Presentation
Primary amenorrhea.
Patient Data
Age: 20 years
Gender: Female
From the case:
Mayer-Rokitansky-Küster-Hauser syndrome
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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.
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