Spectrum of MRI Appearance of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in Primary Amenorrhea Patients

Abstract

Introduction: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a malformation in female genital tract due to interrupted embryonic development of para-mesonephric ducts leading to uterine and proximal vagina aplasia or hypoplasia. Aim: To analyse the MRI findings in females suspected of MRKH syndrome in a primary amenorrhea workup. Materials and Methods: A cross-sectional study comprising of 11 patients of MRKH syndrome presented in a tertiary care centre from March 2016 to February 2017 evaluated in 1.5 Tesla MRI scanner. Results: Normal positioned small vestigial uterus demonstrated in two patients (18.2%) and complete uterine and proximal vaginal agenesis were noted in nine patients (81.8%). The mean volume of rudimentary right uterine bud was 2.26 mL ±3.3 (SD) and left uterine bud was 1.27 mL±1.1 (SD). The mean volume of right ovary was 4.74 mL±2.58 (SD) and left ovary was 4.65 mL±2.2 (SD). The mean developed vaginal length was 26.2 mm±6.34 (SD). Conclusion: The rudimentary uterine buds associated with MRKH syndrome always maintained caudal relationship with ovary. Ovaries or rudimentary uterine buds can be ectopic and should be recognized with MRI before undergoing fertility treatment. Following MRI diagnosis, surgery allows patients to have sexual function with possible attainment of reproduction after assisted reproduction technique or surrogacy.

Keywords

• infertility
• mullerian anomalies
• rudimentary uterine buds
• utero-vaginal agenesis