Mayer-Rokitansky-Küster-Hauser syndrome with persistent descending mesocolon
Presentation
The patient presented with primary amenorrhoea. 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.
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 haemorrhagic 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.
Case Discussion
MRKH syndrome is generally characterised by normal external genitalia and absence or reduced development of the uterus and upper two-thirds of the vagina.