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

**Authors:** Elena Grömping, Johanna Hagens, Hans C. Schmidt, Katharina Wenke, Christian Tomuschat, Konrad Reinshagen

PMC · DOI: 10.1055/a-2806-3084 · European Journal of Pediatric Surgery Reports · 2026-02-24

## TL;DR

A premature baby girl was born with a rare combination of three urogenital and abdominal wall abnormalities requiring complex medical care.

## Contribution

The paper reports a rare syndromic triad of urogenital anomalies in a neonate, highlighting diagnostic and therapeutic challenges.

## Key findings

- The neonate exhibited persistent urogenital sinus, Herlyn–Werner–Wunderlich syndrome, and Prune Belly-like syndrome.
- Management involved staged urinary drainage, septum incision, and multidisciplinary care to improve outcomes.
- Early recognition and individualized care are critical for managing such complex congenital anomalies.

## Abstract

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.

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.

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.

## Linked entities

- **Diseases:** hydrops fetalis (MONDO:0015193), oligohydramnios (MONDO:0005881)
- **Species:** Homo sapiens (taxon 9606)

## Full-text entities

- **Genes:** CST3 (cystatin C) [NCBI Gene 1471] {aka ADLDWA, ARMD11, HEL-S-2}
- **Diseases:** prenatal malformations (MESH:D049188), patent ductus arteriosus (MESH:D004374), gastrointestinal (MESH:D005767), uterus (MESH:D014594), peritonitis (MESH:D010538), Renal insufficiency (MESH:D051437), Urinary (MESH:D014548), coagulation abnormalities (MESH:D001778), Central Nervous System (MESH:D002493), hydronephrosis (MESH:D006869), heart defects (MESH:D006330), secondary hyperparathyroidism (MESH:D006962), bronchopulmonary dysplasia (MESH:D001997), urinary tract infections (MESH:D014552), anorectal malformations (MESH:D000071056), Congenital anomalies (MESH:D000013), urinary obstruction (MESH:D001748), Thrombus (MESH:D013927), hematomas (MESH:D006406), cholecystolithiasis (MESH:D041761), hypoplasia (MESH:D000080344), anemia (MESH:D000740), Ascites (MESH:D001201), oligohydramnios (MESH:D016104), inflammatory (MESH:D007249), anatomical malformations (MESH:D020763), hypotonia (MESH:D009123), congenital malformations (OMIM:163000), apnea (MESH:D001049), urinary retention (MESH:D016055), Abdominal wall hypoplasia (MESH:D046449), vesicoureteral reflux (MESH:D014718), cystic dysplastic kidneys (MESH:D052177), intestinal motility disorder (MESH:D007410), hypernatremia (MESH:D006955), tract (MESH:D014570), obstructive uropathy (MESH:C536483), obstruction (MESH:D000402), malformations (MESH:C564254), ischemia (MESH:D007511), hydrocephalus (MESH:D006849), cryptorchidism (MESH:D003456), respiratory acidosis (MESH:D000142), bile duct dilation (MESH:D001649), Infectious complications (MESH:D003141), plexus cyst (MESH:D003560), vomiting (MESH:D014839), dysplastic (MESH:D004416), hydrocolpos (MESH:D052202), prematurity complications (MESH:D005117), dysplastic kidneys (MESH:D007674), abdominal aplasia (MESH:D000007), Urogenital and Renal Anomalies (MESH:D014564), Sinus tachycardia (MESH:D013616), hemorrhage (MESH:D006470), Noonan (MESH:D009634), ventricular dilation (MESH:C566255), respiratory compromise (MESH:D012131), cataracts (MESH:D002386), Megacystis (MESH:C536139)
- **Chemicals:** Macrogol (MESH:D011092), catecholamines (MESH:D002395), propranolol (MESH:D011433), cefotaxime (MESH:D002439), neostigmine (MESH:D009388), vitamin D (MESH:D014807), alpha-cholecalciferol (-), teicoplanin (MESH:D017334), sodium (MESH:D012964), oxygen (MESH:D010100), paracetamol (MESH:D000082), gentamicin (MESH:D005839), creatinine (MESH:D003404), Spironolactone (MESH:D013148), Naloxone (MESH:D009270), ampicillin (MESH:D000667), meropenem (MESH:D000077731)
- **Species:** Homo sapiens (human, species) [taxon 9606], Respiratory syncytial virus (no rank) [taxon 12814]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12932032/full.md

## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC12932032/full.md

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Source: https://tomesphere.com/paper/PMC12932032