Al Dandan Omran, Hassan Ali, Alsaihati Amna, Aljawad Lujain, Almejhim Fathiya
Imam Abdulrahman Bin Faisal University, King Fahd Hospital of the University, Al-Khobar, Saudi Arabia.
Case Rep Womens Health. 2019 Aug 6;24:e00137. doi: 10.1016/j.crwh.2019.e00137. eCollection 2019 Oct.
Primary amenorrhea is failure to reach menarche. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea, after gonadal dysgenesis. Herein, we report a rare case of an unusual subtype of MRKH syndrome.
This report describes the case of a 28-year-old woman with primary amenorrhea and coital difficulties. On examination, she had normal secondary sexual characteristics but a blind-ending vagina. Her hormonal profile was within normal limits; ultrasound examination was inconclusive due to technical difficulties. Subsequently, she underwent pelvic magnetic resonance imaging, which revealed bilateral rudimentary uterine horns in the presence of normal ovaries and normal length of a blind-ending narrow vagina.
MRKH syndrome with bilateral rudimentary uterine horns and normal-length vagina is an unusual form of Müllerian agenesis.
原发性闭经是指月经初潮未出现。迈耶-罗基坦斯基-库斯特-豪泽（MRKH）综合征是继性腺发育不全之后，导致原发性闭经的第二大常见原因。在此，我们报告一例罕见的MRKH综合征不寻常亚型病例。
本报告描述了一名28岁原发性闭经且性交困难的女性病例。检查发现，她的第二性征正常，但阴道盲端。其激素水平在正常范围内；由于技术困难，超声检查结果不明确。随后，她接受了盆腔磁共振成像检查，结果显示双侧子宫残角，卵巢正常，阴道盲端狭窄且长度正常。
伴有双侧子宫残角和正常长度阴道的MRKH综合征是苗勒管发育不全的一种不寻常形式。
