Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes

No AccessJournal of UrologyPediatric Article15 Apr 2024Bladder Exstrophy Consortium (MIBEC) After 8 Years: The Short and Intermediate Term Outcomes Dana A. Weiss, Travis W. Groth, Suhaib Abdulfattah Abdulfattah, Sahar Eftekharzadeh, Ted Lee, Richard Lee, Douglas A. Canning, John V. Kryger, Aseem R. Shukla, Elizabeth B. Roth, Michael E. Mitchell, and Joseph G. Borer Dana A. WeissDana A. Weiss Corresponding Author: Dana A. Weiss, MD, 3500 Civic Center Blvd. 9th HUB, Urology Philadelphia, PA 19104 ( (email protected) ). , Travis W. GrothTravis W. Groth , Suhaib Abdulfattah AbdulfattahSuhaib Abdulfattah Abdulfattah , Sahar EftekharzadehSahar Eftekharzadeh , Ted LeeTed Lee , Richard LeeRichard Lee , Douglas A. CanningDouglas A. Canning , John V. KrygerJohn V. Kryger , Aseem R. ShuklaAseem R. Shukla , Elizabeth B. RothElizabeth B. Roth , Michael E. MitchellMichael E. Mitchell , and Joseph G. BorerJoseph G. Borer View All Author Informationhttps://doi.org/10.1097/JU.0000000000003971AboutPDF ToolsAdd to favoritesDownload CitationsTrack Citations ShareFacebookLinked InTwitterEmail Abstract Background: Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long term protection of the upper tracts. This study reports on our short term complications and intermediate term continence outcomes. Methods: A single prospective database for all patients undergoing surgery with a bladder exstrophy epispadias complex diagnosis at three institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries, including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies and immobilization techniques, and subsequent surgeries. Data on short term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound (RBUS), presence of vesicoureteral reflux (VUR), cortical defects on nuclear scintigraphy (DMSA), and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization (CIC), and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. Results: CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (IQR) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Bucks traction). Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate term outcomes were available for 40 of the 46 patients who have between 4 and 8 years of follow up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals, demonstrated in the summary table. Conclusions: Cumulative efforts of prospective data collection have provided granular data for evaluation. Short term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow up are voiding with dry intervals of > 1 hour. © 2024 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Advertisement Copyright & Permissions© 2024 by American Urological Association Education and Research, Inc.Keywordsbladder exstrophyreconstructive surgical procedurespediatricscomplete primary repair of exstrophy (CPRE)ConsortiumMetrics Author Information Dana A. Weiss Corresponding Author: Dana A. Weiss, MD, 3500 Civic Center Blvd. 9th HUB, Urology Philadelphia, PA 19104 ( (email protected) ). Co-first authors. More articles by this author Travis W. Groth Co-first authors. More articles by this author Suhaib Abdulfattah Abdulfattah More articles by this author Sahar Eftekharzadeh More articles by this author Ted Lee More articles by this author Richard Lee More articles by this author Douglas A. Canning Deceased. More articles by this author John V. Kryger More articles by this author Aseem R. Shukla More articles by this author Elizabeth B. Roth More articles by this author Michael E. Mitchell More articles by this author Joseph G. Borer More articles by this author Expand All Advertisement PDF downloadLoading ...