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.

About a uni-cervical unicorn uterus case with vaginal septum

The unicorn uterus is a common uterine malformation after septate uteri, accounting for 30 to 50% of cases. We report a case of unicorn uterus with an associated vaginal septum discovered during the obstetrical examination of a consultant surgeon for hemorrhage, and exploration of the uterus revealed a uterus during a cesarean section. Maternal-fetal rescue for placenta previa. The interest of this case is to show the double embryological mechanism involved, unilateral aplasia of the muller ducts and the lack of resorption explaining this vaginal septum.

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.

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.

Vaginal Obstruction after Excision of a Transverse Vaginal Septum: A Rare Case Report

A vaginal obstruction often occurs as a result of complication in women who had previously undergone an excision of a transverse vaginal septum. Her we are reporting a case of 12 years old unmarried girl presented with complaint of secondary amenorrhea and cyclic pain lower abdomen since two months. She was operated for cryptomenorrhea four months back. On examination under anaesthesia Thick scarred septum was present in vaginal canal about 6-7 cm from vaginal introitus .After sharp dissection hematocopos bulge seen and drained, residual scar tissue excised , posterior and anterior vaginal wall reconstructed. Vaginal mould inserted to maintain patency

Value of MRI in the pre-operative diagnosis and classification of oblique vaginal septum syndrome / 中华妇产科杂志

Objective@#To explore the role of MRI in the pre-operative diagnosis and classification of oblique vaginal septum syndrome (OVSS) .@*Methods@#A retrospective analysis of the clinical records and pre-operative MRI images of 19 patients with surgery proved OVSS was carried out. Two experienced radiologists reviewed the pre-operative pelvic MRI of the 19 patients in consensus blind to the surgery results. Characteristics including malformations of the uterus, cervix and vagina, the diagnosis of the disorder and classification were evaluated. Pre-operative MRI diagnosis and classification were correlated with surgical findings.@*Results@#Mean age of onset of symptoms for the 19 patients was 15 years (ranged 9-25 years) , and mean age of menarche was 12 years. Ten patients suffered from dysmenorrhea or lower abdominal pain, 5 patients complained of vaginal discharge, 3 patients had a history of irregular menstruation, 1 patient suffered from primary infertility. All 19 patients showed uteri didelphys. Eighteen patients showed vaginal oblique septum.One patient showed cervical atresia.MRI was completely correlated with the surgery in the pre-operative diagnosis of OVSS. MRI classification was in line with surgery in 17 patients, including 9 patients with imperforate septum (typeⅠ) , 6 patients with perforate septum (type Ⅱ) , 1 patient with imperforate septum and cervical fistula (type Ⅲ) , and another one with cervical agenesis (type Ⅳ) . One case of type Ⅱ was misdiagnosed as type Ⅰ, another one of type Ⅰ was misdiagnosed as type Ⅲ. Pre-operative MRI classification was correlated with surgery in 17 out of 19 patients.@*Conclusion@#Pre-operative MRI allows excellent manifestation and accurate diagnosis of OVSS, and could also facilitate the evaluation of the classification.

Clinical application of MRI in the diagnosis of oblique vaginal septum syndrome / 实用放射学杂志

Objective To investigate the clinical value of MRI in the diagnosis of oblique vaginal septum syndrome(OVSS),and to improve the diagnostic level for this disease.Methods Clinical and imaging data of 8 patients (7 adolescent females and 1 female in fertility period)with vaginal septum syndrome were analyzed retrospectively.All participants were evaluated by ultrasound examination before MRI scanning,and 1 of them underwent enhanced MRI scan.Results Among the 8 patients with OVSS,the main findings of MRI includes uterine deformity (4 double uterus,3 mediastinal uterus,1 double angle uterus with uterine septum),oblique vaginal septum (7 right oblique septum and 1 left oblique septum).All subjects presented with ipsilateral renal agenesis,and 4 patients with endometriosis,1 patient with ureteral distortion and ectopic opening and 1 patient with double inferior vena cava.6 patients suffered with hemorrhage in the posterior chamber of oblique septum and 2 patients with empyema.Hemorrhage in the contralateral vagina were observed in 3 cases,while no significant abnormality was found in other 5 cases.Conclusion MRI scan has an important clinical value on the diagnosis of OVSS and can provide crucial information for the optimal individual treatment.

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.

Imaging features of oblique vaginal septum syndrome and its diagnostic value / 实用放射学杂志

Objective To investigate the imaging findings of oblique vaginal septum syndrome and its diagnostic value.Methods Clinical and radiographic data of 13 patients with oblique vaginal septum syndrome were analyzed retrospectively and relevant literatures were reviewed.All 13 patients underwent ultrasonography examination preoperatively,and 8 of them underwent MRI examination,and 3 patients underwent CT examination as well.The position of the oblique septum,the size of the cavity in the rear of oblique septum, the positional relationship between the cavity in the rear of oblique septum and the cervix,and the concomitant changes of the uterine appendages and urinary system were analyzed.Results The ultrasonography and MRI examinations all manifested as uterus didelphys,cervix duplex,double vagina and obstructed hemivagina,and oblique septum originated from the middle of cervix duplex and attached to one side of the vaginal wall,shielding the ipsilateral cervix;oblique vaginal septum was on the right side in 4 cases and on the left side in 9 cases;the average volume of the cavity in the rear of oblique septum in 6 cases of type Ⅰ was 255 mL,and the average volume in 6 cases of type Ⅱ was 74 mL,and the volume in 1 case of type Ⅲ was 56 mL;2 cases were combined with ipsilateral hematosalpinx and 1 case was combined with a chocolate cyst of ipsilateral ovary;12 cases were accompanied with ipsilateral renal agenesis and 1 case was accompanied with ipsilateral renal hypoplasia.CT examinations of 3 cases all presented as uterus didelphys,a cystic hypodensity lesion under unilateral cervix and ipsilateral renal agenesis.Conclusion Imaging findings of oblique vaginal septum syndrome are quite characteristic. Ultrasonography,CT and MRI examinations have certain value for accurate diagnosis of the disease.

Imaging features of oblique vaginal septum syndrome and its diagnostic value / 实用放射学杂志

Objective To investigate the imaging findings of oblique vaginal septum syndrome and its diagnostic value.Methods Clinical and radiographic data of 13 patients with oblique vaginal septum syndrome were analyzed retrospectively and relevant literatures were reviewed.All 13 patients underwent ultrasonography examination preoperatively,and 8 of them underwent MRI examination,and 3 patients underwent CT examination as well.The position of the oblique septum,the size of the cavity in the rear of oblique septum, the positional relationship between the cavity in the rear of oblique septum and the cervix,and the concomitant changes of the uterine appendages and urinary system were analyzed.Results The ultrasonography and MRI examinations all manifested as uterus didelphys,cervix duplex,double vagina and obstructed hemivagina,and oblique septum originated from the middle of cervix duplex and attached to one side of the vaginal wall,shielding the ipsilateral cervix;oblique vaginal septum was on the right side in 4 cases and on the left side in 9 cases;the average volume of the cavity in the rear of oblique septum in 6 cases of type Ⅰ was 255 mL,and the average volume in 6 cases of type Ⅱ was 74 mL,and the volume in 1 case of type Ⅲ was 56 mL;2 cases were combined with ipsilateral hematosalpinx and 1 case was combined with a chocolate cyst of ipsilateral ovary;12 cases were accompanied with ipsilateral renal agenesis and 1 case was accompanied with ipsilateral renal hypoplasia.CT examinations of 3 cases all presented as uterus didelphys,a cystic hypodensity lesion under unilateral cervix and ipsilateral renal agenesis.Conclusion Imaging findings of oblique vaginal septum syndrome are quite characteristic. Ultrasonography,CT and MRI examinations have certain value for accurate diagnosis of the disease.

Study on Combination of Hysteroscopy and Laparoscopy in Diagnosis and Treatment of Herlyn-Werner-Wunderlich Syndrome : Report of 23 Cases / 中国微创外科杂志

Objective To explore the efficacy of hysteroscopy combined with laparoscopy in the diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome (HWWS).Methods The clinical data of 23 patients with HWWS from January 2009 to December 2015 treated with combination of laparoscopy and hysteroscopy were retrospectively analyzed.The patients had different degrees of dysmenorrhea (17 cases),abnormal vaginal discharge (17 cases),and infertility (4 cases) before surgery.During followup,we observed the symptom and pregnancy outcomes after surgery.Results The patients were followed up for 3-36 months (average,24 months).The vaginal irregular bleeding and abnormal secretions disappeared in 17 patients;dysmenorrhea relieved partly in 17 cases;4 patients who had fertility requirements gave birth to babies.Conclusion Combination of hysteroscopy and laparoscopy sufficiently clears genital malformation of HWWS and solves the lower genital tract obstruction.

Value of MRI in diagnosis of oblique vaginal septum syndrome / 中华全科医师杂志

Objective To assess the application of MRI in diagnosis of oblique vaginal septum syndrome (OVSS).Methods Clinical and imaging data of 41 patients with OVSS confirmed by surgery from March 2011 to November 2016 were retrospectively analyzed.Results The average age of patients was 20.5 year (10-46 years).The primary clinical symptoms were menorrhalgia (16 cases) and menorrhagia (13 cases).There were 12 cases of type Ⅰ,23 cases of type Ⅱ,5 cases of type Ⅲ and 1 case of type Ⅳamong 41 cases of OVSS.The resections for OVSS were performed in 35 cases.Forty two cases were diagnosed as OVSS by MRI scan,and 41 were confirmed by surgery,the accuracy of MRI diagnosis was 97.6％ (41/42).MRI showed uterus didelphys,hydrocolpos or hematocolpos with varying degrees,and revealed ipsilateral renal agenesis in all 41 cases.Conclusion MRI scan can accurately diagnose oblique vaginal septum syndrome and provide comprehensive information for clinical treatment.

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.

Two Cases of Microperforation of the Transverse Vaginal Septum

Primary amenorrhoea with microperforate transverse vaginal septum is a rare entity with varied incidence in different parts of the world. It may be asymptomatic, unlike imperforate hymen. Depending on the size of the microperforation in transverse vaginal septum clinical features may vary similar to those found in imperforate hymen cases. We report two cases of a 25 and 22-year-old women who sought the gynaecologist with complains of primary infertility. They had normal menses with one of them having secondary dysmenorrhea. Physical examination and imaging disclosed microperforation of transverse vaginal septum in the upper part of the vagina at mid point position. Resection of the septum under anesthesia was done. The outcome was favourable and 25 year asymptomatic patient with primary infertility at the present moment have conceived carrying six weeks pregnancy and second patient is in follow up. We conclude that this anomaly may be overlooked, interfering on its incidence determination. The finding of transverse vaginal septum in an asymptomatic infertility patient is highly unusual. Clinicians must be aware of atypical presentations and potential multifactorial etiologies of primary infertility. Ensuring a thorough evaluation is essential in order to mitigate long-term effects of a misdiagnosis.

Manejo quirúrgico de un caso de hematocolpos por tabique vaginal transverso

Se presenta un caso de hematocolpos, en una niña de 14 años, sometida a himenotomía en otra institución, sin lograrse evacuar el hematocolpos por presencia de tabique vaginal transverso, siendo transferida a nuestro Hospital. Refería dolor pélvico, disuria, polaquiuria y tumoración en hipogastrio; estaba febril, con presencia de tumoración pélvica hacia fosa iliaca izquierda. La vagina estaba obturada por tabique transversal a 1 cm del introito. Se halló fistula rectovaginal y tumor renitente a tensión de 6 cm hacia pared rectal anterior. Se inició antibioterapia, y la ecografía transperineal localizó el tabique, su grosor y diámetro y distancia del meato urinario. Bajo anestesia epidural y cateterización vesical, se punzó el tabique en el punto medio, obteniéndose 200 mL de material hemopurulento maloliente. Con tracción con sonda Foley triple vía, globo insuflado con 30 mL de suero, se pudo exponer el tabique que fue cortado en rodete con electrobisturí. Se reparó la fístula rectovaginal en tres planos. Por persistencia de fiebre, el piosálpinx fue resuelto al sexto día vía laparoscópica y fue dada de alta al 16º día.

A case of hematocolpos in a 14 year-old girl is presented. She had himenotomy at other institution without evacuation of hematocolpos due to transverse vaginal septum; she was transferred to our Hospital. She complained of pelvic pain, dysuria, polaquiuria and hypogastric tumor; she had fever and a left pelvic tumor. A transversal septum 1 cm from the introitus closed the vagina. Rectovaginal fistula and tense tumor were present at 6 cm towards the anterior rectal wall. Antibiotics were started and transperineal ultrasound localized the septum, its thickness, diameter and distance from the urinary meatus. Under epidural anesthesia and with bladder catheter, the septum was punctured centrally and 200 mL of foul hemopurulent material was obtained. By traction with three-way Foley catheter filled with saline 30 mL the septum was exposed and cut in ring shape with electro scalpel. The rectovaginal fistula was repaired in three layers. Due to persistence of fever pyosalpinx was resolved at the sixth day by laparoscopy and the patient was discharged on day 16.

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.

A case of transverse vaginal septum with modified Garcia technique / 대한산부인과학회지

The incidence of all transverse vaginal septum is 1:2,100 - 1:72,000. The transverse vaginal septum is a developmental defect of vagina which may be fused incompletely between the Mullerian duct component and the urogenital sinus component of vagina. Serious complications may be hematocolpos, hematometra and hematosalpinx. Simple surgical excision or simple incision have been developed to treat congenital transverse vaginal septa, but also caused common complications such as secondary tissue contracture. Garcia technique using eight vaginal mucosa flaps was developed to avoid common complication of secondary vaginal stenosis. Here, we present a case of contracted transverse vaginal septum who has been performed a simple incision 13 years ago, successfully performed the modified Garcia technique without serious postoperative complication.

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.

Case Report: A new surgical technique for Transverse Vaginal Septum / 대한산부인과학회지

Transverse vaginal septum, especially completely imperforate condition, is a rare congenital anomaly of female genital tract. The patient was 26 years old and visited for primary amenorrhea and low abdominal pain. Secondary sexual characteristics were all normal, but the depth of the vagina was only about 1cm. Hematometra was suspected and two large cystic structures were detected in the pelvis on ultrasonography. Intravenous pyelography and other laboratory results were normal. Vaginoplasty was needed to drain out the hematometra. We tried a new surgical technique using radial incisions of the closed vaginal end. To prevent postoperative contracture, we used a sterile pen case. and educated the patient to dilatate the vagina with that, daily. Here, we report a case of complete imperforate transverse septum with reviews of literatures.