Four orifices in the perineum of a girl with imperforate anus : uterus didelphys with rectovestibular fistula or variant cloaca

Abstract EN

In this report, we describe the case of two girls who had imperforate anus with four orifices in the perineum.

First case, 11-month-old girl represented with imperforate anus and rectovestibular fistula and a colostomy done previously.

Second case, 18-month-old girl had an iatrogenic anovaginal fistula after anorectolplasty.

In both cases, the clinical examination and other investigations showed an imperforate anus with rectovestibular fistula, wide urethral meatus, and uterus didelphys.

This malformation was combined in the both cases with congenital pouch colon.

For the first girl, posterior sagittal anorectoplasty was done with an anastomosis of the two vaginas at the midline in one stage, whereas recto-vaginal fistula repair and vaginal anastomosis were done for the second child.

The colostomy closure was done 3 months later and no complications were reported.

The precise clinical examination and other investigations are very important to diagnose the genital malformations that are associated with the anorectal malformations and to treat them in one stage.

And the question remains: is the described anomaly in this report auterus didelphys with a rectovestibular fistula or is it a variant cloaca associated with uterus didelphys and congenital pouch colon whereas the urethral meatus, hemi-vaginal orifices and rectal fistula are conjoined in the very wide and short urogenital sinus ?.