Mayer-Rokitansky-Küster-Hauser syndrome with rudimentary uterine horns
Presentation
Primary amenorrhea.
Patient Data
two rudimentary uterine horns are seen located laterally in the pelvis being caudal to both ovaries. A horizontal band is seen connecting both rudimentary horns
cervicovaginal dysgenesis is noted with small dysgenetic bands of cervical tissues extending from both rudimentary horns
normally functioning ovaries with follicles with right ovarian simple functional cyst
Case Discussion
This case demonstrates a rare form of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) which shows bilateral rudimentary uterine horns located laterally in the pelvis caudal to both ovaries and connected by a horizontal band and associated with cervicovaginal dysgenesis.
Rudimentary uteri are common in patients with MRKH syndrome and it is a common misconception that the affected patients have no uterus at all. Sometimes there is additional intraluminal blood or adenomyosis within the rudimentary uterus which indicates a functioning endometrium.
This case should be classified as U5aC4V4 according to the European Society of Human Reproduction and Embryology (ESHRE) classification.
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