MRI evaluation of pelvis in Mayer–Rokitansky–Kuster–Hauser syndrome: interobserver agreement for surgically relevant structures

Abstract

The paired Mullerian or paramesonephric ducts fuse between 6 and 11 weeks of gestation to form uterus, cervix and upper two-thirds of vagina. Mullerian duct anomalies (MDA) result from disruption in any of the three stages of uterine development, i.e. organogenesis, fusion or septal resorption. 1 Ovaries develop from primordial germ cells, while lower one-third of vagina develops from sinovaginal bulb. Owing to the different embryological origins, ovaries and lower third of vagina are not involved in MDA. 2 The prevalence of MDA is reported to be 1-5%. 2 American Fertility Society (AFS), has classified MDA into seven basic types, from Type I to Type VII. 3 Out of these seven types, Type I MDA also called as Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, which reportedly accounts for nearly 15% of total case load. 2 MRKH syndrome is characterized by complete/partial agenesis of uterus and upper two-thirds of vagina, with normal bilateral ovaries,

Imaging of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome on MRI Examination in dr. Soetomo Hospital.

“…1), with isointense signals at the T2WI sequence frequently occurring among these subjects. This discovery aligned with a prior study by Bhayana (2019), who stipulated that the right and left uterine buds, according to the craniocaudal position to the ovarium, are mainly situated at the caudal site; in consonance with the anterior position, they occur most often at the anterior site.…”

“…3a). This result was discordant with a previous study by Bhayana (2019), in which 13 subjects out of 18 appeared hyperintense and five patients hypointense at the T2WI sequence. 4.4 Ovarium Our study result showed that both right and left ovaries were evident in 35 patients (92.1%), all positioned within intrapelvic (Fig.…”

“…5); meanwhile, it did not exist in the remaining 7 (18.4%). This result was supported by Deb Kumar Boruah et al (2013) and Bhayana (2019), stating the absence of a proximal vagina.…”

“…The American Fertility Society (AFS) classified the Mullerian duct anomalies into seven classes. Class I refers to the Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, making up roughly 15% of all Mullerian duct malformation (Bhayana and Ghasi, 2019). MRKH exhibits a set of clinical symptoms due to the failure of the paramesonephric (Mullerian) tract formation, happening in 1:4,500 baby girls (Wilson D and Bordoni B., 2021).…”

“…MRKH syndrome is characterized by total or partial agenesis of the uterus and the two-thirds portion of the vagina, in spite of 46XX karyotyping with normal bilateral ovaries and fallopian tubes. Even though the uterus fails to develop, the Mullerian residues are still evident, including the uterine buds (rudimental uterus), fibrous bands, and midline triangular soft tissue (Bhayana and Ghasi, 2019).…”