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Hum. Reprod. Advance Access published online on November 10, 2005
Human Reproduction, doi:10.1093/humrep/dei381
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© The Author 2005. Published by Oxford University Press on behalf of the European Society of Human Reproduction and Embryology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Received April 21, 2005
Revised September 22, 2005
Accepted September 28, 2005

Article

Clinical aspects of Mayer-Rokitansky-Kuester-Hauser syndrome: recommendations for clinical diagnosis and staging

Peter Oppelt 1*, Stefan P.Renner 1, Anja Kellermann 1, Sara Brucker 2, Georges A.Hauser 3, Kurt S.Ludwig 4, Pamela L.Strissel 1, Reiner Strick 1, Diethelm Wallwiener 2, and Matthias W.Beckmann 1

1 Department of Gynecology and Obstetrics, University Hospital, Universitätsstrasse 21-23, D-91054 Erlangen
2 Department of Gynecology and Obstetrics, University Tübingen, Calwerstraße 7, 72076 Tübingen, Germany
3 Swiss Medical Association (FMH), Allenwindenstrasse 7, CH-6004 Lucerne
4 Department of Anatomy, University of Basel, Pestalozzistrasse 20, CH-4056 Basel, Switzerland

* To whom correspondence should be addressed.
Peter Oppelt, E-mail: Peter.Oppelt{at}gyn.imed.uni-erlangen.de


  Abstract

BACKGROUND: The Mayer-Rokitansky-Kuester-Hauser (MRKH) syndrome is a malformation of the female genitals (occurring in one in 4000 female live births) as a result of interrupted embryonic development of the Müllerian (paramesonephric) ducts. This retrospective study examined the issue of associated malformations, subtyping, and the frequency distribution of subtypes in MRKH syndrome. METHODS: Fifty-three MRKH patients were investigated using a newly developed standardized questionnaire. Together with the results of clinical and diagnostic examinations, the patients were classified into the three recognized subtypes [typical, atypical and MURCS (Müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia)]. RESULTS: The typical form was diagnosed in 25 patients (47%), the atypical form in 11 patients (21%), and the most marked form--the MURCS type--in 17 patients (32%). Associated malformations were notably frequent among the patients. Malformations of the renal system were the most frequent type of accompanying malformation, with 23 different malformations in 19 patients, followed by 18 different skeletal changes in 15 patients. CONCLUSIONS: In accordance with the literature, this study shows that associated malformations are present in more than a third of cases. Therefore, new basic guidelines for standard diagnostic classification involving patients with suspected MRKH are presented.

Keywords: diagnostic malformations/genital or Müllerian duct malformations/MRKH syndrome/staging malformations/vaginal aplasia.
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