Presentation
Primary amenorrhea
Patient Data
There is a small uterine stump noted with hypoplastic cervix.
A small oval structure noted along the right internal iliac without any follicles suspicious for atretic ovary.
The left ovary is seen lateral to ovarian vessels, likely ectopic.
Case Discussion
Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), also known as Müllerian aplasia, is a congenital anomaly characterized by vaginal and uterine agenesis. MRKH syndrome belongs to class I Mullerian duct anomalies.The typical presentation is that of primary amenorrhea with normal hormonal levels guaranteed by fully functional gonads.
Another differential for uterine agenesis is androgen insensitivity syndrome, in which the imaging features include:
bilateral cryptorchidism +/- juxta-testicular Müllerian duct cysts
absent/rudimentary uterus
shortened vagina
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