WOFAPS 2025 8th World Congress of Pediatric Surgery

Ruijie Zhou ¹, Zhen Zhang ¹, Ya Ma ², Qi Li ¹

¹ Department of General Surgery, Capital Center for Children's Health, Capital Medical University, Beijing, China
² Department of Ultrasound, Capital Center for Children's Health, Capital Medical University, Beijing, China

Introduction

Complete vaginal agenesis in association with uterine agenesis or hypoplasia is usually known as the Mayer–Rokitansky–Kuster-Hauser Syndrome (MRKH). The most common type of anorectal malformation in female patients is a rectovestibular fistula, but concomitant with MRKH it appears to be rare. We describe 2 cases of 2-month-old girls with rectovestibular fistula with MRKH in whom we chose to leave the native rectovestibular fistula to function as a neovagina during laparoscopic-assisted anorectoplasty.

Case Report

2 two-month-old girls were admitted to our hospital due to imperforate anus with rectovestibular fistula. Perineal examination revealed only two openings in the introitus with an absent anus. Abdominal ultrasound revealed absent uterus and vagina. Both ovaries were normal. Hence, the diagnosis of MRKH concurrent with rectovestibular fistula and imperforate anus was made. We chose to leave the rectovestibular fistula in situ for vaginal reconstruction and to perform indocyanine green (ICG) guided laparoscopic-assisted anorectoplasty. Rectosigmoid colon was transected at the level of peritoneal reflection preserving mesenteric vessels of distal part of the rectum, thus native recto-vestibular fistula was left as a neovagina. And final length of the neovagina was approximately 6-8 cm. The operation was completed in approximately 2 hours without complication. Perineum had a very natural appearance with orifices of neovagina and neoanus.

Discussion

For MRKH with anorectal malformation, surgical reconstruction is aimed at creation of a functional neovagina and neoanus with preservation of fecal continence. Laparoscopic-assisted anorectoplasty and vaginal reconstruction was more convenient with less damage and a better cosmetic outcome for the reconstructed perineum. Although the neovaginal function for sexual purposes was hard to evaluate because the patients were too young, we believe performing the anorectoplasty and vaginal reconstruction in a single procedure would help to avoid the fibrosis and scarring created by repeated surgery.