OBJECTIVE: To compare different diagnostic procedures for staging malformations associated with Mayer-Rokitansky-K?ster-Hauser (MRKH) syndrome.DESIGN: Retrospective two-center cohort study (Canadian Task Force classification II-2).SETTING: University hospital.PATIENT(S): One hundred and thirty-eight women with MRKH.INTERVENTION(S): Clinical examinations, abdominal or perineal/rectal ultrasound, magnetic resonance imaging (MRI), and laparoscopy.MAIN OUTCOME MEASURE(S): Agreement between the results obtained with the other methods and the results obtained with the reference methods for correct staging of malformations, presented as kappa values (ê).RESULT(S): The VCUAM (vagina cervix uterus adnex-associated malformation) classification system was used to classify genital malformations in 138 women with MRKH. The reference methods for examining the individual organs were: vagina-clinical examination; cervix/uterus and adnexa-laparoscopy; and urinary tract malformations-MRI. The values obtained were as follows. Vagina was ê 0.74 for MRI versus clinical examination; ultrasound and laparoscopy did not allow adequate description of vaginal malformations. Cervical findings were rarely detailed with any of the imaging methods. Uterus was ê 0.93 for MRI versus laparoscopy, and ê 0.83 for ultrasound. For adnexa, only laparoscopy was able to describe the morphology adequately. The urinary tract was ê 0.87 for ultrasound versus MRI.CONCLUSION(S): For the correct staging of malformations associated with MRKH, MRI or a combination of clinical examination and ultrasound are equivalent. However, none of the imaging methods adequately describes adnexal morphology.
46, XX Disorders of Sex Development Abnormalities, Multiple