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• Mayer–Rokitanski–Kuster–Hauser syndrome is a congenital aplasia of uterus and vagina. • It causes absolute infertility, determining psychological disorders. • Psychological evaluation is needed to plan a management strategy and support patients and partners. Mayer–Rokitanski–Kuster–Hauser (MRKH) syndrome consists of a congenital aplasia of the uterus and the upper part of the vagina. It is the most frequent congenital cause of absolute uterine factor infertility, determining psychological disorders due to infertility and reduced quality of sexual activity. Being the necessity of baseline and prolonged assessments, clinicians need evaluation parameters for the monitoring of patients to plan a suitable management strategy and for efficient support before and after interventions, such as neovagina and uterus transplantation. Research of the literature was performed in PubMed and SCOPUS by searching for the terms "Mayer-Rokitanski-Kuster-Hauser" AND "psychological disorders"; from the 60 articles obtained, only 35 articles regarding neovagina creation and uterus transplantation were considered for the present manuscript. Based on the literature, management of MRKH syndrome by neovagina creation, either surgically or not, can restore a satisfactory sexual life and to reduce stress, signs of mental disorder and depression and improve sexual activity and quality of life. A psychological assessment of candidates to UT and of their partners is necessary. Recipients had low levels of anxiety compared to the normal population at baseline but a transiently lowered physical quality of life 1 year after surgery; elevated anxiety scores are associated with childlessness in the long-term evaluation. Further research is necessary to develop suitable evaluation protocols and adequate supportive services, to improve the outcomes of patients who undergo neovagina creation and uterus transplantation. [ABSTRACT FROM AUTHOR]

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by the congenital absence of the uterus and vagina, sometimes with associated extragenital anomalies. Currently, there is limited literature on pelvic pain and comorbid pain syndromes in people with MRKH. The aims of this scoping review were to summarize existing literature on pelvic and generalized persistent pain syndromes associated with MRKH and to identify knowledge gaps for further research into this field. This scoping review followed the Joanna Briggs Institute framework. The population of interest was patients with a diagnosis of MRKH. MEDLINE, CINAHL, Scopus, Cochrane, Embase, and Emcare databases were searched. Articles that did not meet the inclusion criteria or critical appraisal standards were excluded. The resultant articles were reviewed by 2 independent researchers, and a third was used in cases of disagreement. A descriptive analytical method was used for data analysis. We screened 3348 articles for eligibility. Of these, 39 articles, which described 1353 cases of MRKH, met the criteria. Four studies described baseline pelvic pain in MRKH, 19 described acute presentations, and 13 described postintervention pain levels. Despite the paucity of research, this review found that cyclic pelvic pain was mostly present in women with uterine remnants, whereas pelvic pain in those without remnants was poorly understood. There were no studies exploring generalized persistent pain syndromes in MRKH. Further cross-sectional studies are needed to elucidate the prevalence and levels of pain syndromes in MRKH. [ABSTRACT FROM AUTHOR]

Introduction and Hypothesis: Congenital cervicovaginal agenesis in the presence of a functional endometrium is a rare Müllerian anomaly. The management ranges from hysterectomy historically to various reconstructive procedures more recently. We report our experience with utero-colo-vaginoplasty in the management of this anomaly and its long-term follow-up. Methods: The case records of all the patients with vaginal or cervicovaginal agenesis in our hospital from January 2002 to December 2019 were reviewed retrospectively. The patients were then called for an outpatient visit and examined in detail. The anatomical variations, surgical procedures and outcomes were recorded and analysed. Results: Sixteen patients aged 14 to 26 years were included during the study period. They presented with cyclical painful cryptomenorrhea. Magnetic resonance imaging (MRI) confirmed cervicovaginal or distal vaginal agenesis. All the patients underwent utero-colo-vaginoplasty. Intraoperative rectal injury led to post-operative faecal leak from the perineal wound in one patient in the post-operative period. Restoration of painless menstrual flow was possible in all 16 cases. Long-term complications were seen in 4 patients. These were stenosis of the perineal neovaginal orifice in 2 patients, obstruction at colo-uterine anastomosis in 1 patient and mucosal prolapse at the neovagina in 1 patient. Three of these patients needed secondary surgical procedures. Five were sexually active and reported consummation of penetrative intercourse. None of them had conceived. Conclusion: In our experience, utero-colo-vaginoplasty allows for regular painless menstruation and coitus with minimal long-term complications. The sole disadvantage is the failure to conceive. [ABSTRACT FROM AUTHOR]

Objective: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder that results in vaginal agenesis. Lee's neovaginoplasty is a novel surgery for reconstructing the vagina. Transneovaginal oocyte retrieval completely changes the scope of fertility for patients with MRKH syndrome who have undergone neovaginal reconstruction. Case report: A 22-year-old female with type 1 MRKH syndrome underwent Lee's neovaginoplasty successfully. Four years later, she sought embryo cryopreservation consultation and underwent controlled ovarian hyperstimulation. Upon examination, her anti-Müllerian hormone level was 1.97 ng/ml and she had only eight antral follicles. The neovaginal length was 8 cm with elasticity and extensibility. Transneovaginal oocyte retrieval was performed under ultrasound guidance, and seven oocytes were retrieved. The follicle-to-oocyte index was 87.5%. Conclusion: Lee's neovaginoplasty is a promising surgery for reconstructing the vagina in MRKH syndrome, and this case shows that transneovaginal oocyte retrieval can be successfully performed after vaginal reconstruction. This technique provides a minimally invasive option for retrieving oocytes in patients of MRKH syndrome.

Vaginal agenesis (VA) is frequently associated with mullerian agenesis. VA treatments include mechanical dilation and surgical vaginoplasty. We created a vaginal expansion sleeve (VES) as a novel device to progressively lengthen the vaginal canal. This study evaluated the histologic effects of the VES on rat vaginal tissue. The VES is a spring-like device made of proprietary woven cylindrical material and flat resin caps. The VESs were constructed as 25–30 mm, pre-contracted springs, which were secured into the vaginas of six Sprague Dawley rats and allowed to re-expand post-surgically. After one week, the VESs were removed, and the vaginas were harvested and measured in length. Test (n = 6) and control (n = 4) formalin-fixed paraffin-embedded tissues were stained with hematoxylin and eosin (H&E), Masson’s trichrome, and anti-Desmin antibodies. The VESs achieved significant vaginal lengthening. The mean vaginal canal length increased from 20.0 ± 2.4 mm to 23.8 ± 1.2 mm after removal of the VESs (n = 6, p < 0.001), a 19% increase. There was a positive correlation between the expander/tension generated in the vagina and the amount of acute and chronic inflammation. H&E staining revealed increased submucosal eosinophilia in five of the six test tissues. One VES sample that was lengthened to 30 mm long showed evidence of lymphocytic and neutrophilic inflammation. Desmin immunostaining and Masson’s trichrome stain revealed a thinner muscularis with more infiltrative fibrous tissue between muscle fibers in the test tissue compared to the control tissue. Although effective, the VES may provoke at least a transient increase in eosinophils consistent with a localized immune reaction during muscularis remodeling.

The diagnosis of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is often a lengthy process that typically occurs during late adolescence. To support optimized and patient-centered care, this study aimed to investigate how women with MRKH syndrome experience the diagnostic process. From January 2021 to March 2021, we conducted in-depth interviews with 18 Danish women (≥25 years) diagnosed with MRKH syndrome. The interviews lasted a median of 92 minutes (range: 67-117). Data were analyzed using thematic analysis. As teenagers or young women at the time, all women had experienced the diagnostic process in the nonspecialized healthcare sector as deeply upsetting due to distressing gynecological examinations, use of inappropriate language, and considerable diagnostic delay. When reaching the specialized health care sector, questions could finally be answered, but this information and support did not significantly alter their feelings of being "deviant" or "flawed". The women continued their diagnostic odyssey beyond the health care system and found online communities that gave them valuable support in living with MRKH syndrome. Women experience the diagnostic odyssey of MRKH syndrome as upsetting and potentially traumatizing beyond the diagnosis. Healthcare professionals can influence young women's understanding and experience of MRKH syndrome by using inclusive language (eg, avoiding "deformity") and addressing all that is normal and functioning (eg, external genitalia and potential for sexual pleasure). In nonurgent conditions, young women should be given the choice to delay a genital examination. [ABSTRACT FROM AUTHOR]

The development of the female genital system is a complex process that depends on a series of events involving cellular differentiation, migration, fusion, and recanalization. Failure of any of these processes results in congenital anomalies. Developmental anomalies can occur at various stages, resulting in conditions that impact both the urinary and reproductive systems. In younger patients, such malformations can significantly affect their overall health and quality of life, including aspects such as fertility, sexual function, and psychological well-being. The psychosexual effects of vaginal agenesis should not be overlooked, and clinical care primarily involves comprehensive counseling and support through open communication with the patient. For adult patients, treatment for vaginal agenesis typically starts with therapeutic counseling and education, with non-invasive vaginal dilation being recommended as the first-line approach, or surgery if necessary. Consequently, managing these issues often requires a multidisciplinary approach, engaging specialists such as urologists, gynecologists, endocrinologists, and geneticists, among others. Early detection and timely intervention can greatly enhance the outlook for individuals with these conditions. Besides considering the patient's expectations, the surgeon's experience plays a crucial role in selecting the appropriate surgical technique. This is because the success of the initial surgery is critical to the effectiveness of any subsequent procedures if required. In this review, the evaluation and treatment of vaginal agenesis, which constitutes an important part of congenital anomalies of the vagina, were discussed. [ABSTRACT FROM AUTHOR]

Background Objectives Search Strategy Selection Criteria Data Collection and Analysis Main Results Conclusions Congenital uterine anomalies include a wide diversity of uterine malformations that can compromise reproductive potential. Uterus transplantation (UTx) proposes an innovative treatment for absolute uterine factor infertility; however, there is a lack of standardized protocols to guide clinical management among this population.To describe recipient and donor characteristics and obstetric outcomes in patients undergoing UTx.We performed a literature search using the PubMed database to retrieve available scientific articles. We analyzed the references of included articles to assess additional articles that could be eligible to be included in the review. Likewise, we identified further studies using other methods, including Google Scholar.Titles and abstracts were screened in duplicate to select original reports with information available for the outcomes of interest.This review assessed the advantages and disadvantages of the techniques used, patient characteristics, obstetric and non‐obstetric complications, functional duration of the organ, and neonatal outcomes.Among the 36 reports included in this review we found 55 pregnancies and 38 live births following UTx and a higher success rate for in vivo uterine donations. The most common obstetric complications reported included miscarriage, pre‐eclampsia, and gestational hypertension. The most common non‐obstetric complications reported include episodes of rejection, acute kidney injury, anemia, and cholestasis. Living donors required a comprehensive preoperative workup, decreasing organ rejection, infection, and vascular complications.More studies are needed to standardize the UTx procedure and improve obstetric, fetal, and neonatal outcomes. Further understanding of which recipient and donor characteristics minimize complications will significantly decrease the risk of adverse outcomes. [ABSTRACT FROM AUTHOR]

To analyze the features of the epithelia coating neovaginas after vaginoplasty in women affected by Mayer-Rokitansky-Küster-Hauser syndrome We conducted a retrospective analysis of prospectively collected data. Women affected by Rokitansky syndrome who underwent neovaginal biopsy after vaginoplasty (McIndoe surgery, intestinal vaginoplasty, Vecchietti surgery, and Davydov surgery) were included. Macroscopic mucosal features were assessed through clinical examination and the Schilling test. Each biopsy specimen was prepared for examination by light microscopy and in some cases by scanning electron microscopy (SEM). Thirty-six patients (4 McIndoe, 2 intestinal vaginoplasty, 14 Vecchietti, and 16 Davydov) were included. All biopsies were performed without complications. In McIndoe's neovaginas, the mucosal microscopic features were similar to normal skin, with large areas of preserved epithelium, heterogeneous presence of dermal papillae, and superficial keratinization. The characteristics of the intestinal neovagina's surface were similar to those of a sigmoid colon, with well-shaped glands, cylindrical cells, and a secreting mucosa. In Vecchietti neovaginas, the surface the epithelium was flat and multilayered, highly similar to that of a normal vagina, with the presence of glycogen and superficial desquamation. On medium SEM magnification evaluation, the epithelium presented flattened polygonal cells. Finally, in Davydov neovaginas, none of the specimens had persistent mesothelial elements. The squamous neo-epithelium had regular aspects of differentiation with the presence of glycogen. At greater SEM magnification, microridges were evident, with a regular distribution. Each different technique of vaginoplasty leads to unique histological and structural features of the neovagina's mucosa. Knowledge of these elements must be the basis for the choice of the most appropriate intervention. [ABSTRACT FROM AUTHOR]

Aim: The aim of this paper is to present the evidence on the effectiveness of non‐surgical interventions to improve health and well‐being in women living with Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome. Design: Systematic review guided by Preferred Reporting Items for Systematic Reviews checklist. Data Sources: The search was conducted between June and September 2022 across the following databases: CINAHL, EMBASE, Medline, PsycINFO and Cochrane. Trial registries (clinicaltrials. gov, World Health Organization International Clinical Trials Registry Platform (ICTRP), Cochrane Controlled Trials Register‐CCTR), Google scholar, dissertations, conference proceedings and reference lists of included studies were also searched. Corresponding authors, formal and informal MRKH groups were contacted to obtain any significant studies or reviews. Review Methods: Eligible were only English‐language empirical studies of any time period. The review followed narrative synthesis. Results: Twenty‐three studies were identified that fit the inclusion criteria which included 1540 MRKH syndrome affected women. Four studies were on psychological interventions (n = 85) and 19 studies (vaginal dilation therapy n = 897, coital dilation n = 57) focused on non‐surgical vaginal dilation as a measure to vaginal agenesis in MRKH syndrome. Conclusions: Clearly, vaginal dilation is a viable initial treatment option for women with MRKH syndrome. There is limited evidence that 'coital dilation' is an effective method of dilation for vaginal agenesis. The literature, however, supports the need for psychological intervention to improve health and well‐being. Impact: Women with MRKH syndrome who require dilation can receive guidance and support from their healthcare providers, particularly sexual and reproductive health nurses, clinical nurse specialists and gynaecologists. From the point of diagnosis, clinical psychologists should be involved. As much as feasible, family and partner support can be encouraged. Patient or Public Contribution: No patient or public contribution. [ABSTRACT FROM AUTHOR]

Vaginal agenesis (VA) is frequently associated with mullerian agenesis. VA treatments include mechanical dilation and surgical vaginoplasty. We created a vaginal expansion sleeve (VES) as a novel device to progressively lengthen the vaginal canal. This study evaluated the histologic effects of the VES on rat vaginal tissue. The VES is a spring-like device made of proprietary woven cylindrical material and flat resin caps. The VESs were constructed as 25–30 mm, pre-contracted springs, which were secured into the vaginas of six Sprague Dawley rats and allowed to re-expand post-surgically. After one week, the VESs were removed, and the vaginas were harvested and measured in length. Test (n = 6) and control (n = 4) formalin-fixed paraffin-embedded tissues were stained with hematoxylin and eosin (H&E), Masson's trichrome, and anti-Desmin antibodies. The VESs achieved significant vaginal lengthening. The mean vaginal canal length increased from 20.0 ± 2.4 mm to 23.8 ± 1.2 mm after removal of the VESs (n = 6, p < 0.001), a 19% increase. There was a positive correlation between the expander/tension generated in the vagina and the amount of acute and chronic inflammation. H&E staining revealed increased submucosal eosinophilia in five of the six test tissues. One VES sample that was lengthened to 30 mm long showed evidence of lymphocytic and neutrophilic inflammation. Desmin immunostaining and Masson's trichrome stain revealed a thinner muscularis with more infiltrative fibrous tissue between muscle fibers in the test tissue compared to the control tissue. Although effective, the VES may provoke at least a transient increase in eosinophils consistent with a localized immune reaction during muscularis remodeling. [ABSTRACT FROM AUTHOR]

Puberty transforms a girl into a fertile woman, and its social importance is so great that any deviation from normality may be the cause of considerable embarrassment and anxiety. Delayed puberty in girls is defined as the absence of physical manifestations of puberty by the age of 13 years. Primary amenorrhoea is the absence of menarche and needs to be evaluated in the context of secondary sexual characteristics. Differential diagnoses can be classified according to the level of gonadotrophins. Differences in Sex Development and Complex Obstructed Mullerian Duct Anomalies should be investigated and managed in a specialist centre within a multidisciplinary team. Psychological support is essential. [ABSTRACT FROM AUTHOR]

Vaginal agenesis is the most common condition affecting women known as Mayer-Rokitansky-Kã¼Ster-Hauser syndrome (MRKH). After surgery, vaginal stents are prosthetic devices that are utilized to keep the vagina that was rebuilt from potentially contracting. They ensure that the neovagina keeps its shape by assisting in maintaining the width, depth, and preventing stenosis. But there are some potential problems associated with the use of vaginal stent which needs to be addressed for better functioning and in improving overall quality of life for women. This review article deals with the potential problems associated with the vaginal stent. [ABSTRACT FROM AUTHOR]

Purpose: This systematic review aims to provide a data synthesis about the risk of neovaginal cancer in women with Müllerian anomalies and to investigate the association between the adopted reconstructive technique and the cancer histotype. Methods: PubMed, MEDLINE, Embase, Scopus, ClinicalTrials.gov and Web of Science databases were searched from inception to March 1st, 2023. Studies were included if: (1) only women affected by Müllerian malformations were included, (2) the congenital defect and the vaginoplasty technique were clearly reported, (3) the type of malignancy was specified. Results: Literature search yielded 18 cases of squamous cell carcinoma and two cases of vaginal intraepithelial neoplasia 3 (VAIN 3). Of these, 3 had been operated on according to the Wharton technique, 8 according to the McIndoe technique, 3 with a split-skin graft vaginoplasty, 2 according to the Davydov technique, 2 with a simple cleavage technique, 1 according to the Vecchietti technique and 1 with a bladder flap vaginoplasty. A total of 17 cases of adenocarcinoma and 1 case of high-grade polypoid dysplasia were also described. Of these, 15 had undergone intestinal vaginoplasty, 1 had been operated on according to the McIndoe technique and 1 had undergone non-surgical vaginoplasty. Finally, 1 case of verrucous carcinoma in a woman who had undergone a split-skin graft vaginoplasty, was reported. Conclusion: Although rare, neovaginal carcinoma is a definite risk after vaginal reconstruction, regardless of the adopted technique. Gynaecologic visits including the speculum examination, the HPV DNA and/or the Pap smear tests should be scheduled on an annual basis. [ABSTRACT FROM AUTHOR]

Rubinstein-Taybi syndrome (RSTS) is a multi-system neurodevelopmental condition caused by deficiency of CREBBP (16p13.3) or EP300 (22q13.2). Müllerian agenesis, or Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is defined as congenital agenesis of the uterus, cervix, and upper vagina without a definite genetic cause. We present a 14-year-old female with RSTS type 1 (CREBBP, c.4395-2A>C) and MRKH, the first documented in the literature. Following presentation to Gynecology for anticipatory guidance regarding future menstrual suppression and follow-up of previously diagnosed labial adhesions, exam under anesthesia revealed a single urogenital opening with cystoscopy demonstrating a normal urethra and bladder. Laboratory evaluation was consistent with peripubertal female gonadotropins and estradiol, 46,XX karyotype, and normal microarray, and a pelvic MRI confirmed Müllerian agenesis. Given this case, we assessed our cohort of females with RSTS and found that 4 of 12 individuals also had Müllerian anomalies. Gynecologic evaluation should be a part of medical care for females with RSTS, particularly in individuals with delayed menarche or abnormal menstrual history, on the basis of the observed association between RSTS and Müllerian anomalies in this case series. Although several candidate genes and copy number variants are associated with MRKH, no candidate genes in close proximity to the 16p13.3 region have been identified to explain both RSTS and MRKH in the index patient. Due to the regulatory nature of CREBBP during embryonic development, we theorize that CREBBP may play a role in the migration of Müllerian structures during embryogenesis. [ABSTRACT FROM AUTHOR]

Management of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome patients is by creating functional neovagina through surgical or nonsurgical route. Surgical repair using minimally invasive technique is a favorable option in creating neovagina. In this study, the patients underwent neovaginoplasty. Clinical follow-ups were done at 3, 6, and 12 months postoperatively. The primary outcomes were anatomic and functional successes; anatomical success was defined as a ≥6 cm-long neovagina that allows for easy introduction of two fingers, and functional success was defined with Female Sexual Function Index FSFI-6 questionnaire score above 19. Modified neovaginoplasty using autologous peritoneal graft was performed on the patients (n = 6). Follow-up showed mean vaginal lengths of 8.16 cm, mean surgery time of 175 min, mean blood loss of 59.17 ml, and mean duration of hospital stay of 2 days, with an average FSFI-6 score of 25,2. Therefore, we concluded that laparoscopic approach using modified technique of autologous peritoneal graft provides satisfactory result. [ABSTRACT FROM AUTHOR]

To describe surgical correction of vaginal agenesis via a modified laparoscopic Vecchietti procedure with the goal of disseminating knowledge and improving surgical technique An 18-year-old female presented with primary amenorrhea, age-appropriate secondary sex characteristics, a shallow vagina, and 46,XX karyotype. Imaging showed rudimentary uterine horns and normal ovaries, kidneys, and spine. Diagnosis of Mayer-Rokitansky-Küster-Hauser syndrome type I was made. After an unsuccessful attempt at vaginal dilation and extensive counseling, the patient chose to have a laparoscopic Vecchietti procedure. Vecchietti vaginoplasty eliminates the need for grafts and creates a neovagina with accelerated vaginal dilation by stretching the introital mucosa with a spring mechanism. A modified laparoscopic Vecchietti procedure was performed. Postoperatively, daily suture adjustments were made. When the device was removed after 7 days, the examination revealed a 9-cm vaginal canal, which was maintained with self-dilation. [ABSTRACT FROM AUTHOR]

Objective: To report the use of progressive, high-frequency movement-based dilator therapy (MBDT) to create a neovagina in a patient with congenital vaginal agenesis. Design: Case report. Setting: Tertiary care military hospital. Patient(s): A 22-year-old woman with congenital vaginal agenesis. Intervention(s): Self-directed MBDT. Main Outcome Measure(s): Vaginal elongation by self-directed MBDT. Result(s): The patient achieved a 6.5-cm vaginal length after 6 pelvic health physical therapy sessions over a span of 4 months of progressive, high-frequency MBDT. Conclusion(s): Progressive, high-frequency MBDT should be considered as part of a first-line dilator therapy regimen for patients with congenital vaginal agenesis interested in creating a neovagina.

Background & aim: A vaginal septum is a condition in which the wall of the tissue dividing vagina does not resolve completely. This complication can be transverse and longitudinal. Although the occurrence of a transverse vaginal septum is 2 per 100,000 births, the exact etiology of this anomaly remains still unidentified. In this study, a case of high transverse vaginal septum with vaginal and cervical agenesis is reported.Case report: A 13-year-old single and virgin girl presented with cyclic abdominal pain. On examination and magnetic resonance imaging, vaginal and cervical agenesis was diagnosed. Surgical therapy included an incision in the lower part of the uterus at the site of the bulging and the use of a hysterometer passing down the uterus. The cervix was not touched, cervical agenesis was diagnosed, and the patient underwent abdominal hysterectomy and bilateral tubal salpingectomy. The pathological results confirmed the cervical agenesis.Conclusion: It is recommended that in patients with high transverse vaginal septum, hysterectomy be performed for the treatment of cervical and vaginal agenesis. However, it may not be a suitable treatment choice for some cases, and it is better to decide based on the patient's condition.

Complete androgen insensitivity syndrome (CAIS) occurs in XY individuals due to androgen receptor dysfunction and presents with female phenotype and primary amenorrhea. Patients are typically raised and identify themselves as female and the absence of an upper vagina results in psychological and sexual problems. The cases of two sisters with CAIS that were managed differently regarding the neovagina formation are presented. Non-surgical neovagina formation results in sufficient vaginal depth and satisfying sexual life and may be preferred over the operation. Our findings highlight that the interaction of CAIS patients with each other and the continuous psychological and physical support by the medical team contribute significantly to the outcome.

To understand variations and experiences of vaginal lengthening internationally in individuals with congenital underdevelopment of the uterus, cervix, and upper vagina or Müllerian agenesis In this study, we used a cross-sectional mixed-methods design incorporating quantitative and qualitative questionnaires. Adults with Müllerian agenesis completed questionnaires with quantitative and open-ended qualitative questions about their vaginal lengthening experiences. Data were analyzed using descriptive statistics and inductive thematic analysis. Of 616 respondents meeting inclusion criteria (representing 40 countries), 46% (n = 284) reported no vaginal lengthening intervention. Vaginal lengthening was commonly reported by participants from North America and Europe (59%) and less commonly by participants from Africa, Asia, and South America (16%). Of those who had undergone vaginal lengthening, 72% reported dilator use, 34% coital dilation, and 39% surgery. Four major themes were identified in response to the open-ended vaginal lengthening experience question: (1) difficult physical symptoms, (2) practical and psychosocial challenges, (3) intimate relationships and sexual satisfaction, and (4) impact of experiences with healthcare providers. This study highlights vaginal lengthening practices internationally and shared themes related to significant challenges and positive experiences. The findings show room for improvement in the counseling and care surrounding vaginal lengthening. Future research should investigate factors that influence decision-making about vaginal lengthening and work toward international consensus on best care practices in Müllerian agenesis. [ABSTRACT FROM AUTHOR]

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A common congenital abnormality affecting the female genital system is vaginal agenesis. It may appear on its own as a developmental aberration or as a component of a larger collection of anomalies. This disorder is frequently linked to the MRKH syndrome (Mayer-Rokitansky-Küster-Hauser syndrome). A new vaginal canal must be made and positioned between the bladder and the rectum in order to treat vaginal agenesis. Long-term effectiveness depends on maintaining the width and depth of the surgically created vaginal region and avoiding restriction. In this article, the surgical management of nonsyndromic vaginal agenesis in a 42-year-old lady is described. Digital fabrication methods were used to create a personalised acrylic vaginal stent. This customised vaginal stent helped gain patency of vaginal canal. These stents can be used to prevent neovaginal stricture and shrinking as well as to preserve the width and depth of the vaginal canal.

Congenital malformations that include vaginal agenesis require surgical treatment in childhood, but deforming sequelae present in adulthood constitute a reason for consultation that must be solved. This case is intended to highlight the importance of integrating surgical and complementary techniques in the safe treatment of disabling surgical sequelae in vaginal reconstruction showing the results of the combined treatment with botulinum toxin and adipose stem cells in a patient with Mayer Rokitansky syndrome and post-surgical vaginal stenosis. This treatment decreased the pain and contracture of the vaginal introitus that allowed physical examination and that patient was reintegrated into sexual life. [ABSTRACT FROM AUTHOR]

Introduction: Inflammation and injury within reproductive organs, including artificial vagina, can facilitate human immunodeficiency virus (HIV) transmission. However, HIV infection in a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome has been rarely described. Both MRKH and HIV diagnoses are linked to psychological distress in patients. Case description: We report on a case of a 34-year-old HIV-infected woman with MRKH syndrome. The presented report is the second described case of HIV infection in a woman with MRKH syndrome, the previous one dating back to 1992. Entry of infection were squamous cells, covering the wall of artificial vagina. Acquired immunodeficiency syndrome (AIDS) diagnosis was delayed because symptoms, such as thrombocytopenia, subfebrile illness, cachexia, malaise, and persistent diarrhea were misdiagnosed by physicians. Conclusions: HIV infection is rarely described in women with MRKH syndrome; however, the procedure of artificial vagina can favor sexually transmitted diseases (STD) transmission, including HIV. In any case, a patient with unexplained symptoms or illnesses that may have any connection with HIV infection should be tested for HIV, even in the absence of risk factors. Moreover, these two diagnoses combined can be considered as huge psychological burden for the patient. Receiving information about incurable infertility due to MRKH during young adolescence might cause severe psychological complications. Despite many sources of HIV infection, ways of transmission, and risk factors, HIV patient still can face stigma associated with the infection. Psychological circumstances of HIV infection can include depressive symptoms, feeling of guilt, fear of consequences, and escape from reality. [ABSTRACT FROM AUTHOR]

The demand for sexual life makes vaginoplasty a crucial need for patients who had complete vagina agenesis. We present our experience on vaginoplasty by using a maximum amount of meshing oral mucosal grafts in a case series study on 43 patients. The patients were followed up for an average of 19 months, ranging from 6 months to 4 years. No serious complications were reported. The patients gained good results also at neovagina and donor sites. These results were proved on clinical, endoscopy images, and biopsies. We recommend seven tips in the techniques to be used to achieve these significant results. [ABSTRACT FROM AUTHOR]

Background: Around 1 in 400-5000 live births of females result in vaginal agenesis, a malformation of the female genital tracts. Using an amnion graft to treat vaginal agenesis is a straightforward, widely accessible, economically advantageous, and physiological surgery that leaves no additional scars on the body. This study sought to ascertain whether individuals with vaginal agenesis may successfully undergo laparoscopic-assisted vaginoplasty utilising amnion as a transplant to create a neovagina. Methods: Ten cases of vaginal agenesis linked to Mayor- Rokitansky-Kuster-Hauser (MRKH) Syndrome were included in this case series and were hospitalised within a year. The ladies with MRKH condition were either previously married or unable to consummate their marriage due to a blind vagina when they were brought for surgical intervention. To maintain the patency of the neo-vagina, a laparoscopically guided vaginoplasty was carried out as part of the care. Results: In all but one of the patients, laparoscopy-guided vaginoplasty with an amnion graft was effective. The procedure was stopped when the rectum was hurt, and the harm to the rectum was repaired. Except for one example, the functional outcomes of the neovagina were quite good. None experienced any major postoperative problems. The patients were happy with the postoperative results. Also, the neovagina was psychologically acceptable and sufficiently patent for sexual function. Conclusion: Over the past few years, the new vaginoplasty technique has developed. The most common practice, however, is a laparoscopic approach using several graft materials. A safe and efficient method for treating MRKH syndrome patients with vaginal agenesis is vaginoplasty with amnion grafts. [ABSTRACT FROM AUTHOR]

Vaginal atresia is seen in genetic disorders such as Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome, which can cause significant sexual dysfunction. Current treatments include surgical reconstruction or mechanical dilation of the vaginal canal. Mechanical dilation requires patients to be highly motivated and compliant while surgical reconstruction has high rates of complications. This study evaluated a novel vaginal expansion sleeve (VES) method as an alternative treatment for vaginal atresia. The proprietary cylindrical VES is a spring-like device consisting of polyethylene terephthalate helicoid trusses capped at each end with a fixed diameter resin cap for fixation within tissues. Following the development of the VES and mechanical characterization of the force–length relationships within the device, we deployed the VES in Sprague Dawley rat vaginas anchored with nonabsorbable sutures. We measured the VES length–tension relationships and post-implant vaginal canal expansion ex vivo. Vaginal histology was examined before and after implantation of the VES devices. Testing of 30 mm sleeves without caps resulted in an expansion force of 11.7 ± 3.4 N and 2.0 ± 0.1 N at 50% and 40%, respectively. The implanted 20 mm VES resulted in 5.36 mm ± 1.18 expansion of the vaginal canal, a 32.5 ± 23.6% increase (p = 0.004, Student t test). Histological evaluation of the VES implanted tissue showed a significant thinning of the vaginal wall when the VES was implanted. The novel VES device resulted in a significant expansion of the vaginal canal ex vivo. The VES device represents a unique alternative to traditional mechanical dilation therapy in the treatment of vaginal atresia and represents a useful platform for the mechanical distension of hollow compartments, which avoids reconstructive surgeries and progressive dilator approaches. [ABSTRACT FROM AUTHOR]

Objectives: To assess the postoperative functional, anatomical outcome and complications of various surgical procedures of vaginoplasty performed for patients with vaginal agenesis at our institution. Methods: This was a cross-sectional study of 14 patients (age range 17-40 years), who underwent vaginoplasty at the Aga Khan University Hospital, Karachi, Pakistan between January 2008 to December 2018. We aimed to assess the anatomical outcomes in terms of vaginal depth, axis and functional outcome as painless and satisfactory vaginal intercourse. Results: The mean age and mean body mass index (BMI) of the cases were 26.8±8.1 years and 27.7143±4.6 respectively. All were phenotypically female, with only two cases of XY genotype. Two patients were married on presentation. On evaluation, four cases had Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, three had androgen insensitivity syndrome (AIS), one case had congenital adrenal hyperplasia and six cases did not fit into any diagnosis. Associated renal anomalies were diagnosed in 14.3% of cases. The performed procedures were; Singaporean flap vaginoplasty (in four patients), Lee’s, modified McIndoe and pull-through vaginoplasty (in three each patients), and Davydov vaginoplasty (in one patient). One patient was complicated by intraoperative bladder injury (p<0.63) and two cases by vaginal stenosis (p<0.43). The mean operative time was 120 minutes and the mean estimated blood loss was 200mls. Postoperatively, the vaginal length varied from 6-10 cm with a normal vaginal axis and satisfactory sexual activity. Conclusions: Vaginal agenesis is associated with several sexual disorders and despite the various surgical options available, the best procedure in terms of fewer complications and best surgical outcome is yet to be determined. [ABSTRACT FROM AUTHOR]

Imperforate hymen is a common condition that with classic appearance and presentation in puberty. This may be diagnosed in a neonate when mucocolpos is noted. There are many great pretenders of this condition, but the hallmarks include a bulging hymen with blue hue at puberty. If this is not seen on external genital exam, the provider should proceed with magnetic resonance imaging to better assess the reproductive tract anatomy before engaging in surgery. For providers uncomfortable in managing conditions often confused with an imperforate hymen, referral to a Pediatric and Adolescent Gynecologist or specialist comfortable managing these conditions, is recommended. [ABSTRACT FROM AUTHOR]

Introduction Meyer-Rokitanski-Küster-Hauser syndrome, also reffered to as Müllerian aplasia, is a congenital disorder characterized by agenesis or hypoplasia of the uterus and upper part of the vagina in female with normal female karyotype (46,XX). In these women the external genitalia have a normal structure and their internal endocrine organs function properly. Most often, this syndrome is diagnosed in adolescence due to primary amenorrhea. Material and methods This review was based on available data collected in the PubMed data base and Google Scholar data and published between 2011 and 2021. The research was done by looking through keywords such as:“Mayer-Rokitansky-Küster-Hauser syndrome”, “MRKH syndrome”, “uterine agenesis” and “primary amenorrhea”, "vaginal agenesis", "uterus transplantation". Conclusions Recent scientific achievements have shown how much can be changed in the lives of patients with MRKH syndrome. It should be remembered that this is a disease that needs treatment by specialists in many fields. Treatment should include not only medical side of problem but also should take care of psychical side of patients life. During the last 10 years several advances have been made in MRKH syndrome research, especially within the fields of genetics, non-surgical management, and uterus transplantation as the first available fertility treatment.

Introduction: Obstructive vaginal anomalies such as transverse septum and vaginal agenesis usually present with primary amenorrhea and pelvic pain. This study was performed with aim to evaluate the results of vaginal reconstructive surgery (vaginoplasty) in patients with vaginal agenesis (Rokitansky syndrome) and transverse vaginal septum.Methods: This cohort study was performed on all patients with vaginal agenesis and transverse vaginal septum who had underwent vaginoplasty in the pelvic floor disorders department of Imam Khomeini Hospital ,Tehran in 2014-2019. Basic information, clinical symptoms at admission, type of Mullerian anomaly, type of surgery, complications during surgery, vaginal re-stenosis and pregnancy were collected from patients' files and by phone calls and follow up visits after surgery. Central and dispersion indices were used to describe the data. Frequency distribution was presented in the form of tables.Results: 33 patients had transverse vaginal septum and 35 cases had vaginal and uterine agenesis (Rokitansky syndrome). In terms of early symptoms at admission, the most common symptoms were amenorrhea, pelvic pain, and inability to sexual intercourse or dyspareunia. In 11 cases (33.33%), vaginal re-stenosis occurred after correction of transverse vaginal septum, all of which occurred in the first 3 months after surgery due to incorrect use of vaginal dilator. Laparotomy was performed in 2 cases (6%) due to severe abdominal pain after initial surgery that was diagnosed as pelvic endometriosis. In the vaginal agenesis group, vaginal re-occlusion occurred in5 cases (14.28%) after the surgery.Conclusion: The most common complication after surgery was vaginal re-obstruction, so training the patient and emphasizing on the regular use of vaginal dilator is very important to prevent this complication.

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Context: Understanding mental health issues facing individuals with disorders/differences of sex development (DSD) is crucial for optimizing evidence-based practices in this population., Objectives: To compare the prevalence of psychiatric diagnoses among patients diagnosed with complete androgen insensitivity syndrome (CAIS) or Müllerian duct aplasia/agenesis (MA) to male and female reference groups., Design: Retrospective cohort study., Setting: Three large integrated health systems., Participants: All individuals with confirmed CAIS or MA enrolled in one of three Kaiser Permanente healthcare systems between January 1, 1988, and January 31, 2017. For each DSD patient, age-, race/ethnicity- and health system-matched male and female referents with typical sex development were randomly selected., Outcomes/measures: Mental health diagnoses and use of psychiatric medications., Results: The prevalence of anxiety and depressive disorders in the CAIS and MA cohorts was approximately twice as high as in male referents without DSD, but the corresponding differences relative to female referents were less evident. A subgroup of MA patients with uterine agenesis had higher prevalence of bipolar disorder than either reference group, but these results were accompanied by wide confidence intervals. Women with CAIS and MA more frequently filled psychiatric medications compared to male but not female referents., Conclusion: On balance, these findings are reassuring, albeit requiring confirmation in other settings. Future studies using longitudinal designs and patient-reported outcomes are needed to evaluate changes in mental health status of CAIS and MA patients at different ages and different intervals following initial diagnosis., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com. See the journal About page for additional terms.)

Objective: Several graft options can be used to construct a neovagina. This study aimed to evaluate the efficacy of creating a neovagina using a fresh human amnion in women with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome., Methods: Ten patients were analyzed retrospectively. Anatomical success was defined by a postoperative vaginal length ≥5 cm, and a width sufficient to comfortably accommodate the insertion of two fingers. Functional success was achieved when a score of >26.5 was attained on the Arabic validated version of the functional sexual function index (FSFI)., Results: The mean vaginal length before surgery was 2.15±0.85 cm (range, 1.5-4.5). The mean vaginal length after surgery was 5.5±0.53 cm (range, 5-6), and all patients (n=10) achieved anatomical success. The FSFI score after surgery was 29.2±2.4, and eight patients achieved functional success. The mean operative time and estimated blood loss were 83.5±50.31 minutes (range, 42-210) and 122.0±75.69 mL (range, 20-250), respectively. None of the patients required intraoperative blood transfusion or experienced injury to vital organs. Four patients developed postoperative leukocytosis within 48 hours, and one patient experienced chronic pelvic pain that lasted more than 6 months postoperatively. No major postoperative complications, such as pelvic abscesses, open wounds, chronic vaginal discharge, or reoperation, were reported., Conclusion: Surgical dissection of the vesicorectal space and utilization of fresh human amnion to create a neovagina over a vaginal mold are technically feasible and safe, and are linked to favorable anatomical and functional outcomes in MRKH patients with vaginal agenesis.

Objective: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a rare congenital disorder that results in vaginal agenesis. Lee's neovaginoplasty is a novel surgery for reconstructing the vagina. Transneovaginal oocyte retrieval completely changes the scope of fertility for patients with MRKH syndrome who have undergone neovaginal reconstruction., Case Report: A 22-year-old female with type 1 MRKH syndrome underwent Lee's neovaginoplasty successfully. Four years later, she sought embryo cryopreservation consultation and underwent controlled ovarian hyperstimulation. Upon examination, her anti-Müllerian hormone level was 1.97 ng/ml and she had only eight antral follicles. The neovaginal length was 8 cm with elasticity and extensibility. Transneovaginal oocyte retrieval was performed under ultrasound guidance, and seven oocytes were retrieved. The follicle-to-oocyte index was 87.5%., Conclusion: Lee's neovaginoplasty is a promising surgery for reconstructing the vagina in MRKH syndrome, and this case shows that transneovaginal oocyte retrieval can be successfully performed after vaginal reconstruction. This technique provides a minimally invasive option for retrieving oocytes in patients of MRKH syndrome., Competing Interests: Conflict of interest statement The authors have no conflicts of interest relevant to this article., (Copyright © 2024. Published by Elsevier B.V.)

Introduction and hypothesis: Mayer-Rokitansky-Küster-Hauser syndrome affects about 1 in 5000 live female births and is associated with gonadal dysgenesis and primary amenorrhea. Neovaginoplasty has been established as an appropriate treatment option for patients who have failed or denied dilation therapy. In search of accessible, economical material with low risk of complications, the team proposed the use of Nile tilapia fish skin (NTFS) as an innovative biomaterial in the neovaginoplasty procedure for vaginal agenesis management. NTFS has noninfectious microbiota, morphologic structure comparable to human skin and high in vivo bioresorption. Methods: In this descriptive study, the method offered an anatomical and functional neovagina to 11 patients efficiently, quickly and safely. Correct post-surgical dilation is still extremely important to keep the neovagina's size > 6 cm. Results: Histological and immunohistochemical analysis demonstrated the formation of a stratified squamous epithelium with strong marking for cytokeratins, FGF and EGFR, similar to healthy adult vaginal tissue. Conclusions: Since NTFS is a low cost and easily accessible biomaterial, this technique proves to be an inexpensive therapeutic possibility for the health system with excellent advantages for patients. [ABSTRACT FROM AUTHOR]

• The use of autologous buccal mucosa grafts for vaginal reconstruction provides graft material histologically similar to vaginal tissue, a hidden donor site scar, and avoids major abdominal surgery. • An intravaginal wound vacuum can be easily constructed and has the potential to promote excellent graft take and graft healing. Vaginal reconstruction with autologous buccal mucosa graft offers a promising alternative to the use of skin grafts and vascularized intestinal segments. Given the novelty of this procedure, the optimal approach to postoperative wound management remains unclear with current practices often requiring many months of vaginal stents/molds. This study aims to evaluate a newly developed negative pressure intravaginal wound vacuum placed at the conclusion of the vaginoplasty with the goals of facilitating graft take and healing. A retrospective review of patients (age 12–21 years) who underwent eight primary and secondary vaginoplasty procedures using autologous buccal mucosa coupled with intravaginal wound vacuum placement was performed. Vaginal reconstruction with fenestrated full-thickness buccal mucosa graft and intravaginal wound vacuum placement was successfully performed eight times in seven patients at a median age of 15.6 years. Four patients underwent robotic vaginal pull-through with buccal mucosa serving as an interposition graft, and four patients underwent vaginoplasty with buccal graft alone. All cases had excellent engraftment at time of wound vacuum removal on postoperative day seven and had healthy-appearing buccal mucosa at a mean follow-up of 148 days. Postoperatively, one patient developed a stricture at the anastomosis between native vagina and buccal mucosa graft, requiring a second buccal mucosa graft six months after the first operation. The use of autologous buccal mucosa graft for primary and secondary vaginal reconstruction coupled with intravaginal wound vacuum therapy offers a promising new approach. Negative pressure wound vacuum therapy may provide a more optimal wound healing environment for improved outcomes. Retrospective Study Level IV [ABSTRACT FROM AUTHOR]

Fraser syndrome is a rare disorder with autosomal recessive inheritance having a wide spectrum of phenotypic appearances. A fourteen-year-old female presented to us with a chief complaint of acute retention of urine without any cyclical abdominal pain with syndromic appearance. She had partial vaginal agenesis which was treated by successful reconstructive surgery by lotus petal flap technique followed by recanalisation. Objective of this report is to highlight the rare disease of Fraser syndrome along with successful rare surgical management. [ABSTRACT FROM AUTHOR]

Background: Sigmoid colpoplasty is a surgical method for the treatment of vagina agenesis. Malignant tumors of neovaginas derived from sigmoid colons are rare. Case presentation: We report a 33-year-old woman who underwent sigmoid colpoplasty for vaginal agenesis and presented 18 years later with vaginal bleeding. Examination revealed cancer of the neovagina with involvement of the cervix and endometrium. The patient was administered four cycles of chemotherapy because she refused surgery. Conclusions: Patients with a history of colpoplasty should undergo long-term comprehensive testing after reconstruction, including regular gynecological, colposcopic, and gastrointestinal examinations. In patients with cancer of the neovagina, a comprehensive treatment plan should be developed in consultation with gynecologists and surgeons. There is no standard treatment, although surgery plus chemotherapy or radiotherapy appears to be effective. [ABSTRACT FROM AUTHOR]

Aims: To introduce and compare the modified laparoscopic Vecchietti and Davydov techniques for vaginoplasty in patients with Mayer‐Rokitansky‐Küster‐Hauser (MRKH) syndrome. Moreover, the long‐term treatment of vaginal agenesis was followed‐up. Methods: This comparative retrospective cohort study enrolled a total of 53 women with MRKH syndrome. The patients underwent surgical creation of a neovagina including 32 patients who underwent the modified laparoscopic Vecchietti technique, and 21 patients who underwent the modified laparoscopic Davydov technique from January 2009 to February 2019. The perioperative parameters, complications, anatomical, and functional outcomes of the two groups were compared. Patients' sexual functions were evaluated over a long‐term follow‐up using the female sexual function index (FSFI) and the revised female sexual distress scale (FSDS‐R). Results: The medians (25th–75th) of the surgery duration for modified Vecchietti procedures was 50.0 (40.0–59.0) minutes, comparing to 135.0 (117.5–162.5) min for Davydov procedures (p < 0.001). The intraoperative blood loss was 20 (7.5–20.0) mL versus 50.0 (50.0–100.0) mL using the modified Vecchietti and Davydov approaches (p < 0.001), respectively. In the 39 follow‐up cases, the lengths of the neovagina of the patients for Vecchietti group versus Davydov group were 7.9 ± 1.0 cm versus 8.6 ± 1.2 cm at 6 months after the vaginoplasty and 8.3 ± 0.7 cm versus 8.5 ± 0.9 cm after 2 years. There was no statistical difference in the FSFI and FSDS‐R scores between the two groups. Conclusions: Both the modified Davydov and Vecchietti laparoscopic procedures successfully achieved optimal anatomic and functional outcomes in treatments of vaginal agenesis. The modified Vecchietti technique is relatively simpler than the modified Davydov technique. [ABSTRACT FROM AUTHOR]

Background: The ideal timing of genital surgery in differences/disorders of sex development (DSD) is controversial and differs according to the underlying type of DSD. Increasing numbers of persisting sinus as a result of delayed feminizing genitoplasty in DSD patients require interdisciplinary collaboration of pediatric surgeons/urologists and gynecologists. This study focusses on surgical techniques other than bowel vaginoplasties and results of gender assigning surgery in young adolescents. Methods: Data of adolescent and adult patients treated between 2015 and 2022 were analyzed retrospectively: underlying type of malformation, techniques of vaginoplasty, vaginal length and caliber, possibility of sexual intercourse, and temporary vaginal dilatation. Results: A total of 9 patients received a primary vaginoplasty at a median age of 16.75 years (range 10.3–29.25). The underlying anatomical conditions were persistent urogenital sinus (UGS) in 8 patients (3 patients with CAH, 2 patients with XY-DSD, 1 patient with cloacal malformation and missed UGS, 2 patients with UGS only). One patient had a MURCS association. Surgical techniques were total urogenital mobilization and perineal flap vaginoplasty in 4 patients, modified McIndoe vaginoplasty in 4 patients, and a laparoscopic vaginal pull-through in 1 patient. In a median follow-up of 45 months (2–84), all but 1 patient presented with physiological vaginal length and width. Conclusions: If possible, modern treatment concepts delay gender assigning surgery until the participation of the patient in the decision-making process is possible. Optimal treatment concepts are given by transfer of surgical techniques from pediatric urology/surgery by multidisciplinary teams. Techniques other than bowel vaginoplasties are favorable. [ABSTRACT FROM AUTHOR]

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, as congenital anomaly of the female reproductive tract, is characterized by the congenital absence of uterus and vagina. Dilation maneuver was recommended as the nonsurgical treatment for these patients. However, only several cases were reported depicting the prolapse of the dilated neovagina of patients with MRKH syndrome. We report our own experiences in treating 2 patients with neovaginal prolapse MRKH syndrome receiving ischial spinous fascia fixation (ISFF) and reviewed neovaginal prolapse occurrence in MRKH syndrome. In our approach to evaluating the anatomic and functional outcome after operation, we compared the prolapse stage with Pelvic Organ Prolapse Quantification score and analyzed sexual activities by the standardized Female Sexual Function Index 1 (FSFI) and Modified Body Image Scale 2 (MBIS). Both subjective and objective improvement were observed in these 2 patients. [ABSTRACT FROM AUTHOR]

Background: Vaginal agenesis is an uncommon condition with an estimated incidence of 1/5000 live female births. There are surgical and non‐surgical treatment options for neo‐vagina creation. Vaginal dilatation has been used to treat vaginal agenesis for over 80 years, but outcome data to date have had poor definitions for 'success'. The optimal method for neo‐vagina creation varies with vaginal dilator therapy being the most successful first line therapy in women with Mayer‐Rokistansky‐Kuster‐Hauser syndrome, with little literature to guide best practice in women with more complex congenital conditions. There is a paucity of data from Australian and New Zealand paediatric and adolescent gynaecology services on management of women requiring a functional vagina, for any underlying aetiology. Aims: To determine if creation and maintenance of a functional neo‐vagina for vaginal agenesis with non‐surgical management (via vaginal dilators) should be the first line treatment. Materials and Methods: Retrospective observational study between January 2005 and June 2015. Results: Of the 23 women, 21 (91.3%) achieved a successful vaginal length defined as achieving vaginal length >6 cm and maximum width using the largest dilator if not sexually active, or ability to have sexual intercourse without discomfort. Conclusion: Vaginal dilator therapy remains an effective first line treatment for neo‐vagina creation and maintenance and can be achieved in an outpatient setting with appropriate support. [ABSTRACT FROM AUTHOR]

Objective: To compare, in terms of anatomical, functional, and sexual aspects, two types of treatment for women with vaginal agenesis: progressive dilation or surgical neovaginoplasty. Methods: Women with vaginal agenesis underwent either dilation treatment using the Frank method or surgical treatment using the modified Abbé-McIndoe technique with oxidized cellulose. Patients were evaluated 3–6 months after treatment for a follow-up including medical history, physical examination, general satisfaction, clinical aspect of the vagina, Female Sexual Function Index, and three-dimensional pelvic floor ultrasound. Results: In total, 20 women with vaginal agenesis were included in the present study; nine in the dilation group and 11 in the surgical group. A comparison between the groups (vaginal dilation and surgical neovaginoplasty) showed efficacy in neovagina formation after both treatments, with a statistically significant difference between the pre- and post-treatment periods (P value pre- × post-dilation group <0.0001 and P value pre- × post-surgical group <0.0001). There were no statistical differences in total vaginal length measurements (P value post-dilation × post-surgical = 0.09) or Female Sexual Function Index scores (P = 0.72) after both treatments. Conclusion: Both treatments had satisfactory efficacy and positive outcomes for patients with vaginal agenesis concerning anatomical, functional, and sexual aspects, with minimum complications in the surgical group. Dilation treatment can remain the first-line therapy. [ABSTRACT FROM AUTHOR]

Vaginal agenesis is a rare condition worldwide. Most reported cases were accompanied by the absence of uterus or uterine hypoplasia; for patients with functional endometrium, hysterectomy was most likely to be conducted to lower postoperative complications. Based on our successful experience in vaginoplasty with autologous buccal mucosal, the purpose of this article is to discuss the surgical strategies in the reconstruction of neovaginal for vaginal agenesis patients with functional uterus and cervical hypoplasia. The uterus was preserved in our procedure, and the cervicoplasty was performed to connect the uterine cavity with the neovagina. After the vaginal cavity was formed, the cervix was confirmed and fixed. With the assistance of laparoscope, the direction and angle of the cervix and the uterine body were observed and confirmed. An incision was made in cervix to connect the uterine cavity, and a Foley's catheter was inserted. The newly formed opening of cervix and neovagina was covered by autologous buccal mucosal. The connection between neovagina and cervix uteri was successfully conducted in patient with functional uterus. Unimpeded and regular menstrual was achieved, and the cyclic abdominal pain was disappeared. No complications were observed. For patients without functional uterus, vaginoplasty with autologous buccal mucosal can be conducted. However, fertility-preserving surgery should be the primary choice in patients with functional endometrium. It can be concluded from our experience that the utero-vaginal connection with the assistance of laparoscope and the use of autologous buccal mucosa is a promising way to achieve ideal outcomes. [ABSTRACT FROM AUTHOR]

Vaginal agenesis is a rare congenital condition, with an incidence of 1 in 4500 female births. We present a clinical case of vaginal aplasia with cervical atresia in a 31-year-old woman with primary amenorrhea. We aim to report the diagnostic process and provide a comprehensive outline of different possible treatments. The most common etiology of these agenesis cases is Mayer-Rokitansky-Küster-Hauser syndrome associated with uterine aplasia. However, vaginal aplasia can occur in 9 % of cases where the uterus is present. During embryogenesis, the Müllerian ducts give rise to the fallopian tubes, uterus, and upper two-thirds of the vagina, while the lower portion of the vagina develops from the urogenital sinus. Vaginal aplasia arises from a failure in the development of the terminal portion of the paramesonephric ducts. Abdominal pain, especially periodic pain, is the most common symptom, followed by primary amenorrhea. MRI is considered the gold standard for the diagnosis and precise description of female genital tract anomalies. Total hysterectomy remains a preferred option for cases of complete vaginal atresia to mitigate the risk of cervical or vaginal stenosis, adhesions, and pelvic inflammation resulting from poor menstrual blood drainage. • Vaginal agenesis is rare, with the most common symptoms being periodic abdominal pain and primary amenorrhea, and a pelvic mass. • A clinical case of a 31-year-old woman with vaginal aplasia and cervical atresia is presented. • MRI is the gold standard for diagnosing female genital tract anomalies. • Total hysterectomy is often preferred in complete vaginal atresia cases to prevent complications [ABSTRACT FROM AUTHOR]