Gynecologic anatomic abnormalities following anorectal malformations repair

• Published in: Journal of pediatric surgery 2018-04, Vol.53 (4), p.698-703
• Main Authors: Vilanova-Sanchez, Alejandra, Reck, Carlos A., McCracken, Kate A., Lane, Victoria A., Gasior, Alessandra C., Wood, Richard J., Levitt, Marc A., Hewitt, Geri D.
• Format: Article
• Language: English
• Subjects: Anal Canal - pathology; Anal Canal - surgery; Anorectal malformation; Anorectal Malformations - surgery; Child; Child, Preschool; Female; Follow-Up Studies; Genital Diseases, Female - diagnosis; Genital Diseases, Female - etiology; Genital Diseases, Female - surgery; Gynecologic complication; Humans; Infant; Perineal body; Perineoplasty; Perineum - pathology; Perineum - surgery; Posterior anorectoplasty reoperation; Postoperative Complications - diagnosis; Postoperative Complications - surgery; Reconstructive Surgical Procedures; Rectovaginal Fistula - diagnosis; Rectovaginal Fistula - etiology; Rectovaginal Fistula - surgery; Retrospective Studies; Treatment Outcome; Vagina - pathology; Vagina - surgery
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2017.07.012

Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes. A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded. During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6years (0.5–12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n=11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n=2, 13.4%), acquired rectovaginal fistula (n=1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n=1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n=4, 26.6%), retained vaginal septum (n=3, 20%) and remnant recto v