Mayer-Rokitansky-Küster-Hauser syndrome with persistent descending mesocolon
Presentation
The patient presented with primary amenorrhea. She is newly married with no successful intercourse.
Patient Data
Complete absence of the uterus and upper vagina, with a remnant vaginal canal measuring about 6.3 cm in length. Both kidneys are normal in size, shape and position.
Features are consistent with Müllerian agenesis (Type A Mayer-Rokitansky-Küster-Hauser syndrome).
There is a well defined T1 hyperintense cystic lesion in the left ovary with T2 hypointensity and diffusion restriction, measuring about 15 x 12 mm, suggesting a hemorrhagic cyst. Right ovarian ruptured corpus luteum cyst. Otherwise, both ovaries are unremarkable.
Evidence of left-sided double ureters, which seem to join at the level of mid-ureter.
Incidental finding of oblique orientation of the descending colon, which seems to have an intraperitoneal course with small bowels lateral to it. Features are consistent with persistent descending mesocolon.
Evidence of a fat-containing small left obturator hernia.
Case Discussion
MRKH syndrome is generally characterized by normal external genitalia and absence or reduced development of the uterus and upper two-thirds of the vagina.
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