Robert’s uterus: a rare mullerian anomaly mystery unfolded

Anomalies of female genital tract may not be detected until after menarche when they present a cyclical pain due to outlet obstruction. Mullerian anomalies represent a vast array of structural abnormalities resulting from improper development and fusion of embryological mullerian ducts. 19-year-old girl attained menarche at the age of 14, had progressive dysmenorrhoea and diagnosed as right haemotosalphinx and ovarian endometrioma which were removed in 2008. As pain progressed, she underwent laparoscopic adhesiolysis in 2013. Since, pain persisted, diagnosed as right haematometra, and drainage done by laparotomy. Left adnexa were normal. She was given depot provera till she completed schooling. She developed recurrent dysmenorrhoea after stopping depot provera. USG and MRI revealed recurrent haematometra on right side with normal left horn. The possibility of atypical septum was thought about and hystero laparoscopy was done. It showed right side haemetometra with absent right adnexa. Left adnexa normal. Hysteroscopy showed normal left horn with septum with a bulge towards the left side. Hence, proceeded with hysteroscopic septostomy and haemetometra was drained to the left horn. Later patient was free from dysmenorrhea and repeat hysteroscopy was found to be normal. This case highlighting mullerian anomalies have to be considered when young girls present with severe progressive dysmenorrhoea and diagnosis remains a challenge most of the clinicians. This rare entity has to be kept in mind while evaluating such patients. Prompt diagnosis and early surgical correction are essential to avoid future morbidity in the form of repeated unnecessary surgeries.

A case of successful conception after Jone's metroplasty in woman with recurrent pregnancy loss with septate uterus

Anomalies of the reproductive tract are common and are seen in approximately 3-5% of the general population. These are usually asymptomatic, but are sometimes associated with recurrent pregnancy loss or infertility. Among these anomalies, septate uterus is the most common anomaly to be associated with obstetric complications and infertility. A 27 years old female patient P0A2L0 with first spontaneous abortion at 3 months and second spontaneous abortion at 4 months came to OPD with complaint of recurrent pregnancy loss. She was advised USG of pelvic region which shows septate uterus with normal cervix, fallopian tubes and ovaries. Patient prepared for DHL complete septum present normal B/L ostium and Jone's metroplasty done in which wedge shaped incision kept on uterine fundus, common cavity created by un roofing septum myometrium closed and AT the end Copper T 375 placed in intrauterine cavity for 6 months. 1 year after surgery patient came with UPT positive for antenatal visit at sola civil. Patient is now with 30 weeks of pregnancy with normal single intrauterine live cephalic fetus, metroplasty is an accepted method of treatment in women with recurrent abortions and septate uterus and it significantly improves the subsequent reproductive outcome. combined data from several published series and reported that the incidence of spontaneous abortion and preterm delivery rate decreases significantly after metroplasty, whereas, the incidence of term delivery rate increases.

Septate uterus with double cervix and longitudinal vaginal septum – A report of two cases of rare uterine anomaly

Background: Embryological maldevelopment of the Mullerian or paramesonephric duct results in congenital uterine anomalies. A rare developmental anomaly is a septate uterus with cervical duplication and a longitudinal vaginal septum. This anomaly cannot be explained by the classical unidirectional Mullerian developmental theory and does not fit into American Society for Reproductive Medicine (ASRM) classification of uterine anomalies. The bidirectional Mullerian developmental theory holds good for this type of anomalies and the new ESHRE/ESGE classification for female urogenital tract anomaly (CONUTA) is more suitable for effective categorization, diagnosis, and treatment.Case Report: Two cases of the complete uterine septum with double cervix and longitudinal vaginal septum who presented with dysmenorrhea, dyspareunia, and infertility were diagnosed and managed by hysteroscopy and laparoscopy. The vaginal and the uterine septum were resected. Both the patients had an uneventful post-operative recovery period and were followed up for a period of 1 year. The symptoms such as dysmenorrhea and dyspareunia improved significantly without any effect on the fertility outcome.Conclusion:Very few of such cases are reported in the literature. This rare anomaly is explained by the bidirectional Mullerian developmental theory and the application of the new ESHRE/ESGE classification of the urogenital tract (CONUTA) is more suitable for such anomalies for effective categorization, diagnosis, and treatment.

Successful outcome in a perplexing case of Roberts uterus: a rare uterine anomaly, a rare case report with review of literature

Robert uterus is a rare Mullerian development anomaly with very few cases reports available. It presents with triad of morphologic features of - Blind hemi cavity with or without unilateral hematometra, contralateral unicornuate uterine cavity and normal uterine fundus with or without small external indentation. The major difficulty lies in making the diagnosis of Robert’s uterus. All the reported cases of Robert’s uterus have been managed differently according to patient’s complaints. A 25-year-old married female, resident of Agra presented to gynecology OPD of Dr RML Hospital and associated PGIMER in June 2013, with primary infertility and cyclical left sided dysmenorrhoea since menarche. Patient was consulting at her hometown Agra for 2 years and had an USG and MRI pelvis report of Feb. 2013 with her showing unicornuate uterus with rudimentary horn. Infertility investigations were done in our hospital. HSG report was of localized spill on right with left tubal block. Patient was posted for diagnostic hystero-laproscopy which was further followed by laprotomy, after which we could reach to a diagnosis of Robert uterus with non-communicating left cavity and with severe endometriosis. Patient needed a second hysteroscopy for lysis of septum and subsequently conceived with IVF. She delivered a healthy male baby of 2.65 kg by elective LSCS at 37 weeks with associated breech presentation with gestational hypertension with severe IHCP on 20 June 2019. Paediatric surgeons and gynecologists should be aware of this rare atypical obstructive Mullerian malformation and its management to avoid inappropriate management delays in these patients. A timely diagnosis and definite treatment have a great impact on future reproductive and endocrine function.

Operative hysteroscopy-assisted pregnancy termination after failed surgical abortion in missed abortion of woman with complete septate uterus

A discussion of diagnostic value and diagnostic indicator in distinguishing arcuate uterus from septate uterus by three dimensional sonography / 中华医学超声杂志（电子版）

Objective To discuss the accuracy of three-dimensional (3D) sonography in diagnosing arcuate uterus and septate uterus and propose a quantitative indicator in distinguishing arcuate uterus and septate uterus.Methods Ninety-six patients with suspected congenital uterine malformation underwent 3D sonography to assess uterine anomalies at Obstetrics and Gynecology Hospital of Fudan University from August 2011 to March 2013.Uterine coronal section was reconstructed and measured in onmi view imaging mode of 3D sonography.Then qualitative diagnosis and categorization was done.The results were compared with diagnosis of hysteroscopy,hysteroscopy combined with laparoscope to determine the sensitivity,specificity,positive predictive values,negative predictive values and total accuracy of 3D sonography in diagnosing arcuate uterus,partial and complete septate uterus respectively.The variable was created in coronal section of uterus constructed through 3D imaging mode to differentiate arcuate uterus from septate uterus.Results There were 33 cases (34.4％,33/96) of arcuate uterus,54 cases (56.2％,54/96) of partial septate uterus and 9 cases (9.4％,9/96) of complete uterus in 96 cases diagnosed by 3D sonography.The accuracy of 3D sonography in diagnosing arcuate uterus and septate uterus was 86.5％.We concluded that 3D sonography had 100％ sensitivity,82.9％ specificity,60.6％ positive predictive value and 100％negative predictive value in the diagnosis of arcuate uterus.The sensitivity of 3D sonography for diagnosis of partial septate uterus was 80.6％,the specificity was 100％,the positive predictive value was 100％,and the negative predictive value was 69.1％.The sensitivity,specificity,positive and negative predictive values of 3D sonography for detecting complete septate uterus were all 100％.The ratio of depth of uterine internal indentation and depth from uterine fundus to the top of intemal indentation was more than 50％ for septate uterus,while less than 50％ for most of the arcuate uterus.Conclusions The diagnostic efficiency of 3D sonography in specific uterine malformation is different.The new quantitative indicator maybe helpful in improving the accuracy of 3D sonography in diagnosing arcuate uterus and septate uterus.

Fertilidad posterior a la septoplastia histeroscópica de consultorio. Una propuesta de clasificación del septo uterino / Fertility after hysteroscopic septoplasty surgery. A proposed classification for the septum uterine

Resumen ANTECEDENTES: Las malformaciones müllerianas son anomalías del desarrollo de los genitales internos femeninos. La incidencia mundial es de 0.16 a 10%. La más común es el útero septado, consecuencia de una falla en la reabsorción del tabique útero-vaginal. OBJETIVO: Evaluar, en la población estudiada, la repercusión de la septoplastia histeroscópica en la fertilidad de mujeres con útero septado. MATERIALES Y MÉTODOS: Estudio abierto, observacional, retrospectivo y transversal de pacientes de la Clínica de Histeroscopia del Hospital General Dr. Manuel Gea González, con diagnóstico de infertilidad establecido con auxilio de la técnica de acceso por vaginoscopia, según Betocchi, siguiendo el concepto de "ver y tratar". RESULTADOS: Se revisaron 508 expedientes y de estos se seleccionaron 313 de pacientes a quienes se practicó histeroscopia diagnóstica de las que 32 resultaron con septo uterino. Se efectuaron 27 septoplastias histeroscópicas en consultorio y 5 programadas en quirófano. En 27 (84.3%) se logró el embarazo y de éstos 5 mediante fertilización asistida; hubo 8 nacimientos prematuros y 17 a término y 2 abortos diferidos. CONCLUSIÓN: Hoy día la septoplastia histeroscópica es la alternativa de tratamiento del septo uterino debido a su facilidad de realización en el consultorio por personal adiestrado, baja morbilidad, menores costos, rápida recuperación y excelente resultado reproductivo. Se propone una clasificación de acuerdo con el componente del septo.

Abstract BACKGROUND: Müllerian malformations are defined as anomalies development of female internal genitalia. Its incidence worldwide is 0.16% to 10%. The most common of these is the septate uterus which is due to a failure in the reabsorption of uterovaginal septum. OBJECTIVE: To evaluate the impact on fertility hysteroscopic septoplasty in women with uterine septum. MATERIAL AND METHODS: This is an open, observational, retrospective and cross-sectional study. 508 cases of which 313 patients underwent diagnostic office hysteroscopy hysteroscopy clinic were reviewed Hospital General Dr. Manuel Gea González, diagnosed with infertility, you access using the technique vaginoscopic according Betocchi, following the concept of" see and treat ", proceed to perform septoplasty with bipolar energy at that moment. RESULTS: 32 patients were diagnosed with uterine septum. 27 hysteroscopic septoplasty were performed in office and five scheduled in the operating room. 27 of them achieving pregnancy (84.38%) spontaneously regarding perinatal outcomes of those 27 patients who achieved pregnancy 2 had missed abortion, premature 8 were 17 were concluded; 5 required assisted fertilization. CONCLUSION: The hysteroscopic septoplasty is now recommended for the treatment of uterine septum due to ease of performance by trained personnel, low morbidity, the feasibility of its implementation in practice, reduced cost, speedy recovery of patients and excellent reproductive outcome alternative. Classification according to the component of the septum is proposed.

Complete septate uterus, obstructed hemivagina, and ipsilateral adnexal and renal agenesis in pregnancy

Most cases of double uterus with obstructed hemivagina and ipsilateral renal agenesis were diagnosed at adolescents after menarche. This is the first reported case of complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis in addition to ipsilateral agenesis of fallopian tube and ovary in which the diagnosis was delayed until pregnancy. The pregnancy was uneventful in spite of intermittent vaginal spotting. During the cesarean section, the septum of the uterus was resected and about a 3-cmx3-cm window was made on the vaginal septum to allow an opening for the obstructed vaginal discharge. We followed the patient up for one and half years, and she has not had symptoms such as dysmenorrhea or abnormal vaginal bleeding.

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.

Hysteroscopic Septotomy of a Complete Septate Uterus Using a Balloon with Methylene Blue / 대한불임학회지

A complete septate uterus with cervical duplication and a longitudinal vaginal septum is a rare uterine malformation. The dissection of the septum can be difficult because it is difficult for hysteroscopists to find out initial point, direction and final point of a complete septum. This study aimed the introduction of more efficient surgical procedure using a balloon with methylene blue. We have experienced three cases with a complete septate uterus. We performed hysteroscopic dissection of a complete uterine septum using a balloon with methylene blue and obtained good reproductive outcomes. So we report three cases with a brief review of literatures.

A variant of complete septate uterus with double cervix and vagina: A case report / 대한산부인과학회지

Rare mullerian anomalies without any present classification were sometimes reported. A 30-year-old nulligravid woman was referred to our hospital with 2-year history of primary infertility. Laparoscopic examination revealed a relatively intact uterine fundus with both patent fallopian tubes. Hysteroscopic exam confirmed the presence of double vagina and cervix, as well as complete uterine septum with opening at the lower segment. Hysteroscopic septotomy was successfully performed through the right-sided cervix. A variant of complete septate uterus with double cervix that communicated at the isthmic portion could be successfully treated by hysteroscopic operation.

Hysteroscopic treatment of septate uterus with Neodymium: YAG laser / 中国微创外科杂志

Objective To investigate the clinical effect of hysteroscopic treatment of septate uterus with Neodymium: YAG laser. Methods Forty-six sterile patients with septate uterus received hysteroscopic metroplasty with Nd: YAG laser.The metroplasty was completed under the guidance of B-ultrasonography in 41 patients with incomplete septate uterus,and with the assistance of laparoscopy in other 5 patients with complete septate uterus.Results The metroplasty was successfully conducted on one session in 45 patients,the success rate being 98%(45/46).Postoperative follow-up examinations in 28 patients found pregnancy within 1 year in 20 patients,16 of whom delivered of a viable term infant,the pregnancy rate within 1 year being 71%.Conclusions Hysteroscopic treatment of septate uterus with Nd: YAG laser is effective.The uterine neck may not be excessively dilated,the intraoperative bleeding can be thoroughly controlled,and the procedure under general anesthesia is relatively safe.

Three cases of successful pregnancy outcome after intensive care in the patients with complete uterine septum, cervical duplication and longitudinal vaginal septum / 대한산부인과학회잡지

Three cases of successful pregnancy outcome after intensive care in the patients with rare mullerian anomaly of complete uterine septum, hypoplastic cervical duplication and longitudinal vaginal septum in infertility clinic are reported. These presented three cases are the reports of supporting the embryologic hypothesis of bidirectional mullerian development which fusion and resorption begins at the isthmus and proceeds simultaneously in both the cranial and caudal directions rather than the classical views of unidirectional(caudal to cranial) mullerian development. Thus, we present here three cases of rare mullerian anomaly and their pregnancy outcomes in an attempt to alert gynecologist to the possible occurrence of such a malformation. In 2 cases, successful pregnancy outcomes were achieved after abdominal or hysteroscopic uterine septotomy before pregnancy and in the last case, successful pregnancy outcome intensive obstetric care was achieved even under uncorrected uterine anomaly and complete removal of uterine septum resulted in uneventful postoperative courses at the same time of cesarean section.

A Case of Hematometrocolpos due to Complete Septate Uterus and Obstructed Hemivagina associated with Ipsilateral Renal Agenesis / 대한산부인과학회잡지

We report a case of a 13-year-old girl who was noted to have combined malformation of complete septate uterus, obstructed hemivagina and ipsilateral renal agenesis with complaints of dysmenorrhea. Hematometrocolpos was developed in the result of retained menstrual blood since menarche. This rare congenital anomaly is supposed to be derived from disorders of mesonephric and M llerian developement in embryogenesis of female genitouinary tract. An accurate preoperative diagnosis of this syndrome is critical because of the difference in therapeutic strategy and prognosis. Simple and definite treatment of the condition is excision of the obstructed vaginal septum with prompt exit of retained blood. We present here a interesting case of M llerian anomaly in attempt to alert gynecologist to the possible occurrence of such a malformation with a brief review of concerned literatures.

Septate Uterus with Duplication of the Cervix and Longitudinal Vaginal Septum with Hemivaginal Stricture / 대한산부인과학회잡지

M llerian anomalies have been infrequently encountered clinical problem for the obstetrician gynecologist, incidence was estimated to occur in 1% to 6% of all women. Recently, the use of vaginal ultrasound, MRI and endoscopic techniques have led to more accurate description and classification of m llerian anomalies. This report of a complete uterine septum with duplication of the cervix and a longitudinal vaginal septum with hemivaginal stricture represents a unique case of septate uterus, which to our knowledge previously has not been reported.