Two orifices in the perineum of a girl with imperforate anus: possibility of uterovaginal agenesis associated with rectovestibular fistula.

The authors describe 4 cases of rectovestibular fistula associated with uterovaginal atresia. The diagnosis of the associated uterovaginal atresia was missed in the neonatal period in all 4. It was diagnosed in 2 cases at puberty while investigating for amenorrhea; the other 2 were discovered during the repair of anorectal malformation in early childhood. A variety of techniques were used successfully for vaginal reconstruction. The authors believe that the incidence of such an association is very similar to that of imperforate anus with rectovaginal fistula and therefore suggest that if there are 2 orifices in the perineum of a girl with imperforate anus, it could be either a rectovaginal fistula or a rectovestibular fistula with uterovaginal agenesis. They recommend that all such neonates have an endoscopy or a magnetic resonance imaging done to distinguish between the 2 conditions. This distinction is of importance because the latter would require a concomitant vaginal reconstruction procedure along with anorectoplasty.