Abstract

The association of müllerian anomalies with concomitant gonadal development abnormalities is very rare. The literature revealed only a few cases of unicornuate uterus with unilateral ovarian agenesis. The pathophysiology of this rare combination is not clear. A 31-year-old primiparous woman at 38 weeks' gestation admitted to our labor room due to painful uterine contractions. An emergency cesarean section was performed and a 3,100-g healthy male infant was delivered. At cesarean delivery, she was found to have unicornuate uterus with no evidence of a rudimentary horn and the absence of a left (unilateral) ovary. The right fallopian tube, round ligament, and ovary were all normal. The left fallopian tube, round ligament, and ovary were all absent. Intraabdominal exploration, intravenous pyelography, postoperative abdominal and transvaginal ultrasonography were failed to reveal additional gynecologic, renal and urinary tract anomalies. To our best knowledge this case is unique since the incidental diagnosis of unicornuate uterus with ipsilateral ovary was made during cesarean delivery.

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