Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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Herlyn-Werner-Wunderlich Syndrome: A Mini-review

  • Lee, Jiwon M. (Department of Pediatric Nephrology, Chungnam National University Children's Hospital)
  • Received : 2017.09.04
  • Accepted : 2018.03.16
  • Published : 2018.04.30
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Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

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References

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