Child Kidney Dis.
2018 Apr;22(1):12-16. 10.3339/jkspn.2018.22.1.12.
Herlyn-Werner-Wunderlich Syndrome: A Mini-review
- Affiliations
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- 1Department of Pediatric Nephrology, Chungnam National University Children's Hospital, Daejeon, Korea. jwmleemd@gmail.com
- KMID: 2435349
- DOI: http://doi.org/10.3339/jkspn.2018.22.1.12
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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