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