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.

METHODS

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.

RESULTS

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).

CONCLUSION

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.