Mayer-Rokitansky-Küster-Hauser Syndrome: A rare case of a crossed-fused pelvic renal ectopia with variant vasculature and common ureter

Abstract

The association of renal abnormalities with Mullerian anomalies is well recognized and assessment of the renal tract forms part of the routine assessment of patients presenting with Mullerian anomalies. This is a case of a 20-year-old nulliparous female who had abdominopelvic ultrasonography on account of primary amenorrhea showing bilateral ectopic kidney fused in the pelvis, complete absence of the uterus, the cervix, and the upper part of the vagina; while normal sized ovaries seen. These were confirmed on computed tomography (CT). Renal CT angiograms showed a renal artery arising anteriorly from the abdominal aorta just above its bifurcation into the common iliac arteries. An interesting feature in this patient was the existence of the median sacral artery in the blood supply of the fused pelvic kidney. No skeletal anomalies were visualized in dorsolumbar spine or pelvis.