Presentation
Amenorrhea.
Patient Data
The uterus is not visualized.
The right ovary is located in the inguinal canal through the internal inguinal ring (canal of Nuck hernia).
The left ovary is intrapelvic just deep to the internal inguinal ring.
Both ovaries show otherwise normal MRI features.
Right renal agenesis.
Solitary left kidney showing compensatory hypertrophy.
Case Discussion
Mayer-Rokitansky-Küster-Hauser sundrome belongs to class I Müllerian duct anomalies (uterine agenesis). In this case; the uterus is missing and upper segment of the vagina with the presence of both ovaries, the right ovary is extra-pelvic at the inguinal region. This is associated with right renal agenesis. There is an uncommon association with renal agenesis and extrapelvic ovary that is demonstrated in this case. Primary amenorrhea is the most common presentation as the presence of functioning ovaries and normal hormonal levels preserve the external genital development and puberty.
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