Anomalous vasculature in Mayer-Rokitansky-Kuster-Hauser syndrome

To report a case of unique anomalous pelvic vasculature in a woman with Müllerian agenesis.

Case report.

University hospital.

A 61-year-old woman with Müllerian agenesis and stage IIIc papillary serous ovarian cancer.

Surgery was performed, including bilateral salpingo-oophorectomy, omentectomy, right terminal ileum and right ascending colon resection, ileoascending/transverse colon reanastomosis, and debulking of pelvic plaques along bilateral ureters. The patient had subsequent chemotherapy with taxane and platinum agents and at the time of writing was in remission.

None.

Absence of the uterus was confirmed; rudimentary uterine horns and associated fallopian tubes and ovaries were noted bilaterally. The right ureter coursed below and behind the right common iliac artery, which did not bifurcate into external and internal arteries at the pelvic brim. Instead there was only an external iliac artery that gave off a pelvic branch just before the inguinal ligament. On the left, there was only an internal iliac artery that gave off an external branch after diving into the pelvis.

It is possible that the aberrant vasculature was partially responsible for the absence of the uterus. Preoperatively, it is important to recognize the possibility of abnormal pelvic vasculature in patients with Müllerian agenesis.