Uterine Adenomyosis in Mullerian Remnant with Transverse Vaginal Septum with Haematocolpos in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Case Report

The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder characterised by failure of the uterus and vagina to develop properly in women who have normal ovarian function and normal external genitalia and secondary sexual characters. Mullerian agenesis accounts for approximately 10% cases of primary amenorrhoea. We report a case of 40-year-old female with MRKH syndrome with primary amenorrhea who presented with lower abdominal pain. She had well developed secondary sexual characters. Her ultrasound showed bulky and heterogeneous uterus like mass with ill defined endometrial-myometrial interface with multiple uterine fibroids. Primary amenorrhea with normal secondary sexual characters and phenotype of 44XX lead us to the diagnosis of MRKH syndrome with uterine myoma in mullerian remnant. Surgical intervention showed the presence of transverse vaginal septum with haematocolpos while the histopathology of the remnant showed adenomyosis. Because of rarity of the condition we report this case.