P-196: Atypical Presentations of Primary Amenorrhea with Multifactorial Etiologies, Hypothalamic Amenorrhea with Transverse Vaginal Septum No Hematocolpos: A Case Report

Background: Patients with a transverse vaginal septum generally present at the age of menarche with complain of cyclic pelvic or abdominal pain due to retention of blood above the obstruction. In the absence of menstruation this women did not have typical hematocolpos. Materials and Methods: A rare case of hypothalamic amenorrhea and transverse vaginal septum were reported. A 28- year-old woman presents with primary amenorrhea and no complaint of abdominal pain. Her laboratory testing showed FSH 0.2 mIU/ml and LH 0.3 mIU/ ml. Laparoscopy revealing a small rudimentary uterus with streak ovaries and a vaginal pouch. The patient with diagnosis of Mayer-Rokitansky- Kuster-Hauser syndrome was subject to a vaginoplasty in another infertilty center. When the patient referred to our center, she was suffering amenorrhea, osteopenia and infertility because of misdiagnosis. Results: After two courses of estrogen and progesterone, sonography revealed that hematocolpos under anesthesia transverse vaginal septum was resected. Hysteroscopy revealed normal uterine cavity.She became pregnant five months postoperatively with controlled ovarian hyper stimulation (COH) in conjunction with intrauterine insemination.Conclusion: This case highlights the importance of careful evaluation of all primary amenorrhea. The clinicians should be aware of presence of more than one etiology which cause atypical presentations and accomplish a systematic strategy for the evaluation of amenorrhea potential to avoid long-term effects of a misdiagnosis.