Síndrome de Herlyn-Werner Wünderlich: reporte de dos casos / Herlyn-Werner-Wünderlich syndrome: Two case report

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

Rol de la laparoscopia en el manejo del síndrome OHVIRA: reporte de un caso y su relación con la endometriosis / Role of laparoscopy in the management of OHVIRA syndrome: a case report and its relation to endometriosis

El síndrome de hemivagina obstruida y anomalía renal ipsilateral (OHVIRA) es producido por una alteración en el desarrollo de los conductos de Müller y Wolff en la vida fetal. El síndrome es poco frecuente, se reporta una prevalencia de 1/2.000 a 1/28.000 casos. La endometriosis se presenta en un 19% de los casos complicando esta patología. El tratamiento del síndrome OHVIRA consiste en resecar el tabique vaginal drenando el hematocolpos. Hasta el momento no existe un consenso en recomendar la realización de una laparoscopia diagnóstica. El objetivo de este estudio es reportar la eventual importancia de la laparoscopia diagnóstica/terapéutica como parte del manejo del síndrome OHVIRA.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is caused by a defect in the development of Müllerian and Wolffian ducts at fetal life. The syndrome is uncommon, with a reported prevalence of 1/2,000 to 1/28,000 cases. Endometriosis is present in 19% of cases complicating this pathology. Treatment of OHVIRA syndrome consists in resecting the vaginal septum and draining the hematocolpos. Until now there isnt an agreement on recommending diagnostic laparoscopy as part of the treatment. The aim of this study is to report the importance of diagnostic/therapeutic laparoscopy in the management of OHVIRA syndrome.

Variante incomum da síndrome de Herlyn- -Werner-Wunderlich: relato de caso / Unsual Herlyn-Werner-Wunderlich syndrome: case report

Malformações müllerianas correspondem a variações anatômicas do trato repro- dutor feminino. Comumente assintomáticas, o diagnóstico e a verdadeira incidên- cia são difíceis de determinar. A síndrome de Herlyn-Werner-Wunderlich, clas- sicamente descrita pela tríade útero didelfo, hemivagina cega e agenesia renal ipsilateral também pode ter variações diferentes. Em virtude da mesma origem embrionária dos tratos genital e urinário, anomalias renais devem ser investigadas nesses casos, sendo a mais comum a agenesia renal. Este artigo relata o caso de uma paciente de 18 anos, do sexo feminino, com história de piocolpo por cinco anos. Em propedêutica complementar, foi identificado útero com septação com- pleta associado a hemissepto de terço superior de vagina, formação de piocolpo e agenesia renal à direita. Apesar de não ser a definição clássica, o quadro está incluído nos casos de síndrome de Herlyn-Werner-Wunderlich.

Mullerian malformations correspond to anatomical variations of the female repro- ductive tract. Commonly asymptomatic, the diagnosis and true incidence are difficult to determine. The Herlyn-Werner-Wunderlich syndrome, classically described by the triad: uterus didelphus, blind hemivagina and ipsilateral renal agenesis, can also have different variations. Due to the same embryonic origin of the genital and urinary tracts, renal anomalies must be investigated in these cases, the most common being renal agenesis. This article reports the case of an 18-year-old female patient with a 5-year history of pyocolpus. In complementary exams, a uterus with complete septa- tion was identified, associated with a hemiseptum in the upper third of the vagina, formation of pyocolpus and renal agenesis on the right side. Despite not being the classic definition, it is included in the cases of Herlyn-Werner-Wunderlich syndrome.

Malformaciones müllerianas: actualización y revisión a propósito de casos clínicos, 2022 / Müllerian malformations: update and review of clinical cases, 2022

Las malformaciones müllerianas (MM) son un grupo de anomalías estructurales originadas por fallas de desarrollo de los conductos paramesonéfricos o de Müller durante las primeras 16 semanas de gestación. Un oportuno diagnóstico y una correcta clasificación permiten ofrecer el mejor manejo y diferenciar aquellas pacientes que requieren tratamiento quirúrgico. Se realizó una revisión de la literatura sobre MM en las bases de datos Epistemonikos, SciELO, Cochrane y PubMed. Se rescataron todas las pacientes ingresadas con diagnóstico de MM. En el año 2021, la American Society of Reproductive Medicine publicó un consenso en el que se estandarizó la nomenclatura, se amplió el espectro y se simplificó la clasificación. La clínica es variada, e incluye pacientes asintomáticas cuyo diagnóstico es un hallazgo por imágenes. Los mejores estudios imagenológicos son la resonancia magnética (RM) y la ultrasonografía 3D, dejando la histeroscopia y la laparoscopia (método de referencia) como procedimiento diagnóstico-terapéutico. Se presentan casos clínicos desarrollados durante el primer trimestre de 2022. Recomendamos la utilización sistemática de la RM para el diagnóstico de anomalías complejas u obstructivas del aparato genital. El tratamiento de estas patologías debe ser realizado por ginecólogos endoscopistas expertos, e incluye tratamiento médico y quirúrgico, el cual debe ser enfocado en cada paciente dependiendo del tipo de MM y de los deseos de fertilidad.

Müllerian malformations (MM) are a group of structural anomalies caused by developmental failure of the paramesonephric or Müllerian ducts during the first 16 weeks of gestation. Timely diagnosis and classification allow us to offer the best management and to differentiate those patients who require surgical treatment. Literature review on MM in Epistemonikos, SciELO, Cochrane and PubMed databases. All patients admitted with a diagnosis of MM were rescued. In 2021, the American Society of Reproductive Medicine publishes a consensus where it standardizes the nomenclature, broadens the spectrum, and simplifies the classification. The clinical picture is varied and includes asymptomatic patients whose diagnosis is an imaging finding. The best imaging studies are magnetic resonance imaging (MRI) and 3D ultrasonography, leaving hysteroscopy and laparoscopy (gold standard) as diagnostic therapeutic. Clinical cases developed during the first trimester 2022 are presented. We recommend the routine use of MRI for the diagnosis of complex and/or obstructive anomalies of the genital tract. The treatment of these pathologies should be performed by expert endoscopic gynecologists and include medical and surgical treatment, which should be focused on each patient, depending on the type of MM and fertility desires.

Riñón pélvico fusionado con uréter único, hidrometrocolpos y ano imperforado: conjunción de malformaciones infrecuentes en una adolescente / Pancake kidney with a single ureter, hydrometrocolpos, and imperforate anus: a set of uncommon malformations in a female adolescent

Una niña de 11 años de edad con antecedentes de ano imperforado, infección urinaria y episodios de constipación intermitentes se presentó a la consulta con cólicos abdominales y náuseas de una semana de evolución. Estudios radiológicos revelaron hidrometrocolpos y fusión renal pélvica con uréter único hidronefrótico. El examen vaginal evidenció un tabique transverso no permeable. Se evacuó temporalmente la colección con resolución de los síntomas. La paciente fue programada para cirugía vaginal reconstructiva definitiva. Se destaca en este caso no solo la asociación de malformaciones infrecuentes, sino una sintomatología muy común en la práctica pediátrica a causa de una patología rara vez considerada en el diagnóstico diferencial, y la importancia de una evaluación precoz y completa de este tipo de malformaciones para un tratamiento oportuno.

An 11-year-old girl with a history of imperforate anus, urinary tract infection, and intermittent episodes of constipation presented with abdominal pain and nausea for 1 week. The x-rays revealed hydrometrocolpos and fused pelvic kidney with a single hydronephrotic ureter. The vaginal examination revealed a non-permeable transverse vaginal septum. The collection was temporarily drained and symptoms resolved. The patient was scheduled for definitive vaginal reconstructive surgery. In this case, it is worth noting the association of infrequent malformations and also the signs and symptoms very common in pediatric practice due to a pathology rarely considered in the differential diagnosis, and the importance of an early and complete assessment of this type of malformations for a timely treatment.

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 asociado con malformación anorectal / Mayer-Rokitansky-Küster-Hauser syndrome associated with anorectal malformation

La agenesia vaginal es una entidad poco frecuente que puede presentarse en forma aislada o acompañada de otras anomalías congénitas. El desconocimiento de esta asociación puede retardar su diagnóstico, causar complicaciones indeseadas y comprometer el resultado del tratamiento. Se describen tres casos clínicos con agenesia vaginal y malformación anorrectal, cuyos diagnósticos no fueron realizados en el momento del nacimiento. Aunque la coexistencia de anomalías vaginales y anorectales sean poco frecuentes, los médicos responsables de su tratamiento deben ser conscientes de su existencia y practicar examen perineal cuidadoso.

Vaginal agenesis is a rare condition. It may exist in isolation or be associated with other congenital anomalies. A lack of awareness on this association may delay diagnosis, cause undesirable complications and compromise treatment outcomes. We describe three cases of vaginal agenesis associated with anorectal malformation which were not diagnosed at birth. Although the coexistence of vaginal and anorectal anomalies is uncommon, treating physicians should be aware of them and perform a detailed perineal examination.

Management of transverse vaginal septum by vaginoscopic resection: hymen conservative technique / Manejo do septo vaginal transverso por ressecção vaginoscópica: técnica conservadora do hímen

Abstract Transverse vaginal septum is a rare female genital tract anomaly, and little is described about its surgical treatment. We report the case of a patient who wished to preserve hymenal integrity due to social and cultural beliefs. We performed a vaginoscopic resection of the septum under laparoscopic view, followed by the introduction of a Foley catheter in the vagina, thus preserving the hymen. After 12 months of follow-up, no septal closure was present, and the menstrual flow was effective. Vaginoscopic hysteroscopy is an effectivemethod of vaginal septum resection, even in cases in which hymenal integrity must be preserved due to social and cultural beliefs.

Resumo Septo vaginal transverso é uma anomalia rara do trato genital feminino, e pouco é descrito sobre o tratamento cirúrgico. Relatamos o caso de uma paciente que desejava preservar a integridade do hímen devido a crenças sociais e culturais. Realizamos ressecção vaginoscópica do septo sob visão laparoscópica, seguida da introdução de um cateter de Foley na vagina, preservando assim o hímen. Após 12 meses de acompanhamento, não havia fechamento do septo, e o fluxo menstrual era eficaz. A histeroscopia vaginoscópica é um método eficaz de ressecção dos septos vaginais, incluindo os casosemque a integridade do hímen deve ser mantida devido a crenças sociais e culturais.

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.

Doença do nó sinusal em paciente jovem com síndrome de Mayer-Rokitansky-Küster-Hauser: coincidência ou comorbidade associada? / Sick sinus syndrome in young woman with Mayer-Rokitansky-Küster-Hauser syndrome: coincidence or associated comorbidity?

Relato do caso de uma paciente de 39 anos de idade com síndrome de Mayer-Rokitansky-Küster-Hauser, apresentando doença do nó sinusal e necessidade de implante de marcapasso bicameral. Por se tratar de paciente jovem, sem outras causas secundárias de bradicardia, sugerese a hipótese de relação entre as duas doenças, visto que já há descrição de associação de síndrome de Mayer-Rokitansky-Küster-Hauser e cardiopatias estruturais, sem referências de relação com bradicardia até o momento

Case report of a 39-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome and sick sinus syndrome requiring a definitive pacemaker. As a young patient, without secondary causes of bradycardia, we suggest the possibility of a relationship between these two diseases, since there are reports of the association of Mayer-Rokitansky-Küster-Hauser syndrome and structural heart diseases, and no reports of a relationship with bradycardia until now

Síndrome de Hemivagina Obstruida, Útero Didelfo y Agenesia Renal Ipsilateral (OVHIRA), Drenado Parcialmente por Fístula Útero-Uterina Espontánea / Obstructed Hemivagina Syndrome, Uterus didelphys and Ipsilateral Renal Agenesis (OVHIRA), Partially Drained by Spontaneous Uterine-Uterine Fistula

RESUMEN Antecedentes: El síndrome de hemivagina obstruida, útero didlefo y anomalía renal ipsilateral (OVHIRA) es una malformación mülleriana infrecuente. Objetivo: Se presenta un caso con una complicación no antes descrita y se hace una revisión de la literatura hasta la fecha. Caso clínico: Mujer de 12 años de edad con antecedentes de diagnóstico de útero bicorne y agenesia renal derecha en la infancia. Veintidós meses post menarquia consulta por algia pelviana y dismenorrea premenstrual. Ecografía revela hematómetra en cuerpo uterino izquierdo, de 142 x 69 x 61 mm. Resonancia nuclear magnética (RNM) describe además un hematocolpos de 7 x 10 cm y un tabique vaginal transverso de hemivagina izquierda. Se diagnostica OHVIRA y se planifica cirugía para resecar el tabique. Días antes del la cirugía, la paciente tiene episodio de metrorragia. Ecográficamente se constata una disminución considerable del volumen de hematómetra. En la cirugía se pesquisa fístula uterina que comunica útero obstruido izquierdo con útero no obstruido derecho por donde había drenado espontáneamente la hematómetra. Al resecar el tabique vaginal izquierdo se termina de drenar hematómetra y hematocolpos. Al alta, paciente se maneja con dilatación vaginal progresiva por seis meses para evitar la estenosis del tabique. Conclusión: Se presenta una complicación no descrita, fístula útero uterina, de una malformación mülleriana infrecuente (OVHIRA). El proceso diagnóstico meticuloso, la cirugía bien planificada y la dilatación vaginal prolongada permitieron tener éxito en su manejo.

ABSTRACT Background: Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome (OHVIRA) is an uncommon Müllerian anomaly. Objective: A case is described and the main complications related to the syndrome are reviewed. Case report: Female, 12 years old, with a medical history of a bicornuate uterus and right renal agenesis diagnosed at birth. Twenty-two months post menarche she seeks medical attention due to pelvic pain, menometrorrhagia and premenstrual dysmenorrhea. An ultrasound revealed a hematometra, of 142 x 69 x 61 mm, in the left uterus. Magnetic resonance imaging also described a 7 x 10 cm hematocolpos and a transverse vaginal septum of the left hemivagina. OHVIRA is diagnosed and surgery is planned to resect the septum. Two days before the surgery, the patient has an episode of abundant metrorrhagia. Ultrasonographically a considerable decrease in the volume of the hematometra is observed. In surgery, a uterine fistula is discovered which communicates the left obstructed uterus with the right unobstructed uterus, where the hematometra had been partially drained. During the procedure, the left vaginal septum is resected, which completes the drainage of the hematometra and hematocolpos. At discharge, the patient undergoes progressive vaginal dilation for six months to avoid stenosis of the septum. Conclusion: An utero-uterine fistula has not been previously described as a complication of OHVIRA. The meticulous diagnostic process, the well planned surgery and the prolonged vaginal dilation allowed for a successful management.

Tabique vaginal transverso diagnosticado en dos hermanas: Reporte de dos casos / Transverse vaginal septum diagnosed in sisters: Cases report

El tabique vaginal transverso es una anomalía que se originaría por un fracaso en la fusión y/o canalización del seno urogenital y de los conductos müllerianos con baja frecuencia, pero con un importante impacto en la salud de las pacientes que lo presentan. El tratamiento es quirúrgico y la vía de abordaje depende de la localización y del espesor del tabique. En ocasiones, se ha relacionado con una herencia que podría ser recesiva asociada al sexo, pero se han documentado pocos casos de asociación familiar. Se exponen los casos de dos hermanas que presentaron esta rara entidad con 5 años de diferencia y que se diagnosticaron por la aparición de dolor abdominal hipogástrico intenso a la edad de 11 y 12 años, cuando presentaban un desarrollo puberal estadio III de Tanner y amenorrea primaria.

Transverse vaginal septum is a disorder that would arise from a failure in the fusion and/or channeling of the urogenital sinus and the müllerian ducts with low frequency but with a significant impact on the health of patients. The treatment is surgical and it will depend on the location and thickness of the septum. Sometimes, it described a female sex-limited autosomal recessive transmission but few cases of family association have been reported. We present the cases of two sisters who had this rare entity 5 years apart and were diagnosed by the presentation of an intense hypogastric abdominal pain at the age of 11 and 12 years when they had a Tanner stage III pubertal development and primary amenorrhea.

Pyometra and Pregnancy with Herlyn-Werner-Wunderlich Syndrome / Piometria e gravidez com síndrome de Herlyn-Werner-Wunderlich

ABSTRACT We describe a Herlyn-Werner-Wunderlich syndrome (HWWS) patient with previous history of infertility who got pregnant without treatment and presented a pyometra in the contralateral uterus throughout the gestational period, despite multiple antibiotic treatments. Due to the uterus' congenital anomaly and the possibility of ascending infection with subsequent abortion, this pregnancy was classified as high-risk. We believe that the partial horizontal septum in the vagina may have contributed to the closure of the gravid uterus cervix, thus ensuring that the pregnancy came to term, with an uneventful vaginal delivery.

RESUMO Os autores descrevem uma paciente com síndrome de Herlyn-Werner-Wunderlich (SHWW) e história prévia de infertilidade, que engravidou espontaneamente. Durante todo o período gestacional apresentou, apesar da instituição de antibioticoterapia, um piometra localizado ao útero não gravídico. Devido à anomalia congênita uterina e ao risco de infeção ascendente, com possível desfecho obstétrico desfavorável, esta gravidez foi classificada de alto risco. O septo vaginal horizontal e parcial poderá ter contribuído para ausência de disseminação da infecção, permitindo que a gravidez tenha chegado a termo, com um parto vaginal, sem intercorrências.

Síndrome de Herlyn-Werner-Wunderlich. Síndrome OHVIRA. Reporte de Caso / Herlyn-Werner-Wunderlich Syndrome or OHVIRA Syndrome. Case Report

Paciente Femenina de 11 años con el síndrome de Herlyn-Werner-Wunderlich, manejado multdisciplinariamente y resuelto endoscópicamente con una septotomía y dilataciones vaginales.

This case is about a female patent, 11 years old, with Herlyn-Werner-Wunderlich Syndrome, who received multdisciplinary approach and was endoscopically resolved with septotomy and vaginal dilatatons.

Creation of a Neovagina by Laparoscopic Modified Vecchietti Technique: Anatomic and Functional Results / Criação de neovagina pela técnica de Vecchietti laparoscópica modificada: resultados anatómicos e funcionais

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.

Anomalias do desenvolvimento sexual / Abnormalities of sexual development

As anormalidades da diferenciação sexual são infrequentes na prática clínica. A caracterização de uma ampla variedade de síndromes tem sido muitas vezes confusa, necessitando, com relativa frequência, a consulta de múltiplos livros e uso constante de referências para uma correta compreensão. O presente artigo tem a proposta de revisar as entidades mais frequentes, seus métodos diagnósticos e sua conveniente orientação.(AU)

Abnormal sexual differentiation is not frequently seen in an individual clinician's practice. The categories of many syndromes in this area require special and constant references to review many papers and books to understand these abnormalities. In this paper, the most frequent syndromes are described, and their diagnostic methods and proposals for correct orientation are provided.(AU)

Herlyn-Werner-Wunderlich syndrome: a case report / Síndrome de Herlyn-Werner-Wunderlich: relato de caso

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disorder of the Müllerian ducts in which there is uterus didelphys, obstructed hemivagina and unilateral renal agenesis. The most common presentation is an abdominal mass secondary to hematocolpos, pain and dysmenorrhea. However, in some cases, such as the one we present here, menses are normal due to an obstructed hemivagina, and diagnosis can be delayed. We describe evaluation and surgical management of a 13-year-old girl with this condition who was diagnosed by computed tomography (CT) scan and confirmed by pelvic ultrasound and surgical exploration, as well as a review of the literature.

A síndrome de Herlyn-Werner-Wunderlich (HWW) é uma doença congênita rara dos dutos müllerianos, em que há útero didelfo, hemivagina obstruída e agenesia renal unilateral. A apresentação clínica mais comum é como uma massa abdominal secundária a hematocolpo, dor e dismenorreia. Em alguns casos, porém, como no que apresentamos aqui, a menstruação é normal devido à obstrução de uma hemivagina, e o diagnóstico pode ser tardio. Descrevemos neste trabalho a avaliação e manejo cirúrgico de uma paciente de 13 anos com essa condição que foi diagnosticada com uso de tomografia computadorizada e confirmada por ultrassonografia pélvica e exploração cirúrgica, bem como uma revisão da literatura.

Hallazgos en resonancia magnética de las malformaciones uterovaginales: datos imprescindibles previos a una intervención quirúrgica / Magnetic resonance imaging findings of uterovaginal malformations: essential data prior to surgical intervention

La caracterización de las malformaciones congénitas del aparato genital femenino ha adquirido gran relevancia, principalmente en mujeres jóvenes con problemas de fertilidad. El origen de estas malformaciones procede de una alteración en el desarrollo embriológico de los conductos de Müller. El manejo terapéutico de estas entidades dependerá tanto del tipo de anomalía como de su gravedad. En la actualidad los múltiples avances tecnológicos en imagen permiten un diagnóstico preciso de estas malformaciones así como una posible planificación quirúrgica. Este trabajo tiene como objetivo revisar la clasificación de las malformaciones müllerianas así como estudiar los hallazgos en resonancia magnética que el cirujano debe conocer previo a la cirugía.

Morphologic characterization of congenital malformations of the female genital tract has acquired great importance, especially in the evaluation of female infertility.The origin of these malformations comes from an alteration in the embryological development of the Müllerian ducts. Technological advances in the field of radiology allow the diagnosis of these malformations, its possible treatment and surgical planning. This paper aims to review the classification of Müllerian anomalies and study the magnetic resonance findings that the surgeon should know prior to possible surgery.