An Initial Report of a Novel Multi-Institutional Bladder Exstrophy Consortium: A Collaboration Focused on Primary Surgery and Subsequent Care (original) (raw)
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Prospective Followup in Patients After Complete Primary Repair of Bladder Exstrophy
The Journal of Urology, 2008
The new technique of complete primary repair of bladder exstrophy has offered the promise of improved bladder functional outcomes and yet longitudinal followup in patients with exstrophy who have undergone this form of closure is sparse. We present our median 5-year data on patients who have undergone complete primary repair of bladder exstrophy. Materials and Methods: The records of patients who underwent complete primary repair of bladder exstrophy were analyzed retrospectively. Patients were followed prospectively using case report forms, radiological and clinical data, and semistructured parental and patient interviews. We reviewed parameters including clinical events, scarring on renal scan, vesicoureteral reflux, surgical procedures, urodynamic studies, urinary and fecal continence status, and episodes of urinary tract infection. Results: From 1994 to 2007 complete primary repair of bladder exstrophy was performed in 32 patients. Of these patients 28 underwent closure within 72 hours of life, including 20 males and 8 females, and 4 underwent it after 72 hours at ages 7 days to 12 months. One patient underwent complete primary repair of bladder exstrophy elsewhere. Median followup was 5 years (range 1 to 13). In 32 patients a total of 193 surgical procedures were done, of which 40% were minor endoscopic cases. Six of 23 males (26%) underwent bladder neck reconstruction at a median age of 4.5 years (range 4 to 10). Two boys and 1 girl received Deflux® injection to the bladder neck at ages 3 to 5 years. To date no patient has undergone bladder augmentation. Nine patients (28%) had 1 to 4 episodes of pyelonephritis and 6 had cortical defects on renal scan. A total of 30 patients were voiding or incontinent via the urethra. One patient performed clean intermittent catheterization via the urethra and 1 performed it via appendicovesicostomy. All 4 children who were at least 6 months from bladder neck reconstruction after complete primary repair of bladder exstrophy had urinary continence periods of 2 to 3 hours or greater. Parents did not report any fecal incontinence or soiling in children older than 4 years. Relative to males the females had better urinary continence and a decreased need for bladder neck reconstruction. Conclusions: Complete primary repair of bladder exstrophy has been shown to be safe and efficacious. Prospective followup in this small number of evaluable patients reveals that continence periods of more than 2 hours are possible in patients after bladder neck reconstruction. Long-term followup in patients after complete primary repair of bladder exstrophy continues to be necessary to establish the long-term effects of this procedure.
Complete Primary Repair of Bladder Exstrophy (CPRE): Critical Analysis of the Long Term Outcome
Urology, 2018
To demonstrate the long term outcome of a contemporary series of 64 children who underwent CPRE in a single tertiary referral center. Between 1998 and 2012, 64 children; 47 boys and 17 girls were identified. Only 60 out of the 64 cases were available for follow up. Follow up was done by renal bladder ultrasound (RBUS) and serum creatinine every 3 months and Voiding cystourethrogram (VCUG) between 6 to 12 months post operatively. Continence was defined as dryness ≥3 hours. Median (range) follow up is 14 years (from 5 to 19 years). Voided continence was achieved in 14 children (23%) after CPRE only. Additionally, 6 children were continent after bladder neck reconstruction (BNR) and 2 after bladder neck injection (BNI) raising the percentage of voided continence to 36%. The remaining 38(64%) patients were using clean intermittent catheterization (CIC). All cases were continent at last assessment. The results of BNR and/or BNI were better in de novo than in redo cases (P<0.05). The p...
COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY: INITIAL EXPERIENCE WITH 33 CASES
The Journal of Urology, 2004
Purpose: We evaluated our initial experience with complete primary repair of bladder exstrophy in 33 children. Materials and Methods: Between 1998 and, 33 children with classic bladder exstrophy were treated with 1-stage primary repair for the first time in all except 4, who had undergone previous failed initial bladder closure. Our series included 26 boys and 7 girls with a mean age of 2 months (range 3 weeks to 14 months). The bladder was closed in continuity with the urethra and complete penile disassembly was used for epispadias repair. Anterior transverse innominate osteotomy was performed in all cases. Combined general and caudal anaesthesia were applied in all cases with an indwelling epidural caudal catheter in 7.
Osteotomy for Bladder Exstrophy: Commentary and Ten Tips for Success
Journal of the Pediatric Orthopaedic Society of North America, 2020
Patients with Bladder Exstrophy, though rare, are cared for at most Children's Hospitals. Although osteotomy is not always needed for closure, especially in the neonatal period, it can significantly increase the success treating those presenting late. Specific indications for osteotomy, as part of a reconstruction include wide diastasis as seen in cloacal exstrophy, re-closure after failed initial repair, patients with persistent abnormal perineal appearance, and uterine prolapse due to a wide pelvic floor.
Modern management of bladder exstrophy repair
Current urology reports, 2013
The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall musculature, and pelvic bones. Historically, surgical outcomes in patients with classic bladder exstrophy, the most common presentation of the exstrophy-epispadias complex, were poor. However, modern techniques have increased the success of achieving urinary continence, satisfactory cosmesis, and improved quality of life. Still, recent studies recognize complications that may occur during management of these patients. This review provides readers with an overview of the exstrophy-epispadias complex, the modern management of bladder exstrophy, and potential surgical complications.
Journal of Pediatric Urology, 2008
It is controversial as to whether osteotomy, by restoring a more normal pelvic anatomy, might improve the final outcome of bladder exstrophy (BE) repairs. We compared the functional orthopaedic and urological outcomes in BE patients treated with and without osteotomy. Orthopaedic and urological outcomes were compared in eight BE patients treated with osteotomy and six BE patients treated without osteotomy. Orthopaedic evaluation included an assessment of pubic bones dissymmetry, bending of the spine, presence of Trendelenburg or Thomas sign, and presence of out-toeing. Pubic diastasis was ruled out on a plain X-ray of the pelvis. A Pediatric Orthopedic Society of North America (POSNA) questionnaire was administered to every child or his/her caregiver to assess functional outcome. Urological evaluation included an assessment of required continence surgeries and of contemporary continence status. All patients presented a pubic diastasis. This was in median 49 (24-66) mm in patients treated without osteotomy and 42 (25-101) mm in those treated with osteotomy (p = 0.3). There was no difference either in the orthopaedic outcome or in any features of the POSNA questionnaire between groups. Neither was there a difference in the final continence rate nor in the number of additional continence procedures required. Although osteotomy is an essential step in the treatment of many BE patients in order to achieve a tension-free closure of the abdominal wall and bladder, our preliminary results suggest that it does not improve the eventual orthopaedic or urological outcomes of BE.
Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder
World Journal of Urology, 2006
The surgical management of classic bladder exstrophy has evolved over time. Different techniques are used to address the challenge of reconstructing these patients. We review the long-term outcomes of bladder exstrophy treatment from the published literature with regard to urinary continence, voiding and secondary complications. Continence now can be achieved in up to 80% of children in specialist centres. Whether such success can sustained into adult life is uncertain. About 40% of adults are dry in the best hands. Up to 84% of children can void, but there is some evidence that this function is lost with time in 70%. The need for bladder augmentation is widely variable between series, reported in 0-70% of children. This reduces the ability to void spontaneously to about 50% of children. It brings with it the later risk of metabolic disturbance and stone formation. Adults with exstrophy have a 694-fold increase in the risk of bladder cancer by the age of 40 years.
THE FAILED COMPLETE REPAIR OF BLADDER EXSTROPHY: INSIGHTS AND OUTCOMES
The Journal of Urology, 2005
Purpose: We describe the complications of complete repair and their management. Materials and Methods: A total of 19 patients were referred after failed complete repair. Total dehiscence occurred in 6 males, major bladder prolapse in 3, minor prolapse in 3, pubic separation in 1, impassable stricture in 1, and total hemiglans and corporal loss in 2. Overall, partial glans loss was seen in 7 patients, urethral loss in 5 and penile skin loss in 3. One female had complete dehiscence and 1 had major prolapse, both losing the urethrovaginal septum. One female had an impassable stricture.