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

Reproductive Biology and Endocrinology

Andreas Mueller¹  Katharina Rall²  Sara Brucker²  Matthias W Beckmann¹  Stefan P Renner¹  Christine Schulze¹  Ingo Rettig³  Pamela Strissel¹  Ralf Dittrich¹  Reiner Strick¹  Johannes Lermann¹  Patricia G Oppelt¹ 

[1] Department of Obstetrics and Gynecology, Erlangen University Hospital, Universitätsstrasse 21-23, Erlangen, 91054, Germany;Department of Obstetrics and Gynecology, Tübingen University Hospital, Calwerstr. 7, Tübingen, 72076, Germany;Department of Internal Medicine IV, Tübingen University Hospital, Otfried-Müller-Straße 10, Tübingen, 72076, Germany

关键词: Renal abnormalities;    VCUAM;    Malformations;    Staging;    Diagnosis;    MRKH syndrome;   

Others  :  1150361
DOI  :  10.1186/1477-7827-10-57

 received in 2012-05-03, accepted in 2012-08-11,  发布年份 2012

【 摘 要 】

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.

【 授权许可】

   
2012 Oppelt et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

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