Estimulación ovárica en síndrome de Mayer-Rokitansky: reporte de caso y revisión de la literatura / Controlled ovarian stimulation in Mayer-Rokitansky syndrome: case report and literature review

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.

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.

Mayer-Rokitansky-Kuster-Hauser syndrome – An unusual case of primary amenorrhea

The onset of menstruation is a significant milestone of sexual maturation in a girl child. Although there are numerous causes of primary amenorrhea, Mayer-Rokitansky-Küster-Hauser syndrome, also known as Müllerian aplasia, is one of the extremely rare causes of primary amenorrhea which is usually picked up by suitable imaging techniques like magnetic resonance imaging. Affected females have a normal female hormonal function and a normal karyotype but non-functional vagina and uterus which make it an interesting entity. Although specific treatment is not known for this entity except for vaginoplasty and creation of a neovagina for sexual gratification, the clinical and diagnostic workup of these patients has evolved in recent years and infertility treatment and child-bearing through in vitrofertilization have become a part of the long-term treatment plan for these females.

Mayer Rokitansky Kuster Hauser Syndrome with Concomitant Turner Syndrome Presented with Primary Amenorrhea in an 18 Year Old Woman: A Case Report

@#<p>Primary amenorrhea is a symptom caused by different rare pathologic conditions. It is commonly seen during adolescence due to the absence of menses during this period. Presented here is a rare case of primary amenorrhea in an 18 year old girl with delayed pubertal growth and short stature which on series of investigations revealed hypergonadotropic hypogonadism, absence of the uterus and non-visualized bilateral ovaries on MRI. Karyotyping showed 45,X0. The coexistence of MRKH and gonadal dysgenesis was considered in this case and has been reported in only a few studies up to this date. Its association is uncommon, hence, a multidisciplinary approach is warranted for the management of her case. Further implications on menses and future fertility options are the main considerations, affecting the quality of life.</p>

Rare case: enucleation of multiple fibroids after intraoperative vaginal sonographic detection of the solitary pelvic kidney by patient with Mayer-Rokitansky-Kuster-Hauser syndrome

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is a congenital anomaly of the genital tract that occurs in about 1 in 4000 women. MRKH syndrome can be associated with renal, skeletal, heart and hearing abnormalities. The frequency of renal/urinary tract abnormalities is 33%. Only a few cases of fibroid development in MRKH syndrome have been described in the literature. The diagnosis and surgery of a fibroid in MRKH syndrome may be complicated in associated kidney abnormality by an atypical kidney position, as in this case: pelvic kidney on one side and renal agenesia on the contralateral side. Authors present the case of a 47-year-old female patient with a known MRKH syndrome and a pelvic kidney on the right side who had presented with an unclear tumour in the right lower abdomen. A completed CT scan revealed the tumour directly next to the pelvic kidney. A malignancy could not be excluded with certainty, so that a laparoscopy in laparotomy readiness was indicated and performed. During surgery, two rudimentary uterine horns were found; on the right side retroperitoneally, below the uterine horn, the tumour was located and directly below it there was a soft tissue alteration, probably the kidney. For safety, a vaginal sonography was performed in between, to clearly identify the only kidney and to avoid damaging it. The tumour could be extirpated laparoscopically without kidney injury. The two uterine horns were removed simultaneously. Histologically the fibroid could be confirmed. In addition, three other fibroids (one on the left side and two on the right side) were detected. Due to the high probability of a simultaneous kidney abnormality in the MRKH syndrome, authors suggest an accurate kidney diagnosis preoperatively. If necessary, in the case of a pelvic kidney and/or renal agenesia, as in this case, an additional intraoperative kidney check should be performed.

Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is an unknown congenital etiology disorder characterized by agenesia or hypoplasia of the Müller ductal system, including the upper vagina, uterus and fallopian tubes. The occurrence of an associated ovarian tumor is rare, with fewer than 20 cases reported to date according to the literature. We report the case of a 14-year-old girl, virgin, who had not yet seen her menarche, complaining of an abdomino-pelvic mass associated with pain. The ultrasound performed revealed a large left ovarian tumor and an absence of uterus. The indication of a laparotomy confirmed the ovarian mass and a complete absence of uterus associated with vaginal hypoplasia. The contralateral ovary was present, and of normal appearance. The pathological examination was in favor of a mature benign multi-tissular teratoma. This is the first case described in our service. The mode of transmission of this entity appears to be autosomal dominant with low penetrance and variable expressivity, suggesting that the incidence of this syndrome is likely underestimated. With the development of techniques of medical assistance to procreation, maternity remains possible, particularly through gestational surrogacy.

Study and clinical evaluation of 25 cases of primary amenorrhea

Background: Primary amenorrhea is absence of menstruation and secondary sexual characters by age of 14 years primary amenorrhea is < 1%. Development of female genital organs takes place from mullerian DUCT (paramesonephric duct). The objective of this study was to note the various causes, complete clinical picture and the management in 25 such cases of primary amenorrhea.Methods: This is a prospective study done in 25 cases. They were investigated, managed and patients were called up for follow up for their response to treatment.Results: Out of 25 cases studied, maximum cases presented at 14-16 years of age, with chief complaint of primary amenorrhea, out of which 8% were married, 44% cases were of MRKH syndrome which was the most common cause of primary amenorrhea.Conclusions: Amenorrhea has got multi factorial etiology. For patients with amenorrhea physical examination should focus on pubertal development and possible genital out flow obstruction.

Síndrome Mayer-Rokitansky-Küster-Hauser con prolapso de órganos pélvicos: reporte de un caso / Mayer-Rokitansky-Küster-Hauser syndrome with pelvic organ prolapse: Case report

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.

Prospective study of primary amenorrhoea and its management in a rural tertiary centre

Background: Amenorrhoea (absence of menstruation) is a symptom of varied causes. It results from dysfunction of hypothalamic-pituitary ovarian axis, uterus and vagina. It is a major concern for pubertal girls and their family members. It has a major impact on the physical, mental, psychological and social life of the girl and her family. The objective of the present study was to evaluate the aetiology and management of primary amenorrhoea in young adolescent girls.Methods: It was a prospective study conducted for a period of 2 years from August 2016 to July 2018 at Rajarajeswari medical college and hospital. Patients presenting with history of amenorrhoea that is, absence of menses by the age of 13 years with no visible development of secondary sexual characteristics or by 15 years of age with the presence of normal secondary sexual characteristics were included in our study. Cases of secondary amenorrhoea were excluded. Detailed history, examination, investigations and management was documented and analysed.Results: A total of 25 patients of primary amenorrhea were studied during the study period. In our study outflow tract anomalies were the commonest cause of amenorrhoea accounting for 84%, of which imperforate hymen (32%) and Mayer Rokitansky Küster Hauser syndrome (MRKH) 36% were the two most common Mullerian anomaly causing primary amenorrhoea. Gonadal dysgenesis accounted for 12% of the cases. Amenorrhoea was the commonest complaint patients presented with accounting to 76%, followed by cyclical pain abdomen accounting for 16% of cases.Conclusions: Primary amenorrhoea is multifactorial and is of major concern among adolescent girls. Early diagnosis and intervention has an impact on the physical and psychological wellbeing of the girl.

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.

Long-term follow-up on MURCS (Müllerian duct, renal, cervical somite dysplasia) association and a review of the literature

Müllerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) association is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. The majority of MURCS patients are diagnosed with primary amenorrhea from late-adolescence. However, a few cases with MURCS association are not well diagnosed during childhood and long-term outcomes are not well reported. We report a case of an 8-year-old girl with MURCS association who presented with recurrent urinary tract infections and multiple congenital malformations, and who was followed for 10 years until adulthood. MURCS association should be considered as one of the differential diagnoses when evaluating prepubertal females with vertebral and renal malformations.

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.

Clinical value of MRI in patients with MRKH syndrome / 实用放射学杂志

Objective To evaluate the accuracy of preoperative MRI in the diagnosis of malformations associated with MRKH syndrome and identification of uterine endometrium to optimize the clinical management.Methods 1 8 females with primary amenor-rhea were studied with MRI performed with a 1.5T or 3.0T imager.All patients were examined in the supine position using a phased-array coil and underwent pelvic MRI.Two experienced radiologists evaluated all the examinations in consensus to assess the pres-ence,position and morphology of vagina,uterus,ovaries and any pelvic abnormalities.Results One or two rudimentary uteri were identified in 17 patients (94.4%).A total of 16 patients (88.9%)had bilateral rudimentary uteri,1 (5.6%)had unilateral rudimentary uteri(it was left sided).1 (5.6%)had no uterine remnants.4 (12.1%)of 33 uterine buds in 4 patients showed differentiation of the center of the uterus into three layer.Bilateral ovaries were present in 1 7 patients,and their shapes,sizes and signals are normal.The vagina and cervix were absent in all cases.Conclusion MRI is a useful diagnostic tool in the preoperative evaluation of MRKH syndrome and the endometrium to further optimize the treatment plan.

Mayer-Rokitansky-Küster-Hauser Syndrome with Imperforate Anus: A Rare Association and an Innovative Surgical Management.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is one of the rare disorder of Mullerian agenesis leading to non-development of uterus and vagina. Its association with anorectal malformation is rare. We report a case of MRKH syndrome with recto-vestibular in a female child. The child had undergone a sigmoid loop colostomy in the neonatal period. On clinical examination of the perineum, a fistula was present in the vestibule just below the urethral opening, but no vaginal canal was found. It was managed with an innovative surgical technique, preserving the fistula and lower rectum to function as vagina.

Perioperative nursing for a patient with mayer rokitansky kuster hauser combined with rectovestibullar fistula / 中国实用护理杂志

Objective To investigate the perioperative nursing experience for patients with mayer rokitansky kuster hauser (MRKH).Methods We conducted multidisciplinary care to a MRKH patient,including personalize psychological support,bowel preparation,two surgical nursing convergence and vaginal mold care during hospitalization.Results The patient discharged after clinical symptoms remission,the patient sexual life was satisfaction,no complications were found in the six months follow-up.Conclusion The key of operation success is good coordination and cooperation of multiple departments,close observation and effective nursing in the perioperation period.

Manejo laparoscópico de miomatosis uterina en pacientes con Síndrome de Rokitansky: Reporte de un caso y revisión de la literatura / Laparoscopic management of uterine myomatosis in a patient with Rokitansky syndrome: Case report and review of the literature

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.

Mayer-Rokitansky-Kuster-Hauser syndrome type II: A rare case.

Mayer-Rokitansky-Kuster-Hauser (MRKH) is a malformation complex comprising absent vagina and absent or rudimentary uterus. MRKH syndrome may be attributed to an initial affection of the intermediate mesoderm consequently leading (by the end of the 4th week of fetal life) to an alteration of the blastema of the cervicothoracicsomites and the pronephricducts. These latter subsequently induce the differentiation of the mesonephric and then the Wolffian and Mullerian ducts. There are very sparse such cases reported. We present a case of type II MRKH or Mullerian renal cervical somite association (i.e., Mullerian duct aplasia, renal dysplasia, and cervical somite anomalies).

Uterine Adenomyosis Which Developed from Hypoplastic Uterus in Postmenopausal Woman with Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is characterized by vaginal agenesis with variable Mullerian duct abnormalities. We report here a case of uterine adenomyosis which developed from a hypoplastic uterus in a patient with MRKHS. A 55-year-old postmenopausal woman visited a university hospital for pelvic mass. She had underwent vaginoplasty via the McIndoe procedure for MRKHS at 15 years of age. Pelvic magnetic resonance imaging showed a 5.4 x 4.8 x 4.7 cm mass suspicious for a uterine myoma. She received total abdominal hysterectomy with bilateral salpingo-oophorectomy, and neither the cervix nor endometrium was found grossly in the surgical specimen. The final histologic diagnosis was uterine adenomyosis.

Magnetic Resonance Evaluation of Mullerian Remnants in Mayer-Rokitansky-Kuster-Hauser Syndrome

OBJECTIVE: To analyze magnetic resonance imaging (MRI) findings of Mullerian remnants in young females clinically suspected of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome in a primary amenorrhea workup. MATERIALS AND METHODS: Fifteen young females underwent multiplanar T2- and transverse T1-weighted MRI at either a 1.5T or 3.0T MR imager. Two gynecologic radiologists reached consensus decisions for the evaluation of Mullerian remnants, vagina, ovaries, and associated findings. RESULTS: All cases had bilateral uterine buds in the pelvic cavity, with unilateral cavitation in two cases. The buds had an average long-axis diameter of 2.64 +/- 0.65 cm. In all cases, bilateral buds were connected with fibrous band-like structures. In 13 cases, the band-like structures converged at the midline or a paramedian triangular soft tissue lying above the bladder dome. The lower one-third of the vagina was identified in 14 cases. Fourteen cases showed bilateral normal ovaries near the uterine buds. One unilateral pelvic kidney, one unilateral renal agenesis, one mild scoliosis, and three lumbar sacralization cases were found as associated findings. CONCLUSION: Typical Mullerian remnants in MRKH syndrome consist of bilateral uterine buds connected by the fibrous band-like structures, which converge at the midline triangular soft tissue lying above the bladder dome.