Presentation
Primary amenorrhea and normal secondary sexual characteristics.
Patient Data
Age: 15 years
Gender: Female
From the case:
Mayer-Rokitansky-Küster-Hauser syndrome
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The uterus could not be visualized.
The ovaries demonstrated normal volume, signal, and location.
The right and left ovaries measure 23 x 34mm and 22 x 30 mm respectively.
Both ovaries are seen with multiple small follicles.
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, fallopian 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.
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