A rare form of Mayer–Rokitansky–Küster–Hauser syndrome associated with ovarian endometrioma: a case report

Abstract

Abstract Mayer–Rokitansky–Küster–Hauser syndrome is a congenital malformation that affects the uterus and upper two-thirds of the vagina. Its prevalence is estimated to be 1 in 4500 live births. We present the case of a 19-year-old patient that presented with primary amenorrhea and cyclic abdominal pain. Upon the exploratory laparoscopy, a right rudimentary uterine horn and left unicornuate uterus were found. These two entities were completely separated from each other and from the vaginal vault. In addition, a left ovarian endometrioma was also found. The unicornuate uterus was resected with an intent to resolve the pain. Endometriosis is known to raise the risk of ovarian cancer by 50%. Therefore, a left salpingo-oophorectomy was performed to minimize the risk of ovarian cancer and endometriosis recurrence. In conclusion, ovarian endometriomas should be suspected when obstructive malformations are present with active endometrial remnants. These lesions should be managed appropriately to optimize the postoperative outcomes.