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Variant Presentations of the Exstrophy-Epispadias Complex: A 40-Year Experience
Mahir Maruf1, Karl Benz1, John Jayman1
1Robert D. Jeffs Division of Pediatric Urology, Charlotte Bloomberg Children's Center, James Buchanan Brady Urological Institute, The Johns Hopkins Medical Institutions, Baltimore, MD.
Variant exstrophy-epispadias complex (EEC) requires accurate diagnosis for effective management. Surgical reconstruction, particularly primary bladder closure, yields high success rates and good continence outcomes, comparable to typical EEC cases.
Area of Science:
- Pediatric Urology
- Congenital Abnormalities
- Surgical Reconstruction
Background:
- Variant presentations of the exstrophy-epispadias complex (EEC) are rare and diverse, posing diagnostic and management challenges.
- Understanding these variants is crucial for optimizing patient care and outcomes.
Purpose of the Study:
- To investigate the diagnosis, surgical management, and outcomes of patients with variant exstrophy-epispadias complex (EEC).
Main Methods:
- A review of an institutional database (1975-2018) identified 1336 EEC patients, focusing on variant presentations.
- Variant types included skin-covered bladder exstrophy (BE), duplicate bladders, superior vesical fistula, and epispadias with major bladder prolapse.
- Surgical management and outcomes were assessed for 44 identified EEC variants.
Main Results:
- The most common variant was skin-covered BE (43%).
- Eighty-two percent of variants underwent primary bladder closure, with 58% also receiving pelvic osteotomy; 89% of closures were successful.
- Continence procedures were performed in 17 patients, with 8 achieving continence and volitional voiding even without such procedures.
Conclusions:
- Skin-covered bladder exstrophy is the most frequent EEC variant.
- Accurate initial diagnosis is vital for timely and appropriate management of EEC variants.
- Successful surgical reconstruction of EEC variants can lead to continence rates similar to or better than non-variant EEC.
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