Recto-Anovestibular Fistula With Mayer-Rokitansky-Küster-Hauser Syndrome: A Case Report of a Patient Treated With One-Stage Anterior Sagittal Anorectoplasty and Vaginoplasty Using Small Bowel Graft

Abstract

Herein, we report the case of a girl with a rectovestibular fistula and vaginal agenesis, who underwent one-stage anterior sagittal anorectoplasty and vaginoplasty using small bowel graft. She was born without an anus; thus, colostomy was carried out 2 days after birth. She was introduced to our hospital at the age of 9 months. Vaginal agenesis was found in the contrast examination before the surgery of rectovestibular fistula. Laparoscopy confirmed a complete vaginal agenesis and two hypoplastic uterus horns with rudimentary fallopian tubes. Her ovaries and karyotype were normal. On the basis of these findings Mayer-Rokitansky-Küster-Hauser syndrome was diagnosed. At the age of 1 year and 11 months when her weight increased sufficiently for surgical operation, anterior sagittal anorectoplasty and vaginoplasty using small bowel tissue were performed simultaneously. Female patients with vestibular fistula often also show gynecological organ malformations. Vaginal abnormality is difficult to diagnose on the basis of appearance alone. A vaginal contrast examination should be performed routinely as part of any treatment regimen for rectovestibular fistulas. One-stage anterior sagittal anorectoplasty and vaginoplasty using small bowel graft is a useful strategy for rectovestibular fistula.