Vaginal Duplication Research Articles

Usefulness of 3D reconstruction of low dose pelvic CT as an innovative tool for surgical planning in definitive correction of cloacal malformations

PurposeTo describe the results of the use of a low dose pelvic CT protocol for preoperative imaging in cloacal anomalies at a referral center. MethodsWe reviewed 43 cases of cloacal anomalies treated at our institution from 2012 to 2022. The patients underwent a cystoscopy under general anesthesia, with measurement of the common channel and the urethra, and visualization of the Müllerian structures, and a Foley catheter was placed in the bladder. Then the patient was transferred to the CT suite for a low dose pelvic CT, filling the bladder, the vagina (by overflow) and the sigmoid/rectum (as in a distal colostogram) with diluted meglumine. Volume rendering was obtained at the CT station. Results43 girls with cloaca were treated our institution, 40 of them operated on for correction. The median effective radiation dose was of 2.19mSv (0.81 - 2.96 mSv). The measurement of the common channel had a Pearson coefficient of 0.96 when correlated with the actual measurement. The length was < 1 cm in 3, 1-3 cm in 18, 3-5 cm in 15, and >5 cm in 4. Total urogenital mobilization was possible in 28 patients. We found vaginal duplication in 16, and vaginal reconstruction was performed in 13 others. ConclusionThis technique provided the spatial anatomy of the urinary, vaginal, and colorectal components, with a higher fidelity than fluoroscopy. Since we do not have rotational fluoroscopy, we overcame that limitation with this modality, and found it to be an improvement for planning, allowing for better outcomes.

Инновационный миниинвазивный метод хирургического лечения частичной аплазии гемивлагалища при удвоении матки и влагалища. Первый российский опыт

Инновационный миниинвазивный метод хирургического лечения частичной аплазии гемивлагалища при удвоении матки и влагалища. Первый российский опыт

Treatment of Urethral Intercourse and Impact on Female Sexual Function.

BackgroundUrethral intercourse is a very rare entity which usually presents as urinary incontinence during and after intercourse and is most commonly seen in patients with vaginal agenesis (Mayer–Rokitansky–Hauser Syndrome) or hypoplasia, or other rear vaginal anomalies.AimTo evaluate management and outcomes for vaginal and urethral consequences of urethral intercourse, including urinary incontinence.MethodsBetween February 2006 and March 2021, 8 women aged from 17 to 22 years underwent genital and urethral reconstruction due to consequences of urethral sexual intercourse. Vaginal reconstruction included sigmoid vaginoplasty and introitoplasty with division of the vaginal septum in cases of vaginal agenesis (5 cases) and vaginal duplication (3 cases), respectively. Incontinence was treated by sling procedures in 5 women with longer history of urethral coitus and evident bladder neck prolapse.OutcomesSexual and psychosexual outcomes assessment was based on the Female Sexual Function Index and standardized questionnaires.ResultsFollow-up ranged from 9 to 188 months (mean 78 months). Good esthetical and functional results were achieved in all 8 women. All patients reported satisfactory sexual intercourse. All 5 incontinent women who had underwent sling procedure were continent. In one of 3 nontreated cases, additional sling treatment was indicated 6 months after vaginal reconstruction with satisfactory outcome. One patient with vaginal duplication reported a successful pregnancy with a Caesarean section delivery.Clinical ImplicationsUrinary incontinence with megalourethra in young women, along with the presence of Mullerian anomalies should raise suspicion of urethral coitus. Surgical treatment includes correction of vaginal anomalies and management of consequences.Strengths and LimitationsThis study represents one of the largest series for urethral intercourse, with assessment of psychosexual outcome. The limitation is the lack of statistical analysis due to small sample size.ConclusionUrethral intercourse is very rare, but it can cause severe consequences. It is important to recognize this occurrence and treat it by well-known vaginal or urethral reconstructive procedures.Djordjevic ML, Bizic M, Stojanovic B, et al. Treatment of Urethral Intercourse and Impact on Female Sexual Function. Sex Med 2022;10:100534.

Cervical and Vaginal Duplication with a Normal Uterus — is that Possible?

Cervical and Vaginal Duplication with a Normal Uterus — is that Possible?

Multiple Genitourinary Malformations Associated with Malformations of Other Organs – As a Rarity of Case Report

Congenital anomalies are not uncommon. Urogenital disorders can be isolated or associated with other malformations, but they are usually misdiagnosed at birth and found in physical examination or as urologic signs at adolescence. This case report presents a rare case of a 3-year-old female patient with multiple malformations including ectopic anus, crossed fused ectopic kidney, vaginal duplication, bladder duplication, and multiple organ malformations. Prenatal diagnosis, a detailed physical examination and early surgical treatment are particularly important for prevention of further complications. In this case report, the surgical approach was performed using the cutback technique for rectovaginal separation among other surgical procedures in order to achieve functionality.

Diagnosis and surgical management of Herlyn-Werner-Wunderlich syndrome

Abstract Abdominal pain in the adolescent population is rarely attributed to anatomic variance. Minimal consideration placed on this pathology can present a diagnostic dilemma and delay in care if such options are not investigated. Our case report describes the workup, step-by-step imaging and multidisciplinary approach to a twelve-year-old female who presented with abdominal pain at an outside facility, where she was originally diagnosed with a pelvic mass seeming to arise from the left ovary on ultrasound. Subsequent CT at the authors’ institution indicated a hydrometrocolpos and possible uterine and vaginal duplication; exam under anesthesia revealed uterine didelphys with obstructed left hemivagina. MRI confirmed the diagnosis of Herlyn-Werner-Wunderlich Syndrome and return to the operating room resulted in successful evacuation of the obstructed hemivagina by vaginal septum resection. Post-operatively, she is doing well, menstruating without difficulty, and has not had recurrence of symptoms. This case elucidates the need for consideration of Mullerian duct anomalies in female pediatric patients presenting with lower abdominal pain.

Laparoscopic anterior and posterior native tissue repair: a new pelvic floor approach

Introduction: Traditionally, a cystocele caused by a midline defect of the pelvic fascia is treated by vaginal fascia duplication, also known as anterior colporraphy. The rectocele is managed by suturing the posterior fascia and, frequently, the levator ani muscles. We developed the approach of laparoscopic anterior and posterior fascia repair by native tissue.Material and methods: The methods were based on anterior and posterior exposure of pelvic fascia similar to the preparation of an extended sacral colpopexy. The fascia was compressed and narrowed by absorbable woven sutures, size 1. Twenty-seven patients were followed up for 6–13 months. All patients received additional apical fixation by pectopexy.Results: In the examination group, 13 patients underwent anterior laparoscopic fascia repair and 23 had posterior repair. We detected one apical and one posterior relapse, and also one in the anterior repair group. The patient with the apical relapse reported pain and de novo urgency. Anatomical reconstruction was achieved in all other patients.Summary: Laparoscopic anterior and posterior native tissue repair appears to be a feasible method for the treatment of midline cystocele and rectocele. No new risks were observed. The technique leaves no scar in the vagina and is well accepted.Abbreviations: POPQ: Pelvic Organ Prolapse Quantification System; FDA: Food and Drug Association; US: United States; Fig: Figure; ICIQ: International Consultation on Incontinence Questionnaire

Complete duplication of bladder, urethra, clitoris and vagina associated with bilateral ureteral reflux in an old female patient: unique case.

Complete duplication of the bladder, urethra, vaginal and clitoris is an extreme- ly rare congenital anomaly that is usually diagnosed at pediatric age. We report an unusu- al case of a complete bladder, vaginal, clitoris and urethra duplication occurred in an old female patient (81 years old). This case highlighted the fact that an anatomical anomaly should be considered in all patients with persistent urinary tract problems, especially in old women, due to patients' reluctance to consult physicians for this kind of problem. (Urologia 2009; 76: 258-9)

Adult Gli2+/-;Gli3Δ699/+ Male and Female Mice Display a Spectrum of Genital Malformation.

Disorders of sexual development (DSD) encompass a broad spectrum of urogenital malformations and are amongst the most common congenital birth defects. Although key genetic factors such as the hedgehog (Hh) family have been identified, a unifying postnatally viable model displaying the spectrum of male and female urogenital malformations has not yet been reported. Since human cases are diagnosed and treated at various stages postnatally, equivalent mouse models enabling analysis at similar stages are of significant interest. Additionally, all non-Hh based genetic models investigating DSD display normal females, leaving female urogenital development largely unknown. Here, we generated compound mutant mice, Gli2+/–;Gli3Δ699/+, which exhibit a spectrum of urogenital malformations in both males and females upon birth, and also carried them well into adulthood. Analysis of embryonic day (E)18.5 and adult mice revealed shortened anogenital distance (AGD), open ventral urethral groove, incomplete fusion of scrotal sac, abnormal penile size and structure, and incomplete testicular descent with hypoplasia in male mice, whereas female mutant mice displayed reduced AGD, urinary incontinence, and a number of uterine anomalies such as vaginal duplication. Male and female fertility was also investigated via breeding cages, and it was identified that male mice were infertile while females were unable to deliver despite becoming impregnated. We propose that Gli2+/–;Gli3Δ699/+ mice can serve as a genetic mouse model for common DSD such as cryptorchidism, hypospadias, and incomplete fusion of the scrotal sac in males, and a spectrum of uterine and vaginal abnormalities along with urinary incontinence in females, which could prove essential in revealing new insights into their equivalent diseases in humans.

Congenital renal anomalies in cloacal exstrophy: Is there a difference?

Cloacal exstrophy (CE) is the most severe manifestation of the epispadias-exstrophy spectrum. Previous studies have indicated an increased rate of renal anomalies in children with classic bladder exstrophy (CBE). Given the increased severity of the CE defect, it was hypothesized that there would be an even greater incidence among these children. The primary objective was to characterize renal anatomy in CE patients. Two secondary objectives were to compare these renal anatomic findings in male and female patients, and female patients with and without Müllerian anomalies. An Institutional Review Board-approved retrospective review of 75 patients from an institutional exstrophy database. Data points included: age at analysis, sex, and renal and Müllerian anatomy. Abnormal renal anatomy was defined as a solitary kidney, malrotation, renal ectopia, congenital cysts, duplication, and/or proven obstruction. Abnormal Müllerian anatomy was defined as uterine or vaginal duplication, obstruction, and/or absence. The Summary Table presents demographic data and renal anomalies. Males were more likely to have renal anomalies. Müllerian anomalies were present in 65.7% of female patients. Girls with abnormal Müllerian anatomy were 10 times more likely to have renal anomalies than those with normal Müllerian anatomy (95% CI 1.1-91.4, P=0.027). Patients with CE had a much higher rate of renal anomalies than that reported for CBE. Males and females with Müllerian anomalies were at greater risk than females with normal uterine structures. Mesonephric and Müllerian duct interaction is required for uterine structures to develop normally. It has been proposed that women with both Müllerian and renal anomalies be classified separately from other uterine malformations on an embryonic basis. In these patients, an absent or dysfunctional mesonephric duct has been implicated as potentially causal. This provided an embryonic explanation for uterine anomalies in female CE patients. There were also clinical implications. Women with renal agenesis and uterine anomalies were more likely to have endometriosis than those with isolated uterine anomalies, but were also more likely to have successful pregnancies. Males may have had an analogous condition with renal agenesis and seminal vesicle cysts. Future research into long-term kidney function in this population, uterine function, and possible male sexual duct malformation is warranted. Congenital renal anomalies occurred frequently in children with CE. They were more common in boys than in girls. Girls with abnormal Müllerian anatomy were more likely to have anomalous renal development. Mesonephric duct dysfunction may be embyologically responsible for both renal and Müllerian maldevelopment.

Complexities of Müllerian Anatomy in 46XX Cloacal Exstrophy Patients

Study ObjectiveTo characterize Müllerian anatomy in 46,XX cloacal exstrophy patients. DesignRetrospective review of prospectively maintained, institutionally approved exstrophy-epispadias-cloacal exstrophy database. SettingTertiary care, high-volume exstrophy center (Division of Pediatric Urology, The Johns Hopkins Hospital, Baltimore, Maryland). ParticipantsWe included 31 patients who were genetically female with cloacal exstrophy for whom records included detailed evaluation of Müllerian anatomy. InterventionsNone. Main Outcome MeasuresMüllerian structures, method of evaluation, management, and sexual activity. ResultsOf our patients, 12.9% (3/31) had no identified abnormalities. Vaginal anatomy was described for 30 patients; 3/30 had vaginal agenesis, 14/30 had a single vagina, and 13/30 had vaginal duplication. Of 14 patients with 1 vagina, 5 had atresia/hypoplasia, and 1 had a lateral displacement. One patient with 2 vaginas also had distal atresia. Of the cervices evaluated, 9/14 were duplicated (2/9 with a solitary vagina), and 19/27 of the uteri were duplicated (6/22 with 1 vagina, 1/22 with no vagina). Five patients required imaging to fully characterize their anatomy, and 7 patients had studies that failed to identify Müllerian structures seen in the operating room or on physical examination. Common reconstructive surgeries included vaginoplasties, incisions of vaginal septa, colporrhaphies, and hysterectomies. Sexual activity was confirmed for 3 patients, 2 of whom had conceived. ConclusionMost female cloacal exstrophy patients exhibit abnormalities of the Müllerian system. Axial imaging and ultrasound are helpful diagnostic adjuncts but do not replace careful physical examination and assessment in the operating room. Further studies of sexual activity and fertility are warranted.

Differentiating Between Septate and Bicornuate Uterus: Bi-dimensional and 3-Dimensional power Doppler Findings

Study ObjectiveTo assess if any difference could be found in uterine vascularization between septate and bicornuate uterus. DesignPilot study (Canadian Task Force classification II-2). SettingUniversity hospital infertility clinic. PatientsOne hundred nine women with complete duplication of the uterine cavity. All had already received the final diagnosis of the type of uterine malformation, either septate uterus or bicornuate uterus, via diagnostic hysteroscopy and laparoscopy. Another group of 10 patients with uterine anomalies and affected by ovarian mass were also evaluated via intravenous contrast medium–enhanced ultrasound examination. InterventionsPatients were evaluated using 2-dimensional and 3-dimensional power Doppler imaging of the uterus. Measurements and Main ResultsSeventy-three patients with septate uterus, with or without cervical and vaginal duplication, and 36 patients with bicornuate uterus were selected for inclusion in the study. Irregular vascular networks were detected between the 2 hemicavities in the patients with septate uterus. All bicornuate uteri showed a peculiar vascular network between the 2 hemicavities: the main recognizable vessels formed a network depicting the Greek letter γ at the level of the uterine midline. Detection of the γ sign can be used to differentiate septate from bicornuate uterus. This finding was also confirmed in patients who underwent intravenous contrast medium–enhanced ultrasound examination. Conclusionspower Doppler provides a new and uninvasive tool for differentiation of septate from bicornuate uterus.

DNA Copy Number Variations in Patients with Persistent Cloaca

Persistent cloaca is a devastating female anomaly associated with renal insufficiency/failure, urinary and fecal incontinence and müllerian dysfunction. Genetically engineered murine models of persistent cloaca suggest that this anomaly could have a genetic component in humans. Genomic copy number variations account for previously unexplained genetic diseases by identifying candidate genes in various disorders. We assessed whether novel copy number variations are present in patients with persistent cloaca. With institutional review board approval we performed a retrospective chart review to identify patients with persistent cloaca. Lymphocyte DNA was prospectively tested by whole genome array comparative genomic hybridization. HHAT was Sanger sequenced from genomic DNA. At study recruitment mean age was 12 years (range 0.5 to 23) in 17 females with cloaca. Seven females (41%) had a solitary functioning kidney and 2 each had renal insufficiency and renal replacement therapy. The common cloaca channel was 1.5 to 6 cm long in 6 newborns. Six patients (35%) had vaginal duplication and 4 had spinal anomalies. Array comparative genomic hybridization revealed copy number variations in 7 patients (41%), including 5 gains and 2 losses. Two copy number variations were novel, including a paternally inherited duplication on 16p13.2 and a de novo deletion on 1q32.1q32.3. Subsequent sequencing of the candidate gene HHAT identified no causal mutations. Persistent cloaca is a rare but morbid birth defect. Copy number variations are common in these females but HHAT mutations are not common. Further investigation of these genomic rearrangements may lead to the identification of genetic causes of persistent cloaca.

Septate uterus with cervical and vaginal duplication: A rare Mullerian malformation

A rare case of Mullerian anomaly in a 26-year-old woman who presented with recurrent pregnancy loss and was found to have a longitudinal vaginal septum with cervical duplication and two endometrial cavities separated by a complete septum. The diagnosis of this unusual anomaly was aided by magnetic resonance imaging and was confirmed by hysterolaparoscopy. It was concluded that hysteroscopy aided in the diagnosis and treatment of this Mullerian defect with the highest quality of precision. Hysteroscopy therefore is considered gold standard in the management of septate uterus, thereby helping to regain the anatomical normality and because of its minimally invasive nature, it improves the reproductive outcome.

Cloacal Malformation

Cloacal malformation is a rare but important anomaly. Prenatal diagnosis is possible with knowledge of the distinctive imaging features. The purpose of this case series is to illustrate characteristic prenatal sonographic and magnetic resonance imaging features of cloacal malformation using imaging from 6 cases seen at a single academic center to augment published data. The imaging feature common to all cases was a central cystic pelvic mass containing a characteristic fluid-fluid level. Additional anomalies include uterine and vaginal duplication, hydronephrosis, and lumbosacral anomalies. Prenatal magnetic resonance imaging showed the absence of the normal T1-hyperintense meconium-filled rectum in all cases. Prenatal diagnosis may affect immediate neonatal care (eg, immediate drainage of hydrocolpos) with an ultimate improved outcome.

M499 REPRODUCTIVE FUNCTION OF WOMEN WITH GENITAL ANOMALIES

I. Gonadal dysgenesys: Streak gonad, Ovaryan and Testicular dysgenesys, Ovotestis. II. Uterovaginal anomalies: 1. vaginal aplasia: utero-vaginal aplasia (MRKH syndrome) 2. compete cervico-vaginal aplasia and partial vaginal aplasia 3. unicornuate uterus: with functional uterine horn, unfunctional uterine horn, without horn 4. uterus duplex: symmetric form with duplication of vagina, asymmetric with aplasia of hemivagina 5. bicornuate uterus: complete, incomplete forms and arcuate uterus 6. septate uterus: complete septum with vaginal duplication, incomplete septum 7. fallopian tubes anomaly: absence of tube, additional fimbria. III. External genitalia virilization: I-V degree by Von Prader. Conclusions: The systematic analysis of all clinical cases, allows to suggest new systematyzation and upgrade clinico-morphological classification for optimize the appropriate surgical correction of female patients with various genital malformations.

Covered exstrophy with anorectal malformation and vaginal duplication

Covered exstrophy is a rare variant of the exstrophy-epispadias complex. We report a female newborn with covered exstrophy, absent anal opening and duplication of the introitus and the lower vagina. This rare, previously unreported, combination of anomalies highlights the complexity of the embryological events in the caudal area during separation of the hindgut and allantois.

Complete Duplication of Bladder, Urethra, Clitoris and Vagina Associated with Bilateral Ureteral Reflux in an Old Female Patient: Unique Case

Complete duplication of the bladder, urethra, vaginal and clitoris is an extremely rare congenital anomaly that is usually diagnosed at pediatric age. We report an unusual case of a complete bladder, vaginal, clitoris and urethra duplication occurred in an old female patient (81 years old). This case highlighted the fact that an anatomical anomaly should be considered in all patients with persistent urinary tract problems, especially in old women, due to patients’ reluctance to consult physicians for this kind of problem.

“Perineal Canal”: A Rare Anorectal Malformation of Variable Complexity

Perineal canal (PC) is a fistulous tract that communicates the anus with the vulvar vestibule. Our aim is to show the variable spectrum of complexity of this caudal malformation. Patient 1 had a single urogenital orifice and a normal anus. After sigmoidostomy, a uterovaginal duplication was discovered upon opening the canal that was repaired at puberty. Patient 2 had a single urogenital orifice, a huge dilatation of a single vagina and an apparently normal anus. During perineal operation without diversion, the true anus was found which opened into the PC. Patient 3 had a single urogenital orifice, a uterovaginal duplication and an apparently patent anus. After colonic diversion, the PC was taken down revealing a normal urethral opening, the absence of an anus and a high rectovaginal fistula at the intervaginal wall. A posterior sagittal approach allowed extensive mobilization of the fistula and the rectum, fashioning of a single vagina and positioning of the rectum within the striated muscle complex. The cosmetic results were satisfactory. Patients 1 and 2 have normal continence and a near-normal perineal anatomy. Patient 3 is too young to assess continence. 1) Perineal canal is a malformation that may involve the genitourinary system as well as the rectum and perineum. This developmental disturbance of the caudal end is profound as judged by the vaginal duplication and the nearly cloacal pattern of some cases. 2) Repair may be as challenging as that of a regular cloaca requiring an individualized approach.

Uterine didelphys with duplicated upper vagina and bilateral lower vaginal agenesis: a novel Müllerian anomaly with options for surgical management

To describe a novel Müllerian anomaly and management options. Case series report. Metropolitan tertiary care children's hospital. Three caucasian girls, aged 12, 13, and 15 years, with a novel congenital anomaly. Interval staged vaginoplasties. Functional vagina. Didelphys Müllerian development with vaginal duplication and agenesis of both lower vaginas with staged vaginoplasties resulting in normal functional vaginas. Staged pull-through vaginoplasties led to functional outcome in this unusual anomaly.