青春期功能减退及因低促性腺激素性性腺功能减退导致子宫发育不良的原发性闭经。
Pubertal failure and primary amenorrhea with uterine hypotrophy due to hypogonadotropic hypogonadism.
作者信息
Mulayim Baris, Celik Nilufer Y
机构信息
Department of Obstetrics and Gynecology, Baskent University, Alanya, Antalya, Turkey.
出版信息
J Pediatr Adolesc Gynecol. 2009 Apr;22(2):e1-3. doi: 10.1016/j.jpag.2008.01.071.
BACKGROUND
Primary amenorrhea can be a sign of either delayed puberty or Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.
CASE
A virgin 27-year-old woman with pubertal failure, primary amenorrhea, and uterine hypotrophy due to hypogonadotropic hypogonadism sought treatment at our institution. She was diagnosed as having MRKH syndrome 10 years ago at another institution after pelvic ultrasonography revealed no uterus or ovaries. Unfortunately, no further investigations had been made or treatments implemented during the ensuing decade.
SUMMARY AND CONCLUSION
In female patients in whom the uterus cannot be visualized with ultrasonography, magnetic resonance imaging and/or laparoscopy should be considered to ensure that the diagnosis is correct. Besides further imaging, hormonal assessment and breast development should always be initially considered for the diagnosis of delayed puberty and MRKH syndrome.
背景
原发性闭经可能是青春期延迟或 Mayer-Rokitansky-Küster-Hauser（MRKH）综合征的表现。
病例
一名 27 岁处女，因低促性腺激素性性腺功能减退导致青春期发育不全、原发性闭经和子宫发育不良，前来我院就诊。10 年前，她在另一机构经盆腔超声检查未发现子宫和卵巢后，被诊断为 MRKH 综合征。遗憾的是，在随后的十年里，未进行进一步检查或治疗。
总结与结论
对于超声检查无法显示子宫的女性患者，应考虑进行磁共振成像和/或腹腔镜检查，以确保诊断正确。除进一步的影像学检查外，对于青春期延迟和 MRKH 综合征的诊断，激素评估和乳房发育情况最初也应始终纳入考虑。
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