Mullerian ducts anomaly of 2 divergent uterine horns with cervicovaginal hypoplasia: Is it considered unclassified or under the spectrum of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome?

The Mullerian ducts give rise to the upper part of the female reproductive system, including the uterus, cervix, upper two-thirds of the vagina, and fallopian tubes, which undergo specific processes of development, fusion, and resorption. Any failure in this process will lead to Mullerian duct anomaly (MDA). We present a unique and complex case of MDA, signifying the wide variability and simultaneous existence of combined abnormalities in 1 patient, which do not always fit under a single or particular class from the known classification systems. Therefore, subclassifications may be necessary for each part alone (uterus, cervix, and vagina) or incorporating more than 1 class for a single case. It also shows the role of imaging in the diagnosis; considering that magnetic resonance imaging (MRI) is the standard modality for a detailed description of the reproductive system and its anomalies.

(© 2023 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)