Department of Obstetrics and Gynecology, Turku University Hospital and University of Turku, PL 52, 20521, Turku, Finland.
Department of Radiology, Turku University Hospital and University of Turku, 20521, Turku, Finland.
BMC Womens Health. 2020 Jul 28;20(1):157. doi: 10.1186/s12905-020-01026-1.
Most patients with congenital uterus and vaginal aplasia (i.e., Mayer-Rokitansky-Kuster-Hauser [MRKH] syndrome) have rudimentary pelvic uterine structures that contain smooth muscle. Although leiomyomas and dysplasia of vaginal mucosa are relatively common in the general population, they are rare in MRKH patients. Data on the vulnerability of neovaginas to HPV-associated dysplasia are limited.
A rare case of an MRKH patient with two gynaecological conditions detected during long-term gynaecological follow-up is presented. At the age of 21, the patient was treated for HPV-associated neovaginal dysplasia. At the age of 47, a pelvic leiomyoma was detected with transvaginal ultrasound and confirmed with magnetic resonance imaging.
A Pap smear or human papillomavirus testing is indicated in sexually active MRKH women. Uterine rudiments contain smooth muscle, which facilitates the development of oestrogen-dependent diseases, such as leiomyomas and adenomyosis. Although magnetic resonance imaging is recommended in cases of a pelvic mass, easily attainable and cost-efficient transvaginal ultrasound offers high diagnostic accuracy in patients with a surgically created neovagina and is suitable for the patients' follow-up. Guidelines for the gynaecological follow-up of MRKH patients are warranted.
大多数先天性子宫和阴道发育不全（即 Mayer-Rokitansky-Kuster-Hauser [MRKH] 综合征）患者的盆腔子宫结构都有平滑肌。虽然子宫肌瘤和阴道黏膜发育不良在普通人群中较为常见，但在 MRKH 患者中则较为罕见。关于 HPV 相关发育不良对新阴道易感性的数据有限。
介绍了一位 MRKH 患者在长期妇科随访中发现两种妇科疾病的罕见病例。该患者 21 岁时因 HPV 相关新阴道发育不良接受治疗。47 岁时，经阴道超声检查发现盆腔平滑肌瘤，并经磁共振成像证实。
有性生活的 MRKH 女性应进行巴氏涂片或人乳头瘤病毒检测。子宫残迹含有平滑肌，这有利于雌激素依赖性疾病的发展，如子宫肌瘤和子宫腺肌病。尽管建议对盆腔肿块进行磁共振成像，但对于接受手术重建新阴道的患者来说，易于获得且成本效益高的经阴道超声具有很高的诊断准确性，适合患者的随访。有必要为 MRKH 患者制定妇科随访指南。
