Child Kidney Dis.  2018 Apr;22(1):12-16. 10.3339/jkspn.2018.22.1.12.

Herlyn-Werner-Wunderlich Syndrome: A Mini-review

Affiliations
  • 1Department of Pediatric Nephrology, Chungnam National University Children's Hospital, Daejeon, Korea. jwmleemd@gmail.com

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.

Keyword

Müllerian duct anomaly; renal agenesis; hemivagina; Uterus didelphys; female genital anomalies

MeSH Terms

Abdominal Pain
Adolescent
Female
Hematocolpos
Humans
Incidence
Infant, Newborn
Kidney
Multicystic Dysplastic Kidney
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