Vault prolapse in a patient with rokitansky syndrome

Abstract

INTRODUCTION: The Mayer—Rokitansky—Kuster-Hauser syndrome (MRKHS) is a rare congenital cause of primary amenorrhoea, due to agenesis of the uterus and the upper two thirds of the vagina. Since the vaginal vault is not suspended to the bony pelvis it is predisposed to prolapse. We report a case of MRKH syndrome with vaginal vault prolapse successfully treated with surgery. CASE REPORT: A 30 year old female married for two years, who was diagnosed to have MRKHS, presented with vaginal vault prolapse. This prevented her from having satisfactory sexual intercourse. She did not have symptoms of pelvic floor weakness such as urinary or faecal incontinence. An intravenous urograrn did not reveal any anomaly. At laparotomy normal ovaries with follicular activity and a rudimentary uterus were noted. A tape of proline mesh was fixed to the posterior surface of the rudimentary uterus and was brought forward to the rectus sheath through the broad ligament along the round ligament, to support the vaginal vault. The peritoneal cavity was opened through an incision in the vault of the blind vagina. Vaginal vault was anchored to the previously placed proline tape. A fibreglass mould was introduced and kept in place for seven days to keep the integrity of the dilated vaginal lumen. Two months after surgery the patient was asymptomatic with normal sexual activity. DISCUSSION: MRKHS occurs in one in 4000-5000 women. It may be associated with renal and skeletal anomalies. The blind ended vagina is usually short and originates from the urogenital sinus. The vaginal vault is susceptible to prolapse as it is not anchored to the bony pelvis. The incidence of vault prolapse in MRKHS is unknown and thought to be very rare. Many surgical methods have been described including abdominal sacrocolpopexy and sacrospinous fixation.