Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Dr Bahman Rasuli

Presentation

Primary amenorrhea.

Patient Data

Age: 20 years
Gender: Female

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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