Backgrounds & Aims: Rokitansky-Küster-Hauser (MRKH) syndrome is a rare genetic disease that can be one of the causes of primary amenorrhea in young girls. These patients usually refer with primary amenorrhea despite the normal appearance of the genital tract and normal secondary sexual traits. Although these patients have no other concomitant symptoms, a small percentage of functional endometrial islets are observed. This report investigated a 19-year-old young woman with Rokitansky-Küster-Hauser syndrome with severe endometriosis.
Patient Information: The patient was a 19-year-old woman who referred to the hospital with primary amenorrhea and severe cyclic pain and was diagnosed with uterus larger than normal with vaginal agenesis and cervix in early studies, with primary uterine enlargement and severe endometriosis due to Rokitansky syndrome. So the patient underwent hysterectomy and was a candidate for vaginoplasty.
Conclusion: Functional endometriosis in patients with Rokitansky syndrome is approximately 7-10%, but in the event of primary amenorrhea and symptoms of cyclic pain in the patient, Rokitansky syndrome should be considered and for preventing secondary endometriosis caused by it, cutting off the monthly menstrual cycles or bringing out the uterus containing the endometrium or creating an anastomosis between the functional uterus and vaginal, if it is possible, seem logical techniques.

Keywords: Endometriosis, Mullerian Aplasia, Mayer Rokitansky Kuster Hauser Syndrome

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