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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]

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.)

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]

Rectovaginal fistula is a complication that may occur due to rectal injury during vaginal reconstructive surgery. To prevent these complications, the recognition of the injury is an important factor so that primary repair can be done. The primary repair can reduce the risk of complications such as fistula formation, and also reduce the physical and psychological impact on the patient. A 33-year-old woman, came with a chief complaint of fecal leakage from the vagina and abdominal pain three months before admission with a history of vaginal reconstructive surgery due to vaginal agenesis. Eleven years after the reconstruction, the patient was diagnosed with recurrent obstruction caused by vaginal synechia. During the surgery of synechia release, rectum injury occurred. Even though primary closure repair was done at that time, several months later there was a complication of rectovaginal fistule formation in the form of fecal leakage from the vagina. The corrective surgery is performed in collaboration with a surgical gastroenterologist. Iatrogenic rectal injury may occur during gynecological surgery. A fistula that occurs after the reconstruction of vaginal agenesis is a high-type rectovaginal fistula, making the repairs more complex. Collaboration surgery between surgical gastroenterologist and gynecologist may be an option in such cases. Rectovaginal fistula is a rare but serious complication of vaginal reconstructive surgery. Early recognition, immediate management, and postoperative follow-up are essential in cases of rectal injury during vaginal reconstructive surgery. • Rectovaginal fistula is a complication that may occur due to rectal injury during vaginal reconstructive surgery. • The primary repair can reduce the risk of complication such as fistula formation, and reduce the physical and psychological impact on the patient. • Early recognition and management with postoperative follow-up are essential in cases of rectal injury during vaginal reconstructive surgery. [ABSTRACT FROM AUTHOR]

Objective: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital condition characterized by the underdevelopment or complete absence of the uterus and the upper part of the vagina. Diagnosis is commonly made during adolescence, a sensitive period for psychophysical development, following the absence of menstruation. Having MRKH syndrome can have a profound and multifaceted psychosocial impact that characterizes these women's subjective experiences, although it continues to be qualitatively understudied. This article explores the lived experience of women with MRKH spontaneously recounted on an online support forum. The aim was to gain insight into the features of the syndrome experience to understand deeply the emotional and social impact of the condition and the individual needs expressed online., Methods: Using a naturalistic observation stance, data was collected from an online support forum for MRKH women and systematically analyzed using thematic analysis., Results: Four main interconnected themes are identified: the impact of being diagnosed with MRKH, the difficult interaction with the medical environment, challenging social relationships, and the unmet needs of MRKH women., Conclusion: A multidisciplinary and person-centered approach that provides effective and sensitive management of the condition and its psychosocial implications, is essential. Recommendations for future research and practical clinical implications for healthcare professionals are proposed.

Background: Gonadal dysgenesis, a genetic condition characterized by incomplete of defective formation of the gonads, can present with vaginal agenesis in individuals with 46, XY karyotype., Case: We report an innovative intervention in the management of vaginal agenesis in a 19-year-old female with gonadal dysgenesis. Despite initial attempts with vaginal dilators, the patient presented unresponsive, leading to the adoption of a neovaginoplasty using Nile Tilapia Fish Skin (NTFS) as graft. The procedure, based on the McIndoe technique, involved the creation of a 10 cm x 3 cm vaginal canal with an NTFS-wrapped acrylic mold without complications., Conclusion: The use of NTFS as a graft for neovaginoplasty in gonadal dysgenesis, a novel approach not previously reported in medical literature for this diagnosis, demonstrated favorable outcomes in terms of functionality and patient well-being., Competing Interests: Conflict of interest We declare for all due purposes that there are no conflicts of interest in the conduct and dissemination of this research., (Copyright © 2024. Published by Elsevier Inc.)

Maldevelopment of the vagina and cervix is frequently accompanied by uterine aplasia or hypoplasia. Complete cervico-vaginal aplasia with a normally developing uterus is a very uncommon type of developmental failure. Failure to treat the condition can result in complications such as hematometra and hematosalpinx caused by the retrograde flow of blood into the fallopian tubes. In this case report, we describe the case of a 32-year-old woman experiencing cyclic abdominal pain and primary amenorrhea. The patient exhibited cervico-vaginal agenesis, with a functional uterus that was complicated by hematometra and bilateral hematosalpinx., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Introduction: The bowel remains the favored tissue for neo-vaginoplasty (NeoVP) in pediatric patients with vaginal agenesis. In 2001, the first description of NeoVP using the Yang-Monti technique with a sigmoid double tubular flap was published. We present our experience of NeoVP with a single Yang-Monti tube (SYMT) flap and report on the use of different segments of colon., Methods: We conducted a retrospective review of patients who underwent NeoVP using a bowel SYMT between 2009 and 2021. The procedure was performed under open abdominal surgery by isolating 8 to 12 cm from the rectosigmoid, cecum, or ascending colon. Subsequently, this segment was detubularized near the mesenteric border, folded, and retubularized transversally, leaving the mesentery in a cephalic position. A channel was dissected in the pelvis to accommodate the NeoVP., Results: Seventeen patients were identified. The median age was 16 years old. The principal diagnosis was Mayer-Rokitansky's syndrome (47.1%). The median follow-up was 50 months. The mean harvested bowel length was 9 cm, and the sigmoid was the preferred site (65%). The cecum-ascending colon was used in 3 (17.6%) patients. Complications were recorded in 6 patients (35%). Of these 6 patients, 4 had introital stenosis. There was 1 case of urethrovaginal fistula and another of left hematometrocolpos. Satisfactory sexual function has been reported in sexually active individuals., Conclusions: We described our experience in NeoVP using a large bowel SYMT as a safe and effective technique. It allows decreased tension on the vascular pedicle and the use of shorter colon segments.