Introduction and hypothesis: Congenital vesicovaginal fistula is an exceedingly rare entity. There is no consensus regarding the nature and origin of this condition. We report two cases with congenital vesicovaginal fistula and compile previously reported cases in the English literature. Theories behind the genesis of this anomaly will be briefly presented.
Methods: We describe the presentation, diagnostic workup, and management of two patients with congenital vesicovaginal fistula. Previously reported cases were retrieved through an extensive English literature review using Medline and PubMed. Cases are tabulated based on the presence or absence of vaginal menstrual outflow obstruction.
Results: Two women, aged 23 and 17, had had cyclic hematuria since puberty that was perceived as normal menstruation. One woman presented with an inability to have sexual intercourse, and the other with severe cyclic pelvic pain. Diagnostic workup unveiled congenital vesicovaginal fistula and distal vaginal agenesis in both. One had abnormal ureteric insertion, while the other had a history of anomalies unrelated to the urogenital system. Successful surgical correction of fistula was undertaken in both. An English literature review revealed 23 reported cases of congenital vesicovaginal fistula. While 74 % had concomitant menstrual outflow obstruction, the remaining had normal menstruation per vagina.
Conclusion: Congenital vesicovaginal fistula can present as an isolated anomaly, or associated with complex malformations of a wide spectrum. The presenting symptoms as well as the age at diagnosis vary widely. While the term "congenital" implies its genesis before birth, a congenital vesicovaginal fistula can be a manifestation of faulty embryological development, but also the result of outflow obstruction.