COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY: INITIAL EXPERIENCE WITH 33 CASES (original) (raw)
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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.
The Journal of Urology, 2001
Purpose: The surgical repair of bladder exstrophy remains challenging for the pediatric urologist. We present our preliminary experience with complete primary repair of exstrophy in neonates and children with failed initial closure. Materials and Methods: Between November 1998 and April 1999, 9 boys and 2 girls with bladder exstrophy underwent complete repair. This procedure was performed in the first 72 hours of life in 4 boys and at age 3 months in 1 girl. Complete repair with osteotomy was performed after failed initial closure in 5 boys and 1 girl at a mean age of 28 months (range 15 to 36). The bladder and urethra were closed in continuity and epispadias was repaired by total penile disassembly. All patients were kept in a spica cast for 3 weeks. Ureteral stents and suprapubic tube were removed 10 and 14 days, respectively, after surgery. Ultrasound was performed preoperatively and every 3 months postoperatively, voiding cystourethrography was done 6 to 12 months after surgery.
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.
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.
Pediatric Surgery International, 2009
Purpose to report the early experience with the Kelly procedure for the treatment of bladder exstrophy (BE) in males. Materials and methods Nine boys with BE were treated at our institute. One had an untouched BE, four had epispadias after neonatal bladder closure, and four were secondary phalloplasties. Data on surgical complications, continence status, presence of erections and parental satisfaction with penile appearance and length are reported. Results Mean patient age was 4.7 (1-8.9) years. No intraoperative complications occurred. Two secondary cases experienced formation of a bladder-neck fistula and glans ischemia, respectively. The latter led to glans loss. All the patients had some residual degree of hypospadias after surgery. After a median follow-up of 18.1 (10-22) months, one patient developed chronic bladder outlet obstruction. Overall, five patients are dry (including two on clear intermittent catheterization and one with a Minz II pouch). The other four are still younger than 3 years, all have spontaneous micturitions and dry interval between 30 and 120 min. Of the eight patients without phalloplasty complications, all had erections, and parents judged the penile length and appearance as being satisfactory in six cases. Conclusion The Kelly procedure is feasible in a vast array of BE patients, but may be formidable, especially in secondary phalloplasties. It allows for complete reconfiguration and lightening of the penis, but exposes to potentially catastrophic complications, such as partial or complete penile loss. Longer follow-up is needed to assess the results in terms of continence.
Combined bladder exstrophy and epispadias repair
BMJ Case …, 2011
A 2-year-old male child of exstrophy-epispadias complex presented to us. He had not received any treatment at the time of presentation. It was decided to perform a combined bladder exstrophy and epispadias repair under general anaesthesia. There was a wide diastasis of symphysis pubis, it was decided to perform a bilateral anterior innominate and vertical iliac osteotomy. The bladder was closed in two layers. The urethroplasty and penile reconstruction was done by modifi ed Cantwell-Ransley repair. At the completion of procedure to prevent distraction of pubis, the baby was strapped using elastoplast bandage. The child had a very good cosmetic outcome, good pubic and rectus muscle approximation. On clamping the suprapubic catheter, the patient started passing urine per urethrally and there was a small penopubic fi stula. Thereafter the suprapubic catheter was removed. The urine culture was sterile and the patient was discharged on prophylactic antibiotics. The patient is due for follow-up.
Journal of King Abdulaziz University - Medical Sciences, 2016
Bladder exstrophy is a rare congenital anomaly displaying symptoms such as the urinary bladder turned inside out, an exposed dorsal urethra, foreshortened penis, anteriorly displaced anus and widely separated symphysis pubis. This abnormality is managed by many methods, one of which includes a functional reconstruction. Themajor goals of surgical intervention are: Preservation of the renal function, achieving urinary continence with adequate sexual function and acceptable cosmetic appearance. In this report we present a long term follow up (25 years) of a male patient with classical type bladder exstrophy, managed at our hospital in stages, he is fully continent and voids spontaneously without clean intermittent self-catheterization, with normal renal function, adequate penile erection and has ejaculation.