A Case of Mayer-Rokitansky-Küster-Hauser Syndrome: Case Report of a 22-Year-Old Undergraduate with Primary Amenorrhoea

Amenorrhea in a woman of reproductive age can be sporadic, temporary or permanent. It can result from lesion affecting the hypothalamic-pituitary-ovarian axis, uterus and vagina. A woman is said to have primary amenorrhoea if she fails to attain menarche at the age of fifteen or at thirteen years without evidence of secondary sexual characteristics. The causes of primary amenorrhoea are broadly classified into constitutional delay, anatomical causes such as Mayer-Rokitansky-Küster-Hauser (MRKH) or genetic causes (gonadal dysgenesis) and other physiological causes including isolated deficiency of gonadotropin releasing hormone. MRKH (Mullerian agenesis) is a rare syndromic disorder that usually occurs when there is agenesis or hypoplasia of the paramesonephric ducts (the precursor for the development of the uterus, fallopian tubes and upper two-third of the vagina). We report on a case of a 22-year-old undergraduate with primary amenorrhoea and subsequent diagnosis of MRKH.