In retrospect, our patient was diagnosed to have a congenital VVF with a variant of mesonephric duct-induced müllerian deformity type II anomaly.[1
] She had a solitary crossed renal ectopia, bicornuate uterus with a rudimentary right horn and associated distal vaginal transverse septum.
Congenital VVF is a rare condition with less than 20 cases reported to date.
This patient presented to us with continuous urinary leakage with a probable diagnosis of VVF. Considering the history of cyclical hematuria before excision of the obstructing transverse vaginal septum, the VVF should be considered to be a congenital VVF.
We hypothesize that due to the obstructing low transverse septum, the vaginal collection of the menstrual blood found its way through the congenital VVF (path of least resistance) resulting in cyclical hematuria without vaginal menstruation.
Because of the obstructing vaginal septum, before the age of menarche there was no manifestation of urinary leak even though there was a congenital VVF. Urinary leak manifested only after the obstructing septum was excised. If there was no obstructive septum, this patient would have presented with urinary dribbling since childhood.
Persistent urinary leak following the first VVF repair, however, led us to investigate this patient further. Retrograde catheterization detected a previously unsuspected bifid communication between the bladder, vagina and the solitary ureter.
The uretero–vesicovaginal communication can be explained in two ways. The communication could have been a congenital bifid communication  or an iatrogenic communication. An iatrogenic uretero–vesicovaginal communication could have been caused due to close proximity of the ureteric orifice and the congenital VVF, the first attempted VVF repair converting this into a single common channel.
Figure 3 Line diagram depicting the bifid communication of the fistula. Line arrow depicting the fistulous opening above the bladder neck, block arrow depicting the vaginal communication of the solitary ureter and arrowhead depicting the obstructing vaginal septum (more ...)
Failure to detect the entity of uretero–vesicovaginal communication and its intricacy during the initial evaluation probably led to the recurrence of the fistula following the first VVF repair.
An obstructive vaginal septum will conceal a congenital VVF and manifest as cyclical hematuria at menarche. Excision of the septum in this case unmasked the VVF and manifested as urinary leakage.
Similarly, urinary incontinence occurred only after the obstructing vaginal septum was excised in a patient who had a bizarre communication between the ectopic ureter and the right uterine cervix of an uterine didelphys. The ectopic ureter opened into the bladder neck and its proximal end communicated through a duct-like structure with the right uterine cervix.[2
Congenital VVF has been commonly detected when menouria is associated with an obstructing vaginal septum.[2
] Various types of unusual congenital VVF have been described in the reports.[2
Obstructive genital lesions, although innocuous by themselves, have sexual, reproductive and menstrual sequelae as seen in our patient who developed endometriosis possibly due to retrograde menstruation. A transverse vaginal septum (American fertility society class IIA anomaly) is more common in the upper 1/3rd
of the vagina, but may occur at any level in the vagina, and 14% of the septae are seen in the lower vagina.[7
Among women with unilateral renal agenesis, mullerian anomalies are seen in 25–50% and musculoskeletal defects in 14%.[1
] These associations were also seen in this patient.
A history of cyclical hematuria (menouria) without vaginal menstruation should alert the clinician to the possibility of obstructive vaginal septum with congenital VVF. Considering this rare entity, imaging and contrast studies are warranted to delineate the unique communications that are possible between the genital and the urinary tracts. Although anatomic delineation of the fistula was possible with magnetic resonance imaging, the unique defect in our case was detected only on retrograde cannulation (catheterization). The vaginal 3-swab test of Moir is typically used to differentiate a VVF from a uretero vaginal fistula and was not performed in this case as the patient had a stenotic vaginal introitus at her first visit to our institution and also as she was unwilling for vaginal manipulation because she was sexually inactive. This test would have helped to diagnose the vesicovaginal component of the uretero–vesicovaginal communication. However, the unusual uretero–vesical component would have remained undiagnosed.
This report highlights the diagnostic and therapeutic challenge that a congenital VVF with obstructing transverse vaginal septum and subsequent recognition uretero–vesicovaginal communication can pose to a clinician. This case highlights the intricacies with reference to diagnosis and management of such cases.
Increasing awareness of this rare entity will have a positive impact in future recognition and a more meticulous evaluation before any surgical intervention in patients with complex genitourinary anomalies will decrease the morbidity and improve the quality of life of the patient.
It is therefore essential to review the lessons learnt from this case. An awareness of the rare possibility of a congenital VVF if there is a history of cyclical menouria is necessary. The presence of a VVF does not rule out other unusual uretero–vesicovaginal communications. In the absence of vaginal menstruation, an exploration for an obstructive vaginal anomaly is essential and it is imperative to identify the ureteric orifice before undertaking any surgical intervention.