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1.
J Low Genit Tract Dis ; 27(3): 275-279, 2023 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-37192410

RESUMEN

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.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX , Cíclidos , Anomalías Congénitas , Animales , Femenino , Humanos , Resultado del Tratamiento , Estudios Transversales , Vagina/cirugía , Vagina/patología , Trastornos del Desarrollo Sexual 46, XX/patología , Trastornos del Desarrollo Sexual 46, XX/cirugía , Conductos Paramesonéfricos/cirugía , Anomalías Congénitas/cirugía , Anomalías Congénitas/patología
2.
Int Urogynecol J ; 33(8): 2185-2193, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35312805

RESUMEN

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


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX , Anomalías Congénitas , Procedimientos de Cirugía Plástica , Tilapia , Trastornos del Desarrollo Sexual 46, XX/cirugía , Adulto , Animales , Materiales Biocompatibles , Anomalías Congénitas/cirugía , Femenino , Humanos , Conductos Paramesonéfricos/anomalías , Procedimientos de Cirugía Plástica/métodos , Resultado del Tratamiento , Vagina/patología
3.
Int J Gynaecol Obstet ; 157(3): 574-581, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-34534375

RESUMEN

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


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX , Anomalías Congénitas , Procedimientos de Cirugía Plástica , Trastornos del Desarrollo Sexual 46, XX/cirugía , Anomalías Congénitas/cirugía , Dilatación/efectos adversos , Dilatación/métodos , Femenino , Procedimientos Quirúrgicos Ginecológicos/métodos , Humanos , Masculino , Conductos Paramesonéfricos/anomalías , Conductos Paramesonéfricos/cirugía , Estudios Prospectivos , Procedimientos de Cirugía Plástica/efectos adversos , Resultado del Tratamiento , Vagina/anomalías
4.
J Minim Invasive Gynecol ; 28(11): 1817, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34487890

RESUMEN

OBJECTIVE: To present the first robot-assisted uterus retrieval from a living donor for uterine transplantation in Brazil. DESIGN: Stepwise demonstration of the technique with narrated video footage. SETTING: A uterine transplantation was performed in a 33-year-old patient with Mayer-Rokitansky-Küster-Hauser syndrome. The donor was a 50-year-old woman who underwent a robotic-assisted uterus retrieval. The procedure was held at Barretos Cancer Hospital, Barretos, Sao Paulo, Brazil. INTERVENTIONS: After approval from the national and local institutional review board, the protocol was submitted to ClinicalTrials.gov (NCT04249791), and the first case was performed. The screening of the patients was done. The uterus was retrieved through a Pfannenstiel incision to avoid complications to the uterine vessels. Uterus was transplanted in the recipient by end-to-end anastomosis of the internal iliac arteries and end-to-side anastomosis of the external iliac vein with a gonadal vein from the infundibulopelvic ligament. Surgical intraoperative parameters were measured. The docking time was 4 minutes. Robotic donor surgery took 400 minutes, bench surgery took 62 minutes, and laparotomic recipient surgery was completed in 240 minutes. There were no intraoperative complications. The donor patient was discharged from the hospital in 48 hours and the recipient patient in 5 days. CONCLUSION: To the best of our knowledge, this is the first case in Brazil of uterine transplantation with a living donor. Traditionally, patients who undergo uterine transplantation by minimally invasive surgery are managed by laparoscopy. This video demonstrates a feasible robotic approach to uterine transplantation with superior imaging affording a 3-dimensional vision and stabilization of instruments allowing wrist-like movements.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX , Robótica , Trastornos del Desarrollo Sexual 46, XX/cirugía , Adulto , Brasil , Femenino , Humanos , Donadores Vivos , Persona de Mediana Edad , Útero/cirugía
5.
JBRA Assist Reprod ; 25(4): 581-585, 2021 10 04.
Artículo en Inglés | MEDLINE | ID: mdl-34061487

RESUMEN

OBJECTIVE: To evaluate the newly formed epithelium that develops following a neovaginoplasty performed with Amniotic Membrane. METHODS: A retrospective study conducted at the University Hospital of the Federal University, in Curitiba, Paraná, Brazil. A group of 33 patients with Vaginal Agenesis, most of them amenorrhoeic, either incapable of or having difficulty to perform sexual activity, were separated in Subgroup A (27 patients) with Mayer-Rokitansky-Kuster-Hauser Syndrome, and Subgroup B (six patients) with Androgenic Insensitivity Syndrome (Morris Syndrome). Intervention: Banister-McIndoe neovaginoplasty was performed using amniotic membrane graft in 33 patients of Subgroups A and B and evaluated 60-90 days later by vaginal epithelium biopsies. Main Outcome Measure(s): Transmission Electronic Microscopy (TEM) performed Biopsies of neovaginal epithelium in 10 patients. In 20 patients, we analyzed the levels of intensity and presence of Estrogenic Receptors. RESULTS: Vaginal length was measured (vaginometry) before and after surgery. Before surgery, the vagina was absent in 5 patients (15.15%), vaginal length was 1 cm in 19 patients (57.58%) and in 9 patients (27.27%), it was between 2-3 cm. After surgery, all patients had a vaginal length greater or equal to 5 cm and, in 26 patients (78.8%), vaginal length was 7-8 cm. Seven to eight centimeters average neovagina length allowed patients to have a satisfactory sexual activity after all the surgical procedures to dilate, widen and distend the neomucosa lining. The ERs presented different levels of intensity in the three layers of the neovaginal mucosa. TEM analysis of the vaginal neoepithelium obtained from the amniotic membrane graft revealed all the characteristics of a trophic vaginal epithelium. CONCLUSIONS: In a developing country like Brazil, neovaginoplasty with amniotic membrane graft is considered a great option, being an inexpensive, safe, and easy technique, not requiring any special materials. After a few days (60-90 days), or months, a new epithelium and vagina are obtained allowing patients to have proper sexual activity.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX , Procedimientos de Cirugía Plástica , Trastornos del Desarrollo Sexual 46, XX/cirugía , Epitelio , Femenino , Humanos , Estudios Retrospectivos , Resultado del Tratamiento , Vagina/cirugía
6.
Int Urogynecol J ; 32(8): 2283-2285, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-33661320

RESUMEN

INTRODUCTION AND HYPOTHESIS: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the congenital absence of the vagina with variable uterine development. Different methods have been reported for vaginoplasty treatment. The preferred vaginal approach to treating this condition is usually McIndoe vaginoplasty supported by regular dilatation of the neovagina. We present a case video of a McIndoe modified vaginoplasty technique with a heterologous graft using a covering customized 3D-printed mold, its postoperative follow-up and postoperative complications. METHODS: Video presentation of a McIndoe modified vaginoplasty technique using porcine intestinal submucosa performed in a 18-year-old woman diagnosed with MRKH syndrome. Different sizes of a polylactic acid mold were manufactured with 3D printers. After dissection of the recto-vesical space, a mold tailored in terms of length and width was chosen for this patient. RESULTS: After 13 days of follow-up, the patient presented graft infection and subsequent total graft detachment. However, the patient continued to use the vaginal dilator permanently. After 7 months, 8 cm vaginal length with 90% epithelialization and satisfactory sexual intercourse were achieved. CONCLUSION: Functional, histological and anatomical results were reached despite the graft detachment. New technologies such as 3D printing facilitate the development of techniques using tailored molds.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX , Anomalías Congénitas , Procedimientos de Cirugía Plástica , Trastornos del Desarrollo Sexual 46, XX/cirugía , Adolescente , Animales , Anomalías Congénitas/cirugía , Femenino , Procedimientos Quirúrgicos Ginecológicos , Xenoinjertos , Humanos , Conductos Paramesonéfricos/cirugía , Porcinos , Vagina/cirugía
7.
J Minim Invasive Gynecol ; 27(4): 966-972, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-31546063

RESUMEN

Mayer-Rokitansky-Küster-Hauser syndrome is the second most common cause of primary amenorrhea, trailing only to gonadal dysgenesis. Neovaginoplasty is an appropriate treatment option for patients who have failed dilation therapy. Several biomaterials have been used in this procedure, including peritoneum, amnion, skin grafts, and myocutaneous flaps. Nile Tilapia Fish Skin has noninfectious microbiota, morphologic structure comparable to human skin, and high in vivo bioresorption. In addition, it showed good outcomes when used as a xenograft for burn treatment. Thus, we suggest it as a new biologic graft for vaginal agenesis management. In this descriptive study, neovaginoplasty using Nile Tilapia Fish Skin offered 3 patients an anatomic and functional neovagina via a simple method with potential long-term effectiveness. When postsurgical dilation was performed correctly, a vaginal length greater than 6 cm was maintained at 180 days follow-up. Histologic and immunohistochemical analyses revealed the presence of stratified squamous epithelium with high expression of cytokeratins and fibroblast growth factor, matching the characteristics of normal adult vaginal tissue. We believe that further studies will show Nile Tilapia Fish Skin to be a relevant option in the therapeutic arsenal of Mayer-Rokitansky-Küster-Hauser syndrome.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Cíclidos , Anomalías Congénitas/cirugía , Conductos Paramesonéfricos/anomalías , Procedimientos de Cirugía Plástica/métodos , Trasplante de Piel/métodos , Vagina/anomalías , Administración Intravaginal , Adolescente , Adulto , Animales , Productos Biológicos/uso terapéutico , Brasil , Dilatación/métodos , Femenino , Humanos , Conductos Paramesonéfricos/cirugía , Procedimientos de Cirugía Plástica/efectos adversos , Trasplante de Piel/efectos adversos , Colgajos Quirúrgicos , Trasplante Heterólogo/efectos adversos , Trasplante Heterólogo/métodos , Trasplante Heterotópico/efectos adversos , Trasplante Heterotópico/métodos , Resultado del Tratamiento , Vagina/cirugía , Adulto Joven
8.
Rev. chil. obstet. ginecol. (En línea) ; Rev. chil. obstet. ginecol;84(3): 236-244, jun. 2019. tab, graf, ilus
Artículo en Español | LILACS | ID: biblio-1020642

RESUMEN

RESUMEN INTRODUCCIÓN: La ausencia congénita de vagina es una condición poco común, algunas causas son el síndrome de Mayer-Rokitansky-Kuster-Hauser y la insensibilidad periférica a los andrógenos. Múltiples técnicas quirúrgicas y no quirúrgicas se han descrito para el manejo de esta condición, siendo el objetivo principal la creación de un canal vaginal de diámetro y longitud adecuada que permitan restaurar la función coital. El objetivo de este estudio es detallar la experiencia del procedimiento de neovagina con la técnica quirúrgica de McIndoe en pacientes con Mayer-Rokitansky-Kuster-Hauser realizados en la Unidad de Uroginecología de la Clínica Universitaria Bolivariana. METODOLOGÍA: Reporte de 5 casos de pacientes con agenesia de vagina secundarios al síndrome de Mayer-Rokitansky-Kuster-Hauser, a las cuales se les realizó neovagina con la técnica de McIndoe con algunas modificaciones en el molde para el implante de piel. RESULTADOS: Se incluyeron cinco pacientes con diagnóstico de Mayer-Rokitansky-Kuster-Hauser, todas tenían desarrollo de características sexuales secundarias, perfil hormonal normal, y un cariotipo XX. Se utilizó la técnica quirúrgica de McIndoe para la realización de la neovagina sin complicaciones intraoperatorias asociadas y con adecuada evolución posoperatoria, con una longitud vaginal entre 7-9 cm y 3 pacientes con vida sexual activa. El tiempo de estancia hospitalaria fue 7 a 9 días. CONCLUSIÓN: La técnica quirúrgica de McIndoe es una opción para restaurar la función sexual en mujeres con agenesia vaginal con resultados favorables. El tiempo para decidir su realización es electivo, sin embargo, se debe contar con madurez física y emocional para ser llevado a cabo. Las pacientes de nuestro reporte tenían una edad promedio de 18 años.


SUMMARY INTRODUCTION: The congenital absence of the vagina is an uncommon condition, some causes are the Mayer-Rokitansky-Küster-Hauser syndrome and peripheral insensitivity to androgens. Multiple surgical and non-surgical techniques have been described for the management of this condition, being the main objective the creation of a vaginal canal of adequate diameter and length to restore coital function. The objective of this study is to detail the experience of the neovagina procedure with the McIndoe surgical technique performed in patients with Mayer-Rokitansky-Küster-Hauser syndrome at the Clinica Universitaria Bolivariana. METHODOLOGY: Report of five cases of patients with vaginal agenesis secondary to the Mayer-Rokitansky-Kuster-Hauser syndrome, who underwent neovagina with the McIndoe technique and some modifications in the mold for the skin implant. RESULTS: Five patients with diagnosis of Mayer-Rokitansky-Kuster-Hauser were included, all had development of secondary sexual characteristics, normal hormonal profile, and a XX karyotype. The McIndoe surgical technique was used to perform the neovagina without associated intraoperative complications and with adequate postoperative evolution, with a vaginal length between 7-9 cm and three patients with active sexual life. The length of hospital stay was 7 to 9 days. CONCLUSION: The McIndoe surgical technique is an option to restore sexual function in women with vaginal agenesis with favorable results. The time to decide its realization is elective, however, they must have the physical and emotional maturity to be carried out. The patients in our report have an average age of 18 years.


Asunto(s)
Humanos , Femenino , Adolescente , Adulto , Anomalías Congénitas/cirugía , Anomalías Congénitas/diagnóstico , Anomalías Congénitas/genética , Vagina/cirugía , Procedimientos Quirúrgicos Ginecológicos , Vagina/anomalías , Estructuras Creadas Quirúrgicamente , Trastornos del Desarrollo Sexual 46, XX/cirugía , Conductos Paramesonéfricos/anomalías
9.
Fertil Steril ; 112(1): 174-176, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31103284

RESUMEN

OBJECTIVE: To describe a McIndoe procedure technique for surgical management of Mayer-Rokitansky-Kuster-Hauser syndrome with the use of Nile tilapia skin as a scaffold for the proliferation of new vaginal epithelium. DESIGN: Surgical video article. Local Institutional Review Board approval and written permission from the patient were obtained. There were no conflicts of interest. SETTING: University hospital. PATIENT(S): A 17-year-old woman who presented at our gynecology department with the complaint of primary amenorrhea. At physical examination, she had a phenotypically normal vulva with no vaginal canal. Magnetic resonance imaging of the abdomen and pelvis revealed normal ovaries and absence of uterus and vaginal canal. No other congenital malformations were found. Karyotype was 46,XX. INTERVENTION(S): The McIndoe procedure involved only a vaginal approach. Labia minora were separated, and a transverse midline incision of 3 cm was made. The vesicorectal space was progressively dissected. Blunt dissection was performed initially with digital separation of tissues. The neovagina was then inspected with the introduction of a vaginal speculum, allowing for review of hemostasis. Blunt dissection was continued with the aid of the speculum, to reach the appropriate vaginal dimensions. Subsequently, a vaginal acrylic mold covered with two pieces of processed and sterilized tilapia fish skin was inserted and accommodated into the newly created cavity. The external side of the tilapia skin, which maintained its grayish coloration after the removal of the scales, stayed in contact with the acrylic mold, while the white internal side of the tilapia skin, which was previously attached to the fish's muscle, stayed in contact with the walls of the neocavity. The mold was held in position by four multifilament polyglactin 1.0 sutures in the labia majora, thus preventing expulsion. MAIN OUTCOME MEASURE(S): Anatomic data, such as measurement of the final canal length, and histomorphologic analysis, 180 days after surgery. RESULT(S): The patient remained on bed rest for 9 days, after which the tilapia fish skin had been partially reabsorbed. After this time, the acrylic mold was removed. A larger plastic mold was then inserted and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. The final canal length 180 days after surgery was between 8 and 9 cm. For the histopathologic analysis, fragments of the lateral vaginal wall were removed 180 days after surgery and showed the presence of stratified squamous epithelium with five cell layers, ectasic blood vessels, and occasional desquamated epithelial cells. CONCLUSION(S): The procedure described offered this patient an anatomic and functional neovagina by means of a simple, safe, easy, effective, quick, and minimally invasive procedure. Limitations include the experimental nature of this study, based on a single case report with no long-term outcome results. The tilapia fish skin is a low-cost and widely available biomaterial.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Anomalías Congénitas/cirugía , Procedimientos Quirúrgicos Ginecológicos/métodos , Conductos Paramesonéfricos/anomalías , Procedimientos de Cirugía Plástica/métodos , Trasplante de Piel/métodos , Estructuras Creadas Quirúrgicamente , Tilapia , Vagina/cirugía , Trastornos del Desarrollo Sexual 46, XX/diagnóstico por imagen , Adolescente , Animales , Anomalías Congénitas/diagnóstico por imagen , Femenino , Supervivencia de Injerto , Humanos , Conductos Paramesonéfricos/diagnóstico por imagen , Conductos Paramesonéfricos/cirugía , Trasplante Heterólogo , Resultado del Tratamiento , Vagina/anomalías , Vagina/diagnóstico por imagen
10.
Int J Artif Organs ; 42(6): 307-314, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30838938

RESUMEN

Some biomaterial scaffolds can positively interfere with tissue regeneration and are being developed to successfully repair the tissue function. The possibility of using epithelial cells combined with biomaterials appears to be a new option as therapeutic application. This combination emerges as a possibility for patients with Mayer-Rokitansky-Kuster-Hauser syndrome which requires vaginal repair and can be performed with tissue-engineered solution containing cells and biomaterials. It is expected that tissue-engineered solution containing cells and biomaterials would promote tissue repair in a more efficient, modern, and safe way. This study tested the efficiency of tissue-engineered solution containing human malignant melanoma cell line (HMV-II) and different biomaterials, including Cellprene®, Membracel®, and poly lactic-co-glycolic acid/epoxidized polyisoprene. The cells adhered better on poly lactic-co-glycolic acid/epoxidized polyisoprene, and it was found that tissue-engineered solution may also contain mesenchymal stem cells cultivated on poly lactic-co-glycolic acid/epoxidized polyisoprene. Histological, immunofluorescence, and scanning electron microscopy analyses were performed. These initial in vitro results suggest that tissue-engineered solution containing cells and poly lactic-co-glycolic acid/epoxidized polyisoprene is a potential for tissue reconstruction.


Asunto(s)
Regeneración Tisular Dirigida/métodos , Procedimientos de Cirugía Plástica/métodos , Copolímero de Ácido Poliláctico-Ácido Poliglicólico , Ingeniería de Tejidos/métodos , Andamios del Tejido , Trastornos del Desarrollo Sexual 46, XX/cirugía , Animales , Materiales Biocompatibles/química , Materiales Biocompatibles/farmacología , Línea Celular , Anomalías Congénitas/cirugía , Células Epiteliales , Femenino , Humanos , Células Madre Mesenquimatosas , Conductos Paramesonéfricos/anomalías , Conductos Paramesonéfricos/cirugía , Copolímero de Ácido Poliláctico-Ácido Poliglicólico/química , Copolímero de Ácido Poliláctico-Ácido Poliglicólico/farmacología , Soluciones
11.
Rev Colomb Obstet Ginecol ; 70(4): 266-276, 2019 12.
Artículo en Inglés, Español | MEDLINE | ID: mdl-32142241

RESUMEN

Objective: To present the case of a squamous carcinoma in the neovagina of a patient with Mayer-Rokitansky-Küster-Hauser syndrome and review of the literature related to treatment and prognosis of vaginal neoplasms or HPV infection in transgender women or with MRKH syndrome. Materials and methods: A 56-year-old woman consulted to the Hospital Universitario of Sevilla (Spain). During the clinical examination, a exophytic tumor at the bottom of the vagina was found and the biopsy reported squamous carcinoma and positive nucleic acid amplification test for human papilloma (HPV) type 16. A literature search of case reports, case series and observational studies published from 2000 to October 2019 in English and Spanish was performed in Medline via PubMed, with the follow- ing terms: "congenital abnormalities"; "Mullerian aplasia"; "neovagina"; "Vaginal neoplasms"; "Squamous carcinoma"; "HPV infection" was performed. Results: 14 studies were finally included; seven corresponded to squamous cell carcinoma, three to adenocarcinoma and four reported HPV infection only. All of the squamous cell carcinomas were at advanced stages due to local or lymphatic compro- mise and received radiotherapy with concomitant chemotherapy or radical surgery. The prognosis was bad in three of the cases. Patients with adenocarcinoma also presented with advanced lesions due to local extensión and received surgical treatment and two cases received concomitant chemotherapy. Only one patient was followed-up for five years or more. HPV infection is common in women who underwent neovagina reconstruction. Conclusions: Patients with neovagina are susceptible to develope squamous carcinomas or adenocarcinomas depending if skin or intestinal tissue grafts are used. According to local compromise at the time of diagnosis, radical or combined treatments are required. Which screening strategies for HPV, squamous cell carcinomas and adenocarcinoma is to be investigated.


TITULO: CARCINOMA EPIDERMOIDE DE NEOVAGINA EN PACIENTE CON SÍNDROME DE MAYER- ROKITANSKY-KÜSTER-HAUSER. REPORTE DE UN CASO Y REVISIÓN DE LA LITERATURA. Objetivo: Presentar el caso de una paciente con síndrome de Mayer-Rokitansky-Küster-Hauser, con diagnóstico de carcinoma escamoso en neovagina, y realizar una revisión de la literatura del tratamiento y pronóstico de las neoplasias o la infección por VPH de tejidos neovaginales en mujeres transgénero o con síndrome MRKH. Materiales y métodos: Mujer de 56 años de edad que consulta en un hospital universitario regional de la ciudad de Sevilla (España). Tras la exploración clínica se aprecia una tumoración exofítica en fondo de vagina, con resultado de biopsia de carcinoma escamoso y cultivo positivo para virus del papilloma humano (VPH) tipo 16. Se realizó una búsqueda bibliográfica en la base de datos Medline vía PubMed, con los términos: "congenital abnormalities"; "mullerian aplasia"; "neovagina"; "vaginal neoplasms"; "squamous carcinoma"; "HPV infection", de reportes y series de caso, y estudios observacionales publicados desde el año 2000 hasta octubre de 2019 en inglés y español. Resultados: Se incluyeron 14 estudios; de estos, siete correspondieron a carcinoma escamocelular, tres a adenocarcinoma y cuatro informan infección solo por VPH. Todos los carcinomas escamocelulares presentaban estadios avanzados por compromiso local o linfático y recibieron radioterapia con quimioterapia concomitante o cirugía radical. El pronóstico fue malo en tres de los casos. Las pacientes con adenocarcinoma también presentaron lesiones avanzadas con compromiso local, el tratamiento fue quirúrgico, con quimioterapia concomitante en dos de los casos. Solo una paciente tuvo seguimiento mayor a cinco años. La infección por VPH es frecuente en mujeres sometidas a construcción de neovagina. Conclusiones: Las pacientes con neovagina son susceptibles de desarrollar carcinomas escamosos si se utilizaron injertos cutáneos, o adenocarcinomas en aquellas en las que se reconstruyó la vagina a partir de injertos tisulares intestinales. Dado que al momento del diagnóstico presentan compromiso local, a menudo requieren tratamientos radicales o combinados. Se debe investigar cuáles son las mejores estrategias de tamizaje de VPH, cáncer escamoso y adenocarcinoma en este tipo de pacientes.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Carcinoma de Células Escamosas/diagnóstico , Anomalías Congénitas/cirugía , Conductos Paramesonéfricos/anomalías , Infecciones por Papillomavirus/diagnóstico , Neoplasias Vaginales/diagnóstico , Adenocarcinoma/patología , Biopsia , Carcinoma de Células Escamosas/patología , Carcinoma de Células Escamosas/virología , Femenino , Humanos , Persona de Mediana Edad , Conductos Paramesonéfricos/cirugía , Técnicas de Amplificación de Ácido Nucleico , Infecciones por Papillomavirus/complicaciones , Estructuras Creadas Quirúrgicamente/patología , Vagina/patología , Vagina/cirugía , Neoplasias Vaginales/patología , Neoplasias Vaginales/virología
12.
J Pediatr Adolesc Gynecol ; 32(2): 189-192, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30471353

RESUMEN

BACKGROUND: Müllerian aplasia or Mayer-Rokitansky-Küster-Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea. CASE: An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Transabdominal and transperineal ultrasound examinations did not reveal hematocolpos. Exploratory laparotomy revealed severe endometriosis with bilateral hematosalpinx, markedly distended uterus, no proximal vagina, and normal ovaries. Uterine specimen was filled with blood and no clear cervix was present. SUMMARY AND CONCLUSION: Diagnosis of vaginal and cervical agenesis is complicated in low-resource settings and treatment must be modified when subspecialty care and consistent follow-up are not available.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/diagnóstico , Anomalías Congénitas/diagnóstico , Hematómetra/etiología , Conductos Paramesonéfricos/anomalías , Trastornos del Desarrollo Sexual 46, XX/complicaciones , Trastornos del Desarrollo Sexual 46, XX/cirugía , Adolescente , Anomalías Congénitas/cirugía , Femenino , Haití , Hematómetra/cirugía , Humanos , Laparotomía/métodos , Conductos Paramesonéfricos/cirugía , Ultrasonografía
13.
J Pediatr Urol ; 11(1): 47-8, 2015 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25748526

RESUMEN

INTRODUCTION: Congenital adrenal hyperplasia (CAH) resulting from deficient 21-hydroxylase activity is an autosomal recessive disorder with an incidence of 1:5000-25,000 in Caucasian populations. Despite various techniques to treat CAH, total urogenital mobilization (TUM) has gained popularity. This technique has low morbidity, and can be performed by the perineal route with the patient in the dorsal lithotomy position without the need to separate the urethra from the vagina. We aim to demonstrate in this video the TUM technique step by step. METHODS: A 9-month-old child, born with ambiguous genitalia (Prader 3) with a 46XX karyotype and CAH diagnosis presented to our facility. Endoscopy showed a 2-cm common channel. RESULTS AND DISCUSSION: The patient was treated as described in the video and she had an uneventful clinical outcome with complete healing. TUM is an excellent alternative for treating CAH.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Hiperplasia Suprarrenal Congénita/cirugía , Endoscopía/métodos , Procedimientos Quirúrgicos Urogenitales/métodos , Trastornos del Desarrollo Sexual 46, XX/complicaciones , Hiperplasia Suprarrenal Congénita/complicaciones , Femenino , Humanos , Lactante
14.
Gynecol Endocrinol ; 31(4): 327-31, 2015 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-25561399

RESUMEN

The aim of the study was to compare the expression of oestrogen receptor alpha (ERα) in neovaginal tissue of patients with vaginal agenesis following neovaginoplasty using regenerated, oxidised cellulose in premenopausal women. A prospective, observational case-control study was performed on eight patients with vaginal agenesis following modified Abbé-McIndoe neovaginoplasty and 10 control premenopausal women following benign gynaecologic surgery. 6F11 monoclonal antibody was used to determine ERα expression in the vaginal mucosa. Quantitative and qualitative evaluations were performed, respectively, in vaginal epithelium and stroma. The thickness of the vaginal epithelium was determined as the vertical distance between the basal layer cells and the apical surface of the superficial layer. The percentage of ERα-expressing cells was higher in the control group, except in the superficial zone of the epithelium. In the stromal tissue, ERα was detected in only one patient from the neovagina group compared with nine women in the control group. The neovagina group had a statistically thinner epithelium. Our study suggests that women with vaginal agenesis following modified Abbé-McIndoe neovaginoplasty using regenerated oxidised, cellulose experience relatively local hypo-oestrogenism in the first year after surgery, with repercussion in vaginal trophism.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Anomalías Congénitas/cirugía , Receptor alfa de Estrógeno/metabolismo , Regulación de la Expresión Génica , Regeneración Tisular Dirigida , Procedimientos Quirúrgicos Ginecológicos , Membrana Mucosa/metabolismo , Conductos Paramesonéfricos/anomalías , Vagina/metabolismo , Trastornos del Desarrollo Sexual 46, XX/metabolismo , Trastornos del Desarrollo Sexual 46, XX/patología , Adolescente , Adulto , Atrofia , Biopsia , Brasil , Estudios de Casos y Controles , Celulosa Oxidada/uso terapéutico , Anomalías Congénitas/metabolismo , Anomalías Congénitas/patología , Receptor alfa de Estrógeno/genética , Femenino , Estudios de Seguimiento , Regeneración Tisular Dirigida/efectos adversos , Procedimientos Quirúrgicos Ginecológicos/efectos adversos , Hospitales Universitarios , Humanos , Membrana Mucosa/patología , Membrana Mucosa/cirugía , Conductos Paramesonéfricos/metabolismo , Conductos Paramesonéfricos/patología , Conductos Paramesonéfricos/cirugía , Premenopausia , Estudios Prospectivos , Células del Estroma/metabolismo , Células del Estroma/patología , Andamios del Tejido , Vagina/anomalías , Vagina/patología , Vagina/cirugía , Adulto Joven
15.
Lancet ; 384(9940): 329-36, 2014 Jul 26.
Artículo en Inglés | MEDLINE | ID: mdl-24726478

RESUMEN

BACKGROUND: Several disorders might require vaginal reconstruction, such as congenital abnormalities, injury, or cancer. Reconstructive techniques for which non-vaginal tissue is used can be associated with complications. We assessed the use of engineered vaginal organs in four patients with vaginal aplasia caused by Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS). METHODS: We invited to participate four consecutive patients who presented over a 3-year period with congenital vaginal aplasia due to MRKHS. Patients were aged 13-18 years. We obtained a vulvar biopsy of autologous tissue from every patient. We cultured, expanded, and seeded epithelial and muscle cells onto biodegradable scaffolds. The organs were constructed and allowed to mature in an incubator in a facility approved for human-tissue manufacturing. We used a perineal approach to surgically implant these organs. We recorded history, physical examination, vaginoscopy, serial tissue biopsies, MRIs, and self-administered Female Sexual Function Index questionnaire results for all patients, with a follow-up of up to 8 years. FINDINGS: We noted no long-term postoperative surgical complications. Yearly serial biopsies showed a tri-layered structure, consisting of an epithelial cell-lined lumen surrounded by matrix and muscle, with expected components of vaginal tissue present. Immunohistochemical analysis confirmed the presence of phenotypically normal smooth muscle and epithelia. The MRIs, which showed the extent of the vaginal aplasia before surgery, showed the engineered organs and the absence of abnormalities after surgery, which was confirmed with yearly vaginoscopy. A validated self-administered Female Sexual Function Index questionnaire showed variables in the normal range in all areas tested, such as desire, arousal, lubrication, orgasm, satisfaction, and painless intercourse. INTERPRETATION: Vaginal organs, engineered from the patient's own cells and implanted, showed normal structural and functional variables with a follow-up of up to 8 years. These technologies could be useful in patients requiring vaginal reconstruction. FUNDING: Wake Forest University and Hospital Infantil de México Federico Gómez.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Anomalías Congénitas/cirugía , Conductos Paramesonéfricos/anomalías , Ingeniería de Tejidos/métodos , Vagina/cirugía , Adolescente , Estudios de Cohortes , Femenino , Humanos , Conductos Paramesonéfricos/cirugía , Proyectos Piloto , Vagina/citología
16.
Int Braz J Urol ; 38(3): 380-6; discussions 387-8, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22765864

RESUMEN

PURPOSE: To assess the use of sigmoid colon in vaginal reconstruction of some patients with disorders of sex development. MATERIALS AND METHODS: The study included 31 patients with disorders of sex development of various causes. All were reared as females. Female gender was decided for all cases after complete medical assessment. All patients underwent sigmoid vaginoplasty. Assessment of surgical and functional outcomes was carried out in a follow-up period of up to 6 years. RESULTS: The preoperative diagnoses included mullerian aplasia (16 cases), androgen insensitivity syndrome (12 cases) and previous failed vaginoplasty (3 cases). Associated surgical procedures were gonadectomy in 5 cases and gonadectomy combined with clitoroplasty and vulvoplasty in 7 cases. No intra-operative or early postoperative complications occurred. A cosmetic neovagina with adequate size was achieved in all cases. Long term follow-up showed introital stenosis in 4 cases (12.9 %). Two of them responded to vaginal dilatation. The third one needed y-v plasty while the fourth one presented by acute abdomen secondary to ruptured vagina and was submitted to urgent laparotomy. Mucosal prolapse occurred in 1 case (3.2 %). Reoperation rate was 9.6 %. Sexual satisfaction was achieved among 9 sexually active cases. The subjective satisfaction score of the surgical outcome was 8.03. CONCLUSIONS: For patients with disorders of sex development of various etiologies, sigmoid vaginoplasty is the preferred technique for vaginal replacement. It is a safe technique that provides the patient with a cosmetic neovagina of adequate caliber and a satisfactory functional outcome.


Asunto(s)
Trastornos del Desarrollo Sexual 46, XX/cirugía , Síndrome de Resistencia Androgénica/cirugía , Colon Sigmoide/trasplante , Anomalías Congénitas/cirugía , Vagina/anomalías , Vagina/cirugía , Adolescente , Adulto , Femenino , Procedimientos Quirúrgicos Ginecológicos/métodos , Humanos , Masculino , Conductos Paramesonéfricos/anomalías , Conductos Paramesonéfricos/cirugía , Tempo Operativo , Satisfacción del Paciente , Periodo Posoperatorio , Reproducibilidad de los Resultados , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
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