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Malformations in a cohort of 284 women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH)
Oppelt, Patricia G. ;
Lermann, Johannes ;
Strick, Reiner ;
Dittrich, Ralf ;
Strissel, Pamela ;
Rettig, Ingo ;
Schulze, Christine ;
Renner, Stefan P. ;
Beckmann, Matthias W. ;
Brucker, Sara ;
Rall, Katharina ;
||(2012) Reproductive Biology and Endocrinology 10.57 (2012): 15.11.2012 <http://www.rbej.com/content/10/1/57>
|Freie Schlagwörter (Englisch):
||MRKH syndrome , Diagnosis , Staging , Malformations , VCUAM
||Universität Erlangen-Nürnberg / Von der FAU geförderte Open Access Artikel / 2012
|Kurzfassung in Englisch:
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).
290 women with MRKH syndrome were clinically evaluated with using clinical examinations, abdominal and perineal/rectal ultrasound, MRI, and laparoscopy.
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.
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.