Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome: Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis.
- Author:
Myoung Soo KIM
1
;
Yong Jun PARK
;
Young Jun PARK
;
Noh Hyuck PARK
;
Ji Sun SONG
;
Pyung Kil KIM
Author Information
1. Department of Pediatrics, Kwandong University College of Medicine, Koyang, Korea. pkkim@kwandong.ac.kr
- Publication Type:Case Report
- Keywords:
Herlyn-Werner-Wunderlich syndrome;
Mullerian duct anomaly;
Renal agenesis;
Blind hemivagina;
Thin glomerular basement membrane disease
- MeSH:
Adolescent;
Child;
Diagnosis;
Female;
Follow-Up Studies;
Glomerular Basement Membrane*;
Humans;
Hydrocolpos;
Kidney;
Menarche;
Pelvic Pain;
Puberty;
Unnecessary Procedures;
Urinary Bladder;
Uterus*;
Vagina
- From:Journal of the Korean Society of Pediatric Nephrology
2007;11(2):299-305
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy-proven thin glomerular basement membrane disease which is not related to the above syndrome.
