Bladder Exstrophy Consortium (MIBEC) after 8 years: The short and intermediate term outcomes
Dana A. Weiss, MD1, Travis W. Groth, MD2, Ted Lee, MD3, Suhaib Abdulfattah Abdulfattah, MD1, Katherine Sheridan, BS2, Karl F. Godlewski, MD1, Richard Lee, MD3, John V. Kryger, MD2, Aseem R. Shukla, MD1, Elizabeth B. Roth, MD2, Michael E. Mitchell, MD2, Joseph G. Borer, MD3.
1The Children's Hospital of Philadelphia, Philadelphia, PA, USA, 2Children's Wisconsin/Medical College of Wisconsin, Milwaukee, WI, USA, 3Boston Children's Hospital, Boston, MA, USA. Background Bladder exstrophy (BE) is a complex surgical problem with no optimal repair. A multi-institutional BE consortium utilizing a standardized surgical technique for the complete primary repair of exstrophy (CPRE), real time coaching, video capture and review of footage, and patient data analysis was created in 2013. We hypothesized that this consortium would minimize short-term complications (e.g., glans ischemia, bladder dehiscence), optimize volitional voiding with upper tract protection, and lower incidence of augmentation cystoplasty and/or bladder neck closure. MethodsData from a prospective multi-institutional database were queried to identify children with classic BE who underwent primary CPRE from February 2013-February 2021. Data recorded include sex, age at CPRE, adjunct surgeries at time of CPRE (ureteral reimplantation and hernia repair), osteotomies, subsequent surgeries, upper tract dilation, vesicoureteral reflux (VUR), cortical defects on nuclear scintigraphy (DMSA), and voiding and dryness status. Data on short-term (within 90 days after surgery) outcomes, as well as intermediate-term outcomes for patients with a minimum follow-up of 4 years, were abstracted. ResultsCPRE was performed in 92 patients over 8 years (30 girls, 62 boys), including 46 (17 girls, 29 boys) during the first 4 years. Median (IQR) age at CPRE was 79 (50.3) days. Bilateral osteotomies were performed in 89 (97%). 16 (17%) underwent ureteral
reimplantation and 13 (14%) hernia repair concurrently. Short term complications occurred in 29 (32%): fistulae - 7 (8%) (bladder 1, urethra 6), urethral stricture - 2 (2%), wound dehiscence without bladder involvement - 2 (2%), urinary retention (5 female, 1 male), and febrile UTI - 10 (11%). There were 14 subsequent surgeries within 90 days: cystoscopy and urethral catheterization (7), repair of internal bladder rupture (1), repair of wound dehiscence (1), inguinal hernia repair (3), removal of foreign body (1), and cystoscopy with meatoplasty (1). Intermediate term outcomes were available for 40 of the 46 patients with 4-8 years follow-up. 7/40 had mild hydronephrosis on ultrasound, and no patient had severe hydronephrosis. Cortical scarring on DMSA was identified in 13 of 31 (42%) and 9 of 19 (47%) who had a VCUG after the immediate post-op period had VUR (3 unilateral, 6 bilateral). Subsequent major surgeries included vesicostomy (2), appendicovesicostomy (3), bilateral ureteral reimplantations (6), hypospadias repair (1), and monsplasty (1). No patient underwent bladder neck closure or augmentation. Continence outcomes are in summary table. ConclusionsShort-term outcomes demonstrated no devastating complications, ie., penile injury or bladder dehiscence, however there were episodes of urinary retention, especially in girls. Intermediate-term data corroborate our approach of reserving BNC and AC for rare cases. Continence outcomes are on par with previous reports, with 40% of children with at least 4 years of follow-up having dry intervals of > 1 hour. Since dryness tends to improve with increasing age, further improvements in voiding per urethra are likely expected.
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