Japanese Clinical Medicine Functioning Endometrium and Endometrioma in a Patient with Mayer-Rokitanski-Kuster-Hauser Syndrome

ABSTR ACT: Mayer-Rokitanski-Kuster-Hauser (MRKH) syndrome is a rare disease. A 27-year-old woman was admitted for primary amenorrhea and cyclic pelvic pain. Magnetic resonance imaging (MRI) revealed bilateral Müllerian remnants with functioning endometrium and a pelvic mass considered to be an endometriotic cyst. Bilateral Müllerian remnants were removed, and right ovarian cystectomy was performed at laparoscopic surgery. Accurate evaluation before the operation and informed consent are necessary to treat patients with MRKH syndrome. KEY WORDS: Mayer-Rokitanski-Kuster-Hauser syndrome, vaginal agenesis, endometriosis CITATION: Kawano et al. Functioning endometrium and endometrioma in a patient with Mayer-Rokitanski-Kuster-Hauser syndrome. Japanese Clinical Medicine 2014:5