Presentation
Patient presented with primary amenorrhoea with secondary sexual characteristics.
Patient Data
There are two rudimentary single-layer uterine horns (with volumes of 2.3cc and 1cc on right and left side respectively) seen located laterally in the pelvis being caudal and in relation to both ovaries with a horizontal thin band is seen connecting both rudimentary horns. The cervix and upper two-thirds of the vagina are not visualised.
Normally functioning bilateral ovaries with multiple follicles.
Minimal pelvic free fluid.
N.B: The left kidney is absent with compensatory hypertrophy of the right kidney showing pelvicalyceal dilatation.
Overall appearances are consistent with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) type B with rudimentary single-layer bilateral uterine horn.
Case Discussion
MRKH syndrome belongs to class I Mullerian duct anomalies.
Rudimentary uteri are common in patients with MRKH syndrome. They can be relatively large and have functioning endometrium, which can be associated with pain. Uteri have a constant caudal relationship to ovaries. Ovaries are commonly ectopic.
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