Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome : Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis

Kim, Myoung-Soo; Park, Yong-Jun; Park, Young-Jun; Park, Noh-Hyuck; Song, Ji-Sun; Kim, Pyung-Kil

doi:2007.11.2.299

J Korean Soc Pediatr Nephrol > Volume 11(2); 2007 > Article

J Korean Soc Pediatr Nephrol 2007;11(2): 299-305. doi: https://doi.org/10.3339/jkspn.2007.11.2.299

비박형 사구체 기저막 질환이 동반된 중복 자궁 일측성 폐쇄질 및 동측 신장 무형성 증후군 ( Herlyn - Werner- Wunderlich syndrome ) 1례

김명수, 박용준, 박영준, 박노혁, 송지선, 김병길

¹관동대학교 의과대학 소아청소년과학교실
²관동대학교 의과대학 소아청소년과학교실
³관동대학교 의과대학 산부인과학 교실
⁴관동대학교 의과대학 영상의학과학교실
⁵관동대학교 의과대학 병리학교실
⁶관동대학교 의과대학 소아청소년과학교실

Thin Glomerular Basement Membrane Disease with Herlyn-Werner-Wunderlich Syndrome : Uterus Didelphys, Blind Hemivagina and Ipsilateral Renal Agenesis

Myoung-Soo Kim, Yong-Jun Park, Young-Jun Park, Noh-Hyuck Park, Ji-Sun Song, Pyung-Kil Kim

¹Departments of Pediatrics, Kwandong University College of Medicine
²Departments of Pediatrics, Kwandong University College of Medicine
³Departments of Obstetrics & Gynecology, Kwandong University College of Medicine
⁴Departments of Radiology, Kwandong University College of Medicine
⁵Departments of Pathology, Kwandong University College of Medicine
⁶Departments of Pediatrics, Kwandong University College of Medicine

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.

Key words: Herlyn-Werner-Wunderlich syndrome | Mullerian duct anomaly | Renal agenesis | Blind hemivagina | Thin glomerular basement membrane disease