Mayer-Rokitansky-Küster-Hauser syndrome with bilateral rudimentary uterine horns

Case contributed by Dr Mahmoud Ibrahim Mekhaimar

Presentation

Primary amenorrhea.

Patient Data

Age: 30 years
Gender: Female
  • two rudimentary uterine horns are seen located laterally in the pelvis being caudal to both ovaries. 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 bilateral ovaries with follicles
  • two rudimentary uterine horns are seen located laterally in the pelvis being caudal to both ovaries (yellow arrows). Horizontal band is seen connecting both rudimentary horns (blue arrow) in the first image
  • normally functioning bilateral ovaries with follicles (green arrow) in the second image
  • cervicovaginal dysgenesis is noted with small dysgenetic bands of cervical tissues extending from both rudimentary horns (yellow arrow) in the third image

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 horizontal band. 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 functioning endometrium.

This case should be classified as U5aC4V4 according to the European Society of Human Reproduction and Embryology (ESHRE) classification.

 

Special thanks to Dr. Amr Abd El Fatah. Lecturer of Diagnostic and Interventional Radiology, Cairo University Hospitals.

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