Mayer-Rokitansky-Küster-Hauser syndrome

Case contributed by Dr Erik Ranschaert

Presentation

Primary amenorrhea and dyspareunia.

Patient Data

Age: 20 years
Gender: Female

The patient has no uterus and no distinct vagina. Bilateral ovaries are seen.

Case Discussion

The Mayer-Rokitansky-Küster(-Hauser) Syndrome (MRK or MRKH) presents a congenital female anomaly with agenesis of the uterus. It belongs to the Mullerian duct anomalies

The patient does have normal adnexa and no further renal anomalies, except for a double excretory system on the left side.

Therefore this patient is considered to have the typical form (type A) of this syndrome characterized by congenital absence of the uterus and upper vagina with normal ovaries and fallopian tubes.

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