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European Radiology

, Volume 27, Issue 12, pp 5215–5224 | Cite as

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

Urogenital

Abstract

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.

Keywords

Mayer-Rokitansky-Küster-Hauser syndrome Mullerian duct anomalies Uterine rudiment Magnetic resonance imaging Anatomy 

Abbreviations

MRKH

Mayer-Rokitansky-Küster-Hauser syndrome

MRI

Magnetic resonance imaging

Notes

Acknowledgements

An abstract containing similar content was accepted for oral presentation at ECR 2017

Compliance with ethical standards

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

References

  1. 1.
    Patnaik SS, Brazile B, Dandolu V, Ryan PL, Liao J (2015) Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: a historical perspective. Gene 555:33–40CrossRefPubMedGoogle Scholar
  2. 2.
    Oppelt PG, Lermann J, Strick R, Dittrich R, Strissel P, Rettig I (2012) Malformations in a cohort of 284 women with Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH). Reprod Biol Endocrinol 10:57CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Herlin M, Hojland AT, Petersen MB (2014) Familial occurrence of Mayer-Rokitansky-Kuster-Hauser syndrome: a case report and review of the literature. Am J Med Genet A 164A:2276–2286CrossRefPubMedGoogle Scholar
  4. 4.
    Pizzo A, Lagana AS, Sturlese E, Retto G, Retto A, De Dominici R (2013) Mayer-rokitansky-kuster-hauser syndrome: embryology, genetics and clinical and surgical treatment. ISRN Obstet Gynecol 2013:628717CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Bombard DS 2nd, Mousa SA (2014) Mayer-Rokitansky-Kuster-Hauser syndrome: complications, diagnosis and possible treatment options: a review. Gynecol Endocrinol 30:618–623CrossRefPubMedGoogle Scholar
  6. 6.
    Behr SC, Qayyum A (2012) Imaging of mullerian duct anomalies. Radiographics 32:E233–E250CrossRefPubMedGoogle Scholar
  7. 7.
    Rousset P, Raudrant D, Peyron N, Buy JN, Valette PJ, Hoeffel C (2013) Ultrasonography and MRI features of the Mayer-Rokitansky-Kuster-Hauser syndrome. Clin Radiol 68:945–952CrossRefPubMedGoogle Scholar
  8. 8.
    Yoo RE, Cho JY, Kim SY, Kim SH (2015) A systematic approach to the magnetic resonance imaging-based differential diagnosis of congenital Mullerian duct anomalies and their mimics. Abdom Imaging 40:192–206CrossRefPubMedGoogle Scholar
  9. 9.
    Londra L, Chuong FS, Kolp L (2015) Mayer-Rokitansky-Kuster-Hauser syndrome: a review. Int J Womens Health 7:865–870CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Rall K, Eisenbeis S, Henninger V, Henes M, Wallwiener D, Bonin M (2015) Typical and atypical associated findings in a group of 346 patients with Mayer-Rokitansky-Kuester-Hauser syndrome. J Pediatr Adolesc Gynecol 28:362–368CrossRefPubMedGoogle Scholar
  11. 11.
    Hall-Craggs MA, Williams CE, Pattison SH, Kirkham AP, Creighton SM (2013) Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology 269:787–792CrossRefPubMedGoogle Scholar
  12. 12.
    Preibsch H, Rall K, Wietek BM, Brucker SY, Staebler A, Claussen CD (2014) Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium. Eur Radiol 24:1621–1627CrossRefPubMedGoogle Scholar
  13. 13.
    Rall K, Barresi G, Wallwiener D, Brucker SY, Staebler A (2013) Uterine rudiments in patients with Mayer-Rokitansky-Kuster-Hauser syndrome consist of typical uterine tissue types with predominantly basalis-like endometrium. Fertil Steril 99:1392–1399CrossRefPubMedGoogle Scholar
  14. 14.
    Marsh CA, Will MA, Smorgick N, Quint EH, Hussain H, Smith YR (2013) Uterine remnants and pelvic pain in females with Mayer-Rokitansky-Küster-Hauser syndrome. J Pediatr Adolesc Gynecol 26:199–202CrossRefPubMedGoogle Scholar
  15. 15.
    Allen JW, Cardall S, Kittijarukhajorn M, Siegel CL (2012) Incidence of ovarian maldescent in women with mullerian duct anomalies: evaluation by MRI. AJR Am J Roentgenol 198:W381–W385CrossRefPubMedGoogle Scholar
  16. 16.
    Pan HX, Luo GN (2016) Phenotypic and clinical aspects of Mayer-Rokitansky-Kuster-Hauser syndrome in a Chinese population: an analysis of 594 patients. Fertil Steril 106:1190–1194CrossRefPubMedGoogle Scholar
  17. 17.
    Yoo RE, Cho JY, Kim SY, Kim SH (2013) Magnetic resonance evaluation of Mullerian remnants in Mayer-Rokitansky-Kuster-Hauser syndrome. Korean J Radiol 14:233–239CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Doyle JO, Laufer MR (2009) Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome with a single septate uterus: a novel anomaly and description of treatment options. Fertil Steril 92:e317–e399CrossRefGoogle Scholar
  19. 19.
    Pompili G, Munari A, Franceschelli G, Flor N, Meroni R, Frontino G (2009) Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med 114:811–826CrossRefPubMedGoogle Scholar
  20. 20.
    Huebner M, Rall K, Brucker SY, Reisenauer C, Siegmann-Luz KC, DeLancey JO (2014) The rectovaginal septum: visible on magnetic resonance images of women with Mayer-Rokitansky-Kuster-Hauser syndrome (Mullerian agenesis). Int Urogynecol J 25:323–327CrossRefPubMedPubMedCentralGoogle Scholar
  21. 21.
    Dariane C, Moszkowicz D, Peschaud F (2016) Concepts of the rectovaginal septum: implications for function and surgery. Int Urogynecol J 27:839–848CrossRefPubMedGoogle Scholar

Copyright information

© European Society of Radiology 2017

Authors and Affiliations

  1. 1.Department of Radiology, Peking Union Medical College HospitalPeking Union Medical College, Chinese Academy of Medical SciencesBeijingPeople’s Republic of China
  2. 2.Department of Obstetrics and Gynaecology, Peking Union Medical College Hospital, Peking Union Medical CollegeChinese Academy of Medical SciencesBejingPeople’s Republic of China

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