbers and longer follow-up is needed. Specific outcome kits should be designed to measure more precisely patients' degrees of satisfaction with cosmetic, anatomical, and functional variables. Only if health-care professionals fully appreciate the impact of this condition can optimal care be provided.

Long-term outcomes in males with disorders of sex development.

PURPOSE: Indications that the prenatal action of testosterone in the brain is an important determinant of gender development and improved reconstructive techniques have caused a shift in male gender assignments in patients with 46XY disorders of sex development. We report long-term outcome data on psychosexual development and sexual function of these individuals in a cross-sectional study. MATERIALS AND METHODS: Physical status of 14 men with a mean age of 25 years with disorders of sex development was assessed by structured interview and physical examination. Psychosexual outcome was evaluated by questionnaires and compared to a control group of 46 healthy, age matched men. RESULTS: A total of 13 men underwent 1 to 6 (mean 2) genital surgeries. Mean age at first surgery was 2.7 years. Mean penile length was 6.6 cm. All men reported erections and were able to experience orgasms. Ejaculatory dysfunction was reported by 7 men. Mean penile length was 7.9 cm in patients who were able to achieve penetrative intercourse and 4.9 cm in those who were not. Meatus was glanular in 5 patients, coronal in 7 and at the distal shaft in 1. Compared to controls, men with disorders of sex development were less satisfied with the appearance of the penis and scrotum but not with total body image. These patients reported decreased sexual desire and activities. CONCLUSIONS: Outcome in this group of men with disorders of sex development was poor regarding penile length, ejaculation, satisfaction with external genitalia and frequency of sexual activity. Other aspects, such as overall body image and psychosexual functioning, showed no difference from controls.

Severity of virilization is associated with cosmetic appearance and sexual function in women with congenital adrenal hyperplasia: a cross-sectional study.

INTRODUCTION: Women with the classical form of congenital adrenal hyperplasia (CAH) are born with different degrees of virilization of the external genitalia. Feminizing surgery is often performed in childhood to change the appearance of the genitalia and to enable penile-vaginal intercourse later in life. There are suggestions that this affects sexual functioning. AIMS: The aim is to study the anatomical, surgical, cosmetic, and psychosexual outcomes in women with CAH. METHODS: Forty women with CAH, aged over 15 years, from two referral centers for management of Disorders of Sex Development in the Netherlands were included. Physical and functional status were assessed by a gynecological interview and examination. Sexual functioning was assessed with the Female Sexual Function Index and Female Sexual Distress Scale-Revised scales and compared with a reference group. MEAN OUTCOME MEASURES: Surgery performed, anatomy, cosmetic score, sexual function and distress. RESULTS: Thirty-six of the 40 women had undergone feminizing surgery; 25 women (69%) underwent more than one operation. Resurgery was performed in seven of the 13 (54%) women who had had a single-stage procedure. Anatomical assessment showed reasonable outcomes. Multiple linear regression showed that only level of confluence had a significant effect on cosmetic outcome, the impact depending on the number of surgeries performed. Cosmetic evaluations did not differ between the women and the gynecologists. Only 20 women had experience of intercourse. Eight women reported dyspareunia; seven women reported urinary incontinence. The women's perceived sexual functioning was less satisfactory than in the reference group, and they reported more sexual distress. CONCLUSION: The level of confluence was the major determinant for cosmetic outcome; the impact depended on the number of surgeries performed. Fifty-four percent of the women required resurgery after a single-stage procedure in childhood. Anatomical assessment showed reasonable outcomes. The women evaluated their sexual functioning and functional outcome less favorable than the reference group, and they experienced less often sexual intercourse.

Long-term psychosexual and anatomical outcome after vaginal dilation or vaginoplasty: a comparative study.

INTRODUCTION: In patients with disorders of sex development requiring creation of a neovagina, a number of techniques are available, including surgical vaginoplasty and self-dilation therapy. Vaginal dilation therapy has been recommended as a first-line treatment because of its less invasive character and high success rate. However, no data exist on long-term psychosexual functioning after vaginal dilation as compared with that after vaginal surgery. AIMS: The aim of this study is to compare the psychosexual and anatomical outcome of women with congenital vaginal hypoplasia followed in the same clinical setting after vaginoplasty with that after vaginal dilation. METHODS: The sexual quality of life of 35 women at least 2 years after vaginoplasty (N = 15), vaginal dilation therapy (N = 8), or coital dilation/no treatment (N = 12) was investigated and compared with the Dutch test validation population (as control). MAIN OUTCOME MEASURES: Psychosexual functioning was assessed with the female sexual Function index, the female sexual distress scale-revised, and a semi-structured interview. A gynecological examination was performed to determine the anatomical outcome after both vaginal treatment regimens. RESULTS: After either treatment, 26% of these women had a shortened vaginal length of less than 6.6 cm, i.e., more than two standard deviations below the published mean value (9.6 ± 1.5 cm). Irrespective of the treatment, 47% of the patients had (a) sexual dysfunction(s) and experienced sexual distress. However, after vaginoplasty, patients reported significantly more problems with lubrication (P = 0.025) than after self-dilation therapy. CONCLUSION: Both psychological and physical factors are predisposing for sexual difficulties. To optimize psychosexual comfort, the clinical management of women with vaginal hypoplasia needs to be multidisciplinary and individually tailored. With high success rates reported, vaginal dilation should remain the cornerstone of treatment.

Do surgical interventions influence psychosexual and cosmetic outcomes in women with disorders of sex development?

Clinical practice developed to promote psychosexual well-being in DSD is under scrutiny. Although techniques for genital surgery have much improved lately, long-term studies on psychosexual functioning and cosmetic outcome on which to base treatment and counseling are scarce. We studied 91 women with a DSD. Feminizing surgery was performed in 64% of the women; in 60% of them, resurgery in puberty was needed after a single-stage procedure. Both patients and gynecologists were satisfied with the cosmetic appearance of the genitalia. However, forty percent of these females experienced sexuality-related distress and 66% was at risk for developing a sexual dysfunction, whether they had surgery or not. Recognizing the difficulty of accurate assessment, our data indicate that feminizing surgery does not seem to improve nor hamper psychosexual outcome, especially in patients with severe virilization.