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 left 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 26 x 35 mm and 28 x 38 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.