Embarazo gemelar espontáneo en útero bicorpóreo: una contribución a la experiencia / Spontaneous twin pregnancy in a bicorporeal uterus, a contribution to the experience

Resumen ANTECEDENTES: Las anomalías uterinas congénitas son consecuencia de la formación anormal, fusión o reabsorción de los conductos de Müller durante el desarrollo embriológico. El útero bicorne, unicorne y didelfo (U1, U2, U3 ESHRE-ESGE) son defectos en la unificación con una baja prevalencia en la población general y en la que tiene antecedente de aborto e infertilidad; se estima en alrededor de 0.4 a 1.1%, respectivamente. La presentación del caso puede aportar información útil relacionada con la evolución del embarazo y los desenlaces perinatales cuando aparece esta anomalía. CASO CLÍNICO: Paciente de 35 años, con amenorrea secundaria y prueba inmunológica de embarazo positiva, con antecedentes de dismenorrea primaria severa y anomalía uterina congénita compatible con útero bicorpóreo (U3 completo ESHRE-ESGE), bicorne completo (4a-AFS) y didelfo. El embarazo trascurrió sin complicaciones hasta la semana 37, cuando finalizó mediante cesárea, con hipotonía uterina que no ameritó transfusión de hemoderivados. CONCLUSIÓN: Los embarazos gemelares con útero bicorne son excepcionales y casi todos se logran espontáneamente; los que llegan a término finalizan mediante cesárea electiva.

Abstract BACKGROUND: Congenital uterine anomalies are the result of abnormal formation, fusion, or resorption of the Müllerian ducts during embryological development. The bicornuate, unicornuate and didelphic uterus (U1, U2, U3 ESHRE/ESGE), are unification defects with a low prevalence both in the general population and in the population with a history of abortion and infertility, is estimated at around 0.4 to 1.1%, respectively. The presentation of the case can provide useful information related to with the evolution of the pregnancy and the perinatal outcomes when this anomaly appears. CLINICAL CASE: A 35-year-old patient with secondary amenorrhea and a positive immunological pregnancy test with a history of severe primary dysmenorrhea and previous magnetic resonance imaging with a congenital uterine anomaly compatible with a bicorporeal uterus (U3 complete ESHRE-ESGE), complete bicornuate (4a /AFS) , uterus didelphys (ASRM). The pregnancy was uncomplicated until 37 weeks, when it was terminated by cesarean section, with uterine hypotonia that did not require transfusion of blood products. CONCLUSION: Twin pregnancy with bicornuate uterus is a rare entity, most of them have been achieved spontaneously and those that reach term have been resolved by elective cesarean section.

Accessory and cavitated uterine mass: A rare cause of dysmenorrhea and chronic pelvic pain

Accessory and cavitated uterine mass (ACUM) is a rare developmental uterine anomaly that is said to develop due to duplication of ductal Mullerian tissue. It is an accessory cavity lying along the lateral wall of the uterus near the site of attachment of the round ligament and presents in young females with pelvic pain and dysmenorrhea. Histologically, the cavity is lined with endometrium and smooth muscle bundles similar to the myometrium. Ultrasound is helpful in the identification of the lesion. MRI of the pelvis is the imaging modality of choice for diagnosis. Laparoscopy and histopathology remain confirmatory. This report describes a case of ACUM in a young female who was managed by surgical excision of the mass.

Study of mullerian anomalies over 5 years in a tertiary care centre

Background: Mullerian anomalies-developmental anomalies of the mullerian system might involve the uterus, cervix, fallopian tubes and vagina of which anomalies of the uterus are the most common. They are often regarded as a treatable form of infertility and have a prevalence of 0.5% in the general population. Though most of them remain asymptomatic, they contribute a good proportion of recurrent pregnancy losses, obstetric complications and infertility. Their timely diagnosis, management and evaluation of associated anomalies proves to be necessary.Methods: A prospective observational study was undertaken at KIMS, Hubli from 2014 to 2018. The study involved those women who were admitted in the department of obstetrics and gynecology at KIMS, Hubli for various reasons and were detected to have a mullerian anomaly. Their mode of presentation, method of detection, associated anomalies were analysed and statistical conclusions drawn from the same.Results: During the study period of 5 years, a total of 85 cases of mullerian anomalies were detected amounting to an incidence of 0.15%. 35% of them were asymptomatic, infertility (24%) being the next common mode of presentation. Septate/sub-septate uterus was found to have the poorest obstetric outcome. Mullerian anomalies were seen in combination in 14% of the cases and had an association with other anomalies like renal anomalies in 10% of the cases. Surgical correction was done for most of the cases of vaginal septum and septate/sub-septate uterus.Conclusions: Mullerian anomalies are often regarded as an uncommon but treatable form of infertility. Their timely detection, treatment and watchful obstetric management is in need.

Successful vaginal delivery of spontaneously conceived twins in each horn of a bicornuate uterus with previous caesarean: a case report

Mullerian duct fusion anomalies resulting in uterine malformations have prevalence of 3-4%. Among this, bicornuate uterus has a rare incidence of 0.4%. Uterine malformations pose significant threat in terms of obstetric outcomes. We report an extremely rare case of spontaneous conception of twin pregnancy with the fetus occupying each horn of a bicornuate uterus (bicornis unicollis), in a woman with a history of previous caesarean section. She was booked at our hospital and the pregnancy remained uneventful. At 35 weeks she went into spontaneous labour and delivered vaginally without any maternal-fetal complications. The case is unique and the management is worth discussing as till date no protocols or guidelines have been proposed for the mode of delivery of bicornuate uterus with twins with previous cesarean.  Only 12 cases of twins with bicornuate uterus have been reported till date. This is the first case in literature in which a successful VBAC has been conducted in a woman with bicornuate uterus with twins with previous caesarean.

Obstructive mullerian anamolies: a case series

AIM of the study was to review the heterogeneous clinical presentations and management options for some of the obstructive mullerian anomalies through a case series. Background: Müllerian duct anomalies (MDAs) are a miscellaneous group of entities that result from the non-development, defective vertical or lateral fusion, or resorption failure of the müllerian ducts due to genetic mutation. 5 cases of obstructive mullerian anomaly are reviewed. Cases of OHVIRA (obstructed Hemivagina with Renal Agenesis) syndrome, transverse vaginal septum, imperforate hymen and obstructed rudimentary horn of unicornuate uterus are included. Results: We found that cyclical abdominal pain was the most common presenting complaint. There is a high incidence of associated renal anomalies. Psychosocial counselling before treatment is necessary to address the functional and emotional aspects of the patient. Surgical management was done in all patients with good postoperative outcome. Conclusion: Obstructive mullerian anomalies need to be evaluated by a meticulous examination and imaging studies to reach the diagnosis with precision. The treatment has to be tailored to the specific anomaly.

An observational study of effect of Mullerian anomalies on pregnancy

Background: Mullerian anomalies occur in approximately 3-4% of fertile and infertile women, 5–10% of women with recurrent early pregnancy loss, and up to 25% of women with late first or second-trimester pregnancy loss or preterm delivery. However, due to low prevalence rate and asymptomatic course of the anomalies, Mullerian anomalies remain underdiagnosed and often overlooked as a possible cause of recurrent pregnancy failures, preterm deliveries, IUGR and low birth weight.Methods: Total of 30 cases of Mullerian anomalies with pregnancy, prior diagnosed or incidental during LSCS, were studied for complications during pregnancy, history of gynecological complaints and rate of diagnosis with routine imaging technique.Results: Septate uterus was the most common anomaly seen in this study (36.6%).56.6% were diagnosed incidentally during LSCS despite the fact 26.6% of cases had history of 2 or more abortions and 30% had some or other gynecological complaints previously. 10% of pregnancies ended in abortions, 20% had preterm delivery, 36.6% had malpresentations and there was case of rupture uterus (03.3%).Conclusions: Mullerian anomalies are often asymptomatic or have subtle gynecological symptoms which are often missed by both patient and gynecologists. It is observed that due to the asymptomatic course of Mullerian anomalies, invasive nature of HSG and lack of 1.5 Tesla MRI at many institutes leads to low rate of diagnosis of Mullerian anomalies. Pregnancy with Mullerian anomalies often have preterm delivery, IUGR and malpresentation, so, require proper counselling and close monitoring during antenatal period.

Uterine didelphys with unilateral cervicovaginal agenesis: A case report

@#<p style="text-align: justify;">Unilateral cervicovaginal agenesis in a didelphic uterus is an extremely rare congenital anomaly. Women born with this anomaly present with menarche associated with progressive dysmenorrhea and symptoms of worsening endometriosis. Presented here is a case of a 12 year old nulligravid who presented with progressive cyclic abdominal pain. Physical examination revealed a tender large right adnexal mass and presence of a vaginal canal and cervix tilted to the left. Sonography showed the presence of uterine didelphys, right hematosalpinx and hematocolpos. Preoperative impression was outflow tract obstruction probably due to Herlyn Werner-Wunderlich syndrome. However, intraoperatively, it was discovered this was a case of uterine didelphys with outflow tract obstruction of the right hemiuterus due to unilateral cervicovaginal agenesis and ipsilateral renal agenesis.</p>

Revisión de la clasificación y diagnóstico de malformaciones mullerianas / Review of the classification and diagnosis of mullerian malformations

Resumen Las malformaciones mullerianas son un grupo de alteraciones ginecológicas que presentan una sintomatologia variable, la cual fluctúa entre amenorrea primaria, dispareunia, disfunción sexual, dolor, masas pélvicas, endometriosis, hemorragia uterina anormal, infección, aborto recurrente espontáneo y parto prematuro, e incluso puede ser asintomática. Se requiere una clasificación de estas malformaciones que permitan un adecuado entendimiento, diagnóstico y tratamiento; por otro lado, es de vital importancia el diagnóstico temprano en los casos sintomáticos, que permitan tratamiento quirúrgico adecuado de cada anomalía, con el fin de evitar complicaciones ginecológicas y obstétricas, tales como: esterilidad, hematómetra, piometra, gestación ectópica en cuerno rudimentario y restricción de crecimiento intrauterino. El objetivo de este artículo es presentar la clasificación actual de las malformaciones Mullerianas y determinar cuáles son los estudios imagenológicos, que según la evidencia, son los más recomendados para realizar un diagnóstico adecuado de esta patología. MÉD.UIS. 2018;31(2):57-63.

Abstract Mullerian malformations are a group of gynecological alterations that present a variable symptomatology, which varies between primary amenorrhea, dyspareunia, sexual dysfunction, pain, pelvic masses, endometriosis, abnormal uterine hemorrhage, infection, spontaneous recurrent abortion and premature birth, and can even be asymptomatic. It's necessary a malformation's classification that allows an adequate understanding, diagnosis and treatment; on the other hand, it is of vital importance the early diagnosis in the symptomatic cases that allows the appropriate surgical treatment of each anomaly, in order to avoid gynecological and obstetric complications, such as: sterility, hematoma, pyometra, ectopic gestation in rudimentary horn and Intrauterine growth restriction. The objective of this article is to present the actual classification of Mullerian malformations and to determine which image studies, according to the evidence, are the most recommended to make a suitable diagnosis of this pathology. MÉD.UIS. 2018;31(2):57-63.

Does the ESHRE/ESGE Classification of Mullerian Anomalies Correlate with the Occurrence Of Pregnancy? A Comparison between Two Definitions of Myometrial Thickness.

Introduction: Since the introduction of the European Society of Human Reproduction and Embryology/European Society for Gynaecological Endoscopy (ESHRE/ESGE) classification of Mullerian anomalies, various authors have raised major concern about its clinical implications, as specific diagnostic criteria that clearly correlate to pregnancy have not yet been validated in clinical practice by any prospective or retrospectives studies. In this study, we aimed to correlate the ESHRE/ESGE classification with the occurrence of pregnancy, consideringthetwo different definitions of myometrial thickness. Methods: A retrospective study, including an ultra-selected cohort of 79 patients, from January 2010 to March 2014. All women with fertilityproblems, who had an isolated andsuspected uterine malformation, t ultrasound and hysteroscopy, were retrospectively included in this study. Myometrial thickness was defined as the entire myometrial layer, as suggested by the ESHRE/ESGE criteria, or the free myometrial layer, as suggested by Gubbini. Results: We failed to evidence an association between the occurrence of pregnancy in the two most representative classes (U0 and U2), considering the free myometrial layer, and the entire myometrial layer. When we considered the effect of hysteroscopic surgery on the occurrence of pregnancy, we also failed to obtain a statistically significant difference. Discussion: The ESHRE/ESGE classification may be useful in classifying Mullerian anomalies, but it needs to be applied in larger series. However, we think that new parameters and algorithms are needed for a better prediction of pregnancy. We recommendto associate the fundal uterine vascularization to the ESHRE/ESGE criteria to be analysed in further studies.

Reconstrucción anatómica laparoscópica en útero unicorne no comunicante / Laparoscopic approach for unicornuate uterus

Las alteraciones congénitas de los conductos de Müller son un amplio grupo de patología, siendo el útero unicorne una de sus manifestaciones.Se relaciona con un aumento de complicaciones obstétricas y a su vez la presencia de un cuerno rudimentario no comunicante, es causa de dolor pélvico crónico. Presentamos una paciente de 14 años aquejada de intensa dismenorrea. Mediante técnicas de imagen se evidencia la presencia de útero unicorne con cuerno rudimentario derecho no comunicante. Se realizó abordaje laparoscópico en la que se procedió a exéresis del cuerno rudimentario y posterior reconstrucción anatómica. La paciente cursó un postoperatorio sin complicaciones, obteniendo una mejoría clínica satisfactoria en sus controles posteriores. El abordaje laparoscópico a las pacientes afectas de útero unicorne no comunicante permite restablecer de forma óptima la anatomía y mejorar la sintomatología asociada. Mediante una correcta técnica laparoscópica se consigue disminuir el riesgo de adherencias posteriores, asegurando una baja tasa de complicaciones y una recuperación funcional postoperatoria precoz.

Congenital anomalies of the Müllerian ducts are a large group of pathology, and unicornuate uterus is one of its manifestations. It is associated with increased obstetric complications and the presence of cavitated non communicating rudimentary uterine which cause chronic pelvic pain. We report a 14 years old patient suffering severe dysmenorrhea. Using imaging techniques showed the presence of unicornuate uterus with non communicating rudimentary right horn. Laparoscopy was performed in which we proceeded to resection of rudimentary horn and subsequent reconstruction of the anatomy. The patient had an uneventful postoperative course, obtaining a satisfactory clinical improvement in subsequent tests. The laparoscopic approach in patients with advanced non-communicating unicornuate uterus can optimally restore the anatomy and improving associated symptoms. A proper laparoscopic approach can lower the risk of subsequent adhesions, and ensure few complications and early postoperative functional recovery.

Prevalencia de anomalías müllerianasen un centro de medicina reproductiva / Mullerian anomalies prevalence in a reproductive medicine center

Objetivos: determinar la prevalencia y las características clínicas de las pacientes conanomalías müllerianas que consultan a un centro de reproducción asistida en la región del eje cafetero (Pereira) en Colombia.Métodos: se realizó un estudio descriptivo - transversal en pacientes con diagnóstico de anomalíamülleriana, en el centro de reproducción asistida del eje cafetero, In Ser Pereira, entre el 1 de enerode 2005 y el 30 de abril del 2008.Resultados: de un total de 731 pacientes que asistieron a consulta especializada, 62 pacientescumplieron los criterios de inclusión, hallándose una prevalencia de 8,4 %. Las anomalías másfrecuentes fueron en su orden: útero septado, útero arcuato y útero didelfo. El método de diagnósticoinicial fue la ultrasonografía transvaginal y la confirmación y tratamiento se realizaron mediantehisteroscopia-laparoscopia simultáneas.Conclusiones: las anomalías müllerianas son entidadesfrecuentes con repercusiones importantes reproductivas yobstétricas, las cuales deben ser buscadas sistemáticamenteen todos los casos de infertilidad, pérdida gestacionalrecurrente, partos pretérminos y presentaciones fetalesanormales. La ultrasonografía transvaginal presentauna alta especificidad para el diagnóstico inicial.

Objectives: To determine the prevalenceand clinical characteristics of patients with Mülleriananomalies consulting a fertility clinic inthe coffee-region (Pereira) in Colombia.Methods: We performed a cross-sectional studyin patients with a diagnosis of Müllerian anomalyin the center of assisted reproductionIn Ser Pereira in the coffe-region, between January1, 2005 and April 30, 2008. We used chi-squaretest.Results: Of a total of 731 patients attendingspecialized outpatient medical centerIn Ser-Pereira, in the coffee-region, 62 patientsmet inclusion criteria, we found a prevalence of8.4 %. The most frequent anomalies were in order:septate uterus, arcuate uterus and uterusdidelphys. The method of initial diagnosis wastransvaginal ultrasonography and confirmationand treatment was perfomed with simultaneoushysteroscopy-laparoscopy.Conclusions: Müllerian anomalies are a commoncondition with important implicationsfor reproductive and obstetricians should besought systematically in all cases of infertility, recurrentpregnancy loss, preterm birth and abnormalfetal presentations. Transvaginal ultrasonographyhas a high specificity for the initialdiagnosis.

Conduta frente às malformações genitais uterinas: revisão baseada em evidências / Medical care for uterine genital malformations: evidence-based review

Este trabalho de revisão foi idealizado para analisar as malformações dos ductos de Müller, que, devido à sua frequência de 3 a 7,3% na população em geral, justificam uma análise mais profunda do tema. O objetivo foi avaliar, de acordo com a literatura por meio de metodologia adequada? Os aspectos mais relevantes dessas anomalias, com destaque para a etiopatogenia, classificação, diagnóstico e tratamento. Os resultados obtidos nesta revisão apontaram as melhores evidências, até o momento, de como conduzir as mulheres portadoras dessas malformações.

This review paper was organized in order to analyse Müllerian anomalies, because their frequency from 3 to 7,3% in people in general justify a better evaluation about these malformations. The objective of the study was to evaluate, according to literature - by a proper methodology - the main aspects of these malformations, with special attention to etiology, classification, diagnostic and treatment. The results of this review showed the best evidences up till now of how to manage women with these genital malformations.

Clinical characteristics of women with Mullerian anomaly: Twenty years of experience at Asan Medical Center / 대한산부인과학회지

OBJECTIVE: To investigate the clinical characteristics and reproductive outcomes of women with Mullerian anomalies. METHODS: One hundred and eighty-six patients were diagnosed with Mullerian anomalies at the Asan Medical Center from 1990 to 2009 and their clinical characteristics and reproductive outcomes were analyzed. Mullerian anomalies were categorized according to the classification by the American Fertility Society (1988). RESULTS: Mullerian anomaly was noticed in 1 in 1,326 patients (0.075%). Most cases were found in adulthood (84.9%) whereas only 15.1% cases in adolescent or pediatric period. More than 40% of cases were asymptomatic and found incidentally but others suffered from amenorrhea (12.4%), dysmenorrhea (10.8%), abnormal menstruation (10.2%), etc. Most common type of uterine anomalies was uterine didelphys (30.6%), followed by bicornuate uterus (19.4%), Mayer-Rokitansky-Kuster-Hauser syndrome (10.8%), septate uterus (9.1%) and unicornuate uterus (8.6%). On the reproductive outcomes of 251 pregnancies identified, spontaneous miscarriages and preterm labor were quite common (55.8%), and the overall live birth rate was 48.6%. When each anomaly was individually analyzed, the live birth rate was 60.0% in the arcuate uterus and 58.0% in the uterine didelphys. The unicornuate and bicornuate uterus presented a similar chance of having a living child (42.1%, 46.7%), while the septate uterus showed a relatively lower live birth rate (32.4%). CONCLUSION: Although most cases of Mullerian anomalies are diagnosed in adulthood, many patients may suffer from menstrual abnormalities, dysmenorrhea or recurrent miscarriages since adolescence. The reproductive outcomes of the arcuate uterus and uterine didelphys were better, while those of septate uterus were poor in our study.

Malformaciones uterinas en el Hospital Central de Maracay: revisión de 1999 a 2007 / Uterine malformations in the Hospital Central of Maracay: review of 1999-2007

Conocer la incidencia de malformaciones uterinas y describir las características clínicas asociadas. Estudio descriptivo, retrospectivo y epidemiológico de 24 pacientes egresadas con el diagnóstico de malformación uterina entre los años de 1999 a 2007. Departamento de Obstetricia y Ginecología del Hospital Central de Maracay. Las pacientes en 70,8 por ciento tenían edades comprendidas entre 16 y 25 años. El 50 por ciento eran primigestas. El 33,3 por ciento presentaba diagnóstico de ingreso por presentación fetal anómala. En el 66,6 por ciento de los casos el diagnóstico se realizó por visualización directa de la estructura. En el 62,5 por ciento la malformación más frecuente fue el útero didelfo. En 50 por ciento de los casos el resultado obstétrico fue la cesárea segmentaria. En cuanto al diagnóstico de ingreso el útero didelfo registra el 62,5 por ciento de los casos ingresados. Las mujeres con anomalías uterinas se reproducen normalmente. Las malformaciones uterinas por defectos de fusión de los ductos müllerianos son las más frecuentes. Esporádicamente pueden ser sintomáticas ameritando diagnóstico diferencial y tratamiento

To know the incidence of congenital uterine anomalies and the clinical presentations. Retrospective, epidemiological, descriptive study of 24 cases of patients with congenital uterine anomalies. Department of Obstetric and Gynecology, Hospital Central de Maracay. In the 70.8 percent the age was between 16 and 25 years. The 50 percent were primipara. The 33.3 percent had personal history of abnormal fetal positions. The 66.6 percent were detected by visualization of the uterus. In 62.5 percent the most frequent malformation was the uterus didelphys. In 50 percent the obstetric performance was the cesarean section. The reproductive performance didelphic reported 62.5 percent of patients. Women’s with Müllerian anomalies demonstrate no problems with conception. However comes to medical attention when become symptomatic because of poor obstetric performance

Hysteroscopic Resection of the Vaginal Septum in Uterus Didelphys with Obstructed Hemivagina: A Case Report

Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Excision of the obstructed vaginal septum is the treatment of choice for symptom relief and the preservation of reproductive capability. A 14-yr-old girl complained of persistent vaginal spotting following each menstruation. Pelvic magnetic resonance imaging revealed a uterus didelphys with left hematocolpos and ipsilateral renal agenesis. Instead of conventional transvaginal excision of the vaginal septum, we used hysteroscopic excision under transabdominal ultrasonographic guidance to preserve the integrity of the hymen. The postoperative course was uneventful, and clinical symptoms were completely resolved after this intervention. Resectoscopic excision of the vaginal septum was found to be easy, safe, effective, and appropriate for young women as it preserved hymen integrity. We believe that this is the first Korean report on the use of a hysteroscopy for vaginal septum resection in a patient with uterus didelphys with obstructed hemivagina.

Diagnosis and treatment guidelines of congenital mullerian anomalies / 대한산부인과학회지

Anatomic gynecologic anomalies occur when there is failure of normal embryonic ductal development. These include conditions such as congenital absence of vagina, lateral fusion defect and vertical fusion defect of the Mullerian duct. The treatment of Mullerian anomalies should be based on accurate diagnosis and comprehension of the developmental process. Congenital uterine anomalies occur in approximately 1 in 2000 newborn female infants. It is also important to provide adequate consultation and explanation to all family members with regard to the aim of treatment, possible complications that may ensue, and changes in future fertility potential.