Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 409
Filtrar
1.
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
2.
J Pediatr Urol ; 20(1): 29-34, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37802718

RESUMO

INTRODUCTION: The prostatic utricle (PU) consists of the caudal remnant of the Müllerian duct and the urogenital sinus. The term "vagina masculina" is used if other Müllerian structures are associated with the PU. This work aims to investigate the incidence, management, and follow up of enlarged PUs and Müllerian remnants in males with posterior hypospadias. PATIENTS AND METHODS: This study presents a retrospective review of cases presented with posterior hypospadias over a 5-year period. Prior to hypospadias repair, retrograde urethrograms were used to investigate enlarged PU. Subsequently, they were classified according to the Ikoma score and further assessed by karyotyping and cystoscope. Surgical excision was indicated in cases with symptomatic utricles or vagina masculina. RESULTS: Thirty patients were included in the study in the period between 2015 and 2020 (Table). All cases were asymptomatic initially. Twelve patients were diagnosed with enlarged PU; three of them had vagina masculina. One case with perineal hypospadias had a separate perineal opening for PU. Following hypospadias repair, three of the eight cases treated conservatively turned symptomatic. DISCUSSION: The incidence of enlarged PU and Müllerian remnants varied among different studies. However, it increased as the severity of hypospadias increased. Preoperative urethrogram was helpful in the diagnosis and classification of PU, but it had its limitations. Cystoscope was more advantageous in diagnosing vagina masculina. Although most cases were asymptomatic, some turned symptomatic after hypospadias repair. Some cases with perineal hypospadias had PU with a separate perineal opening. CONCLUSION: The incidence of enlarged PUs or Müllerian remnants was 40%. Although cases were asymptomatic before hypospadias surgery, some cases turned into symptomatic after hypospadias repair. In some cases, the PU or Müllerian remnants had a separate perineal opening. They can be classified as a particular form of Ikoma grade III necessitating surgical intervention.


Assuntos
Hipospadia , Masculino , Feminino , Humanos , Hipospadia/cirurgia , Hipospadia/complicações , Ductos Paramesonéfricos/cirurgia , Uretra , Hipertrofia , Sáculo e Utrículo , Supuração/complicações
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.
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
5.
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
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 Pak Med Assoc ; 73(11): 2280-2283, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-38013548

RESUMO

Persistent Müllerian Duct syndrome is a rare male disorder of sexual development. The phenotypically and genotypically male patient presents with female internal organs (i.e., uterus, cervix, fallopian tubes and upper part of vagina) due to deficiency of anti-mullerian hormone or insensitivity of tissues to Anti Mullerian Hormone. We present a 19 year old male who came with complaint of right iliac fossa pain. He was investigated for acute appendicitis and on imaging, he was diagnosed to have bilateral cryptorchidism with rudimentary uterus. Computed tomography followed by pelvic ultrasonography was done which indicated two testes in abdomen and a soft tissue density structure, identified as a rudimentary uterus located posterior to the urinary bladder. CT scan findings were further confirmed by magnetic resonance imaging pelvis. A trial of stepwise orchidopexy followed by orchidectomy with removal of rudimentary uterus was performed laparoscopically. Additionally, he was counselled for long term sex hormone replacement and reproductive failure in future.


Assuntos
Criptorquidismo , Transtorno 46,XY do Desenvolvimento Sexual , Humanos , Masculino , Feminino , Adulto Jovem , Adulto , Transtorno 46,XY do Desenvolvimento Sexual/diagnóstico , Transtorno 46,XY do Desenvolvimento Sexual/cirurgia , Criptorquidismo/diagnóstico , Criptorquidismo/cirurgia , Hormônio Antimülleriano , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/patologia
9.
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
10.
Rev. chil. obstet. ginecol. (En línea) ; 88(4): 237-247, ago. 2023. ilus
Artigo em Espanhol | LILACS | ID: biblio-1515215

RESUMO

Las malformaciones müllerianas (MM) son un grupo de anomalías estructurales originadas por fallas de desarrollo de los conductos paramesonéfricos o de Müller durante las primeras 16 semanas de gestación. Un oportuno diagnóstico y una correcta clasificación permiten ofrecer el mejor manejo y diferenciar aquellas pacientes que requieren tratamiento quirúrgico. Se realizó una revisión de la literatura sobre MM en las bases de datos Epistemonikos, SciELO, Cochrane y PubMed. Se rescataron todas las pacientes ingresadas con diagnóstico de MM. En el año 2021, la American Society of Reproductive Medicine publicó un consenso en el que se estandarizó la nomenclatura, se amplió el espectro y se simplificó la clasificación. La clínica es variada, e incluye pacientes asintomáticas cuyo diagnóstico es un hallazgo por imágenes. Los mejores estudios imagenológicos son la resonancia magnética (RM) y la ultrasonografía 3D, dejando la histeroscopia y la laparoscopia (método de referencia) como procedimiento diagnóstico-terapéutico. Se presentan casos clínicos desarrollados durante el primer trimestre de 2022. Recomendamos la utilización sistemática de la RM para el diagnóstico de anomalías complejas u obstructivas del aparato genital. El tratamiento de estas patologías debe ser realizado por ginecólogos endoscopistas expertos, e incluye tratamiento médico y quirúrgico, el cual debe ser enfocado en cada paciente dependiendo del tipo de MM y de los deseos de fertilidad.


Müllerian malformations (MM) are a group of structural anomalies caused by developmental failure of the paramesonephric or Müllerian ducts during the first 16 weeks of gestation. Timely diagnosis and classification allow us to offer the best management and to differentiate those patients who require surgical treatment. Literature review on MM in Epistemonikos, SciELO, Cochrane and PubMed databases. All patients admitted with a diagnosis of MM were rescued. In 2021, the American Society of Reproductive Medicine publishes a consensus where it standardizes the nomenclature, broadens the spectrum, and simplifies the classification. The clinical picture is varied and includes asymptomatic patients whose diagnosis is an imaging finding. The best imaging studies are magnetic resonance imaging (MRI) and 3D ultrasonography, leaving hysteroscopy and laparoscopy (gold standard) as diagnostic therapeutic. Clinical cases developed during the first trimester 2022 are presented. We recommend the routine use of MRI for the diagnosis of complex and/or obstructive anomalies of the genital tract. The treatment of these pathologies should be performed by expert endoscopic gynecologists and include medical and surgical treatment, which should be focused on each patient, depending on the type of MM and fertility desires.


Assuntos
Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/diagnóstico por imagem , Doenças Uterinas/cirurgia , Doenças Uterinas/congênito , Doenças Uterinas/diagnóstico por imagem , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/classificação , Anormalidades Congênitas/diagnóstico por imagem , Útero/anormalidades , Vagina/anormalidades , Doenças Vaginais/cirurgia , Doenças Vaginais/congênito , Doenças Vaginais/diagnóstico por imagem , Infertilidade Feminina
11.
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
12.
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
13.
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
14.
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
15.
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
16.
J Minim Invasive Gynecol ; 30(9): 742-747, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37172890

RESUMO

STUDY OBJECTIVE: To investigate the possibility of conservative management of rudimentary uterine horns associated with vaginal agenesis. DESIGN: Observational study on cohort of consecutive cases treated with the same criteria from 2008 to 2021. SETTING: Two academic institutions and teaching hospitals in Milan, Italy. PATIENTS: Eight patients with vaginal agenesis associated with rudimentary cavitated uterine horns treated by the same team and postoperatively followed. INTERVENTIONS: All the subjects underwent the same standardized surgical procedure: laparoscopy, intraoperative ultrasound, and horn-vestibular direct anastomosis. Postoperatively vaginoscopy was performed every 6 months. MEASUREMENTS AND MAIN RESULTS: The postoperative course was generally uneventful and the mean hospital stay was 4.3 ± 2.5 (SD) days. All the patients began to menstruate a few months after the operation. Menstrual flows were light but regular. All patients had a neovaginal length > 4 cm at 1 year postoperatively, reaching approximately 6 cm at 2 years. During the follow-up period, 5 patients were sexually active without dyspareunia. In all cases, surgery restored the continuity of the neovagina and uterine horn through the creation of a "vaginal-horn fistula tract." CONCLUSION: In patients with vaginal agenesis associated with the presence of a uterine cavitary horn, it is possible to recover not only sexual activity but also menstrual function. The horn-vestibular anastomosis may be considered a valid, safe, and effective therapeutic option but requires accurate preoperative and intraoperative evaluation of rudimentary uterine structures.


Assuntos
Anormalidades Múltiplas , Anormalidades Congênitas , Laparoscopia , Procedimentos de Cirurgia Plástica , Fístula Vaginal , Feminino , Humanos , Anormalidades Múltiplas/cirurgia , Anormalidades Congênitas/cirurgia , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Útero/cirurgia , Útero/anormalidades , Vagina/cirurgia , Vagina/anormalidades , Fístula Vaginal/cirurgia
17.
J Pediatr Adolesc Gynecol ; 36(5): 476-483, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37182810

RESUMO

STUDY OBJECTIVE: To understand variations and experiences of vaginal lengthening internationally in individuals with congenital underdevelopment of the uterus, cervix, and upper vagina or Müllerian agenesis METHODS: 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. RESULTS: 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. CONCLUSION: 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.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Adulto , Feminino , Humanos , Estudos Transversais , Resultado do Tratamento , Vagina/cirurgia , Vagina/anormalidades , Útero , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Ductos Paramesonéfricos/cirurgia , Ductos Paramesonéfricos/anormalidades , Anormalidades Congênitas/diagnóstico
20.
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
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...