COMPLETE REPAIR OF BLADDER EXSTROPHY: PRELIMINARY EXPERIENCE WITH NEONATES AND CHILDREN WITH FAILED INITIAL CLOSURE (original) (raw)
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Complete Repair of Bladder Exstrophy in Boys: Can Hypospadias Be Avoided?
European Urology, 2005
Introduction: Complete repair of classic bladder exstrophy in male newborns has been successful with minimal morbidity. However, the technique may create hypospadias in some cases. We have recently adopted a modification to obtain an orthotopic meatus in bladder exstrophy boys. Materials and methods: Between November 1998 and December 2002 complete repair of classic bladder exstrophy was carried out in 27 boys. Complete penile disassembly was performed in 22 boys including 4 newborns and 18 older children; mean age 3 AE 2 years old. Modified disassembly was used in the last 5 boys including 4 newborns and a 9-month-old boy. During repair of epispadias, the dissection starts on the ventral aspect of the penis as usual. The urethral plate is separated from both corpora cavernosa to allow ventral transposition of the plate. In the complete disassembly technique, the urethral plate is completely separated from both hemiglans. In this modification, while the urethral plate is completely separated from both corpora cavernosa, the extreme distal end of the urethral plate remains attached to the distal ends of both hemiglans. Thus, when the plate is tubularized with fine interrupted sutures the meatus ends up at the tip of the glans penis. The symphysis pubis is re-approximated as usual and corpora cavernosa are approximated dorsally in the midline. Results: There was no major complication. Mean follow-up was 43 AE 7 and 7 AE 4 months for complete and modified disassembly groups respectively. Of the 22 boys, who underwent complete disassembly, 15 (68%) ended up with hypospadias and 7 (32%) had an orthotopic meatus. Modified disassembly has resulted in an excellent cosmetic appearance and orthotopic meatus in the 5 boys. Penile length was not shorter than boys who underwent complete disassembly. Parents of the 5 boys noticed normal straight morning erection. Conclusion: Although complete penile disassembly allows ventral placement of the urethra, hypospadias is created in approximately two thirds of the cases. When the extreme distal end of the urethral plate remains attached to the distal ends of both hemiglans (modified disassembly), orthotopic meatus can be obtained in all boys. With modified disassembly, posterior mobilization of the bladder and urethra does not result in corporal angulations or shortening because of the proportionate inward movement of the corpora that accompanies symphyseal approximation. The modification is feasible in newborns and infants. These short-term results may obviate the need for later penile reconstructive procedure.
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.
Long Term Follow Up of Complete Bladder Exstrophy Repair. A Case Report
Folia Medica, 2014
ABSTRACTOBJECTIVE: Bladder exstrophy is a congenital anomaly which is not always successfully managed by surgery. Major goals of surgical intervention in such cases are preservation of normal renal function, development of adequate bladder function and urinary continence and avoidance of future urinary tract infections. We present 5-year data on a patient who underwent complete repair of the bladder exstrophy. CASE REPORT: We describe a full term female infant who presented at birth with complete bladder exstrophy. Complete repair of the condition was performed 3 days after birth (Ransley technique). During hospitalization the patient had a positive urinary culture with Candida lusitaniae, enterococcus and septicemia with Klebsiella pneumoniae ESBL. The patient had no complications until the age of 20 months when she developed an episode of pyelonephritis and five further episodes of cystitis with E. coli. Radiographic testing showed small bladder capacity (23 ml at the age of 3 yea...
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 Repair of Bladder Exstrophy: Management of Resultant Hypospadias
The Journal of Urology, 2005
Purpose: In our hands complete primary repair (CPR) of bladder exstrophy results in hypospadias in two-thirds of boys. To our knowledge hypospadias repair following CPR of bladder exstrophy has not been reported previously. We report our experience with hypospadias repair following CPR of bladder exstrophy. Materials and Methods: A total of 22 boys with bladder exstrophy underwent CPR using Mitchell's technique between November 1998 and January 2002. The procedure resulted in hypospadias in 15 boys (68%). The site of the meatus was distal penile in 5 patients, mid penile in 3 and proximal penile in 7. Hypospadias repair was performed in all 15 boys. The 5 patients with distal penile hypospadias underwent repair consisting of Thiersch-Duplay urethroplasty with dorsal incision in 3 and tubularized vertical island flap with glanular tunnel in 2. All patients with mid (3) or proximal (7) penile hypospadias underwent Mustarde repair with glanular tunnel. Results: Followup ranged from 6 to 18 months (median 13). The Thiersch-Duplay procedure with dorsal incision resulted in fistula/stenosis in 2 of 3 boys. None of the remaining 12 boys with tubularized penile flap urethroplasty (tubularized vertical island flap or Mustarde) with glanular tunneling had development of fistula or stenosis. Complete degloving of the penis and penile skin redistribution were not necessary with the Mustarde technique. Conclusions: Thiersch-Duplay urethroplasty with dorsal incision does not seem to be a good option for hypospadias repair following CPR of bladder exstrophy. The use of penile skin as a tubularized flap with glanular tunneling seems to yield excellent functional/cosmetic results.
Urology, 2006
Objectives. To determine the continence and spontaneous voiding rate after neonatal reconstruction of bladder exstrophy without formal bladder neck reconstruction in patients undergoing primary reconstruction and treated with clean intermittent catheterization (CIC) after closure. Methods. From 1987 to 2003, 15 consecutive patients (8 boys and 7 girls) with bladder exstrophy underwent neonatal reconstruction. Reconstruction focused on bringing the bladder neck and proximal urethra intra-abdominally and meticulously closing the pelvic floor muscles around the urethra. Three weeks postoperatively, CIC was started until toilet-training age. Bladder capacity, continence status, renal anatomy and function, and additional urologic surgical procedures during follow-up were analyzed. Results. Nine patients (60%) became socially continent after primary closure without any additional bladder neck surgery. Twelve patients (80%) were continent when those who underwent endoscopic bulking injection were included. One patient became socially continent after bladder neck reconstruction, and one was dry and used CIC after bladder neck reconstruction and ileocystoplasty. One patient remained incontinent because of the parents' refusal of surgery. Ultimately, of 15 patients, 14 were dry (93%) of whom 10 were completely continent, 3 were partially continent (dry intervals of 1 to 3 hours), and 1 was dry by catheterizable stoma. The bladder capacity was adequate for age in 80% of patients. Febrile urinary tract infection occurred in 33% of patients, and 67% needed endoscopy for urethral stenosis. Upper tract dilation and loss of renal function was not seen. Conclusions. The results of our study have shown that primary repair of bladder exstrophy followed by CIC has encouraging continence and bladder capacity rates, with preservation of the upper urinary tract and limited need for additional bladder neck surgery. UROLOGY 67: 394-399, 2006.
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.