Self-esteem, depression, anxiety and sexual function in Mayer-Rokitansky-Küster-Hauser syndrome with neovagina: A case series.

BACKGROUND: The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare condition with significant psychological implications. However, our understanding of its impact on postoperative sexual function and mental health is still limited. AIM: Evaluate the mental health status and sexual functioning of women with MRKH syndrome after vaginoplasty surgery. METHODS: We enrolled 53 cases with MRKH syndrome who underwent artificial vaginoplasty. The participants were asked to participate in a two-round survey conducted between February 2021 during the covid-19 period and March 2023. The survey included questionnaires to measure depression, anxiety, self-esteem, and sexual functioning. Differences between scores over time were analysed using a paired sample t-test, and we assessed the correlation between mental health and sexual functioning. RESULTS: In the first round, patients' mean ± SD age at surgery was 23.6 ± 4.5 years old, and the mean ± SD time that had elapsed since surgery at the time of the survey was 34.2 ± 20.8 months. None of the patients reported low self-esteem, 45.3 % reported mild-to-moderate depression, and 34.0 % reported mild anxiety. Thirty patients have had vaginal intercourse during the last six months. The mean ± SD Female Sexual Functioning Index score was 24.6 ± 4.4, and 60.0 % had a score of 23.5 or higher, indicating high sexual functioning. The sexual functioning scores were positively correlated with self-esteem scores and negatively correlated with depression or anxiety scores (p < 0.05). There was no significant improvement in patient's mental health status and sexual function between the second round survey (71.3 ± 17.8 months after surgery) and the first round survey (p > 0.05). In contrast, the sexual arousal of FSFI were significantly higher in the second survey round (p < 0.05). CONCLUSION: Most patients undergoing vaginoplasty reported persisting mental health challenges. However, the majority reported good sexual functioning.

Sexual health and sexual well-being of women with Mayer-Rokitansky-Kuester-Hauser syndrome after vaginal reconstruction: a qualitative analysis.

BACKGROUND: Contradictory findings on sexual health in women with Mayer-Rokitansky-Kuester-Hauser syndrome (MRKHS) after vaginal reconstruction point toward the need for more profound assessment of this subject, particularly as it is still unclear what constitutes sexual well-being, especially genital self-image or sexual self-esteem, in women with MRKHS and neovagina. AIM: The aim of this qualitative study was to assess individual sexual health and sexual well-being in the context of MRKHS after vaginal reconstruction, with an emphasis on genital self-image, sexual self-esteem, sexual satisfaction, and coping with MRKHS. METHODS: Qualitative semistructured interviews were conducted with women with MRKHS after vaginal reconstruction (n = 10) with the Wharton-Sheares-George surgical method and a matched control group without MRKHS (n = 20). Women were surveyed about their previous and current sexual activities, perception of and attitudes toward their genitals, disclosure to others, coping with the diagnosis, and perception of surgery. Data were analyzed through qualitative content analysis and compared with the control group. OUTCOMES: The primary outcomes of the study were major categories, such as sexual satisfaction, sexual self-esteem, genital self-image, and dealing with MRKHS, as well as subcategories related to the content analysis. RESULTS: Although half the women in the present study indicated that they were coping well with their condition and were satisfied with sexual intercourse, most felt insecure about their neovagina, were cognitively distracted during intercourse, and showed low levels of sexual self-esteem. CLINICAL IMPLICATIONS: A better understanding of expectations and uncertainties regarding the neovagina might help professionals to support women with MRKHS after vaginal reconstruction to increase sexual well-being. STRENGTHS AND LIMITATIONS: This is the first qualitative study focusing on individual aspects of sexual well-being, especially sexual self-esteem and genital self-image, in women with MRKHS and neovagina. The qualitative study indicates good interrater reliability and data saturation. The limitations of this study include the inherent lack of objectivity resulting from the method but also the fact that all the patients had a particular surgical technique, consequently resulting in limited generalizability of these findings. CONCLUSIONS: Our data indicate that integrating the neovagina into the genital self-image is a prolonged process that is essential for sexual well-being and should thus be the focus of sexual counseling.

[Burden of Affected Persons with MRKH Syndrome: Effect of an Intervention to Support Surgical Neovaginal Placement]. / Belastung von Betroffenen mit MRKH-Syndrom: Effekt einer Intervention zur Unterstützung bei Neovagina-Anlage.

The diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), a rare variant of female sexual development, is usually made during puberty. The uncertainty in self-image and the impos-sibility of becoming pregnant often lead to considerable stress. Although psychosomatic support is consistently recommended in the literature, there have been only a few studies on the psychological aspects of MRKHS. The aim of the present study is to investigate the quality of life or distress of women with MRKHS undergoing neovaginal surgery and, on the other hand, to evaluate effects of the intervention for support during treatment. Methods In an explorative quasi-experimental pre-post study at a national centre for neovaginal surgery, all patients were offered a psychosomatic intervention (intervention group IG, n=23) and their sexual function (FSFI), psychological distress (PHQ-D) and health-related quality of life (SF-12) were assessed before surgery (t0) and six months after (t1). These were compared with data from a sample collected before and after the intervention period (comparison group VG, n=30). Results While the physical quality of life (SF-12) of both groups was unremarkable at both time points, there was a significant impairment in the psychological quality of life. Both groups (IG, VG) improved from t0 to t1 in their sexual function (FSFI) and showed lower depression scores (PHQ-D). The specific intervention developed was well accepted by those affected and rated as helpful. However, this subjectively perceived effectiveness of the intervention was not reflected by improvement on the quality of life scale (SF-12) and depression scale (PHQ-D). Conclusion Those affected show a clear, clinically relevant distress (SF-12), but this is not reflected in the form of psychological comorbidity (PHQ-D). This apparent discrepancy points to psychologically stable women with acute distress due to the diagnosis of variant sex de-evolution. For them, a low-threshold support service with a supportive character seems to be necessary and helpful during the surgical treatment. The reconstructive therapy for the creation of a neovagina seems to have a positive influence on the psychological quality of life. The fact that pregnancy is still not possible due to the missing uterus could be a reason for not reaching the quality of life of the average population.

Sexual function and quality of life after the creation of a neovagina in women with Mayer-Rokitansky-Küster-Hauser syndrome: comparison of vaginal dilation and surgical procedures.

OBJECTIVE: To compare sexual function and outcomes of quality of life of patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome after vaginal dilation and surgical procedures. STUDY DESIGN: Cross-sectional study from January 2019 to June 2019. SETTING: Tertiary teaching hospital. PATIENT(S): Women with MRKH syndrome treated with vaginal dilation (n = 88) or surgical procedures (n = 45). INTERVENTION: WeChat-based questionnaires were distributed to every group member in our MRKH support group. MAIN OUTCOME MEASURE(S): Sexual functional were assessed by means of the Female Sexual Function Index (FSFI). Outcomes of quality of life were assessed by means of the 12-item World Health Organization Disability Assessment Schedule 2 (WHODAS2). Vaginal length was defined as the maximum depth of the placement of the vaginal mold. RESULT(S): The FSFI scores were similar between the dilation (24.49 ± 4.51) and surgery (23.79 ± 3.57) groups. Except for the higher orgasm score in the dilation group (9.96 ± 3.60 vs. 8.20 ± 2.67), the other dimensions of the FSFI were not significantly different between the groups. No significant differences were found in the WHODAS2 scores between the dilation group (median 8.33 [interquartile range 4.17-15.62]) and the surgery group (6.25 [2.08-14.58]). However, the vaginal length was significantly shorter in the dilation group (6.5 ± 2.04 cm) than in the surgery group (8.1 ± 1.59 cm). CONCLUSION(S): Although the vaginal length was shorter in the dilation therapy group than in the surgical therapy group, sexual function and quality of life were similar between these two groups. Vaginal dilation should be proposed as the first-line therapy for MRKH patients.

Treatment of the Mayer-Rokitansky-Küster-Hauser syndrome with autologous in vitro cultured vaginal tissue: descriptive study of long-term results and patient outcomes.

OBJECTIVE: Evaluating sexual function and quality of life (QoL) in patients treated with a modified Abbé-McIndoe technique using in vitro cultured autologous vaginal mucosa. DESIGN: Descriptive study. SETTING: Policlinico Umberto I, Sapienza University of Rome. POPULATION: From 2006 to 2016, 39 women affected by Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) underwent vaginoplasty at our centre using a modified Abbé-McIndoe technique with in vitro cultured autologous vaginal tissue. METHODS: For each patient, vaginal tissue was obtained by full-thickness biopsy of the vaginal vestibule. Following enzymatic dissociation, cells were cultured for 2-3 weeks before the transplant. MAIN OUTCOME MEASURES: Each patient completed two validated questionnaires to quantify sexual function and QoL: the Female Sexual Function Index (FSFI), administered at 12, 36, and 60 months, and the Psychological General Well Being Index (PGWBI) administered at 0, 6, and 36 months after surgery. RESULTS: Twelve months after surgery, 29 patients were engaging in regular sexual activity. The FSFI test results showed a satisfactory sexual function compared to the general population, with median values of 25.85 (range 4.6-30.5) at 12 months, 27.2 (range 4.4-33.6) at 36 months, and 29.6 (range 23.9-33.6) at 60 months. The PGWBI questionnaire showed a median score of 420.5 (range 108-540) before surgery, and 459 (range 252-533) at the 60-month follow-up. CONCLUSIONS: Vaginoplasty performed with the use of autologous vaginal tissue, besides ensuring a long-term satisfying sex life, helps in achieving an improvement in QoL that is maintained over time. TWEETABLE ABSTRACT: Vaginoplasty using in vitro vaginal tissue ensures a satisfactory sexual function and improves quality of life.

The Interest of Women with Mayer-Rokitansky-Küster-Hauser Syndrome and Laparoscopic Vecchietti Neovagina in Uterus Transplantation.

STUDY OBJECTIVE: The goal of this study was to assess a group of women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with surgically created neovaginas in the interest of uterus transplantation (UTx) and to recruit the first group of applicants for a UTx trial. DESIGN AND SETTING: This was an original prospective study using semistructured interviews. PARTICIPANTS: A study group of 50 women with MRKH syndrome with Vecchietti neovaginas was recruited via letter of invitation. INTERVENTIONS AND MAIN OUTCOME MEASURES: Interest of MRKH women in obtaining experimental UTx for the treatment of absolute uterine factor infertility. RESULTS: A total of 50 women responded via e-mail and 31 (62% [31 of 50] of the study group) expressed serious interest in UTx after complete information about its risks and benefits was provided during the first semistructured interview. They subsequently agreed to participate in additional interviews and further examinations. Because of various reasons, only 9 women were prepared to enter our UTx trial (18% [9 of 50] of the study group). Three recipients/donors were accepted into the living donor arm and 6 into the deceased brain donor arm of the trial. CONCLUSION: Nearly two-thirds of our MRKH syndrome study group women with surgically created neovaginas were interested in UTx and motivated to undergo this method of absolute uterine factor infertility treatment. Therefore, this group of women might be approached to participate in ongoing and future UTx trials. Future studies of women with MRKH syndrome might confirm or disprove the results of our survey.

ACOG Committee Opinion No. 728: Müllerian Agenesis: Diagnosis, Management, And Treatment.

Müllerian agenesis, also referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,500-5,000 females. Müllerian agenesis is caused by embryologic underdevelopment of the müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with müllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. The most important steps in the effective management of müllerian agenesis are correct diagnosis of the underlying condition, evaluation for associated congenital anomalies, and psychosocial counseling in addition to treatment or intervention to address the functional effects of genital anomalies. The psychologic effect of the diagnosis of müllerian agenesis should not be underestimated. All patients with müllerian agenesis should be offered counseling and encouraged to connect with peer support groups. Future options for having children should be addressed with patients: options include adoption and gestational surrogacy. Assisted reproductive techniques with use of a gestational carrier (surrogate) have been shown to be successful for women with müllerian agenesis. Nonsurgical vaginal elongation by dilation should be the first-line approach. When well-counseled and emotionally prepared, almost all patients (90-96%) will be able to achieve anatomic and functional success by primary vaginal dilation. In cases in which surgical intervention is required, referrals to centers with expertise in this area should be considered because few surgeons have extensive experience in construction of the neovagina and surgery by a trained surgeon offers the best opportunity for a successful result.

Anatomic and Functional Outcomes of Paramesonephric Remnant-Supported Laparoscopic Double-Layer Peritoneal Pull-Down Vaginoplasty Technique in Patients with Mayer-Rokitansky-Küster-Hauser Syndrome: Uncu Modification.

OBJECTIVE: To describe modifications to the double-layer peritoneal pull-down laparoscopic vaginoplasty technique (Davydov operation) and evaluate anatomic and functional outcomes of the new technique, known as the Uncu modification. DESIGN: Case series (Canadian Task Force classification III). SETTING: Tertiary care university hospital. PATIENTS: Women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) who underwent surgery between 2010 and 2016. INTERVENTIONS: Laparoscopic double-layer peritoneal pull-down vaginoplasty with paramesonephric remnant support to the neovagina. MEASUREMENTS AND MAIN RESULTS: Long-term anatomic and functional satisfaction results. Twenty-seven women with MRKHS underwent surgery with the Uncu-modified Davydov procedure. At 1 year after surgery, the mean vaginal length in these patients was 7.91 ± 1.4 cm. Among the 23 patients who had regular vaginal intercourse, the mean functional satisfaction score was 8.65 ± 1.2. One patient had a perioperative bladder injury, and another patient had a rectovaginal fistula at 3 months after the operation. One woman who did not comply with the prescribed postoperative mold exercises had complete closure of the introitus. CONCLUSION: The Uncu modified laparoscopic double-layer peritoneal pull-down technique appears to be an effective and safe surgical management option that is easy to learn and perform by gynecologic surgeons.

Gender Role, Gender Identity and Sexual Orientation in CAIS ("XY-Women") Compared With Subfertile and Infertile 46,XX Women.

The perception of gender development of individuals with complete androgen insensitivity syndrome (CAIS) as unambiguously female has recently been challenged in both qualitative data and case reports of male gender identity. The aim of the mixed-method study presented was to examine the self-perception of CAIS individuals regarding different aspects of gender and to identify commonalities and differences in comparison with subfertile and infertile XX-chromosomal women with diagnoses of Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) and polycystic ovary syndrome (PCOS). The study sample comprised 11 participants with CAIS, 49 with MRKHS, and 55 with PCOS. Gender identity was assessed by means of a multidimensional instrument, which showed significant differences between the CAIS group and the XX-chromosomal women. Other-than-female gender roles and neither-female-nor-male sexes/genders were reported only by individuals with CAIS. The percentage with a not exclusively androphile sexual orientation was unexceptionally high in the CAIS group compared to the prevalence in "normative" women and the clinical groups. The findings support the assumption made by Meyer-Bahlburg ( 2010 ) that gender outcome in people with CAIS is more variable than generally stated. Parents and professionals should thus be open to courses of gender development other than typically female in individuals with CAIS.

Male gender identity in children with 46,XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood.

BACKGROUND/AIM: Girls with congenital adrenal hyperplasia (CAH) diagnosed at birth have some masculine behaviors but rarely convert to male gender. In developing countries, however, diagnosis and treatment (with secondary androgen suppression) are delayed. We aimed to assess effect of delayed treatment of CAH on gender identity. METHODS: As part of a cross-sectional, case-control study of children with disorders of sex development (DSD), there were 11 patients with CAH. Patients and caregivers answered a questionnaire about gender identity, and behavior was assessed by observing toy play. Patients were examined for Prader score and gender identity. RESULTS: Of 11 CAH patients initially raised as girls, 3 (27%) had converted to male gender at presentation (5, 9, 9years) (Prader 3, 4, 4). Of the remaining 8 patients, one 4-year-old (Prader 2) had a male gender identity score. The remaining girls (2-13years, mean 8.1) (Prader 1-3) had gender identity scores in the female range. CONCLUSIONS: One third (4/11) of CAH patients presenting in mid-childhood had male gender identity scores, and ¾ had assumed male gender role. Although social and cultural factors are important in developing countries, this result suggests that delayed treatment may trigger male gender identity, and delayed female genital surgery may be unwise.

Characterizing Early Psychosocial Functioning of Parents of Children with Moderate to Severe Genital Ambiguity due to Disorders of Sex Development.

PURPOSE: We examined the psychosocial characteristics of parents of children with disorders of sex development at early presentation to a disorders of sex development clinic. Parental anxiety, depression, quality of life, illness uncertainty and posttraumatic stress symptoms were assessed. Additionally we evaluated the relationship of assigned child gender to parental outcomes. MATERIALS AND METHODS: A total of 51 parents of children with ambiguous or atypical genitalia were recruited from 7 centers specializing in treatment of disorders of sex development. At initial assessment no child had undergone genitoplasty. Parents completed the Cosmetic Appearance Rating Scale, Beck Anxiety Inventory, Beck Depression Inventory, SF-36, Parent Perception of Uncertainty Scale and Impact of Event Scale-Revised. RESULTS: A large percentage of parents (54.5%) were dissatisfied with the genital appearance of their child, and a small but significant percentage reported symptoms of anxiety, depression, diminished quality of life, uncertainty and posttraumatic stress. Few gender differences emerged. CONCLUSIONS: Although many parents function well, a subset experience significant psychological distress around the time of diagnosis of a disorder of sex development in their child. Early screening to assess the need for psychosocial interventions is warranted.

Sexual and Psychosocial Functioning in Women with MRKHS after Neovaginoplasty According to Wharton-Sheares-George: A Case Control Study.

BACKGROUND: Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) has a major impact on a woman's psychological and sexual well-being. In most of the studies that addressed treatment techniques, postoperative sexual function was reported to be satisfactory; however, comparatively few studies have additionally provided an accurate analysis of psychosocial functions in MRKHS patients following different kinds of neovaginal treatment. This study is to evaluate sexual and psychosocial functioning after creation of a neovagina according to Wharton-Sheares-George in women with MRKHS. METHODS: We performed a case-control-study using multiple measures to assess sexual and psychosocial functioning. Ten MRKHS patients and 20 controls of a University hospital and tertiary center for pediatric and adolescent gynecology were assessed. The follow-up assessment comprised 6 standardized questionnaires (Female Sexuality Function Index, FSFI; Patient Health Questionnaire, PHQ; Brief Symptom Inventory, BSI; World Health Organization Quality of Life Assessment, WHOQoL-BREF; Parental Bonding Instrument, PBI; and a German questionnaire on body image). The main outcome measures were sexual function, psychological status, quality of life, body image, and parental bonding styles. FINDINGS: Sexual function, psychological status (including depressive and somatic symptoms), quality of life, and own-body experience were at least as good in operated MRKHS patients as in controls. In some measures (FSFI, PHQ-15, psychological domain of the WHOQoL-BREF, and BSI Positive Symptom Total), patients scored significantly better than controls. The results of the PBI indicated a close and sustainable mother-daughter-relationship in MRKHS patients. CONCLUSIONS: We found no evidence for an impairment of sexual or psychosocial functioning in patients after neovaginoplasty according to Wharton-Sheares-George. MRKHS may not necessarily compromise sexual and psychological well-being, provided that the syndrome is properly managed by a multidisciplinary team of health professionals.

[Sexual activity and quality of life in patients with Mayer-Rokitansky-Kuster-Hauser syndrome]. / Activité sexuelle et qualité de vie chez les patientes atteintes d'un syndrome de Mayer-Rokitansky-Kuster-Hauser.

OBJECTIVES: This study evaluated the sexual activity and the quality of life in patients with a Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. PATIENTS AND METHODS: The study is based on a series of patients hospitalized for the treatment of MRKH syndrome in CMCO between 1997 and 2010. Through the medical reports, we recovered information about the pathology, the treatment given and the psychological support proposed to or wished by the patients. The patients also had to answer a questionnary concerning the quality of the informations they received about the treatment, the different types of supports proposed and wanted, their life as a couple and how they communicated about their disease. RESULTS: Eleven medical reports have been reviewed. The patients were aged 21 in average at the moment of the treatment. Three patients had some associated malformations. Different methods of treatment were represented (Frank's method, Vecchietti's technique, sigmoid vaginoplasty, McIndoe's technique). Sexual intercourse was assessed as satisfactory by 4 patients. In 8 cases, the partner accepted well the pathology. Only 4 out of the eleven medical reports mentioned a psychological support. Nine out of the eleven patients accepted to answer the questionnary. DISCUSSION AND CONCLUSION: The review of the literature and our own results show that the patients with MRKH syndrome have a real need for psychological support. They are able to have sexual intercourse that is satisfactory in the domains of excitation, lubrification and desire, especially after treatment. The main problem is the inability to bear children, a problem which might by solved by the future researches about uterus graft and the evolution of the laws concerning gestational surrogacy.

Systematic review of sexual function and satisfaction following the management of vaginal agenesis.

Historically, sexual satisfaction following the management of vaginal agenesis was assessed subjectively. Standardized sexual function questionnaires are being used more frequently as instruments to accurately and more objectively assess the subjective nature of sexual outcomes as part of a more holistic approach to the care of women with vaginal agenesis. Articles concerning the management of vaginal agenesis were systematically reviewed, with specific focus on those that discussed functional outcomes, sexual satisfaction and psychosomatic outcomes, and in particular attempted to measure these outcomes. A total of 6,691 articles on vaginal agenesis were identified, with 106 of these reporting sexual satisfaction and psychosomatic outcomes. Only 1 randomized control trial (RCT) was identified, the remaining articles being made up of case series or case reports. Only 17 articles used standardized objective assessment of sexual satisfaction. While the bowel technique had the longest vaginal length at 12.87 cm, it had the most number of complaints of dyspareunia (4.8%), stenosis (10.5%) and the lowest average subjective sexual satisfaction. The Davydov method used standardized sexual function assessments most frequently. This technique had a higher average score than both the bowel vaginoplasty technique in the only RCT and the Vecchietti method in a prospective assessment. Overall, the management of vaginal agenesis requires a multidisciplinary approach to fully support these patients from initial diagnosis, through management decision-making and long-term follow-up, through transition to adulthood.

And god created woman? The link between female sexuality and the mother-daughter relationship in Mayer-Rokitansky-Kuster-Hauser syndrome in adolescents.

The authors' clinical experience with young girls with Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH), who are facing the often hastily suggested, and accepted, surgical treatment of vaginal reconstruction brings new light to the question of female sexuality and its specific modes of access: its traumatic aspects, the mother-daughter conflict of ambivalence and the associated risk of depression, as well as the importance of the relational factor in the construction of bodily interiority.

Psychosexual and social consequences in a woman with undiagnosed Mayer-Rokitansky-Küster-Hauser syndrome.

PURPOSE: To report a woman with devastating psychosexual and social consequences as a result of undiagnosed Mayer-Rokitansky- Küster-Hauser syndrome (MRKH) syndrome. MATERIALS AND METHODS: An 18-year-old woman was referred after being divorced for "absent vagina and uterus". On examination, secondary sexual characteristics were normal. Vaginal and rectal examinations revealed absent vagina and uterus. Investigations showed normal hormonal profile, 46 XX karyotype, and normal intravenous pyelography. Pelvic ultrasonography and magnetic resonance imaging (MRI) confirmed the absence of the uterus and presence bilateral ovaries. RESULTS: A diagnosis of MRKH syndrome was made and she underwent successful modified laparoscopic Vecchietti operation for creation of a new vagina. Vaginal dilators were used after the surgery. Two years of follow up confirmed that vaginal length was about ten cm. CONCLUSION: The proper diagnosis, counseling, and prompt treatment of MRKH syndrome can prevent tragic consequences.

[A questionnaire for the assessment of women's perception of their own femininity: a study on women with Mayer-Rokitansky-Kuster-Hauser-syndrome and women with polycystic ovary syndrome]. / Fragebogen zum Erleben der eigenen Weiblichkeit (FB-W): Ergebnisse von Frauen mit Mayer-Rokitansky-Küster-Hauser Syndrom und Frauen mit polyzystischem Ovarsyndrom.

Women with Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) or polycystic ovary syndrome (PCOS) experience substantial changes in female body characteristics. It was investigated how this is associated with changes concerning the experience of one's own femininity. A questionnaire was developed to measure the experience of one's own femininity. The question-naire assesses how important several aspects are to women for their experience of their own femininity. Data from 49 women with MRKHS and 55 women with PCOS were compared to a non-clinical sample (932 women). The experience of their own femininity differed between the clinical groups as well as in comparison to the control sample. Diagnosis-specific characteristics emerged, which should be considered in the treatment of affected women. The developed questionnaire proved to be suitable for measuring differences in the experience of one's own femininity between groups of gynecological -patients.

[Morphology of the neovagina and sexual functioning of patients with Mayer-Rokitansky-Küster-Hauser syndrome who underwent modified Wharton vaginoplasty]. / Morfologiczna, czynnosciowa i seksuologiczna ocena efektu wytworzenia pochwy zmodyfikowana metoda Whartona u pacjentek z zespolem Mayera-Rokitansky'ego- Küstera-Hausera.

OBJECTIVES: To evaluate the anatomical results of Wharton vaginoplasty performed with Friebe modification for the surgical correction of vaginal aplasia in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and to assess multidimensionally the sexual functioning of the women. MATERIAL AND METHODS: 20 out of 36 patients with MRKH syndrome who underwent surgical creation of neovagina between October 1995 and September 2009 participated in the study In the group 1 women (55%) had the typical form, 3 women (15%) had the atypical form and 6 women (30%) had the MURCS type of the MRKH syndrome. The dimensions of the vagina (length and width) and the quality of vaginal epithelium (Schiller test) were evaluated. The sexual functioning of the patients was assessed with the Female Sexual Function Index (FSFI) questionnaire and scored in 6 domains (desire, arousal, lubrication, orgasm, satisfaction and pain). RESULTS: The patients underwent the surgery at the median age of 21.7 years (range 16.3-36.6) and were followed-up from 6 months to 11.3 years (median 7.9 years) after the surgery at the median age of 24.7 years (range 18.3-37.3). The vaginal length and width ranged from 4.5 to 12cm (median 6.3) and from 1.5 to 4cm (median 3), respectively. The total FSFI score and 6 domains scores (medians and ranges) obtained within the group of 18 patients (2 patients reported no sexual activity) were as follows: desire 3.6 (1.2-6), arousal 4.5 (2.4-6), lubrication 5.7 (1.2-6), orgasm 4.2 (1.2-6), satisfaction 5.6 (2.4-6), pain 4.8 (0-6), total score 28.2 (10.8-36). No correlation was found between the vaginal dimensions and the total FSFI scores but in 16 (80%) patients the value of the latter was higher than the cut-off value of 26.55, discriminating women with and without sexual dysfunction. CONCLUSION: The modified Wharton vaginoplasty enables women with MRKH syndrome to get a functional vagina of normal anatomy.

Sexuality after sigmoid vaginoplasty in patients with Mayer-Rokitansky-Küster-Hauser syndrome.

OBJECTIVE: To investigate the functional and sexual outcome of sigmoid vaginoplasty in patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. DESIGN: Prospective study. SETTING: University hospital. PATIENT(S): Fifty-nine consecutive patients with MRKH syndrome. INTERVENTION(S): Forty-eight patients underwent sigmoid vaginoplasty, and 11 were treated using the Frank method of dilatation. MAIN OUTCOME MEASURE(S): Functional results and sexuality were evaluated with the use of two standardized questionnaires: the Female Sexual Function Index (FSFI) and the revised Female Sexual Distress Scale (FSDS-R). Questions were added to analyze depression, body image perception, and desire of motherhood. RESULT(S): Out of the 68% of patients who answered the questionnaire, 73% had regular sexual intercourse. The mean total FSFI score was 28 ± 3.1 in the operated group and 30 ± 5.3 in the group treated with the Frank method. Their mean FSDS-R scores were 21 ± 12.1 and 18 ± 13.8, respectively. CONCLUSION(S): Sigmoid vaginoplasty is an effective technique providing a nearly normal sexual function to patients with vaginal aplasia. Despite this, psychologic distress related to sexuality persists in most patients, demonstrating the need for a multidisciplinary support.

Rectosigmoid vaginoplasty in patients with vaginal agenesis: sexual and psychosocial outcomes.

BACKGROUND: The main goal in women with Mayer-Rokitansky-Küster-Hauser syndrome (vaginal agenesis) is creation of a neovagina that will satisfy the patient's desire. We evaluated sexual and psychosocial adjustment in patients who underwent rectosigmoid vaginoplasty because of vaginal agenesis. METHODS: A total of 36 women, aged 21 to 38 years (mean=26) who underwent rectosigmoid vaginoplasty from 1997 to 2006 were evaluated. Sexual and psychosocial appraisal included the Female Sexual Function Index (FSFI), Beck's Depression Inventory (BDI), and standardised questionnaires about postoperative satisfaction, social and sexual adjustment. RESULTS: Mean FSFI score was 28.9 (range=11.5-35.7) with a cut-off score of 26.55 for sexual dysfunction. Out of the 36 women, 10 (27, 8%) had sexual dysfunction. Mean BDI score was 7.55 (cut-off score=0-9 for non-depression). Twenty-eight women (77.8%) were without symptoms of depression, six women (BDI range=10-29) had moderate and two women had severe depression (BDI=42). There were a significant number of patients (P<0.01) with a high satisfaction score in FSFI and low BDI results. Thirty-two (88.9%) of the patients believed that surgery was done at the right time and the main postoperative support came from their family. Thirty-four (94.4%) of the patients reported satisfactory femininity, with a heterosexual orientation. Thirty patients (83. 3%) were very satisfied with the surgery, while 34 considered surgery as the best treatment. CONCLUSIONS: The sexual function and psychosocial status of these patients should be followed long-term to estimate their quality of life.