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.

Sexuality and quality of life of patients operated for Rokitansky syndrome Mayer Kuster Hauser.

INTRODUCTION: Rokitansky syndrome or Mayer Rokitansky Kuster Hauser (SRKMH) is a rare congenital malformation defined by uterovaginal aplasia. The aim of the treatment is to create a neovagina and restore sexual life. However, postoperative results in terms of sexual and overall quality of life of patients remain controversial. AIM: To evaluate the quality of life and sexuality of patients operated on for Rokitansky syndrome. METHODS: This was a retrospective and comparative study between two groups of patients. The first group consisted of patients who had undergone vaginoplasty as part of SRKMH. The second group was that of the controls. Control in terms of sexual function by the Ar FSFI (Arab FemaleSexualFunction Index) and quality of life by the SF36 (Short Forms Health Survey) were used in both groups. RESULTS: The average age of patients operated on for SRKMH was 22.53 years. Eighteen of these patients (60%) were sexually active at the time of the study. Patients operated on for SRKMH had a significantly lower Ar FSFI score compared to the control group. The two areas most affected were lubrication and pain. Likewise, patients who underwent vaginoplasty had a significantly lower SF 36 score compared to the control group. The psychological component was the most affected of the different components of quality of life. CONCLUSION: Sexual function and quality of life after vaginoplasty in the context of Rokitansky syndrome remains unsatisfactory despite the anatomical result.

Issues of identity, perceptions and isolation: An interpretative phenomenological analysis of women's experience of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

The objective of this study was to explore the personal experience of women with MRKH, a rare condition characterised by congenital abnormalities of the genital tract. There are very few qualitative studies into the lived experiences of women with MRKH. Interviews were conducted with 13 women with MRKH and analysed using interpretative phenomenological analysis. Four superordinate themes are discovered: maintaining a viable female identity; acceptance and coping; normality, secrecy and shame and the isolating impact of a lack of knowledge amongst the medical profession. This study also suggests that societal ideals of a 'normal woman' are influencing how MRKH impacts on the self-experienced psychological health. It is suggested that the psychological wellbeing of women with MRKH could be improved with continued multidisciplinary support beyond the initial diagnosis and alongside any vaginal correction.

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 and Psychosocial Outcome After Neovaginoplasty Using Interceed in Females with Mayer-Rokitansky-Küster-Hauser Syndrome: A Case-Control Study.

There is a paucity of research on psychosexual outcomes after neo-vaginoplasty in women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. We compared anatomical and psychosexual outcomes between women with MRKH syndrome who had undergone vaginoplasty and age-matched, sexually active, childless women. Outcomes were assessed using the female sexual function index (FSFI), World Health Organization Disability Assessment Schedule (WHODAS 2.0), General Anxiety Disorder-7 (GAD-7) and Patient Health Questionnaire (PHQ-9) tools. Although mean vaginal length was shorter in women with MRKH syndrome than controls (8.6 ± 1.8 cm vs 9.3 ± 2.6 cm), FSFI scores were comparable in both groups (27.8 ± 1.4 vs. 27.3 ± 4.1). Women with MRKH syndrome reported fewer depressive symptoms, and WHODAS 2.0 scores were comparable in both groups (8.9 ± 2.4 vs. 7.8 ± 6.7). Therefore, psychosexual outcomes for women who had undergone neo-vaginoplasty for MRKH syndrome were not different from those of the general population.

The Sexuality of Adolescents and Young Women With MRKH Syndrome: A Qualitative Study.

BACKGROUND: Vaginal agenesis, most commonly referred as Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome, is mostly diagnosed as primary amenorrhea in teenage girls; although there is plenty of literature concerning the formation of a neovagina, limited research has focused on the psychological burden of this diagnosis to the girls. AIM: To enlighten health providers into the finer aspects of sexuality through the own words and experiences of girls with MRKH under our care. METHODS: Women currently undergoing vaginal dilation or who had completed vaginal dilation within the past year were recruited from February 2019 to January 2020. A gynecologist with training in Sexual Medicine conducted a semistructured interview, which was recorded and then transcribed to identify common themes among interviewees. OUTCOMES: The main outcome explored was the narrative experiences of women with MRKH. RESULTS: 7 women participated, with a mean age of 19.7 (range 17-22 years). None of the girls felt stigmatized, however one reported significant distress at diagnosis, stemming from the attitude of health care professionals and exacerbated by an earlier age at disclosure. All girls accepted that VDT was successful, when it was initiated after they had felt sexual interest and arousal. Exact quantification of the vaginal length at onset, worried 4 as they felt pressurized to achieve a specific length. A few girls reported anxiety over sharing the diagnosis with an intimate partner. All of them pretended at some point to have menses. Childbearing was an important issue for most of the interviewees, but it did not concern them for the time being. All girls had supporting families. However, 5 did not want to share information about VDT with them. One girl reported that openness in discussing genital anatomy, VDT and sexuality, helped her both in completing treatment and adapting in a sexual relationship. CLINICAL IMPLICATIONS: A multidisciplinary team should aim for age-appropriate disclosure and consultation and guide women through VDT and their sexual relations. STRENGTHS AND LIMITATIONS: This is a thorough account of women's perceptions regarding VDT and sexuality in MRKH. However, our conclusions may be limited by the small number of participants. CONCLUSION: Gradual provision of information at disclosure and adjusted timing at VDT may reduce stress in girls with MRKH. Tsitoura A, Michala L. The Sexuality of Adolescents and Young Women With MRKH Syndrome: A Qualitative Study. J Sex Med 2021;18:2012-2019.

Perspectives of 281 patients with Mayer-Rokitansky-Küster-Hauser Syndrome on uterine transplantation.

OBJECTIVES: To investigate the personal, ethical, and financial perspectives of individuals with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), a congenital uterine factor infertility condition, regarding uterine transplantation (UTx). DESIGN: Cross-sectional, quantitative survey. SETTING: A 60-item anonymous electronic questionnaire was disseminated via social media sites. PATIENTS: International members of the Beautiful You MRKH Foundation. INTERVENTIONS: None. MAIN OUTCOME MEASURES: The survey contained UTx educational materials followed by questions assessing participants' baseline knowledge, global perceptions, financial concerns, and ethical considerations regarding UTx. RESULTS: We received 281 responses, with a mean participant age of 28.2 ± 9.8 years. After reviewing the education material, most participants considered receiving a UTx (73%), believed that it should be an option for all women with uterine factor infertility (86%), and believed that it should be covered by health insurance (78%). Respondents perceived the benefits of the procedure to outweigh the risks (67%) and considered it to be an ethical procedure (82%). Almost one-half (49%) were willing to spend more than $10,000 out of pocket to receive the procedure. When asked to rank the risk of UTx to self, donor, and fetus in order of personal importance, 21% ranked their own safety last. CONCLUSION: There is a profound desire in the MRKH community for UTx to become more widely available and affordable. MRKH patients may represent a vulnerable population requiring special considerations for informed consent and rigorous evaluation for UTx. Providers caring for MRKH patients should be prepared to provide education about UTx and to thoughtfully engage with news and media outlets to communicate evidence-supported information.

The Missing Uterus, the Missed Diagnosis, and the Missing Care. Mayer-Rokitansky-Küster-Hauser Syndrome in the Lives of Women in Malaysia.

STUDY OBJECTIVE: To explore the effect of the diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome on affected Malaysian women. DESIGN: Qualitative study with a quantitative component. SETTING: Pediatric and adolescent gynecology unit at Universiti Kebangsaan Malaysia Medical Centre, Malaysia. PARTICIPANTS: Twelve women with MRKH. INTERVENTIONS: Face-to-face interview and short questionnaire. MAIN OUTCOME MEASURES: Thematic analysis was used to understand participants' experiences. RESULTS: There were 7 themes identified: (1) delayed diagnoses; (2) doctors' roles and attitudes; (3) gender identity; (4) family and society's response; (5) reaction toward infertility; (6) managing sexual intimacy; and (7) coping mechanisms. Several participants consulted their physicians regarding their primary amenorrhea at an opportunistic setting. When they were referred to the gynecologists, they were dismayed at the lack of information given. The term, "MRKH" plays an important role to ease information-seeking. Participants felt that the doctors were insensitive toward them. Mental illness is a significant complication of MRKH. All participants acknowledged that infertility was the hardest part of the condition. The importance of blood lineage affects their outlook on childbearing options. Some were afraid of sexual intimacy and worried that they would not be able to satisfy their partners. Participants gained support and bonded with their counterparts in the MRKH support group. CONCLUSION: A multidisciplinary approach including medical, psychological, and social support is essential for the management of MRKH. Adequate information and sexual education plays the utmost importance in preventing social-related complications of MRKH.

From Engulfment to Enrichment: Associations Between Illness Representations, Self-Concept, and Psychological Adjustment in Mayer-Rokitansky-Küster-Hauser Syndrome.

STUDY OBJECTIVE: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a female reproductive disorder characterized by the absence or underdevelopment of the uterus, cervix and vagina. Limited research has examined factors related to psychological adjustment in MRKH. This study aimed to explore associations among illness representations, self-concept, psychological distress, and self-esteem in MRKH. DESIGN: Cross-sectional. SETTING: Participants were recruited globally online and from patient meetings. PARTICIPANTS: A total of 263 patients with MRKH (age 16.1-74.4 years; mean = 31.7 years) completed questionnaires. INTERVENTION: None. MAIN OUTCOME MEASURES: Validated self-reported measures of psychological distress and self-esteem (outcomes) and illness representations, self-concept, social support-seeking, and positive affect (hypothesized correlates) were explored in correlation and hierarchical regression analyses, alongside demographic and clinical variables. RESULTS: Younger age and shorter time since diagnosis was associated with higher distress and lower self-esteem. Patients with MRKH reported significantly higher distress and lower self-esteem than the general population. Higher distress and lower self-esteem were associated with higher reported engulfment (defining one's identity or feeling consumed by MRKH) and beliefs about the serious consequences of MRKH, and lower reported MRKH coherence, enrichment (positive changes to self-identity because of MRKH) and positive affect. CONCLUSIONS: Findings suggest that the impact of MRKH on identity plays an important role in adjustment. High perceived coherence and maintenance of positive affect may play a protective role in psychological adjustment. A 12-month follow-up study is planned to examine associations among these variables longitudinally. Baseline data suggest that early availability of psychological support would be beneficial, and interventions focused on identity and psychoeducation about MRKH would be valuable.

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.

[Iatrogenic urethral coitus]. / Iatrogene urethrale coïtus.

This case discussion on urethral coitus in Mayer-Rokitansky-Küster-Hauser syndrome shows that this rare syndrome can have major physical and psychological consequences. Further consideration shows that a lack of common sense and a lack of action concerning sexual history-taking and careful vulvar examination is the reason that an iatrogenic sexual problem arose in this case. This was not the result of the initial denial of a rare diagnosis. Both attention to these aspects during training and supervision are necessary, since other disorders and medical treatments can also have a negative impact on sexuality.

Anxiety symptoms in patients with Mayer-Rokitansky-Küster-Hauser syndrome: a cross-sectional study.

BACKGROUND: As a congenital malformation that results in infertility and an inability to have vaginal intercourse, Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome places a considerable psychological burden on patients, which results in anxiety symptoms. However, only single case studies or a few small to medium-sized cross-sectional studies were identified to focus on anxiety symptoms in MRKH patients. Thus, the aim of this study was to explore the status of anxiety symptoms and the related factors in patients with MRKH syndrome. METHODS: This cross-sectional study involving 141 patients with MRKH syndrome and 178 healthy women was conducted from January 2018 to December 2018. All participants were required to complete a demographic questionnaire and the Generalized Anxiety Disorder 7-item scale (GAD-7), Patient Health Questionnaire-9, Eysenck Personality Questionnaire-Revised, Short Scale for Chinese, and Chinese Version of the Female Sexual Function Index. The main outcome was the anxiety symptoms measured by the GAD-7. Main outcome was compared between the MRKH syndrome group and the healthy control group. Then, we explored the related factors by comparing patients with and without anxiety symptoms. RESULTS: Of the respondents, 24.1% experienced moderate to severe anxiety symptoms. Patients with MRKH syndrome manifested more severe anxiety symptoms than healthy women. Negative self-evaluation of femininity (odds ratio [OR] 2.706, 95% confidence interval [CI] 1.010-7.247), neurotic personality traits (OR 1.100, 95% CI 1.029-1.175), and coexisting depressive symptoms (OR 4.422, 95% CI 1.498-13.049) were more prevalent in anxious patients. CONCLUSION: The findings stress the importance of anxiety symptom screening in MRKH patients and identify patients at risk of anxiety symptoms, providing a possible basis for future intervention.

Sexual functioning, sexual esteem, genital self-image and psychological and relational functioning in women with Mayer-Rokitansky-Küster-Hauser syndrome: a case-control study.

STUDY QUESTION: Do sexual functioning, sexual esteem, genital self-image and psychological and relational functioning in women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome differ from a comparison group of women without the condition? SUMMARY ANSWER: In comparison to controls, women with MRKH with a non-surgically or surgically created neovagina did not differ in psychological and relational functioning but reported lower sexual esteem and more negative genital self-image, intercourse-related pain, clinically relevant sexual distress and sexual dysfunction, with sexual esteem levels strongly associated with sexual distress and sexual dysfunction. WHAT IS KNOWN ALREADY: Studies on sexual functioning measured with standardized questionnaires in women with MRKH syndrome compared with women without the condition have yielded contradictory results. Factors associated with sexual functioning in this patient population have rarely been investigated. STUDY DESIGN, SIZE, DURATION: Between November 2015 and May 2017, 54 women with MRKH syndrome with a neovagina and 79 age-matched healthy women without the condition were enrolled in this case-control study. PARTICIPANTS/MATERIALS, SETTING, METHODS: All participants had to be at least 18-years old and had to live in a steady heterosexual relationship. Women with MRKH syndrome were asked to participate by their (former) gynecologists at three university hospitals and by MRKH peer support group. Controls were recruited via advertisement in local newspapers and social media. Standardized questionnaires were administered to assess sexual functioning, sexual esteem, genital self-image and psychological and relational functioning. MAIN RESULTS AND THE ROLE OF CHANCE: Women with MRKH syndrome with a surgically or non-surgically created neovagina reported significantly more pain during intercourse (P < 0.05, d = 0.5), but did not differ in overall sexual functioning from control women. More women with MRKH syndrome reported clinically relevant sexuality-related distress (P < 0.05, odds ratio (OR): 2.756, 95% CI 1.219-6.232) and suffered a sexual dysfunction (P < 0.05, OR: 2.654, 95% CI: 1.088-6.471) in comparison with controls. MRKH women scored significantly lower on the sexual esteem scale (SES) (P < 0.01, d = 0.5) and the female genital self-image scale (FGSIS) (P < 0.01, d = 0.6) than controls. No significant differences were found between the two groups regarding psychological distress, anxiety and depression, global self-esteem and relational dissatisfaction. Sexual esteem was significantly associated with the presence of clinically relevant sexual distress (ß = 0.455, P = 0.001) and suffering a sexual dysfunction (ß = 0.554, P = 0.001) and explained, respectively, 40% and 28% of the variance. LIMITATIONS, REASONS FOR CAUTION: Given the nature of the study focusing on sexual functioning, a potential selection bias cannot be excluded. It is possible that those women with the most severe sexual and/or psychological disturbances did or did not choose to participate in our study. WIDER IMPLICATIONS OF THE FINDINGS: The study results add new data to the very limited knowledge about psychosexual functioning of women with MRKH syndrome and are of importance for more adequate counseling and treatment of these women. STUDY FUNDING/COMPETING INTEREST(S): The research was financially supported by the Dutch Scientific Society of Sexology (Nederlandse wetenschappelijke Vereniging Voor Seksuologie). The funding was unrestricted, and there was no involvement in the conduct of the research. There are no conflicts of interest to declare.

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.

Living with permanent infertility: A German study on attitudes toward motherhood in individuals with Complete Androgen Insensitivity Syndrome (CAIS) and Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS).

In this study the authors examined the issue of permanent infertility in two diagnoses of the diverse sex developments (DSD) spectrum: Complete Androgen Insensitivity Syndrome (CAIS) and Mayer-Rokitansky-KÏster-Hauser Syndrome (MRKHS). The participants with CAIS (n = 12) was older, showed a lower wish for a child and was less distressed about their infertility compared to participants with MRKHS (n = 49). Our data indicated an "indifferent" attitude toward motherhood in CAIS and an "ambivalent" attitude in MRKHS. Depression was frequent in both. Infertility is a source of distress. However, the two groups seem to cope in different ways. Comprehensive medical information and psychological support should be provided.

Multiorgan system structural malformations associated with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) type 2: avoiding pitfalls in diagnosis, counseling and treatment.

An 18-year-old virginal woman was referred to the reproductive endocrinology clinic with primary amenorrhoea and secondary sexual development in the absence of pelvic pain. Additionally, she had significant congenital sensorineural hearing loss, autism, bipolar disorder and class III obesity. On physical examination, secondary sexual development was confirmed (Tanner 5 breasts and Tanner 4 pubic hair). She refused further pelvic examination following prior attempts by the referring physicians. Serum leutinizing hormone (LH), follicle sitmulating hormone (FSH). prolactin, estradiol and total testosterone values were within normal limits. Karyotype was 46,XX. MRI demonstrated complete uterine agenesis, short vagina, sacral dysgenesis with complete absence of the coccyx and a horseshoe kidney. Diagnosis of Mayer-Rokitansky-Küster-Hauser Syndrome type 2 was established based on clinical, laboratory and MRI findings. The patient and family were counselled regarding the disease process, techniques for vaginal elongation, sexual activity and future reproductive options.

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.