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Association Between Exstrophy-epispadias Complex And Congenital Anomalies: A German Multicenter Study
Anne-Karoline Ebert1, Nadine Zwink2, Ekkehart Jenetzky3
1Department of Pediatric Urology, University Hospital for Urology and Pediatric Urology Ulm, Ulm, Germany.
Urology
|August 5, 2018
Summary
The exstrophy-epispadias complex (EEC) often includes associated anomalies beyond the typical spectrum. Early identification of these congenital uro-rectal malformations is crucial for patient management and long-term outcomes.
Area of Science:
- Pediatric Surgery
- Medical Genetics
- Developmental Biology
Background:
- The exstrophy-epispadias complex (EEC) is a spectrum of congenital anomalies.
- While often considered isolated, EEC may present with associated defects.
- Previous studies suggest links to renal, anorectal, and neurotubular anomalies.
Purpose of the Study:
- To investigate associated anomalies in patients with the exstrophy-epispadias complex (EEC).
- To analyze data from the congenital uro-rectal malformations network (CURE-Net) database.
Main Methods:
- Systematic screening of the CURE-Net database.
- Analysis of 73 prospective and 162 cross-sectional EEC patients.
- Classification of anomalies using ICD and the London Dysmorphology Database.
Main Results:
- Majority of EEC patients were male (68%) with classical bladder exstrophy (71%).
- Exstrophy variants were more common in newborns (21%).
- Associated anomalies outside the EEC spectrum were present in 59% of prospective and 48% of cross-sectional patients.
Conclusions:
- Multicenter data confirm associated anomalies within and outside the EEC spectrum.
- Detected anomalies impact primary reconstruction and long-term follow-up.
- Associated anomalies in EEC patients require spotlighting during routine check-ups.
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