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KMID : 0358420140570040310
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Korean Journal of Obstetrics and Gynecology
2014 Volume.57 No. 4 p.310 ~ p.313
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Complete septate uterus, obstructed hemivagina, and ipsilateral adnexal and renal agenesis in pregnancy
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Kim Mi-Sun
Nam Sun-Young
Lee Guisera
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Abstract
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The presentation of a complete obstructed hemivagina caused by a longitudinal vaginal septum is associated with uterine malformation and ipsilateral renal agenesis. Burgis reported its incidence as 0.12% to 3.8% [1]. We present here a case of complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis in addition to the agenesis of the ipsilateral fallopian tube and ovary in pregnancy. This Mullerian duct anomaly is a variant of the Herlyn-Werner-Wunderlich syndrome (HWWs) which is didelphys uterus with an obstructed hemivagina and ipsilateral renal agenesis. Most cases were diagnosed in adolescence after menarche and the patients conceived successfully and 33% of the patients delivered vaginally after surgical treatment [2]. The agenesis of the ipsilateral fallopian tube can coincide with the development of the Mullerian duct anomalies. However, ipsilateral ovarian agenesis has a different mechanism of development because the ovary develops from germ cells, not from the Mullerian duct. Complex anomalies involving the urogenital system and ovaries are much more rare than those not involving the ovary. To the best of our knowledge, this is the first report that such complex anomalies have been confirmed during pregnancy.
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KEYWORD
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Hemivagina, Ovarian agenesis, Pregnancy, Renal agenesis, Septate uterus
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