Evaluation of Mayer-Rokitansky-Küster-Hauser syndrome with magnetic resonance imaging: Three patterns of uterine remnants and related anatomical features and clinical settings

Objective To characterize the anatomical features and clinical settings of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and correlate them with patterns of uterine involvement.

Pelvic magnetic resonance images and medical records of 92 MRKH patients were retrospectively reviewed. Patients were subgrouped by uterine morphology: uterine agenesis, unilateral rudimentary uterus and bilateral rudimentary uteri. Uterine volume, presence of endometrium, location of ovary, endometriosis and pelvic pain were compared among groups.

The mean uterine volume was 33.5 ml (17.5–90.0 ml) for unilateral uterine remnants, and 16.1 ml (3.5–21.5 ml) for bilateral uterine rudiments (p<0.01). The incidence of presence of endometrium (100% vs. 22%, p<0.001), haematometra (56% vs. 3%, p<0.001) and ovarian endometriosis (22% vs. 3%, p<0.01) was significantly increased in the group of unilateral rudimentary uteri as compared with the group of bilateral uterine remnants. Thirty-one patients (38%) showed ectopic ovaries. Pelvic pain was more common in individuals with unilateral rudimentary uterus than those who had no (56% vs. 5%, p<0.01) or bilateral uterine remnants (56% vs. 14%, p<0.05).

MRKH patients with different patterns of uterine involvement may have differentiated anatomical features and clinical settings. Key Points • Rudimentary uteri, especially bilateral rudimentary uteri, were quite common in MRKH syndrome. • Uterine remnants can be relatively large, especially the unilateral rudimentary uterus. • Presence of endometrium and related complications are not rare in MRKH syndrome. • Existence of endometrium and complications are more frequent in unilateral rudimentary uterus. Similar content being viewed by others Abbreviations - MRKH: - Mayer-Rokitansky-Küster-Hauser syndrome - MRI: - Magnetic resonance imaging

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An abstract containing similar content was accepted for oral presentation at ECR 2017 Author information Authors and Affiliations Corresponding authors Ethics declarations Guarantor The scientific guarantor of this publication is Prof. Jingjing Lu. Conflict of interest The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article. Funding The study was funded by the ‘Excellent Talents of Beijing Dongcheng District program, year 2012 and year 2015’. Statistics and biometry No complex statistical methods were necessary for this paper. Informed consent Written informed consent was waived by the Institutional Review Board. Ethical approval Institutional Review Board approval was obtained. Methodology • retrospective • observational • performed at one institution Rights and permissions About this article Cite this article Wang, Y., Lu, J., Zhu, L. et al. Evaluation of Mayer-Rokitansky-Küster-Hauser syndrome with magnetic resonance imaging: Three patterns of uterine remnants and related anatomical features and clinical settings. Eur Radiol 27, 5215–5224 (2017). https://doi.org/10.1007/s00330-017-4919-4 Received: Revised: Accepted: Published: Issue date: DOI: https://doi.org/10.1007/s00330-017-4919-4