The Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome Associated with Anorectal Malformation ? Rectovaginal Fistula; A Rare Combination: Case Report

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by either isolated uterovaginal agenesis or associated with other organ anomalies in genetic women with normal development of secondary sexual characteristics. It affects at least 1 out of 4500 Women. We report an uncommonly seen unique sub type of MRKH syndrome, co-existing with imperforate anus and rectovaginal fistula (RVF) in a 13 year old adolescent girl presented with passage of stool through introitus. We have discussed in brief the management including vaginal reconstructive surgery and detailed counseling regarding the potentials of coitus and fertility issues