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European Radiology

, Volume 24, Issue 7, pp 1621–1627 | Cite as

Clinical value of magnetic resonance imaging in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: diagnosis of associated malformations, uterine rudiments and intrauterine endometrium

  • H. Preibsch
  • K. Rall
  • B. M. Wietek
  • S. Y. Brucker
  • A. Staebler
  • C. D. Claussen
  • K. C. Siegmann-Luz
Magnetic Resonance

Abstract

Objectives

The aim of this study was to evaluate the accuracy of preoperative magnetic resonance imaging (MRI) in the diagnosis of malformations associated with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and identification of uterine endometrium to optimise the clinical management.

Methods

We retrospectively reviewed 214 consecutive MRKH patients, mean age 19 years, who underwent laparoscopy-assisted neovagina creation. A total of 115 patients (53.7 %) met the inclusion criterion of sufficient preoperative MRI. In 110 of them (95.7 %), MRI findings were correlated with laparoscopy and associated malformations. In 39 cases (35.5 %) uterine rudiments were removed and analysed histopathologically.

Results

Ten per cent (11/110) of the patients showed complete uterine agenesis. The others presented with either unilateral (n = 16; 14.5 %) or bilateral (n = 83; 75.5 %) uterine rudiments. MRI detection of uterine rudiments agreed in 78.2 % (86/110) with laparoscopy. In 85.4 % of the removed rudiments, MRI could correctly diagnose the existence of the endometrium. Compared to laparoscopy, MRI could exactly detect ovaries in 97.3 % (107/110). Renal or ureteral malformations were seen in 32 cases (27.8 %). In 83 % of unilateral renal agenesis and unilateral rudiment, the latter was located at the side of the kidney.

Conclusions

MRI is useful for preoperative detection of MRKH-associated malformations and assessment of the endometrium to further optimise MRKH patient treatment.

Key points

Pelvic MRI is useful for preoperative detection of MRKH-associated malformations.

MRI can diagnose uterine endometrium in MRKH patients with high precision.

Preoperative MRI can optimise clinical management of patients with MRKH syndrome.

Keywords

MRKH syndrome MR imaging Laparoscopy-assisted neovagina Renal malformations Uterus abnormalities 

Notes

Acknowledgements

The scientific guarantor of this publication is Dr. med. Katja Siegmann-Luz. 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. The authors state that this work has not received any funding. No complex statistical methods were necessary for this paper. Institutional review board approval was obtained. Written informed consent was waived by the institutional review board. Methodology: retrospective, diagnostic or prognostic study, performed at one institution.

References

  1. 1.
    Giusti S, Fruzzetti E, Perini D, Fruzzetti F, Giusti P, Bartolozzi C (2011) Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings. Abdom Imaging 36:753–755PubMedCrossRefGoogle Scholar
  2. 2.
    Morcel K, Camborieux L, Programme de Recherches sur les Aplasies Müllériennes, Guerrier D (2007) Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Orphanet J Rare Dis 2:13PubMedCentralPubMedCrossRefGoogle Scholar
  3. 3.
    Duncan PA, Shapiro LR, Stangel JJ, Klein RM, Addonizio JC (1979) The MURCS association: Müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. J Pediatr 95:399–402PubMedCrossRefGoogle Scholar
  4. 4.
    Strübbe EH, Willemsen WN, Lemmens JA, Thijn CJ, Rolland R (1993) Mayer-Rokitansky-Küster-Hauser syndrome: distinction between two forms based on excretory urographic, sonographic, and laparoscopic findings. Am J Roentgenol 160:331–334CrossRefGoogle Scholar
  5. 5.
    Carranza-Lira S, Forbin K, Martinez-Chéquer JC (1999) Rokitansky syndrome and MURCS association–clinical features and basis for diagnosis. Int J Fertil Womens Med 44:250–255PubMedGoogle Scholar
  6. 6.
    Oppelt P, Renner SP, Kellermann A et al (2006) Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging. Hum Reprod 21:792–797PubMedCrossRefGoogle Scholar
  7. 7.
    Edmonds DK, Rose GL, Lipton MG, Quek J (2012) Mayer-Rokitansky-Küster-Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril 97:686–690PubMedCrossRefGoogle Scholar
  8. 8.
    Rall K, Barresi G, Wallwiener D, Brucker SY, Staebler A (2013) Uterine rudiments in patients with Mayer-Rokitansky-Küster-Hauser syndrome consist of typical uterine tissue types with predominantly basalis-like endometrium. Fertil Steril 99:1392–1399PubMedCrossRefGoogle Scholar
  9. 9.
    Brucker SY, Gegusch M, Zubke W, Rall K, Gauwerky JF, Wallwiener D (2008) Neovagina creation in vaginal agenesis: development of a new laparoscopic Vecchietti-based procedure and optimized instruments in a prospective comparative interventional study in 101 patients. Fertil Steril 90:1940–1952PubMedCrossRefGoogle Scholar
  10. 10.
    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 26:199–202PubMedCrossRefGoogle Scholar
  11. 11.
    Rousset P, Raudrant D, Peyron N, Buy JN, Valette PJ, Hoeffel C (2013) Ultrasonography and MRI features of the Mayer-Rokitansky-Küster-Hauser syndrome. Clin Radiol 68:945–952PubMedCrossRefGoogle Scholar
  12. 12.
    Lermann J, Mueller A, Wiesinger E et al (2011) Comparison of different diagnostic procedures for the staging of malformations associated with Mayer-Rokitansky-Küster-Hauser syndrome. Fertil Steril 96:156–159PubMedCrossRefGoogle Scholar
  13. 13.
    Fedele L, Dorta M, Brioschi D, Giudici MN, Candiani GB (1990) Magnetic resonance imaging in Mayer-Rokitansky-Küster-Hauser syndrome. Obstet Gynecol 76:593–596PubMedGoogle Scholar
  14. 14.
    Pompili G, Munari A, Franceschelli G et al (2009) Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome. Radiol Med 114:811–826PubMedCrossRefGoogle Scholar
  15. 15.
    Janssens F, Verswijvel G, Mestdagh G (2004) Mayer-Rokitansky-Küster-Hauser syndrome. JBR-BTR 87:140–141PubMedGoogle Scholar
  16. 16.
    Maubon A, Ferru JM, Courtieu C, Mares P, Rouanet JP (1996) Gynecological malformations. Classification and contribution of different imaging methods. J Radiol 77:465–475PubMedGoogle Scholar
  17. 17.
    Troiano RN, McCarthy SM (2004) Mullerian duct anomalies: imaging and clinical issues. Radiology 233:19–34PubMedCrossRefGoogle Scholar
  18. 18.
    Kara T, Acu B, Beyhan M, Gökçe E (2013) MRI in the diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome. Diagn Interv Radiol 19:227–232PubMedGoogle Scholar
  19. 19.
    García-Valtuille R, García-Valtuille AI, Abascal F, Cerezal L, Argüello MC (2006) Magnetic resonance urography: a pictorial overview. Br J Radiol 79:614–626PubMedCrossRefGoogle Scholar
  20. 20.
    Vecchietti G (1980) Neovagina in Rokitansky-Kuster-Hauser syndrome. Gynakologe 13:112–115PubMedGoogle Scholar
  21. 21.
    Fedele L, Busacca M, Candiani M, Vignali M (1994) Laparoscopic creation of a neovagina in Mayer-Rokitansky-Küster-Hauser syndrome by modification of Vecchietti’s operation. Am J Obstet Gynecol 171:268–269PubMedGoogle Scholar
  22. 22.
    Oppelt PG, Lermann J, Strick R et al (2012) Malformations in a cohort of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH). Reprod Biol Endocrinol 10:57PubMedCentralPubMedCrossRefGoogle Scholar
  23. 23.
    Fiaschetti V, Taglieri A, Gisone V, Coco I, Simonetti G (2012) Mayer-Rokitansky-Kuster-Hauser syndrome diagnosed by magnetic resonance imaging. Role of imaging to identify and evaluate the uncommon variation in development of the female genital tract. J Radiol Case Rep 6:17–24PubMedCentralPubMedCrossRefGoogle Scholar
  24. 24.
    Minto CL, Hollings N, Hall-Craggs M, Creighton S (2001) Magnetic resonance imaging in the assessment of complex Müllerian anomalies. BJOG 108:791–797PubMedGoogle Scholar
  25. 25.
    Hall-Craggs MA, Williams CE, Pattison SH, Kirkham AP, Creighton SM (2013) Mayer-Rokitansky-Kuster-Hauser syndrome: diagnosis with MR imaging. Radiology 269:787–792PubMedCrossRefGoogle Scholar
  26. 26.
    Yoo RE, Cho JY, Kim SY, Kim SH (2013) Magnetic resonance evaluation of Müllerian remnants in Mayer-Rokitansky-Küster-Hauser syndrome. Korean J Radiol 14:233–239PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© European Society of Radiology 2014

Authors and Affiliations

  • H. Preibsch
    • 1
  • K. Rall
    • 2
  • B. M. Wietek
    • 1
  • S. Y. Brucker
    • 2
  • A. Staebler
    • 3
  • C. D. Claussen
    • 1
  • K. C. Siegmann-Luz
    • 1
  1. 1.Department of Radiology, Diagnostic and Interventional RadiologyUniversity Hospital TuebingenTuebingenGermany
  2. 2.Department of Gynecology and ObstetricsUniversity Hospital TuebingenTuebingenGermany
  3. 3.Institute of Pathology and NeuropathologyUniversity Hospital TuebingenTuebingenGermany

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