PD48-11 VARIANT PRESENTATIONS OF THE BLADDER EXSTROPHY-EPISPADIAS COMPLEX: A 40 YEAR EXPERIENCE

Field: Medicine (Health Sciences)

You have accessJournal of UrologyTrauma/Reconstruction/Diversion: Transitional Urology & GU Congenitalism1 Apr 2018PD48-11 VARIANT PRESENTATIONS OF THE BLADDER EXSTROPHY-EPISPADIAS COMPLEX: A 40 YEAR EXPERIENCE Mahir Maruf, Karl Benz, John Jayman, Jason Michaud, Matthew Kasprenski, Heather Di Carlo, and John Gearhart Mahir MarufMahir Maruf More articles by this author , Karl BenzKarl Benz More articles by this author , John JaymanJohn Jayman More articles by this author , Jason MichaudJason Michaud More articles by this author , Matthew KasprenskiMatthew Kasprenski More articles by this author , Heather Di CarloHeather Di Carlo More articles by this author , and John GearhartJohn Gearhart More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2018.02.2315AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES Variant presentations of bladder exstrophy-epispadias complex (BEEC) exhibit a wide range of abnormalities. The rarity and diversity of such BEEC variants can lead to challenges in the diagnosis and subsequent management of this population. Herein, the authors report their experience in the diagnosis, surgical management and outcomes in patients with variant BEEC. METHODS The authors reviewed a prospectively maintained institutional database of 1311 exstrophy-epispadias patients from 1975 to 2017 for variant presentations of BEEC. Variant presentations included those with pseudoexstrophy, epispadias with prolapsing bladder, skin covered bladder exstrophy (BE), duplicate bladders, and superior/inferior vesical fistula. Surgical outcomes of reconstruction and urinary continence were assessed. RESULTS In total, 37 BEEC variants were identified. Nine patients (24%) presented with epispadias with bladder prolapse. Bladder closure was delayed by 287 days [IQR 169-537] in this group, significantly later than the other BEEC variants (23 days [IQR 4-245]; p=0.010). Nineteen patients (51%) presented with a skin covered BE variant. A primary closure was conducted in 16 of these patients, in which 14 (88%) were successful. Non-skin-covered variants were categorized as either duplicate exstrophy (n=5) or superior/inferior vesical fistula (n=4). Duplicate exstrophies underwent either reconstruction to combine bladder halves or excision of one bladder duplicate. All of the superior/inferior vesical fistulas required a bladder closure, and 2 ultimately required a continence procedure. No pseudoexstrophy patients were observed. Overall, thirty patients underwent primary bladder closure within a median of 135 days (range 1-2010) after birth, with 20 (65%) undergoing pelvic osteotomy. Primary closures were successful in 87% of cases. Continence procedures were performed in 15 patients, including 8 bladder neck reconstructions (BNR) and 7 continent urinary diversions (CUD). With a median follow-up time of 11.1 years (range 0.2-36.3), 15 patients were continent, of which 7 did not require any continence procedure. CONCLUSIONS The most common BEEC variant is the skin covered form. Delayed closures may occur, particularly in epispadias with prolapsing bladder, potentially due to a delay in diagnosis. In order to expedite appropriate management, accurate diagnosis upon initial presentation is crucial. Successful surgical reconstruction often results in continence that is similar or better to non-variant BEEC presentations. © 2018FiguresReferencesRelatedDetails Volume 199Issue 4SApril 2018Page: e964 Advertisement Copyright & Permissions© 2018MetricsAuthor Information Mahir Maruf More articles by this author Karl Benz More articles by this author John Jayman More articles by this author Jason Michaud More articles by this author Matthew Kasprenski More articles by this author Heather Di Carlo More articles by this author John Gearhart More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...