European journal of pediatric surgery reports · 2026
A Case Report of a Syndromic Triad of Persistent Urogenital Sinus, Herlyn-Werner-Wunderlich Syndrome, and Prune Belly Syndrome in a Neonate.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Grömping Elena, Hagens Johanna, Schmidt Hans C, Wenke Katharina, Tomuschat Christian, Reinshagen Konrad
Аннотация
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: A female infant was born at 34 3/7 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.
CASE REPORT: A female infant was born at 34 3/7 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.