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Open Access 01-12-2012 | Research

Malformations in a cohort of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH)

Authors: Patricia G Oppelt, Johannes Lermann, Reiner Strick, Ralf Dittrich, Pamela Strissel, Ingo Rettig, Christine Schulze, Stefan P Renner, Matthias W Beckmann, Sara Brucker, Katharina Rall, Andreas Mueller

Published in: Reproductive Biology and Endocrinology | Issue 1/2012

Abstract

Background

The aim of this retrospective study was to describe the spectrum of genital and associated malformations in women with Mayer-Rokitansky-Küster-Hauser syndrome using evaluated diagnostic procedures and the Vagina Cervix Uterus Adnex – associated Malformation classification system (VCUAM).

Methods

290 women with MRKH syndrome were clinically evaluated with using clinical examinations, abdominal and perineal/rectal ultrasound, MRI, and laparoscopy.

Results

Classification of female genital malformation according to the Vagina Cervix Uterus Adnex – associated Malformation classification system was possible in 284 women (97.9%). Complete atresia of Vagina (V5b) and bilateral atresia of Cervix (C2b) were found in 284 patients (100%). Uterus: bilateral rudimentary or a plastic uterine horns were found in 239 women (84.2%). Adnexa: normal Adnexa were found in 248 women (87.3%). Malformations: associated malformations were found in 126 of 282 evaluable women (44.7%), 84 women (29.6%) had malformations of the renal system. Of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome 212 women (74.7%) could be classified as V5bC2bU4bA0. The most frequent classification was V5bC2bU4bA0M0 (46.8%) diagnosed in 133 of 284 women.

Conclusions

Complete atresia of vagina and cervix were found in all patients, variable malformations were found with uterus and adnexa. A variety of associated malformations were present, predominantly of the renal system. It is therefore recommended that all patients with genital malformations should be evaluated for renal abnormalities.

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Title: Malformations in a cohort of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH)
Authors: Patricia G Oppelt
Johannes Lermann
Reiner Strick
Ralf Dittrich
Pamela Strissel
Ingo Rettig
Christine Schulze
Stefan P Renner
Matthias W Beckmann
Sara Brucker
Katharina Rall
Andreas Mueller
Publication date: 01-12-2012
Publisher: BioMed Central
Published in: Reproductive Biology and Endocrinology / Issue 1/2012
Electronic ISSN: 1477-7827
DOI: https://doi.org/10.1186/1477-7827-10-57

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Malformations in a cohort of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH)

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on sleep in brain health

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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