The Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome Associated with Anorectal Malformation – Rectovaginal Fistula; A Rare Combination: Case Report
نویسندگان
چکیده
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.
منابع مشابه
Mayer-Rokitansky-Küster-Hauser Syndrome with Imperforate Anus: A Rare Association and an Innovative Surgical Management
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is one of the rare disorder of Mullerian agenesis leading to non-development of uterus and vagina. Its association with anorectal malformation is rare. We report a case of MRKH syndrome with recto-vestibular in a female child. The child had undergone a sigmoid loop colostomy in the neonatal period. On clinical examination of the perineum, a fistula...
متن کاملPregnancy in a case of Mayer-Rokitansky-Küster-Hauser Syndrome Gravidez num caso de Síndrome Mayer-Rokitansky-Küster-Hauser
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome refers to the congenital absence of the upper part (2/3) of the vagina with variable uterine development. In this disorder, infertility may be the most difficult aspect for the patient to accept. This review will describe a rare case of pregnancy in a woman with MRKH syndrome through assisted reproductive
متن کاملSuccessful Surgical Treatment for Vaginal Agenesis: Report of Two Cases of Mayer–Rokitansky–Küster–Hauser (MRKH) Syndrome
Introduction: Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome is a rare disorder in women which identified as agenesis of vagina and hypoplasia or agenesis of uterine. Case presentation: in this study, two female cases (17, and 19 years old) diagnosed with MRKH (first one type 2, and second one type 1) are described. Both patients were presented with amenorrhea, and absence of vagina orifice. On...
متن کاملMayer-Rokitansky-Kuster-Hauser syndrome associated with rectovestibular fistula
A female neonate with two openings in the introitus and an absent anal opening at the anal site presents a diagnostic challenge. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome associated with rectovestibular fistula, though rare, should be kept in mind as a differential diagnosis of this presentation. We present such a case in a one-year-old female child with MRKH syndrome and rectovestibular f...
متن کاملThe Coexistence of Gonadal Dysgenesis With Mayer-rokitansky-küster-hauser Syndrome, and Dandy-Walker Variant
Background: Gonadal dysgenesis, the most common cause of primary amenorrhea, is characterized by absent or underdeveloped ovaries. Although the coexistence of gonadal dysgenesis and Mayer-Rokitansky-Küster-Hauser (MRKH) has been reported, it is still quite infrequent. To the extent that authors searched, just one study reported the association between Rokitansky sequence and Dandy-Walker malfor...
متن کامل