Magnetic Resonance Imaging in Mayer-Rokitansky-Kuster-Hauser Syndrome: A Retrospective Study

Abstract

Introduction: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a congenital anomaly in female genital tract owing to irregular embryonic development of para-mesonephric ducts and thus leads to uterine and proximal vagina aplasia or hypoplasia. MRKH syndrome has devastating effects for fertility and sexual intercourse in young women. After diagnosis by the imaging features of Magnetic Resonance Imaging (MRI) surgery may allow patients to have sexual function with possible attainment of reproduction after assisted reproduction technique or surrogacy. Aim: To analyse the MRI findings in females suspected of MRKH syndrome in a primary amenorrhea workup. Materials and Methods: A retrospective study comprising of 11 patients of MRKH syndrome presented in a tertiary care centre from May-2013 to April-2019 was evaluated in a 1.5 Tesla MRI scanner. The diagnostic confirmation of MRKH syndrome was made on the basis of the following features: i) presence or absence of uterine buds; ii) fibrous band like structures connecting bilateral uterine buds; and iii) Midline soft tissue at uterine region. The data was entered in the excel sheet and results were expressed in terms of frequency and percentages. Results: MRI revealed small vestigial uterus in the form of uterine buds in five patients (45.4%) and rudimentary streaky uterus in six patients (54.54%). The presence of the endometrium with prominent cavitation could be detected only in four patients (36.3%), while the remaining seven patients (63.6%) showed no cavitation. The uterine buds were connected with fibrous bandlike structures in three patients (27.2%) and in the remaining 8 (72.7%) patients the uterine buds were located laterally without any apparent connection. The midline soft tissue posteriosuperior to the urinary bladder dome is seen in three cases (27.2%). Bilateral ovaries were normal in size and morphology in all cases. In good number of cases the uterine buds were closely associated with ovaries. Most of them were located in the iliac fossa. All patients displayed only lower 1/3rd of the vagina with the absence of upper 2/3rd vagina. Associated malformations were found in four cases, which were related to renal in 9.09% case, vertebral in 27.2% cases and congenital vesicovaginal fistula in 9.09% case. In brief Type I MRKH is seen in seven cases (63.6%) and Type II MRKH is seen in four cases (36.3%). Conclusion: The diagnosis of MRKH syndrome is made based on clinical findings, but radiological evaluation is also essential for the confirmation. MRI is now considered the imaging modality of choice, because of its ability to accurately identify female genital tract malformations along with associated renal and skeletal anomalies.

Keywords

• mrkh
• mullerian
• primary amenorrhoea
• remnant