A rare case of primary amenorrhea with two etiologies, hypothalamic amenorrhea, transverse vaginal septum, and no hematocolpos.

Firouzeh Ghaffari,Arezoo Arabipoor,Fatemeh Keikha

doi:10.1155/2015/989123

Firouzeh Ghaffari, Arezoo Arabipoor + Show 1 more

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https://doi.org/10.1155/2015/989123

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Abstract

We reported a rare case of hypothalamic amenorrhea and transverse vaginal septum. A 28-year-old woman presented with primary amenorrhea and no complaint of abdominal pain. Laparoscopy revealed a small rudimentary uterus with streak ovaries and a vaginal pouch. The patient with diagnosis of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome was subjected to a vaginoplasty in another fertility center. In our institute, after two courses of estrogen and progesterone, sonography revealed hematocolpos, while, under anesthesia, transverse vaginal septum was resected. Hysteroscopy revealed normal uterine cavity. She became pregnant 5 months postoperatively with controlled ovarian stimulation (COS) in conjunction with intrauterine insemination, and she has two healthy babies now. This case highlights the importance of careful evaluation of all primary amenorrheas. Clinicians should be aware of presence of more than one etiology which causes atypical presentations and accomplishes a systematic strategy for the evaluation of amenorrhea potential to avoid long-term side effects of a misdiagnosis.

Highlights

The recommended evaluation for amenorrhea aimed to divide the reproductive system into its components—the genital outflow tract and uterus, the ovary, the pituitary, and the hypothalamus—and to assess the functional integrity of each, starting at the lowest level and progressing to the higher levels of the system until the cause is found [1]
We reported a case of primary amenorrhea with normal secondary sex by Tanner staging, a transverse vaginal septum, and hypothalamic amenorrhea
Distal occlusions of female genital tract are associated with hematocolpos or hematometra which are manifested with cyclic pelvic pain and amenorrhea [4]

Summary

Introduction

The recommended evaluation for amenorrhea aimed to divide the reproductive system into its components—the genital outflow tract and uterus, the ovary, the pituitary, and the hypothalamus—and to assess the functional integrity of each, starting at the lowest level (the genital outflow tract) and progressing to the higher levels (hypothalamus) of the system until the cause is found [1]. The genital examination is abnormal in 15% of patients with primary amenorrhea [2]. We reported a case of primary amenorrhea with normal secondary sex by Tanner staging, a transverse vaginal septum, and hypothalamic amenorrhea.

Results

Conclusion