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BACKGROUND: Congenital uterine anomalies include a wide diversity of uterine malformations that can compromise reproductive potential. Uterus transplantation (UTx) proposes an innovative treatment for absolute uterine factor infertility; however, there is a lack of standardized protocols to guide clinical management among this population. OBJECTIVES: To describe recipient and donor characteristics and obstetric outcomes in patients undergoing UTx. SEARCH STRATEGY: We performed a literature search using the PubMed database to retrieve available scientific articles. We analyzed the references of included articles to assess additional articles that could be eligible to be included in the review. Likewise, we identified further studies using other methods, including Google Scholar. SELECTION CRITERIA: Titles and abstracts were screened in duplicate to select original reports with information available for the outcomes of interest. DATA COLLECTION AND ANALYSIS: This review assessed the advantages and disadvantages of the techniques used, patient characteristics, obstetric and non-obstetric complications, functional duration of the organ, and neonatal outcomes. MAIN RESULTS: Among the 36 reports included in this review we found 55 pregnancies and 38 live births following UTx and a higher success rate for in vivo uterine donations. The most common obstetric complications reported included miscarriage, pre-eclampsia, and gestational hypertension. The most common non-obstetric complications reported include episodes of rejection, acute kidney injury, anemia, and cholestasis. Living donors required a comprehensive preoperative workup, decreasing organ rejection, infection, and vascular complications. CONCLUSIONS: More studies are needed to standardize the UTx procedure and improve obstetric, fetal, and neonatal outcomes. Further understanding of which recipient and donor characteristics minimize complications will significantly decrease the risk of adverse outcomes.
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Resumen El síndrome de Mayer-Rokitansky-Küster-Hauser (SMRKH) es una anomalía del tracto genital femenino caracterizada por ausencia congénita del útero y porción superior de la vagina. Ocurre en uno de cada 4,500 nacimientos y se diagnostica normalmente durante la adolescencia al presentarse amenorrea primaria. Su función ovárica está preservada, pero la información actual respecto al potencial reproductivo de estas pacientes es limitada. Se presenta el caso de una mujer con diagnóstico de SMRKH sometida a estimulación ovárica para transferencia de embriones a útero subrogado y se discute su potencial reproductivo: técnicas de reproducción asistida, intervenciones e impacto psicológico.
Abstract Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital anomaly of the female genital tract characterized by congenital absence of the uterus and upper part of the vagina. It occurs in 4,500 female births and diagnosis is usually made during adolescence when primary amenorrhea presents. They have functioning ovaries but data regarding their reproductive potential is limited. We hereby report the case of a woman diagnosed with MRKH syndrome in whom assisted reproductive techniques were used to try to achieve pregnancy by gestational surrogacy and their reproductive potential is discussed: assisted reproductive techniques, procedures, and psychological impact.
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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.
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Humanos , Feminino , Vagina/anatomia & histologia , Impressão TridimensionalRESUMO
We revisit the optimal control problem with maximum cost with the objective to provide different equivalent reformulations suitable to numerical methods. We propose two reformulations in terms of extended Mayer problems with state constraints, and another one in terms of a differential inclusion with upper-semi-continuous right member without state constraint. For the latter we also propose a scheme that approximates from below the optimal value. These approaches are illustrated and discussed in several examples.
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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.
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Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Tilápia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Adulto , Animais , Materiais Biocompatíveis , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Ductos Paramesonéfricos/anormalidades , Procedimentos de Cirurgia Plástica/métodos , Resultado do Tratamento , Vagina/patologiaRESUMO
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.
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Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Dilatação/efeitos adversos , Dilatação/métodos , Feminino , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Masculino , Ductos Paramesonéfricos/anormalidades , Ductos Paramesonéfricos/cirurgia , Estudos Prospectivos , Procedimentos de Cirurgia Plástica/efeitos adversos , Resultado do Tratamento , Vagina/anormalidadesRESUMO
Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is the major cause of vaginal agenesis. Vaginoplasty with oxidized cellulose has been used by gynecologists as a surgical option in vaginal agenesis; however, it is not very widespread among pediatric surgeons. A case of MRKHS who underwent vaginoplasty with oxidized cellulose is reported here.
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Vaginal dilation, currently considered as the first-line therapy for vaginal aplasia in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a safe and effective treatment that aims to create a functional neovagina. However, rigid vaginal dilators classically described in the literature usually cause physical discomfort and side effects that can lead to vaginal necrosis. Here, we present two cases of MRKH syndrome patients with vaginal agenesis whose main complaint was the inability to have sexual intercourse with their partners. Considering unavailability of acrylic dilators and previous studies reporting good responses with the use of silicone dilators in women with post-radiotherapy vaginal stenosis, the medical team and patients opted for creation of a neovagina through the daily use of silicone vaginal dilators. Patient 1 developed an 8-cm vagina after 6 months of treatment and had a satisfactory sex life with her partner. Patient 2 developed a 7-cm vagina and reported significant symptom improvement. None of the patients developed side effects after the treatment. The use of inexpensive and easily accessible silicone vaginal dilators may be an effective and noninvasive alternative with few side effects for women with vaginal agenesis, particularly in the developing countries.
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Transtornos 46, XX do Desenvolvimento Sexual/terapia , Anormalidades Congênitas/terapia , Dilatação , Ductos Paramesonéfricos/anormalidades , Vagina/anormalidades , Vagina/patologia , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Adulto , Coito/fisiologia , Anormalidades Congênitas/patologia , Tratamento Conservador , Dilatação/instrumentação , Dilatação/métodos , Feminino , Procedimentos Cirúrgicos em Ginecologia/efeitos adversos , Procedimentos Cirúrgicos em Ginecologia/instrumentação , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Ductos Paramesonéfricos/patologia , Resultado do Tratamento , Adulto JovemRESUMO
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.
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Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ciclídeos , Anormalidades Congênitas/cirurgia , Ductos Paramesonéfricos/anormalidades , Procedimentos de Cirurgia Plástica/métodos , Transplante de Pele/métodos , Vagina/anormalidades , Administração Intravaginal , Adolescente , Adulto , Animais , Produtos Biológicos/uso terapêutico , Brasil , Dilatação/métodos , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Procedimentos de Cirurgia Plástica/efeitos adversos , Transplante de Pele/efeitos adversos , Retalhos Cirúrgicos , Transplante Heterólogo/efeitos adversos , Transplante Heterólogo/métodos , Transplante Heterotópico/efeitos adversos , Transplante Heterotópico/métodos , Resultado do Tratamento , Vagina/cirurgia , Adulto JovemRESUMO
Resumen ANTECEDENTES: El síndrome de Mayer-Rokitansky-Küster-Hauser aparece en 1 de cada 4500 a 5000 mujeres; se origina por subdesarrollo embrionario de los conductos de Müller que resulta en agenesia vaginal o de útero. El tratamiento consiste en dilataciones vaginales o formación de una neovagina mediante un procedimiento quirúrgico. La falta de estructuras de soporte vaginal es una de las causas del prolapso de la cúpula vaginal, posterior al tratamiento conservador o quirúrgico. CASO CLÍNICO: Paciente de 26 años, con diagnóstico de síndrome de Mayer-Rokitansky-Küster-Hauser; inicio de la vida sexual activa a los 16 años. Acudió a consulta por sensación de cuerpo extraño en los genitales y un "bulto" vaginal de dos años de evolución. En la exploración física ginecológica se encontró un prolapso total vaginal. Se realizó la sacrocolpopexia laparoscópica y se colocó una malla tipo I, con monofilamento, para la corrección del prolapso vaginal. A los tres meses posteriores al procedimiento quirúrgico no volvió a reportar síntomas de sensación de cuerpo extraño en la vagina y reinició la vida sexual activa sin problemas. CONCLUSIONES: La sacrocolpopexia laparoscópica, junto con la colocación de una malla, es una opción de tratamiento con buenos resultados en la restauración de la anatomía, función sexual y satisfacción de la paciente con prolapso de la cúpula, posterior a creación de una neovagina.
Abstract BACKGROUND: The Mayer-Rokitansky-Küster-Hauser syndrome, has an incidence of 1 per 4,500 to 5,000 women, is caused by an embryonic underdevelopment of the müllerian ducts, resulting in agenesis of the vagina or uterus. There is treatment based on dilatations and creation of neovagina by surgery. Prolapse of the vaginal vault in Mayer-Rokitansky-Küster-Hauser syndrome is rare and may occur after conservative or surgical treatments. The lack of vaginal support structures can lead these patients to develop a vaginal vault prolapse CASE: A 26-year-old woman, who started sexual life at 16 years of age. He came to the clinic due to a sensation of a foreign body in the genitals and vaginal bulge of two years of evolution. Physical examination: normal external genitalia without alterations, with presence of total vaginal prolapse. Reflex of the sacral plexus S2-S4 (clitoral, perineal and anal) present and normal. Pelvic organ prolapse quantification (POP Q): 0, 0, +2, 3, 4, 5, -1, -1, x. IP: II, Gossling 2. Laparoscopic sacrocolpopexy was performed and type I mesh with monofilament was used to correct vaginal prolapse. At 3 months after the procedure, she denies vaginal bulge symptom in vagina, satisfactory active sexual, denies dyspareunia. CONCLUSIONS: Laparoscopic sacrocolpopexy with mesh placement is a treatment option with good results in the restoration of the anatomy, sexual function and satisfaction of the patient in a patient with dome prolapse posterior to the neovagina.
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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.
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Humanos , Bioética , Saúde da Mulher , Sexualidade , Pessoalidade , Qualidade de Vida , Reprodução , Útero , Vagina , Jornalismo , Estigma SocialRESUMO
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.
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Humanos , Feminino , Adolescente , Adulto , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/genética , Vagina/cirurgia , Procedimentos Cirúrgicos em Ginecologia , Vagina/anormalidades , Estruturas Criadas Cirurgicamente , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Ductos Paramesonéfricos/anormalidadesRESUMO
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.
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Humanos , Feminino , Adulto , Adulto Jovem , Útero/anormalidades , Vagina/anormalidades , Útero/diagnóstico por imagem , Vagina/diagnóstico por imagem , Atenção Secundária à SaúdeRESUMO
Abstract Purpose To evaluate the anatomic and functional results of a laparoscopic modified Vecchietti technique for the creation of a neovagina in patients with congenital vaginal aplasia. Methods Retrospective study of nine patients with congenital vaginal aplasia submitted to the laparoscopic Vecchietti procedure, in our department, between 2006 and 2013. The anatomical results were evaluated by assessing the length, width and epithelialization of the neovagina at the postoperative visits. The functional outcome was evaluated using the Rosen Female Sexual Function Index (FSFI) questionnaire and comparing the patients' results to those of a control group of 20 healthy women. The statistical analysis was performed using SPSS Statistics version 19.0 (IBM, Armonk, NY, USA), Student t-test, Mann-Whitney U test and Fisher exact test. Results The condition underlying the vaginal aplasia was Mayer-Rokitansky-KüsterHauser syndrome in eight cases, and androgen insensitivity syndrome in one case. The average preoperative vaginal length was 2.9 cm. At surgery, the mean age of the patients was 22.2 years. The surgery was performed successfully in all patients and no intra or postoperative complications were recorded. At the first postoperative visit (6 to 8 weeks after surgery), the mean vaginal length was 8.1 cm. In all cases, the neovagina was epithelialized and had an appropriate width. The mean FSFI total and single domain scores did not differ significantly from those of the control group: 27.5 vs. 30.6 ( total); 4.0 vs. 4.2 (desire); 4.4 vs. 5.2 (arousal); 5.2 vs. 5.3 (lubrication); 4.2 vs. 5.0 ( orgasm); 5.3 vs. 5.5 (satisfaction) and 4.4 vs. 5.4 ( comfort ). Conclusions This modified laparoscopic Vecchietti technique is a simple, safe and effective procedure, which allows patients with congenital vaginal aplasia to have a satisfactory sexual activity, comparable to that of normal controls.
Resumo Objetivo Avaliar os resultados anatômicos e funcionais da técnica laparoscópica modificada de Vecchietti para a criação de uma neovagina em pacientes com aplasia vaginal congênita. Métodos Estudo retrospectivo de nove pacientes com aplasia vaginal congênita submetidas à técnica laparoscópica modificada de Vecchietti, no nosso departamento, entre 2006 e 2013. Os resultados anatômicos foram aferidos através da avaliação do comprimento, largura e reepitelização da neovagina nas consultas pós-operatórias. Os resultados funcionais foram avaliados com recurso à versão em português do questionário Female Sexual Function Index de Rosen, comparando os resultados das pacientes aos de um grupo de controle de 20 mulheres saudáveis. A análise estatística foi realizada utilizando o programa SPSS Statistics versão 19.0), o teste t de Student, teste U de Mann-Whitney e teste exato de Fisher. Resultados A etiologia subjacente à aplasia vaginal foi a síndrome de Mayer-Roki-tansky-Küster-Hauser em oito casos, e a síndrome de insensibilidade aos andrógenos em um caso. O comprimento vaginal médio pré-operatório era de 2,9 cm. À data da cirurgia, a média de idade das pacientes era de 22,2 anos. A cirurgia foi realizada com sucesso em todos os casos, sem registo de complicações intra ou pós-operatórias. Na primeira avaliação pós-operatória (6 a 8 semanas após a cirurgia), o comprimento vaginal médio foi de 8,1 cm. Em todos os casos, a neovagina estava reepitelizada e com amplitude adequada. As pontuações médias, total e de cada domínio, obtidas no questionário de avaliação da função sexual não diferiram significativamente das do grupo controle: 27,5 vs 30,6 (total); 4.0 vs 4.2 (desejo); 4,4 vs 5,2 (excitação); 5,2 vs 5 , 3 (lubrificação); 4,2 vs 5,0 (orgasmo); 5,3 vs 5,5 (satisfação) e 4,4 vs 5,4 ( conforto ). Conclusões A técnica laparoscópica modificada de Vecchietti é um procedimento simples, seguro e eficaz, permitindo às pacientes com aplasia vaginal congênita uma atividade sexual satisfatória, comparável à dos controles normais.
Assuntos
Humanos , Feminino , Adolescente , Adulto , Adulto Jovem , Procedimentos Cirúrgicos em Ginecologia/métodos , Laparoscopia , Vagina/anormalidades , Vagina/cirurgia , Recuperação de Função Fisiológica , Estudos Retrospectivos , Autorrelato , Sexualidade , Resultado do Tratamento , Vagina/fisiologiaRESUMO
Introducción: el síndrome de Mayer-Rokitansky-Küster-Hauser (SMRKH) es un espectro de anomalías de los conductos müllerianos caracterizado por la la ausencia congénita de útero y vagina en mujeres fenotípicamente normales, con cariotipo 46 XX, con un estado endocrino normal. Muchas de estas mujeres poseen remanentes uterinos. La presencia de miomas en dichos remanentes es un hallazgo de muy escasa ocurrencia por lo que se reporta el caso. Objetivo: describir la técnica quirúrgica laparoscópica y revisar el diagnóstico clínico y el diagnóstico diferencial. Materiales y métodos: se presenta el caso de una mujer con diagnóstico previo de síndrome de Mayer-Rokitansky-Küster-Hauser (SMRKH), quien consulta a la la Unidad de Endoscopia Ginecológica de la Clínica del Prado en Medellín, institución privada de tercer nivel de complejidad y que es centro de referencia para manejo laparoscópico de pacientes con dolor pélvico crónico del departamento de Antioquia, Colombia. La ecografía reporta una masa sólida de ocho centímetros en la pelvis. Durante la laparoscopia se encuentra un mioma en uno de los remanentes uterinos. Se realiza miomectomía y se resecan ambos remanentes por laparoscopia. Se realizó una búsqueda sistemática de la literatura a través de Medline en la página de Pubmed utilizando los siguientes términos: “Mayer-Rokitansky-Hauser una mujer con diagnóstico previo de síndrome de Syndrome”, “leiomyoma” y “laparoscopy”. Resultados: se encontraron 25 artículos en los últimos 20 años de los cuales 17 estaban relacionados con el tema de manera directa; de estos, 2 incluían el manejo con laparoscopia. Conclusiones: la presencia de leiomioma en un remanente uterino de una paciente con MRKH es poco frecuente. El caso aquí presentado es el segundo reportado en Colombia y el primero manejado por vía laparoscópica. En pacientes con síndrome de Mayer-Rokitansky-Küster-Hauser y masas pélvicas, la laparoscopia es una opción diagnóstica y terapéutica por considerar.
Introduction: The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a spectrum of müllerian duct anomalies characterized by the congenital absence of the uterus and vagina in phenotypically normal women with a 46 XX karyotype and normal endocrine status. Many of these women have uterine remnants. The presence of fibroids in those remnants is a very rare finding, hence this case report. Objective: To describe the laparoscopic surgical technique and review the clinical and differential diagnoses. Materials and methods: Female patient with a prior diagnosis of MRKH syndrome who presented to the Gynecological Endoscopy Unit at Clinica del Prado, a private, level 3 institution in Medellin, which is a referral center for the laparoscopic management of patients with chronic pelvic pain in the Department of Antioquia, Colombia. A solid, nine-centimeter pelvic mass was found on ultrasound. During laparoscopy, a leiomyoma was found in one of the uterine remnants. Laparoscopic myomectomy was performed and the two remnants were resected. A systematic review of the literature was conducted through Medline in the Pubmed site using the following terms: “Mayer-Rokitansky-Hauser Syndrome”, “leiomyoma” and laparoscopy. Results: In total, 25 articles of the past 20 years were found; of those, 17 were related directly to the topic, and 2 of these included laparoscopic management. Conclusions: The presence of leiomyoma in a uterine remnant in a patient with MRKH syndrome is infrequent. The case presented here is the second reported in Colombia and the first managed laparoscopically. In patients with the Mayer-Rokitansky-Küster-Hauser syndrome and pelvic masses, laparoscopy is a diagnostic and therapeutic option.
Assuntos
Humanos , Feminino , Adulto , Laparoscopia , LeiomiomaRESUMO
La agenesia vaginal o Síndrome de Mayer-Rokitansky es una rara anomalía congénita, cuya prevalencia aproximada es de 1 de cada 5 000 mujeres recién nacidas. El tratamiento de estos pacientes representa un reto para el cirujano; se requieren buenos resultados cosméticos y funcionales. Se presenta el caso de una paciente de 41 años con síndrome de Mayer-Rokitansky, a la cual se le construyó una neovagina asistida por laparoscopia, según la técnica de Davydov modificada. No hubo complicaciones inmediatas o mediatas. Se obtuvo una adecuada longitud de vagina, de 7 cm una semana después de la cirugía, y adecuada función sexual seis meses después.
Vaginal agenesis or Mayer-Rokitansky syndrome is a rare congenital anomaly, with prevalence about 1 in 5 000 newborn females. Treatment is a challenge for the surgeon, in order to obtain good cosmetic and functional results. We present the case of a 41 year-old patient with Mayer-Rokitansky syndrome who underwent laparoscopic assisted neovagina according to modified Davydov technique. There were no immediate or mediate complications. An adequate vaginal length was achieved, 7 cm long a week after surgery and adequate sexual function six months later.
RESUMO
En el presente artículo se presenta un caso de paciente femenina de 16 años, que acudió por un cuadro de amenorrea, posteriormente al ser estudiado mediante el uso de estudios de diagnóstico por imágenes (Tomografia Multislice) se llego al diagnostico de sindrome de Mayer Rokitansky Kuster-Hauser.
Assuntos
SíndromeRESUMO
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.
Assuntos
Humanos , Feminino , Adulto , Leiomioma , ÚteroRESUMO
Introducción: la agenesia de vagina representa una malformación infrecuente cuyas principales causas son el síndrome de Rokitansky-Mayer-Kuster-Hauser y la insensibilidad a los andrógenos o síndrome de feminización testicular. Su tratamiento se enfoca a la restauración de la función sexual y la mayoría de técnicas quirúrgicas descritas se caracterizan por su complejidad y requerimiento de instrumentos especiales. El objetivo de este documento es la presentación de una técnica simplificada de neovagina. Metodología: reporte de 4 pacientes con agenesia de vagina a las que se les realizó una neovagina a través de una modificación de la técnica de McIndoe mediante la utilización de compresa adsorbente de bacterias, con seguimiento de 8 a 18 meses. Resultados: se intervinieron tres pacientes con síndrome Rokitansky-Mayer-Kuster-Hauser y la cuarta con síndrome de feminización testicular y cariotipo XY. En uno de los casos se evidenció ruptura de recto distal al inicio de la cirugía, complicación que se identificó y corrigió de manera primaria con adecuada evolución. El tiempo de hospitalización varió de 2 a 3 días. No hubo evidencia de complicaciones a mediano y largo plazo, y se documentó el inicio de relaciones sexuales alrededor de 40 días posteriores a la intervención. Conclusión: la técnica quirúrgica descrita surge como una opción que hace el procedimiento más sencillo para restaurar la función sexual de mujeres con esta afección.
Introduction: vaginal agenesis represents a rare malformation, mostly associated with Mayer-Rokitansky-Kuster-Hauser syndrome and androgen insensitivity syndrome or testicular feminisation syndrome. Treatment focuses on restoring sexual function and most surgical techniques are characterised by their complexity and require special instruments. This paper was aimed at presenting a simplified neovagina technique. Methodology: four cases of patients suffering vaginal agenesis are reported; they underwent a modification of the McIndoe technique by using absorbent antimicrobial barrier dressings which simplifies the procedure and reduces implementation costs. They were followed-up for 8-18 months and suitable results were obtained. Results: three patients suffering from MRKH syndrome were treated; the fourth one had testicular feminisation syndrome (XY karyotype). A rupture of the distal colon or rectum was seen at the start of surgery in one case; such complication was identified and corrected, presenting suitable evolution. Hospital stay varied from 2-3 days. No medium or long-term complications were presented. Sexual relationships were documented as having begun around 40 days later. Conclusion: the surgical technique described here represents an option for restoring sexual function in women suffering from this condition.
Assuntos
Humanos , Adulto , Feminino , Anormalidades Congênitas , Doenças VaginaisRESUMO
A study about the herpetological legacy of the German naturalist Andreas Mayer (1907-1986) (Bérnils & Moura-Leite, 1990) raised 83 specimens from 20 reptile species captured in the Brazilian states of Paraná and Mato Grosso do Sul (years 40, 50, and 60) and currently housed at the Museu de História Natural Capão da Imbuia (Curitiba). Just in Mayer's birth centenary, 15 more snakes collected by him and not presented in the former publication were founded. This material is relevant because (1) came from areas currently under human-altered environments; (2) was collected in areas that were poorly sampled at that time; and (3) embraced some species hard to find in Paraná, including the first occurrence of Phimophis cf. guerini for the state. Label data for the added snakes and some important corrections to the former article, with taxonomic and geographic updates, are being presented.
Um estudo sobre o legado herpetológico do naturalista alemão Andreas Mayer (1907-1986) para o estado do Paraná (Bérnils & Moura-Leite, 1990) levantou 83 exemplares de 20 espécies de répteis, obtidos por ele no Paraná e no Mato Grosso do Sul (anos 40, 50 e 60) e tombados no atual Museu de História Natural Capão da Imbuia (Curitiba). Acreditava-se serem os únicos répteis coletados por Mayer e guardados em coleção científica, mas justamente no centenário de seu nascimento foram encontradas 15 serpentes coletadas por ele e não listadas na publicação anterior. O material é relevante, pois procede de áreas que atualmente se encontram ambientalmente degradadas, foi obtido numa época com raros registros de répteis para o Paraná, e contém espécies de difícil captura no Estado, entre as quais se destaca a primeira ocorrência de Phimophis cf. guerini para o Paraná. As 15 serpentes são arroladas com seus dados de etiqueta e tombo, e são corrigidas informações incompletas ou equivocadas no artigo de 1990, com a devida atualização taxonômica.