Pitfalls in the Diagnosis and Management of Obstructive Uterovaginal Duplication: A Series of 32 Cases

Abstract

Obstructive uterovaginal duplication is a benign pathology when properly managed. Therefore, management of abdominal pain in peripubertal girls should include systematic abdominal and gynecologic examinations completed by radiologic pelvic and renal evaluation. Surgical treatment should be performed by vaginal approach to avoid infertility. Concerning the origin of the malformation, the high association of prenatal dysplastic kidneys and postnatal absent kidneys suggests anomalies of both wolffian and müllerian duct development, contrasting with the classic definition of this malformation.

A case of didelphic uterus and blind hemivagina with renal dysplasia and ectopic ureter presenting with vulvodynia and recurrent fever

“…Capito et al hypothesize that when an anomaly of the distal part of a Wolffian duct is present, a unilateral anomaly of incorporation of this duct within the bladder ensues, resulting in the anomalous incorporation of the attached ureteric bud within the bladder with subsequent ectopic (vaginal) ureteral ending. This situation would lead to a renal misdevelopment as the formation of the definitive kidney derives from cranial growth of the ureteral bud (the metanephric blastema is induced by the ureteric bud before it incorporates in the posterior wall of the bladder) [9,10].…”

“…If the upper vagina has a Müllerian (paramesonephric) origin, the failure of fusion of both the Müllerian ducts could lead to the malformative duct not reaching the urogenital sinus, thus impeding the resorption of the vaginal plate on the affected side. If the upper vagina has a Wolffian origin, the absence or distal agenesis of a mesonephric duct would result in lack of its opening into the urogenital sinus causing the blind hemivagina [10].…”

“…Another study by Capito et al presents a series of 32 patients in which the median delay of diagnosis after menarche was 9 months (range 1-48 months). When there was a communication between the obstructed and the non-obstructed hemi tract, this period was even longer (median 20 months) [10].…”

“…Therefore, performing an uretero-nephrectomy associated with the resection of the vaginal septum is recommended to prevent postoperative urinary incontinence [2,10]. Some authors suggest an evaluation of the function of the ipsilateral and contralateral kidney in advance-e.g., by intravenous pyelogram or renal scintigraphy-to consider a ureteric reimplantation in case of functional renal parenchymal tissue [19].…”

“…Some authors suggest an evaluation of the function of the ipsilateral and contralateral kidney in advance-e.g., by intravenous pyelogram or renal scintigraphy-to consider a ureteric reimplantation in case of functional renal parenchymal tissue [19]. However, in most cases described in the literature, no or minimal functional kidney is present, probably through a mechanism of dysplasia followed by non-functional hypoplasia [10]. For a blind ectopic ureter, no further procedure is required.…”