The Mayer Rokitansky Kuster Hauser Syndrome (MRKHS) is characterized by congenital absence of the uterus and the middle to upper thirds of the vagina. Women with this syndrome are genotypically female presenting with primary amenorrhea, female secondary sexual characteristics, normal ovarian function, absent or short blind vagina and the presence of uterine remnants or rudimentary uteri. The occurrence of leiomyoma in a patient with MRKHS arising from the fibromuscular tissues of uterine remnants is very rare. As the mullerian ducts have smooth muscles, the presence of a myoma is a possibility. This paper aims to explain the pathogenesis of leiomyoma arising from the uterine remnants in patients with MRKHS and to present a comprehensive review on the clinical presentation, diagnostic evaluation, and management of this condition. There is paucity of documented cases of leiomyoma associated with MRKHS reported in literature. This is a the first case reported locally.
MAYER ; ROKITANSKY ; KUSTER HAUSER ; SYNDROME ; MULLERIAN ; AGENESIS ; PRIMARY ; AMENORRHEA ; LEIOMYOMA ; UTERI

Conservative surgery is the treatment of choice for pelvic endometriosis. However, one of the most frustrating aspects of treating pelvic endometriosis is disease recurrence. As endometriotic tissue is hormonally sensitive, postoperative cyclic or continuous low dose monophasic oral contraceptive pills may be effective in preventing or delaying recurrence. Our results showed no statistically significant difference between the recurrence rates of patients who were cyclic or continuous OCP users. Symptom and bigger anatomic recurrences were observed among cyclic OCP users. Post operative OCP use and duration of treatment did not significantly influence time to disease recurrence. The risk of recurrence of endometriotic cyst seemed higher among cyclic OCP users. Further, statistically powered randomized controlled trials with bigger sample size, long term use and longer follow up period are necessary to better provide recommendations regarding the OCP administration regimen.
Human ; ENDOMETRIOSIS ; RECURRENCE ; PELVIC PAIN ; LAPAROSCOPY ; CONTRACEPTIVES, ORAL