Association of the rectovestibular fistula with MRKH Syndrome and the paradigm shift in the management in view of the future uterine transplant

Yogesh Kumar Sarin

doi:10.47338/jns.v9.551

Abstract

Uterine transplantation in Mayer-Rokitansky-Küster-Hauser (MRKH) patients with absolute uterine function infertility have added a new dimension and paradigm shift in the management of females born with rectovestibular fistula coexisting with vaginal agenesis. The author reviewed the relevant literature of this rare association, the popular and practical classifications of genital malformations that the gynecologists use, the different vaginal reconstruction techniques, and try to know what shall serve best in this small cohort of these patients lest they wish to go for uterine transplantation in future.

Highlights

In a female newborn, a single perineal opening with shorter appearing introitus suggests cloaca
Rectovestibular associated with vaginal atresia is a rare variant of anorectal malformations in females with little more than 100 cases have been reported globally
A lot of literature is available about non-surgical and surgical treatment of vaginal atresia associated with MRKH syndrome, it is a different scenario when it coexists with rectovestibular fistula

Summary

INTRODUCTION

A single perineal opening with shorter appearing introitus suggests cloaca. The MRKH syndrome is regarded as an inhibitory malformation of the Mullerian (paramesonephric) ducts This malformation of the female genital organs presents as a rudimentary solid bipartite uterus with a solid vagina. 64%, 24%, and 12% patients respectively; or in other words, associated extra-genital congenital malformations were present in more than a third of cases.[58] the spectrum of types 1 and 2 of the MRKH syndrome is known to vary across different races and geographical locations; the incidence of MURCS association was only 3% in a large cohort of. Associated Malformation) classification in the same year 2005.[60] The external and internal female genital organs were divided into the following subgroups in accordance with the anatomy: vagina (V), cervix (C), uterus (U), and adnexa (A). (Reproduced with permission from Oppelt et al Fertil Steril. 2005;84:1493-7)[60]

C Cardiac

McIndoe Procedure

Findings

CONCLUSION