Multiple Fibroids in a Postmenopausal Woman with Mayer Rokitansky Kuster Hauser Syndrome

Abstract

The Mayer Rokitansky Kuster Hauser (MRKH) syndrome is a congenital abnormality of the genital tract which occurs in approximately 1 in 5000 women. It is characterized by a maldevelopment of the Mullerian ducts at their lower portion, which results in congenital absence of the vagina and either the absence of uterine tissue or the presence of two uterine anlage, which are nonfunctioning myometrial tissue lying laterally on the pelvic side walls. Finally, a small redundant piece of uterine tissue may lie in the midline, but it is again nonfunctional. Leiomyomata are common occurrences in women with normal uteri but the occurrence of fibroids in MRKH syndrome is a rarely reported finding. There are only 15 reported cases in the existing medical literature.1–15 This case is unique in that it occurred in a postmenopausal woman and this has never been previously described.