Vaginal malformations: a proposed classification based on embryological, anatomical and clinical criteria and their surgical management (an analysis of 167 cases)

Congenital urethrovaginal fistula is an extremely rare genitourinary anomaly. Literature search identified only five reported cases, all of which were associated with urogenital abnormalities. Transverse vaginal septum is another rare condition, resulting from abnormalities in the vertical fusion between the vaginal components of the Mullerian ducts and the urogenital sinus; and associated fistulous connection of the vagina with the urethra is even rarer. Herein we describe the case of a 35-year-old woman who presented with dyspareunia, and a 1-year history of infertility, who was found to have a urethrovaginal fistula with low transverse vaginal septum. The patient was successfully treated with excision of the septum and closure of the urethrovaginal fistula.

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“…This theory could explain the development of high CUVF. In conclusion, however, the developmental process of CUVF remains unknown …”

Section: Discussionmentioning

confidence: 91%

“…Occasionally, patients will present with vaginal voiding or “pseudo‐incontinence”, due to accumulation of urine within the vaginal vault. These patients will leak when rising from a seated position after voiding …”

Section: Discussionmentioning

confidence: 99%

Congenital urethrovaginal fistula with transverse vaginal septum

Amer

Ahmed

Ali

2016

J of Obstet and Gynaecol

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“…This could be due to the vaginal orifice acting as a valve causing backflow of urine into the vagina during micturition [3,4]. In a case series of 167 vaginal malformations, high urogenital sinus was most commonly associated with congenital adreno-genital syndrome [5]. In addition to persistent UGS, hydrometrocolpos has also been associated with other anomalies such as ano-rectal malformations, vaginal atresia and cloacal anomalies [3].…”

Section: Discussionmentioning

confidence: 99%

“…Low UGS can be successfully treated with a simple flap vaginoplasty. Intermediate UGS, characterized by a higher confluence of the urethra and vagina, but with acceptable urethral length (greater than 1.5 cm), is suitable for en bloc or total mobilization without jeopardizing urinary continence [1,2,4,5]. For high UGS, surgical correction via the anterior sagittal transanorectal approach (ASTRA) has been associated with less fecal or urinary incontinence [14].…”

Section: Discussionmentioning

confidence: 99%

Urinary ascites due to persistent urogenital sinus: A case report and review of literature

Loganathan

Kamaluddeen

Soraisham

2014

Journal of Neonatal-Perinatal Medicine

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BACKGROUND: Persistent urogenital sinus is one of the rare urogenital anomalies, which commonly presents as hydrometrocolpos. Fetal urinary ascites as a presentation of persistent urogenital sinus is extremely rare. CASE REPORT: We report on a preterm infant with antenatal diagnosis of hydrometrocolpos and massive urinary ascites secondary to urogenital sinus without any bladder or renal abnormalities. CONCLUSION: This case report emphasizes the importance of maintaining a high index of suspicion in the diagnosis of persistent urogenital sinus especially in infants presenting with urinary ascites along with hydrometrocolpos.

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“…Cryptorchidism, hypospadias, and incomplete scrotal sac fusion are the most common congenital complications in males [2, 3]. In females, disorders of sexual development typically present as congenital uterine anomalies, with particular cases such as vaginal duplication threatening child delivery [4, 5]. Despite the clinical significance of such anomalies in both males and females, there is a lack of equivalent animal models.…”

Section: Introductionmentioning

confidence: 99%

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

Akbari

et al. 2016

PLoS ONE

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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.

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Vaginal malformations: a proposed classification based on embryological, anatomical and clinical criteria and their surgical management (an analysis of 167 cases)

Abstract: An early diagnosis is desirable to correct adequately vaginal malformations, which becomes mostly evident around puberty. The better timing for surgery is early age, to obtain better results and to avoid many of the psychological problems that arise at a later age.

Cited by 53 publications

References 13 publications

Congenital urethrovaginal fistula with transverse vaginal septum

Congenital urethrovaginal fistula with transverse vaginal septum

Urinary ascites due to persistent urogenital sinus: A case report and review of literature

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

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