A Case Report of a Syndromic Triad of Persistent Urogenital Sinus, Herlyn–Werner–Wunderlich Syndrome, and Prune Belly Syndrome in a Neonate

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Authors: Elena Grömping, Johanna Hagens, Hans Christian Schmidt, Katharina Wenke, Christian Tomuschat, Konrad Reinshagen
Cited by: 1
References: 15

Abstract

Background: We present a case of a premature female neonate with a triad of persistent urogenital sinus with urinary ascites, bilateral hydrocolpos in a duplex uterus, and abdominal wall hypoplasia resembling Prune Belly-like syndrome, combined with severe bilateral cystic dysplastic kidneys and complex urinary obstruction. Case Report: weeks' gestation via cesarean section due to prenatal detection of hydrops fetalis and massive urinary ascites. Prenatal ultrasound had shown oligohydramnios, ascites, megacystis, and hydrocolpos. Postnatally, urinary ascites, a persistent urogenital sinus, severe upper urinary tract dilation, bilateral dysplastic kidneys, and an obstructive vaginal septum were confirmed. Management included staged urinary drainage, vaginal septum incision, intensive respiratory and renal support, and multidisciplinary care. Conclusion: This unique combination of anomalies presents significant diagnostic and therapeutic challenges. Early recognition and individualized multidisciplinary management are essential to improve postnatal outcomes and guide long-term planning in such cases.

Fields of Study & Subjects

Pediatric Urology and Nephrology Studies Congenital gastrointestinal and neural anomalies Fetal and Pediatric Neurological Disorders

Publication details

Journal / source
European Journal of Pediatric Surgery Reports
Publisher
Georg Thieme Verlag KG
Volume
14
Issue
01
Pages
e17–e21
Publication date
1 January 2026
Language
EN