Presentation
Primary amenorrhea.
Patient Data
Age: 20 years
Gender: Female
From the case:
Mayer-Rokitansky-Küster-Hauser syndrome
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The uterus is small and is located at the left superior aspect of the pelvic cavity. The uterine cervix is not seen and the vagina is atretic. The ovaries are normal-appearing.
Case Discussion
Rudimentary ectopic uterus with vaginal atresia in a young patient with primary amenorrhea indicate 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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