Rectovestibular fistula with absent vagina: A unique anorectal malformation

Abstract

Background/Purpose: A very unusual anorectal defect consisting of a vestibular fistula and absent vagina is presented. Only two previous isolated reports were found in the literature dascribing a repair that included both abdominal and perineal approach. The authors present another technical alternative that allows the repair via posterior sagittal. Methods: Of 1,007 patients with anorectal malformations analyzed, eight female cases shared the common anatomic features of a vestibular fistula and absent vagina. What appeared to be the vagina was actually the rectal orifice ending in the vestibule, and the vagina was absent. With the posterior sagittal approach, the distal rectum was used to create the neovagina. The proximal rectum was mobilized and placed within the limits of the sphincter mechanism. Two of the eight patients were born with a patent upper third remnant of the vagina. In one case, the remnant was anastomosed to the neovagina at the time of colostomy closure; in the second one, the anastomosis was performed during the main repair. Results: Four patients are continent of urine and have voluntary bowel movements. One patient is younger than 3 years and therefore has not been evaluated. One patient has a very poor sacrum and therefore is incontinent. One patient had a primary repair elsewhere and therefore is excluded, and another patient is lost to follow-up. One patient is already sexually active. Conclusions: It is mandatory to perform a meticulous inspection of the perineum in female patients with anorectal malformations to detect unusual defects. Also, a high index of suspicion is necessary to establish the diagnosis of this defect and avoid an inadequate treatment. The posterior sagittal approach represents another alternative to treat these defects without the need for a laparotomy.