Herlyn-Werner-Wunderlich Syndrome: A Mini-review
10.3339/jkspn.2018.22.1.12
- Author:
Jiwon M LEE
1
Author Information
1. Department of Pediatric Nephrology, Chungnam National University Children's Hospital, Daejeon, Korea. jwmleemd@gmail.com
- Publication Type:Review
- Keywords:
Müllerian duct anomaly;
renal agenesis;
hemivagina;
Uterus didelphys;
female genital anomalies
- MeSH:
Abdominal Pain;
Adolescent;
Female;
Hematocolpos;
Humans;
Incidence;
Infant, Newborn;
Kidney;
Multicystic Dysplastic Kidney
- From:Childhood Kidney Diseases
2018;22(1):12-16
- CountryRepublic of Korea
- Language:English
-
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.
