Presentation
Primary amenorrhea.
Patient Data
The rudimentary uterus is located at the right posterolateral aspect of the bladder dome
The uterine cervix is not seen and the vagina is atretic
The ovaries are normal-appearing
Right and left ovaries measuring about 18 x 27 mm and 21 x 29 mm respectively.
Normal features of the rectosigmoid colon and urinary bladder.
Case Discussion
A rudimentary uterus with vaginal atresia in a young patient with primary amenorrhea indicates Mayer-Rokitansky-Küster-Hauser syndrome (MRKH), which is a congenital anomaly characterized by vaginal agenesis associated with a spectrum of other genitourinary tract abnormalities. MRKH syndrome belongs to class I Mullerian duct anomalies.