Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Dr Mohammad Taghi Niknejad


Primary amenorrhea.

Patient Data

Age: 20 years
Gender: Female

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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Case information

rID: 63269
Published: 22nd Sep 2018
Last edited: 14th Aug 2019
System: Urogenital
Inclusion in quiz mode: Included
Institution: Tabesh medical imaging center

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