Eur J Pediatr Surg 2017; 27(06): 495-502
DOI: 10.1055/s-0036-1593610
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Menstrual, Sexual, and Obstetrical Outcomes after Vaginal Replacement for Vaginal Atresia Associated with Anorectal Malformation

Clare Skerritt1, Alejandra Vilanova Sánchez2, Victoria Alison Lane1, Richard J. Wood1, Geri D. Hewitt1, Lesley L. Breech3, Marc Levitt1
  • 1Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, Ohio, United States
  • 2Cirugía Pediátrica, Hospital Universitario La Paz, Madrid, Spain
  • 3Department of Obstetrics and Gynecology, Pediatrics and Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
Further Information

Publication History

07 June 2016

19 August 2016

Publication Date:
15 November 2016 (eFirst)


Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence.

Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile–vaginal intercourse.

Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births.

Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.