Presentation
Primary amenorrhea and normal secondary sexual characteristics.
Patient Data
Age: 20 years
Gender: Female
From the case:
Mayer-Rokitansky-Kuster-Hauser syndrome
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The uterus could not be visualized and the ovaries demonstrated normal volume, signal, and location.
Right and left ovaries measuring about 47 x 31 mm and 28 x 16 mm respectively.
Both ovaries are seen with multiple small follicles.
Follicular cyst measuring about 29 mm is seen in the right ovary.
The vaginal canal could not be visualized.
Case Discussion
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder characterized by aplasia or hypoplasia of the uterus, tubes, and vagina due to an early arrest in Mullerian duct development but ovaries are normal and functional. At birth parents have a normal karyotype.