BACKGROUND/PURPOSE: A very unusual anorectal defect consisting of @entity292 and absent vagina is presented. Only two previous isolated reports were found in the literature describing 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 @entity1 with @entity2463 analyzed, eight female cases shared the common anatomic features of @entity292 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 @entity1 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 @entity254 was performed during the main repair. RESULTS: Four @entity1 are continent of urine and have @entity717 . One @entity1 is younger than 3 years and therefore has not been evaluated. One @entity1 has a very poor sacrum and therefore is incontinent. One @entity1 had a primary repair elsewhere and therefore is excluded, and another @entity1 is lost to follow-up. One @entity1 is already sexually active. CONCLUSIONS: It is mandatory to perform a meticulous inspection of the perineum in female @entity1 with @entity2463 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.

@entity292 with absent vagina: a unique XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity254 :: ('MESH:C563598', 'Disease') :: ['anastomosis'] @entity2463 :: ('MESH:C537771', 'Disease') :: ['anorectal malformations'] @entity292 :: ('MESH:D005402', 'Disease') :: ['a vestibular fistula'] @entity717 :: ('MESH:D009069', 'Disease') :: ['voluntary bowel movements']
@entity2463 :: (MESH:C537771,Disease) :: ['anorectal malformation']
