Anovestibular fistula with imperforate anus in two adults.

To the Editor: Imperforate anus occurs in one of 2500 to 5000 live births.1 It is reported that the majority of girls with imperforate anus will have a fistula to the perineum, fourchette, or vestibule.1 Anovestibular fistula (AVF) is the most common form of anorectal anomaly in female infants.2 Treatment of these lesions can be by a variety of techniques including anal transposition, or posterior or anterior sagittal anorectoplasty in the neonatal period.2–4 These procedures can be safely performed with or without a diverting colostomy. To our knowledge, only two cases of AVF presenting in adulthood have been described in the literature.5 Posterior and anterior sagittal anorectoplasty were performed in these cases.5 We report anal transposition for AVF for the first time in two adults.

An 18-year-old female patient was referred to our institution with the chief complaint of defecating from the vulvovaginal vestibule. The family recognized the abnormal place of defecation at birth, but no attempt was done for the management of this pathology. There was also a history of intermittent constipation but no history suggestive of intestinal obstruction. However, the patient denied a history of frequent urogenital infections. On physical examination, the patient was found to have imperforate anus and AVF (Figure 1). No additional urogynecologic abnormality was found on detailed physical examination. An abdominopelvic ultrasound did not reveal any abnormality of the urogenital system. Anal transposition without colostomy was performed in the gynecologic position under general anesthesia (Figure 2,3). She had an uneventful postoperative recovery and was discharged on the seventh postoperative day. She was well doing on the 33-month follow-up without any complication. The Cleveland Clinic Incontinence Score (CCIS) was 5 (good continence). She had no complaints about her social life after marriage.

Figure 1

Preoperative image of patient.

Figure 2

Dissection step for anal transposition procedure.

Figure 3

Pull-through step of fistula in to neoanus.

The second case was a 19-year-old female patient referred to our institution with the complaint of defecating from a vulvovaginal vestibule and constipation. Admission for treatment was due to the decision of marriage. The medical history and complaints of the patient were similar to the first case. On physical examination, she was diagnosed with an imperforate anus and AVF. No additional urogynecologic abnormality was found on a detailed physical examination or on abdominopelvic ultrasound. Laboratory and physical investigations showed no other systemic pathologies. An anal transposition procedure without colostomy was performed in the gynecologic position under general anesthesia. She had an uneventful postoperative period and was discharged on the sixth postoperative day. She was doing well on 31-month follow-up with satisfactory continence and perineal cosmesis without any complication. Postoperative infection dehiscence and fistula recurrence did not occur, hence no secondary surgery was necessary. The CCIS scale was 7 (good continence). The patient did not consent to providing information about her social life after marriage.

The number of untreated adult patients with anorectal malformations is so few that only case reports appear in the literature. For this reason, there is no consensus on the surgical management of these adult cases. On the contrary, various operative techniques have been reported for the treatment of patients in the neonatal period with this abnormality. Large case series with various operative techniques have been published in infants. Currently, posterior sagittal anorectoplasty (PSARP) defined by Alberto Pena in 1982, is the most popular and frequently preferred surgical technique for neonatal period. Vijay et al. reported two adult cases with AVF. They performed limited ASARP in one patient and PSARP in the other.5 We performed anal transposition without colostomy in the treatment of both cases. Colostomy may not be necessary in adult patients in the case of optimal fecal passage. Colostomy is a poorly tolerated procedure in adult patients compared with neonatal patients. For this reason, surgical interventions without colostomy is probably a better surgical option in adults with AVF. Single staged anal transposition without colostomy as a minor, safe operation may be the technique of choice for treatment of adults with imperforate anus and anovestibular fistula with satisfactory continence and perineal cosmesis (Figure 4).

Figure 4

Postoperative images of patient 2 years after operation.