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1.
J Pediatr Adolesc Gynecol ; 37(3): 353-359, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38246338

RESUMO

OBJECTIVE: To analyze the features of the epithelia coating neovaginas after vaginoplasty in women affected by Mayer-Rokitansky-Küster-Hauser syndrome STUDY DESIGN: 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). RESULTS: 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. CONCLUSION: 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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Ductos Paramesonéfricos , Vagina , Humanos , Feminino , Vagina/cirurgia , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Estudos Retrospectivos , Adolescente , Útero/anormalidades , Útero/cirurgia , Estruturas Criadas Cirurgicamente , Adulto , Epitélio/patologia , Adulto Jovem , Procedimentos de Cirurgia Plástica/métodos , Biópsia , Somitos/anormalidades , Microscopia Eletrônica de Varredura
2.
Arch Gynecol Obstet ; 309(3): 801-812, 2024 03.
Artigo em Inglês | MEDLINE | ID: mdl-37466686

RESUMO

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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Adenocarcinoma , Carcinoma de Células Escamosas , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Neoplasias Vaginais , Humanos , Feminino , Vagina/patologia , Neoplasias Vaginais/cirurgia , Neoplasias Vaginais/patologia , Carcinoma de Células Escamosas/patologia , Adenocarcinoma/patologia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/patologia , Procedimentos Cirúrgicos em Ginecologia/métodos , Resultado do Tratamento
3.
J Pediatr Surg ; 59(1): 124-128, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37802758

RESUMO

PURPOSE: Various techniques for neovaginal construction have been employed in the pediatric and adult populations, including the use of intestinal segments, buccal mucosal grafts, and skin grafts. Small intestinal submucosa (SIS) extracellular matrix grafts have been described as a viable alternative, though prior experience is limited. Our purpose was to assess operative characteristics and patient outcomes with neovaginal construction using SIS grafts. METHODS: Thirteen patients underwent vaginoplasty with acellular porcine SIS grafts at our institution between 2018 and 2022. Operative and clinical data, postoperative mold management, vaginal dilating length, and complications were reviewed. RESULTS: Age at time of repair ranged from 13 to 30 years (median 19 years). Patient diagnosis included cloacal anomalies (n = 4), Mayer-Rokitansky-Küster-Hauser syndrome (n = 4), isolated vaginal atresia with or without a transverse vaginal septum (n = 4), and vaginal rhabdomyosarcoma requiring partial vaginectomy (n = 1). Following dissection of the neovaginal space, a silicon mold wrapped with SIS graft was placed with retention sutures and removed on postoperative day 7. Median (IQR) operative time was 171 (118-192) minutes, estimated blood loss was 10 (5-20) mL, and length of stay was 2 (1-3) days. The follow-up period ranged from 3 to 47 months (median 9 months). Two patients developed postoperative vaginal stenosis that resolved with dilation under anesthesia. Mean vaginal length on latest follow-up was 8.97 cm. All thirteen patients had successful engraftment and progressed to performing self-dilations or initiating intercourse to maintain patency. There were no cases of graft reaction or graft extrusion. CONCLUSIONS: We conclude that acellular small intestinal submucosa grafts are effective and safe alternatives for mold coverage in neovaginal construction. Our experience demonstrates minimal perioperative morbidity, early mold removal, and progression to successful dilation with maintenance of a functional vaginal length. Future study on sexual outcomes, patient satisfaction, and comparison against alternative techniques has been initiated. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Retrospective Study.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Adulto , Humanos , Animais , Suínos , Feminino , Criança , Adolescente , Adulto Jovem , Vagina/cirurgia , Vagina/anormalidades , Estudos Retrospectivos , Constrição Patológica/cirurgia , Satisfação do Paciente , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Resultado do Tratamento
4.
Int Urogynecol J ; 35(2): 467-470, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37823899

RESUMO

INTRODUCTION AND HYPOTHESIS: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a condition with an underdeveloped or absent vagina and uterus due to embryological growth failure of the Müllerian ducts. Many techniques have been described to construct a neovagina with an acceptable depth that allows penetrative intercourse. This is a step-by-step video tutorial on the Wharton-Sheares-George surgical technique for vaginoplasty in patients with MRKH syndrome. METHOD: With Wharton-Sheares-George vaginoplasty, the rudimentary Müllerian ducts are incrementally dilated by pushing Hegar dilators in the direction of the pelvic axis, and the resulting median raphe is then intersected using diathermy. As a result, a neovagina is created and an estriol-coated vaginal mold is inserted for 3 days. The patient receives comprehensive discharge instructions, a self-dilation program three times a day, and a monthly follow-up. RESULTS: A 3-month follow-up showed a high subjective degree of satisfaction with surgery and sexual satisfaction in both patients. CONCLUSION: Wharton-Sheares-George vaginoplasty is a safe and efficient technique for creating a neovagina for patients with MRKH syndrome.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Ductos Paramesonéfricos , Ductos Paramesonéfricos/anormalidades , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Útero/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Vagina/cirurgia
5.
J Minim Invasive Gynecol ; 31(1): 37-42, 2024 01.
Artigo em Inglês | MEDLINE | ID: mdl-37820829

RESUMO

STUDY OBJECTIVE: To describe the long-term anatomic and sexual functional results of vaginoplasty with acellular dermal matrix (ADM) in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) and to evaluate the changes in body image pre- and postoperatively in these patients. DESIGN: A retrospective study from March 2015 to September 2021. SETTING: A tertiary teaching hospital. PATIENTS: Forty-two patients with MRKH syndrome who underwent vaginoplasty with ADM (the MRKH group) and 30 sexually active, nulliparous, aged-matched women (the control group). INTERVENTION: The relevant data were retrospectively collected via our electronic medical record system and were analyzed statistically. MEASUREMENTS AND MAIN RESULTS: Vaginal length was assessed using a 3-cm-diameter mold. The Chinese version of the Female Sexual Function Index questionnaire was used to evaluate sexual function. The Chinese version of the modified body image scale was applied to evaluate body image. The median follow-up time was 57 months (range, 13-91 months). Granulomatous polyps in the neovagina were the most common postoperative complication (7 of 42, 16.7%). Patients with MRKH syndrome can achieve long-term satisfactory outcomes both anatomically and functionally after vaginoplasty with ADM, comparable with those of healthy control women. The vaginal length in the MRKH group was comparable to that in the control group ( 8.04 ± 0.51 cm vs. 8.15 ± 0.46 cm, respectively). The FSFI scores were similar between the MRKH (26.54 ± 3.44) and control (26.80 ± 2.23) groups. The modified body image scale score was significantly decreased after vaginoplasty with ADM. CONCLUSION: Vaginoplasty with ADM is a minimally invasive and effective procedure for patients with MRKH syndrome.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Derme Acelular , Anormalidades Congênitas , Feminino , Humanos , Idoso , Estudos Retrospectivos , Imagem Corporal , Vagina/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia
6.
Fertil Steril ; 121(4): 679-692, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38104886

RESUMO

OBJECTIVE: To summarize the available evidence on the laparoscopic Davydov and Vecchietti methods to create a neovagina and to compare these techniques with a focus on neovaginal length, sexual function, operative time, and complications. DESIGN: A systematic electronic search up to August 2022 using PubMed and Embase is performed. SETTING: Not applicable. PATIENTS: Women with Mayer-Rokistansky-Küster-Hauser syndrome. INTERVENTIONS: All published clinical studies concerning the laparoscopic Davydov and laparoscopic Vecchietti procedures as a surgical technique to create a neovagina in women with Mayer-Rokistansky-Küster-Hauser syndrome were obtained. The guidelines for the preferred reporting items for systematic reviews and meta-analysis were followed. The following data were extracted: operative time, hospital stay, major early complications (within 3 months postsurgery), dilation therapy, neovaginal length, vaginal discharge, vaginal stenosis, time to sexual activity, sexual satisfaction, penetrative sexual activity, dyspareunia, score on the Female Sexual Function Index (FSFI), and duration of follow-up. The Newcastle Ottawa Scale was used to assess the quality of articles. MAIN OUTCOME MEASURES: Neovaginal length, FSFI scores, operative time, and complications. RESULTS: A total of 1,163 articles were identified, of which 33 studies were included in this systematic review. Of these, 12 studies (380 patients) are related to the Davydov method, 19 studies (1,126 patients) to the Vecchietti method, and 2 articles concern both. There is clinical heterogeneity and variety in the quality of the studies. Eighteen studies were included in the meta-analyses. The mean neovaginal length 12 months after the Davydov method is 8.3 cm (95% confidence interval [CI] 8.1-8.6), vs. 8.7 cm (95% CI 7.2-10.3) after the Vecchietti method. The mean FSFI score after the Davydov method is 28.9 (95% CI 26.8-31.1), compared with 27.5 (95% CI 25.0-30.1) after the Vecchietti method. The operative time of the Davydov method is 126 minutes (95% CI 109-143), compared with 40 minutes (95% CI 35-45) of the Vecchietti method. CONCLUSIONS: The operations yield comparable neovaginal length, sexual function, and complication rates. The mean FSFI scores indicate no sexual dysfunction in either group. The operative time of the Davydov method is significantly longer. There is no superiority shown for one of the surgical techniques in functional terms.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Ductos Paramesonéfricos/anormalidades , Humanos , Feminino , Vagina/cirurgia , Constrição Patológica/cirurgia , Laparoscopia/efeitos adversos , Laparoscopia/métodos , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/cirurgia , Resultado do Tratamento
7.
BMC Womens Health ; 23(1): 634, 2023 11 27.
Artigo em Inglês | MEDLINE | ID: mdl-38012663

RESUMO

BACKGROUND: In the past, the primary treatment for MRKH syndrome (Mayer-Rokitansky-Küster-Hauser syndrome) with a functional primordial uterus was surgical removal of the functional primordial uterus. In rare instances, the endometrium of the functional primordial uterus is well developed, and surgical preservation of the functional primordial uterus provides the possibility of preserving reproductive function for these patients. CASE PRESENTATION: A 14-year-old female was diagnosed with type I MRKH syndrome with a functional primordial uterus through physical examination and imaging investigations. We freed the functional primordial uterus through laparoscopic surgery and excised a portion of the lower myometrium to create an outlet at a lower uterine segment, which we then intermittently anastomosed to the tip of the artificial vagina. The patient recovered well after the surgery, and a re-examination showed no significant abnormalities. CONCLUSION: We were successful in preserving the functional primordial uterus using laparoscopic surgery in a patient with MRKH syndrome and connecting it to an artificial vagina through reconstructive surgery to ensure unobstructed menstrual drainage and preserve the reproductive potential of the patient.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Feminino , Humanos , Adolescente , Útero/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Vagina/cirurgia , Ductos Paramesonéfricos/cirurgia , Laparoscopia/métodos , Anormalidades Congênitas/cirurgia
8.
J Gynecol Obstet Hum Reprod ; 52(10): 102674, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37805077

RESUMO

OBJECTIVE: To better understand patients' conditions and expectations before starting a uterus transplantation (UTx) program for women suffering from Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome). METHOD: A web-based survey was conducted among MRKH patients via the French national association network from March to August 2020. The questionnaire comprised twenty-eight questions about their desire for parenthood, their condition's characteristics and previous reconstructive procedures, opinions and knowledge about UTx. RESULTS: Among the 148 participants, 88 % reported a desire for parenthood, and 61 % opted for UTx as their first choice to reach this aim. The possibility of bearing a child and having the same genetic heritage were the main motivations. Once informed about the usual course of an UTx protocol, only 13 % of the participants changed their mind and 3 out of 4 of them opted for UT. CONCLUSION: Uterus transplantation seems to be the first option to reach motherhood in patients suffering from MRKH syndrome. The development of UTx programs could meet the demands of this already well-informed population.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Motivação , Criança , Humanos , Feminino , Útero , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia
9.
Int Urogynecol J ; 34(11): 2647-2655, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37490063

RESUMO

BACKGROUND: Bowel vaginoplasty is a surgical method for neovagina construction that, despite its advantages over other techniques, is still burdened by complications such as prolapse. The incidence of sigmoid neovagina prolapse (SNP) is difficult to determine, and there are no evidence-based recommendations for treatment. We present a case of SNP and a systematic review of previous cases. CASE: A 73-year-old woman presented with stage III prolapse of her sigmoid neovagina constructed 51 years prior. Dynamic pelvic MRI revealed that the majority of the prolapse was due to the mucosa's loss of support. Due to the presence of numerous pelvic adhesions, an alternative to the laparoscopic approach was evaluated by a multidisciplinary team which led to the patient being treated using a modification of Altemeier's procedure. SYSTEMATIC REVIEW: After PROSPERO Registration (CRD42023400677), a systematic search of Medline and Scopus was performed using specific search terms. Study metadata including patient demographics, prolapse measurements, reconstruction techniques, recurrence rates, and timing were extracted. Fourteen studies comprising 17 cases of SNP were included. Vaginal resection of the redundant sigmoid, comprising Altemeier's procedure, was the most definitive surgery, but it was also associated with recurrences in three cases. Laparoscopic sacropexy was the second most definitive surgery with no recurrence reported. CONCLUSION: Our review shows that the recurrence after correction of sigmoid neovagina prolapses is higher than previously reported. Laparoscopy colposacropexy appeared to be the best approach, but it's not always feasible. In these scenarios, a mucosal resection using the Altemeier's procedure is the most effective surgery.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Humanos , Feminino , Gravidez , Idoso , Colo Sigmoide/cirurgia , Prolapso , Vagina/cirurgia , Laparoscopia/métodos , Colpotomia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia
10.
J Minim Invasive Gynecol ; 30(9): 705-715, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37271411

RESUMO

OBJECTIVE: In patients with Rokitansky syndrome, vaginal agenesis can be treated using various surgical techniques, and various factors can affect each surgery outcome in the long term. This meta-analysis aimed to evaluate sexual function outcomes in patients with Rokitansky syndrome after various surgery techniques. DATA SOURCES: Searches were conducted in Google Scholar, PubMed, Cochrane database, ScienceDirect, Web of Science, and ClinicalTrials.gov. Systematic searches were conducted on studies published until November 2022 (CRD42022370735). METHODS OF STUDY SELECTION: During the first stage of database scanning and reference check, 1820 results were identified, and an evaluation of the total 10 studies was finally conducted. The inclusion criteria involved selecting randomized controlled trials that focused on assessing sexual function after surgical treatment in patients of all ages with Rokitansky syndrome. TABULATION, INTEGRATION, AND RESULTS: EndNote version 20 software was used to organize and identify duplicate articles through screening. The Joanna Briggs Institute's critical appraisal tool was used to evaluate each study's quality for bias potential. The results showed that the total scores of female sexual functions in patients with Rokitansky syndrome after vaginoplasty were significantly lower than in healthy women (standardized mean difference, -0.233; p <.05; range, -0.376 to -0.090). The 6-domain analysis of the Female Sexual Function Index questionnaire revealed that lubrication (p <.05) and satisfaction (p <.05) were significantly lower in patients undergoing vaginoplasty. CONCLUSION: All surgical techniques to create a neovagina for patients with Rokitansky syndrome have successfully affected the sexual function outcomes. Considering all other factors affecting sexual function outcomes in the long term, more quantitative and qualitative studies are needed to assess sexual satisfaction in patients treated with surgical techniques.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Múltiplas , Anormalidades Congênitas , Feminino , Humanos , Resultado do Tratamento , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades
11.
J Pediatr Adolesc Gynecol ; 36(6): 556-559, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37354985

RESUMO

OBJECTIVE: To describe surgical correction of vaginal agenesis via a modified laparoscopic Vecchietti procedure with the goal of disseminating knowledge and improving surgical technique CASE: 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. RESULTS: 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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Laparoscopia , Procedimentos de Cirurgia Plástica , Feminino , Animais , Humanos , Adolescente , Vagina/cirurgia , Vagina/anormalidades , Laparoscopia/métodos , Útero/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Anormalidades Congênitas/cirurgia , Resultado do Tratamento
12.
BMJ Case Rep ; 16(5)2023 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-37156565

RESUMO

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a condition caused by Müllerian anomalies, is characterised by congenital vaginal aplasia and a rudimentary uterus. Case reports concerning uterine fibroids associated with MRKH syndrome are limited, and differentiating between uterine fibroids and ovarian solid tumours prior to surgical intervention is often challenging. Here, we present the case of a patient with MRKH syndrome and asymptomatic bilateral pelvic solid tumours located close to both ovaries. Based on intraoperative and histopathological findings, the tumours were diagnosed as adenomyomas of the rudimentary uterus. This is the first reported case of a uterine adenomyoma associated with MRKH syndrome. Moreover, our report highlights the fact that diagnostic laparoscopy is a valuable method to evaluate pelvic tumours in MRKH syndrome.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Adenomioma , Anormalidades Congênitas , Laparoscopia , Leiomioma , Neoplasias Pélvicas , Feminino , Humanos , Adenomioma/complicações , Adenomioma/diagnóstico , Adenomioma/cirurgia , Neoplasias Pélvicas/cirurgia , Útero/cirurgia , Útero/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Vagina/cirurgia , Vagina/anormalidades , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Laparoscopia/métodos , Leiomioma/cirurgia , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/cirurgia
13.
Aust N Z J Obstet Gynaecol ; 63(4): 599-602, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37200477

RESUMO

Uterine transplantation (UT) is an emerging medical treatment for women affected by absolute uterine factor infertility (AUFI). To date there have been over 90 documented cases of UT performed worldwide, with over 50 live births. UT allows women affected by AUFI the opportunity to carry and deliver a childd. The Royal Prince Alfred Hospital (RPAH) introduced a UT study in 2019; however, due to the impacts of the COVID pandemic the study was placed on hold for two years. In February 2023, RPAH performed the centre's first UT from a living unrelated donor to a 25-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome. The donor and recipient surgeries were uncomplicated and both are recovering well in the early post-operative period.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , COVID-19 , Anormalidades Congênitas , Infertilidade Feminina , Feminino , Humanos , Adulto , Útero/cirurgia , Infertilidade Feminina/etiologia , Infertilidade Feminina/cirurgia , Hospitais , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia
14.
J Low Genit Tract Dis ; 27(3): 275-279, 2023 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-37192410

RESUMO

OBJECTIVES: To study the clinical, cytological, and vaginal microbiota findings in patients with Mayer-Rokitansky-Küster-Hauser syndrome who underwent neovaginoplasty using Nile tilapia fish skin. METHODS: This is a cross-sectional study with 7 cisgender women with Mayer-Rokitansky-Küster-Hauser syndrome who had previously undergone neovagina reconstruction using Nile tilapia fish skin at a university hospital. Local institutional review board approval and written permission from the patient were obtained. Between August 2019 and November 2021, within 12 to 24 months after surgery, vaginal specimens were obtained for conventional oncotic and hormonal cytology, and for Gram staining. The Nugent scores were calculated. Colposcopy was also performed. RESULTS: Squamous cells without atypia were found in all patients. Five patients had intermediate vaginal microbiota (Nugent score of 4), which was determined by the presence of few lactobacilli on Gram staining. In hormonal cytology, 4 patients presented with findings compatible with menacme. No colposcopic change was observed. When postsurgical dilation was performed correctly, a mean vaginal length of 8.3 cm was maintained after 1 year of follow-up. CONCLUSIONS: Squamous cells without atypia were present in neovaginas with Nile tilapia fish skin. Most vaginal contents revealed intermediate microbiota and hormonal results compatible with menacme. Studies with a greater number of patients are necessary for a more comprehensive understanding of the microbiome in neovaginas with this new technique, thereby providing support for the treatment and prevention of associated pathologies.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Ciclídeos , Anormalidades Congênitas , Animais , Feminino , Humanos , Resultado do Tratamento , Estudos Transversais , Vagina/cirurgia , Vagina/patologia , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/patologia
15.
Gynecol Obstet Fertil Senol ; 51(9): 420-424, 2023 09.
Artigo em Francês | MEDLINE | ID: mdl-37024089

RESUMO

Uterine transplantation is now a possible treatment for absolute uterine infertility. It is currently proposed to women with Mayer-Rokitansky-Küster-Hauser syndrome but indications will likely to expand in the upcoming years. Despite the progressive standardization of the surgical technique and the reduction in perioperative morbidity for both donors and recipients, the number of transplants performed worldwide remains very low compared to the number of women potentially in need. This is partly due to the singularity of uterine transplantation: the uterus is not a vital organ since one can live without a uterus. It is a temporary transplantation that is not performed to extend life but to improve its quality, responding above all to a desire to conceive and bear a child. Beyond the strictly technical aspect, these particularities raise many ethical questions, both on an individual and social level, which should make us question the real place uterine transplantation should take in our society. Answering these questions will allow us to provide better guidance for future eligible couples and to anticipate ethical problems on the long run.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Infertilidade Feminina , Feminino , Humanos , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Previsões , Infertilidade Feminina/cirurgia , Doadores de Tecidos , Útero/transplante
18.
J Sex Med ; 20(1): 57-64, 2023 01 14.
Artigo em Inglês | MEDLINE | ID: mdl-36897238

RESUMO

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


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Saúde Sexual , Humanos , Feminino , Reprodutibilidade dos Testes , Comportamento Sexual/psicologia , Vagina/cirurgia , Coito , Síndrome , Ductos Paramesonéfricos/cirurgia , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/psicologia , Anormalidades Congênitas/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Transtornos 46, XX do Desenvolvimento Sexual/psicologia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia
19.
Int Urogynecol J ; 34(8): 1983-1985, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36790559

RESUMO

INTRODUCTION: The objectives of this video are to provide a brief overview of Müllerian agenesis, discuss a case of partial vaginal agenesis with a functional uterus, and present the steps of a staged McIndoe procedure for the creation of a neovagina and utero-neovaginal unification. METHODS: We give an overview of Mayer-Rokitansky-Küster-Hauser syndrome, and review its incidence, clinical presentation, diagnostic evaluation, and treatment options. We present the case of a 23-year-old woman with partial vaginal agenesis, and her clinical course through conservative management with hormonal suppression and dilator therapy leading up to urogynecological surgical treatment. We describe a staged surgical approach that highlights the value of cystoscopy and laparoscopy to better delineate our patient's anatomical variations. Additionally, a mini-laparotomy and placement of an intrauterine Malecot catheter allowed for the drainage of prominent hematometra, relief of menstrual outflow obstruction, and epithelialization of a tract between the uterus and the planned neovaginal space. Ultimately, a neovagina was created using a staged McIndoe technique, leading to utero-neovaginal unification and unobstructed menses. CONCLUSION: In conclusion, our approach should be considered a feasible option for anatomical restoration via the creation of a neovagina in patients with Müllerian anomalies, even in the presence of a functional uterus.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Humanos , Feminino , Adulto Jovem , Adulto , Útero/cirurgia , Útero/anormalidades , Vagina , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Anormalidades Congênitas/cirurgia
20.
BJOG ; 130(6): 645-652, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36660801

RESUMO

OBJECTIVE: To investigate the long-term outcomes for Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) patients undergoing vaginoplasty using acellular porcine small intestinal submucosa grafts (SIS). DESIGN: A case series. POPULATION: Seventy-eight MRKH syndrome patients and a post-SIS patient who delivered a baby following the world's first robot-assisted uterus transplantation. METHODS: Mayer-Rokitansky-Küster-Hauser syndrome patients were grouped based on the postoperative time and the diagnosis-surgery interval. Outcomes of sexual function and psychological status were assessed using the female sexual function index (FSFI), self-rating scale of body image (SSBI) and self-acceptance questionnaire (SAQ). Anatomical outcomes were measured by clinicians. MAIN OUTCOME MEASURES: The primary outcome was restoration of sexual function, defined by an FSFI score in the 'good' range. Anatomical and psychological outcomes were also analysed. RESULTS: Sexual function was restored in 42.3% (33/78) of patients and the total FSFI score was 23.44 ± 4.43. Three factors (body defect, recognition of physical appearance and willingness to change physical appearance scores) in the SSBI and two in the SAQ decreased as the postoperative time increased. Based on the interval between diagnosis and surgery, the total SSBI score was lower in the short-interval group than in the long-interval group (7.25 ± 5.55 versus 12.04 ± 10.21, p = 0.038). CONCLUSIONS: Nearly half of MRKH patients in our study had good long-term sexual function after SIS vaginoplasty. Sexual function and psychological status improved as postoperative time increased. In addition, reducing the diagnosis to surgery interval was associated with improved psychological function.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Feminino , Suínos , Animais , Humanos , Vagina/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Útero/cirurgia , Anormalidades Congênitas/cirurgia
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