Terapia regenerativa en secuela postquirúrgica de neovagina. Caso clínico con síndrome Mayer-Rokitansky / Regenerative therapy applied to post-surgical sequelae of neovagina. Case presentation with Mayer Rokitansky síndrome

Las malformaciones congénitas que incluyen agenesia vaginal requieren tratamiento quirúrgico en la infancia, pero las secuelas deformantes se presentan en edad adulta y son un motivo de consulta al que se debe dar solución.Con este caso pretendemos resaltar la importancia de la integración de técnicas quirúrgicas y complementarias en el tratamiento seguro de las secuelas quirúrgicas invalidantes en pacientes sometidas a reconstrucción vaginal, mostrando los resultados del tratamiento combinado con toxina botulínica y células madre adiposas en una paciente con síndrome de Mayer Rokitansky y estenosis vaginal postquirúrgica.Este tratamiento disminuyó el dolor y la contractura del introito vaginal que permitió la exploración física y la reintegración de la paciente a la vida sexual. Nivel de evidencia científica 4d Terapéutico.(AU)

Congenital malformations that include vaginal agenesis require surgical treatment in childhood, but deforming sequelae present in adulthood constitute a reason for consultation that must be solved.This case is intended to highlight the importance of integrating surgical and complementary techniques in the safe treatment of disabling surgical sequelae in vaginal reconstruction showing the results of the combined treatment with botulinum toxin and adipose stem cells in a patient with Mayer Rokitansky syndrome and post-surgical vaginal stenosis.This treatment decreased the pain and contracture of the vaginal introitus that allowed physical examination and that patient was reintegrated into sexual life.Level of evidence 4d Terapeutic.(AU)

Three-dimensional Printer Molds for Vaginal Agenesis: An Individualized Approach as Conservative Treatment / Moldes de impressão tridimensionais para agenesia vaginal: Uma abordagem individualizada como tratamento conservador

Abstract Objective The aim of this study was to evaluate the use of vaginal molds, made with three-dimensional (3D) printing, for conservative treatment through vaginal dilation in patients with vaginal agenesis (VA). Methods A total of 16 patients with a diagnosis of VA (Mayer-Rokitansky-Küster-Hauser syndrome, total androgen insensitivity syndrome, and cervicovaginal agenesis) from the Federal University of São Paulo were selected. Device production was performed in a 3D printer, and the polymeric filament of the lactic polyacid (PLA) was used as raw material. A personalized treatment was proposed and developed for each patient. Results There were 14 patients who reached a final vaginal length of 6 cm or more. The initial total vaginal length (TVL) mean (SD) was 1.81(1.05) and the final TVL mean (SD) was 6.37 (0.94); the difference, analyzed as 95% confidence interval (95% CI) was 4.56 (5.27-3.84) and the effect size (95% CI) was 4.58 (2.88-6.28). Conclusion The 3D printing molds for vaginal dilation were successful in 87.5% of the patients. They did not present any major adverse effects and offered an economical, accessible, and reproducible strategy for the treatment of VA.

Resumo Objetivo O objetivo deste estudo foi avaliar o uso de moldes dilatadores vaginais, confeccionados com impressão tridimensional (3D), para tratamento conservador através da dilatação vaginal em pacientes com agenesia vaginal (AV). Métodos Foram selecionadas 16 pacientes com diagnóstico de AV (síndrome de Mayer-Rokitansky-Küster-Hauser, síndrome de insensibilidade androgênica total e agenesia cervicovaginal), da Universidade Federal de São Paulo. A produção dos dispositivos foi realizada em uma impressora 3D e, como matéria-prima, foi utilizado o filamento polimérico do poliácido lático (PLA). Um tratamento personalizado foi proposto e desenvolvido para cada paciente. Resultados Quatorze pacientes atingiram um comprimento vaginal final (CVF) de 6 cm ou mais. A média inicial do CVF (DP) foi de 1,81 (1,05) e a média final do CVF (DP) 6,37 (0,94); a diferença (IC 95%) foi de 4,56 (5,27-3,84) e o tamanho do efeito (IC 95%) foi de 4,58 (2,88-6,28). Conclusão Os moldes de impressão 3D para dilatação vaginal obtiveram sucesso em 87,5% das pacientes. Como impacto secundário, não apresentaram efeitos adversos importantes e ofereceram uma estratégia econômica, acessível e reprodutível para o tratamento da AV.

Tabique vaginal transverso: revisión bibliográfica a propósito de un caso / Transverse vaginal septum: case report and literature review

El tabique vaginal transverso es una malformación genital femenina muy infrecuente con una etiología desconocida y un manejo clínico aún por discernir. Presentamos el caso de una mujer de 17 años que presentaba estrechez del introito vaginal asociada a una intensa dismenorrea, con el diagnóstico final de esta entidad tras una exploración bajo anestesia. El conocimiento de esta anomalía junto con sus hallazgos clínicos y posibles entidades extragenitales asociadas permiten realizar un apropiado diagnóstico de sospecha y una correcta clasificación, lo que conlleva un manejo terapéutico más óptimo.(AU)

Transverse vaginal septum is a rare female genital malformation of unknown aetiology, and for which there is still no clear clinical management. The case is presented of a 17-year-old woman who had a narrow vaginal introitus associated with intense dysmenorrhoea. She was diagnosed with this disorder after an examination under anaesthesia. The knowledge of this anomaly, as well as its symptomatology and associated extra-genital pathologies, can lead to an appropriate suspected diagnosis and correct classification, and to a more optimal therapeutic management.(AU)

Reporte de serie de casos: neovagina en pacientes con Síndrome Mayer-Rokitansky-Kuster-Hauser / Case series report: neovagina in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome

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.

Uropatía obstructiva en una paciente con síndrome de Mayer-Rokitansky-Küster-Hauser / Obstructive uropathy in a patient with Mayer-Rokitansky-Küster-Hauser syndrome

El síndrome de Mayer-Rokitansky-Küster-Hauser (MRKH) es una aplasia congénita del útero y los dos tercios superiores de la vagina, debido a falla en el desarrollo de los conductos de Müller entre la quinta y sexta semana de gestación. Se caracteriza por la presencia de amenorrea primaria en mujeres jóvenes que tienen un desarrollo normal de los caracteres sexuales secundarios femeninos, genitales externos normales y un cariotipo normal. Ocurre en aproximadamente una de cada 4500 mujeres y se ha relacionado con mutaciones específcas en genes que median el desarrollo embrionario. La técnica no quirúrgica más utilizada es el método dilatador de Frank, mientras que las alternativas quirúrgicas incluyen las técnicas desarrolladas por McIndoe, Williams, Vecchietti, Davydov y Baldwin

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital aplasia of the uterus and the upper two thirds of the vagina, due to developmental failure of the Müllerian ducts between the fifth and sixth week of gestation. Be characterized by the presence of primary amenorrhea in young women who have a normal development of female secondary sexual characters, Normal external genitalia and a normal karyotype. It occurs in approximately one in 4500 women and has been linked to specific mutations in genes that mediate embryonic development. Nonsurgical technique The most widely used is Frank's dilator method, while surgical alternatives include the techniques developed by McIndoe, Williams, Vecchietti, Davydov and Baldwin