Mayer-Rokitansky-Küster-Hauser syndrome (MRKH)

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Primary amenorrhea.

Patient Data

Age: 25 years
Gender: Female

Congenital absence of the uterus and upper 2/3 vagina. Both ovaries are normal in size, containing numerous small follicles.

Normal appearance and location of both kidneys on coronal T2 sequence.

Case Discussion

Case of Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) atypical form (type A) which is characterized by a congenital absence of the uterus and upper 2/3 vagina with normal ovaries and fallopian tubes and no associated renal anomalies.

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