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.

Síndrome de Mayer-Rokitansky-Küster-Hauser: estigma, corpo, mídia e bioética / Mayer-Rokitansky-Küster-Hauser syndrome: stigma, body, mass media and bioethics / Síndrome de Mayer-Rokitansky-Küster-Hauser: estigma, cuerpo, medios de comunicación de masas y bioética

O objetivo do estudo que fundamenta este artigo foi identificar e compreender a construção do estigma social relacionado à síndrome de Mayer-Rokitansky-Kuster-Hauser (SMRKH), uma condição que afeta exclusivamente mulheres. Analisou-se o conteúdo de 43 narrativas jornalísticas veiculadas eletronicamente. O desenho metodológico permitiu a identificação de três temas: (a) o tratamento anedótico da SMRKH; (b) a fragmentação anatomopatológica: mulher-útero ou mulher-vagina; e (c) a retórica do sofrimento da mulher redimido pela medicina. Cerca de 80% do corpus estavam centrados em questões biomédicas, além de haver um flerte com tecnologias experimentais e uma perspectiva de medicina paternalista. Concluiu-se, numa aproximação bioética centrada na dignidade humana, que há necessidade de rever a forma como a mídia apresenta as mulheres afetadas (mulher-útero), evitando ao mesmo tempo modelos de perfeição ou de normalidade que subsumam a mulher ao habitus mulher-esposa-mãe. As mulheres com SMRKH não são corpos ocos e sem úteros, são plenas e podem vivenciar a diferença.

This article bases on a study to identify and understand the construction of social stigma related to Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome, a condition that exclusively affects women, in 43 electronically transmitted journalistic narratives, using the content analysis, based on Laurence Bardin. The methodological design allowed the emergence of three themes: (a) the anecdotal treatment of MRKH syndrome; (b) the anatomopathological fragmentation: woman-uterus or woman-vagina; and (c) the rhetoric of the suffering of the woman redeemed by doctors in medicine. About 80% of the corpus focused their attention on biomedical issues, and there was a flirtation with experimental technologies and a perspective from paternalistic medicine. Based on a bioethical approach centered on human dignity, it was concluded that there is a need to review the way in which the media presents the affected women (womanwomb), avoiding models of perfection or normality that subsume women to the woman-wife-mother characterization. Women affected by MRKH syndrome are not hollow and without uterus bodies, they have plenitude and can experience the difference.

El objetivo del estudio en el cual se funda este artículo ha sido identificar y comprender la construcción del estigma social relacionado con el síndrome de Mayer-Rokitansky-Kuster-Hauser (MRKH), una condición que afecta exclusivamente a las mujeres. Utilizando el análisis de contenido, basado en Laurence Bardin, fueron analizadas 43 narrativas periodísticas transmitidas electrónicamente. El diseño metodológico permitió la identificación de tres temas: (a) el tratamiento anecdótico del síndrome de MRKH; (b) la fragmentación anatomopatológica: mujer-útero o mujer-vagina; y (c) la retórica del sufrimiento de la mujer redimido por la medicina. Cerca del 80% del corpus estabam centrados en cuestiones biomédicasy mostraron flirteo con tecnologías experimentales y una perspectiva de medicina paternalista. Se concluyó, en una aproximación bioética centrada en la dignidad humana, que existe la necesidad de revisar la forma como los medios presentan a las mujeres afectadas (mujer-útero), evitando modelos de perfección o de normalidad que subsuman a la mujer a la caracterización mujer-esposa-madre. Las mujeres con síndrome de MRKH no son cuerpos huecos y sin úteros, son plenas y pueden experimentar la diferencia.

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.

Síndrome de Mayer-Rokitansky-Küster-Hauser o agenesia de útero y vagina en una paciente joven / Mayer-Rokitansky-Küster-Hauser Syndrome or uterus and vagina agenesis in a young patient

Se describe el caso clínico de una paciente con síndrome de Mayer-Rokitansky-Küster-Hauser o agenesia de útero y vagina, pero con ovarios funcionantes, atendida por un equipo multidisciplinario en el Hospital Materno Norte Tamara Bunke Bider de Santiago de Cuba, cuyo diagnóstico se realizó a través del examen físico, el antecedente de amenorrea primaria y estudios complementarios como el cariotipo y la resonancia magnética de la pelvis.

The case report of a patient with syndrome of Mayer-Rokitansky-Küster-Hauser or uterus and vagina agenesis, but with functioning ovaries, assisted by a multidisciplinary team in Tamara Bunke Bider Northern Maternal Hospital in Santiago de Cuba, whose diagnosis was carried out through the physical exam, the history of primary amenorrhoea and complementary studies as the cariotype and magnetic resonance of the pelvis is described.

Leiomioma uterino en paciente con síndrome de Mayer-Rokitansky-Küster-Hauser: reporte de caso / Uterine leiomyoma in a patient suffering from Mayer-Rokitansky-Küster-Hauser syndrome: a case report

Objetivo: el síndrome de Mayer-Rokitansky-Küster-Hauser (MRKH) es una malformación congénita del útero y la parte superior de la vagina en las mujeres que muestran características sexuales secundarias normales y cariotipo 46 XX. Puede ocurrir aplasia completa del útero en presencia de dos cuernos rudimentarios o hipoplasia uterina simétrica o asimétrica acompañada por aplasia de uno de los dos cuernos. Es poco frecuente la aparición de tumores en los rudimentos uterinos. Este caso se presenta con el objetivo de hacer una revisión de la literatura respecto a la asociación de leiomioma uterino y síndrome de MRKH. Materiales y métodos: se presenta el caso y se realiza búsqueda a través de MEDLINE en la página de PubMed con los términos "leiomyoma" y "Mayer-Rokitansky-Küster-Hauser syndrome". Se obtuvieron 8 referencias, de las cuales 7 se identificaron como pertinentes y se incluyeron en la revisión. Además, se hace una búsqueda manual de publicaciones relacionadas con la patología de base. Conclusión: el hallazgo de un leiomioma en el remanente uterino en el síndrome de MRKH es muy poco frecuente, con apenas unas cuantas publicaciones en la literatura médica. En esta paciente, con la impresión diagnóstica de leiomioma uterino, se prefirió realizar laparotomía dado el tamaño del tumor, donde se reportó la patología de biopsia por congelación y la definitiva como leiomioma.

Objective: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital malformation of the uterus and the upper part of the vagina in females having normal secondary sexual characteristics and karyotype 46 XX. Complete uterine aplasia could happen in the presence of two rudimentary horns or symmetric or asymmetric uterine hypoplasia accompanied by aplasia of one or two horns. The appearance of tumours in uterine rudiments is not often described. This case is presented following a review of the literature regarding an association between uterine leiomyoma and MRKH syndrome. Materials and methods: a case has been presented and a search was made through MEDLINE on the PubMed page using the terms "leiomyoma" and "Mayer-Rokitansky-Küster-Hauser syndrome". 8 references were obtained; 7 of them were identified as being pertinent and included in the review. A manual search was also made of publications related to the base pathology. Conclusion: finding a leiomyoma in an uterine remnant in MRKH syndrome is very rare; there are very few publications about it in the pertinent medical literature. It was preferred to carry out laparotomy given the size of the tumour in this patient who had a diagnostic impression of uterine leiomyoma, the pathology being reported by biopsy freezing and the definitive as leiomyoma.