Complete Repair Of Bladder Exstrophy Research Articles

Commentary to ‘Continence after BNR in the complete repair of bladder exstrophy (CPRE): A single institution expanded experience'

Commentary to ‘Continence after BNR in the complete repair of bladder exstrophy (CPRE): A single institution expanded experience'

Continence after BNR in the complete repair of bladder exstrophy (CPRE): A single institution expanded experience

Several surgical methods have been used for primary repair of bladder exstrophy in the newborn. Complete primary repair of exstrophy (CPRE) aims to prevent the need for surgeries beyond the newborn period. Due to the rarity of bladder exstrophy, it has proven difficult in the past to analyze whether use of this method of closure truly does confer acceptable continence outcomes and hence minimizes the requirement for additional surgeries later in life. To describe the continence outcomes of CPRE patients who went on to receive bladder neck reconstruction (BNR), and secondarily, to compare clinical features between those patients who were able to receive undergo a BNR compared to those who were not. An IRB approved database of 1330 exstrophy-epispadias patients was used to identify referred patients after successful CPRE for management of continued urinary incontinence. Urinary continence outcomes were assessed in those who underwent modified Young Dees Leadbetter BNR following CPRE. Sixty-one patients were referred for treatment after successful CPRE between 1996 and 2016. None developed continence or a dry interval after primary closure. Of these, forty-two (68.9%) underwent BNR by a single surgeon at a mean age of 5.8 years (range 5-8.4). The mean bladder capacity at BNR was 147mL (range 102-210mL). Twenty-five (59.5%) achieved day and night continence, 7 (16.7%) gained daytime continence with nocturnal leakage, and 10 (23.8%) remain totally incontinent. Mean follow-up after BNR was 5.9 years. Combined CPRE and pelvic osteotomy were performed in 100% of patients who were continent and 75% of those who were daytime dry. No continent patient had a ureteral reimplantation before BNR, whereas 4 patients with daytime continence and nocturnal leakage and 7 patients who remained continuously incontinent did. This is the largest known series of BNRs in exstrophy patients closed by CPRE. Previous smaller studies have demonstrated mild to moderate success rates of BNR after CPRE, with many patients still requiring additional continence surgeries. The present study found similar results, with additional indication that successful primary closure and use of pelvic osteotomies may correlate with enhanced continence. This study includes outcomes from a single surgeon, with a maximum length of follow up of 13 years. CPRE alone often does not render patients continent of urine, based on the authors' referral population. However, following BNR continence rates in this subgroup were found to reach 76%. Surgeons who treat this population should keep these factors in mind when planning continence surgeries.

Mitchell's Detrusor Wrap-Around Bladder Neck (Dwbn), Ileocystoplasty, and the Extramural Serous-Lined Continent Outlet: An Optimal Triad

Purpose Providing continence for children with non compliant bladder and low detrusor leak point pressure secondary to neuropathic dysfunction or bladder exstrophy is a challenging task. A one stage reconstruction triad is presented. Material and Methods Between May 2005 to July 2008, 21 patients underwent DWBN, ileocystoplasty, and extramural serous lined tunnel as continent outlet in one stage. Patients included 15 boys and 6 girls with mean age of 7.6 years. Nine patients had previous complete repair of bladder exstrophy, 4 had staged repair and 8 had neuropathic bladder dysfunction. The ileum was fashioned in the shape of U in 4, S in 13, and W in 4. The appendix was used in 17 and Monti was used in 4. Neo umbilicus was constructed for the outlet in 13. In all patients the continent outlet was located in the umbilicus or a neo-umbilicus. Results Mean follow-up duration was 19 months (range 4-39). Early postoperative complications included prolonged ileus in 1 and urine leak in 1 and both were conservatively managed. Vesicoureteral reflux persisted in 13/21 (62%) patients. All patients were completely dry on CIC except 1. The leakge was through the urethra and was successfully managed by macroplastique injection at the bladder neck. No patient leaked through the continent outlet. Stomal stenosis was encountered in 2 patients with neo-umbilicus and both had stomal revision. No patient had bladder stones or perforation during follow-up. Conclusions Children with urinary incontinence secondary to the combination of a non-compliant bladder and sphincteric deficiency could be optimally managed with this triad. The DWBN provides optimal bladder neck reconstruction. The extramural serous-lined technique has the advantages of a leak-proof continent outlet, ease of catheterization through a straight channel, and the orthotopic position of the outlet in the umbilicus/neo-umbilicus in all patients.

Results of Bladder Neck Reconstruction After Newborn Complete Primary Repair of Exstrophy

We describe the results of modified Young-Dees-Leadbetter bladder neck reconstruction to achieve continence in patients who underwent complete primary repair of bladder exstrophy as newborns. Using the Johns Hopkins Exstrophy Database we identified patients who underwent bladder neck reconstruction after having undergone complete primary repair of bladder exstrophy as newborns. We determined patient characteristics and surgical outcomes. A total of 30 males and 3 females were referred after complete primary repair of bladder exstrophy, of whom 26 underwent bladder neck reconstruction. Results of complete primary repair of bladder exstrophy were strongly associated with osteotomy use. Of the patients 19 (58%), including 16 males and 3 females, who underwent complete primary repair of bladder exstrophy without osteotomy had complications (dehiscence and bladder prolapse), while none of 14 male patients who underwent complete primary repair of bladder exstrophy with osteotomy had complications, although none were subsequently continent. Of the 19 patients who had complications after complete primary repair of bladder exstrophy 12 underwent bladder neck reconstruction and total continence was achieved in only 3 (25%). Of the 14 patients with successful complete primary repair of bladder exstrophy 8 (57%) are dry day and night, 4 (28%) are dry during the day and wet at night, and 2 (14%) are completely incontinent. Continent children underwent successful complete primary repair of bladder exstrophy with pelvic osteotomy, all underwent hypospadias repair before age 1 year and none required ureteral reimplantation before bladder neck reconstruction. Many patients who undergo newborn complete primary repair of bladder exstrophy will require bladder neck reconstruction. Bladder neck reconstruction is more successful in those in whom complete primary repair of bladder exstrophy was successful. As in all types of repair, failed initial closure usually results in a bladder that is unsuitable for bladder neck reconstruction. These patients often require bladder augmentation and a continent stoma to be dry.

EARLY OUTCOME FOLLOWING COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY IN THE NEWBORN

Complete primary repair of bladder exstrophy (CPRE) represents a paradigm shift from the staged approach for surgical management. We present early clinical outcomes in our patients following CPRE. From 1996 to 2004 all newborns with bladder exstrophy were treated with CPRE within 48 hours of birth. We reviewed parameters including transfusions (packed red blood cells), urethral meatal position, complications, findings on renal ultrasound and renal scan, and post-CPRE procedures and infections. A total of 16 boys and 7 girls were treated with CPRE. Followup ranged from 8 to 96 months. Twelve boys (75%) and 2 girls (29%) received packed red blood cells at CPRE (p = 0.066). The tubularized urethral plate could not be brought to the penile tip, resulting in hypospadias in 9 of 11 boys (82%) with the running suture technique and only 1 of 5 boys (20%) with the interrupted technique (p = 0.036). Of the 23 patients 6 had a total of 8 complications after CPRE. Vesicoureteral reflux was present in 17 of 23 patients. After CPRE a total of 93 endoscopic/surgical procedures (median 4, range 0 to 16 per patient) were performed. Five patients had 1 to 4 episodes of pyelonephritis, 16 of 23 had 1 or more episodes of asymptomatic bacteriuria and 5 had cortical defects on renal scan. We recommend that urethral closure during CPRE be performed with interrupted suture technique to prevent hypospadias. An aggressive approach should be taken toward reflux in the setting of urinary infection. Consideration should be given for repair of all aspects of the defect during CPRE, including bilateral ureteral reimplantation.

Complete Repair of Bladder Exstrophy in Boys: Can Hypospadias Be Avoided?

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 ± 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 ± 7 and 7 ± 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.

LOSS OF THE PENILE GLANS AND/OR CORPORA FOLLOWING PRIMARY REPAIR OF BLADDER EXSTROPHY USING THE COMPLETE PENILE DISASSEMBLY TECHNIQUE

The technique of penile disassembly is increasingly being used for the surgical repair of classical bladder exstrophy. We describe the complications and discuss the possible etiologies of genital injuries we have seen following this operation. A review of the records of patients evaluated for management of genital complications following complete repair of bladder exstrophy from 1996 to 2003 was performed. Nine patients were evaluated for genital injuries following complete repair of bladder exstrophy using the penile disassembly technique. Injuries included the loss of 1 hemiglans and penile urethra in 2 cases; loss of 1 hemiglans and distal corpora in 2; loss of bilateral glans, distal corpora and penile urethra in 2; loss of 1 hemiglans, 1 corporal body, urethral plate and penile shaft skin in 1; loss of 1 hemiglans, distal corporal body and a portion of the urethra in 1; and loss of 1 hemiglans in 1. The exact etiology of these complications is unknown, and possibilities include, a technical mishap, induction of venous congestion/arterial spasm or disruption of a congenitally abnormal blood supply. The finding that primary repair of bladder exstrophy using the penile disassembly technique is associated with the risk of partial or complete penile loss dampens our enthusiasm for this procedure. The association of genital injuries with penile disassembly has resulted in a modification of this surgical technique that would hopefully lessen its risk.

575: Repair of Hypospadias Following Complete Repair of Bladder Exstrophy: An Evolving Experience

575: Repair of Hypospadias Following Complete Repair of Bladder Exstrophy: An Evolving Experience

COMPLETE REPAIR OF BLADDER EXSTROPHY IN THE NEWBORN: COMPLICATIONS AND MANAGEMENT

Complete repair of bladder exstrophy in the newborn includes bladder, and posterior and penile urethral closure, along with epispadias repair and abdominal wall closure without bladder neck reconstruction. While reports have shown the need for early reimplantation of the ureters in 50% of patients and repair of hypospadias in those in whom the urethral plate does not reach the tip of the glans, to my knowledge none has described immediate postoperative complications in these patients. Six males and 1 female were referred after "complete repair" procedure in the newborn period without osteotomy. Complete dehiscence with loss of the proximal urethra and a significant amount of penile skin occurred in 2 male patients. Bladder prolapse with loss of the urethra well onto the penis occurred in 2 newborns, of whom 1 had undergone 2 closures before referral and 1 had partial loss of the glans on 1 side. One patient had only minor bladder prolapse but lost most of the urethra and penile skin. The final male patient lost most of the urethra and had separation of the pubis but the bladder maintained a subcutaneous position and did not prolapse. The female patient had major prolapse of the bladder and loss of the urethrovaginal septum. The 2 patients with complete dehiscence underwent reclosure with bilateral innominate and vertical iliac osteotomy with replacement of the urethral plate with a skin graft and await epispadias repair. The patient with major prolapse and 2 prior closures was treated with reclosure, bladder augmentation and continent stoma reconstruction, and awaits epispadias repair. In the male patient with marked prolapse and partial loss of the glans has undergone reclosure with bilateral innominate and vertical iliac osteotomy was done and he awaits epispadias repair. The patient with minor prolapse was treated with reclosure with bilateral innominate and vertical iliac osteotomy, and reclosure with epispadias repair under testosterone stimulation. The patient in whom the pubis was separated and the urethra was lost underwent reclosure, epispadias repair and combined osteotomy under testosterone stimulation. The female patient was treated with reclosure with combined bilateral osteotomy and mobilization of an anterior bladder tube to use as a urethra with no attempt to establish continence. Complete repair of bladder exstrophy in the newborn period requires experience with the exstrophy condition, appropriate pelvic and patient immobilization, and excellent postoperative care. Complications with this repair are similar to those with modern versions of the staged repair, and its failures are similar. However, penile skin loss with dehiscence or prolapse is particularly difficult because of the paucity of skin already due to the exstrophy condition, for which skin grafting and testosterone stimulation are valuable adjuncts. Other than the patient who underwent augmentation and continent stoma construction, none has undergone any type of continence procedure, all are awaiting an increase in bladder capacity and followup is short. Complete repair of bladder exstrophy, whether in the newborn or older child, is a formidable task and not for the occasional exstrophy surgeon. While earlier reports of this procedure seem encouraging, complications are real and difficult to manage, even in experienced hands.

Complete repair of bladder exstrophy in the newborn: complications and management.

Complete repair of bladder exstrophy in the newborn includes bladder, and posterior and penile urethral closure, along with epispadias repair and abdominal wall closure without bladder neck reconstruction. While reports have shown the need for early reimplantation of the ureters in 50% of patients and repair of hypospadias in those in whom the urethral plate does not reach the tip of the glans, to my knowledge none has described immediate postoperative complications in these patients. Six males and 1 female were referred after "complete repair" procedure in the newborn period without osteotomy. Complete dehiscence with loss of the proximal urethra and a significant amount of penile skin occurred in 2 male patients. Bladder prolapse with loss of the urethra well onto the penis occurred in 2 newborns, of whom 1 had undergone 2 closures before referral and 1 had partial loss of the glans on 1 side. One patient had only minor bladder prolapse but lost most of the urethra and penile skin. The final male patient lost most of the urethra and had separation of the pubis but the bladder maintained a subcutaneous position and did not prolapse. The female patient had major prolapse of the bladder and loss of the urethrovaginal septum. The 2 patients with complete dehiscence underwent reclosure with bilateral innominate and vertical iliac osteotomy with replacement of the urethral plate with a skin graft and await epispadias repair. The patient with major prolapse and 2 prior closures was treated with reclosure, bladder augmentation and continent stoma reconstruction, and awaits epispadias repair. In the male patient with marked prolapse and partial loss of the glans has undergone reclosure with bilateral innominate and vertical iliac osteotomy was done and he awaits epispadias repair. The patient with minor prolapse was treated with reclosure with bilateral innominate and vertical iliac osteotomy, and reclosure with epispadias repair under testosterone stimulation. The patient in whom the pubis was separated and the urethra was lost underwent reclosure, epispadias repair and combined osteotomy under testosterone stimulation. The female patient was treated with reclosure with combined bilateral osteotomy and mobilization of an anterior bladder tube to use as a urethra with no attempt to establish continence. Complete repair of bladder exstrophy in the newborn period requires experience with the exstrophy condition, appropriate pelvic and patient immobilization, and excellent postoperative care. Complications with this repair are similar to those with modern versions of the staged repair, and its failures are similar. However, penile skin loss with dehiscence or prolapse is particularly difficult because of the paucity of skin already due to the exstrophy condition, for which skin grafting and testosterone stimulation are valuable adjuncts. Other than the patient who underwent augmentation and continent stoma construction, none has undergone any type of continence procedure, all are awaiting an increase in bladder capacity and followup is short. Complete repair of bladder exstrophy, whether in the newborn or older child, is a formidable task and not for the occasional exstrophy surgeon. While earlier reports of this procedure seem encouraging, complications are real and difficult to manage, even in experienced hands.

COMPLETE REPAIR OF BLADDER EXSTROPHY: PRELIMINARY EXPERIENCE WITH NEONATES AND CHILDREN WITH FAILED INITIAL CLOSURE

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. 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. Mean followup is 14 months (range 12 to 17). All repairs were successful including 1 case of penile scrotal duplication. Concomitant augmentation was done in 2 girls due to a small bladder plate. Complete closure resulted in hypospadias in 3 of the 9 boys. No patient had dehiscence or fistula. Serial followup ultrasound revealed no hydronephrosis and normal renal growth. Febrile urinary tract infection occurred in 1 case 2 months after surgery and was managed conservatively. Bladder capacity was 200 and 270 ml., at 6 months, respectively, in the 2 patients with an augmented bladder and both are dry on intermittent catheterization. The 4 patients in whom the closure was performed at birth are voiding with dry intervals with mean bladder capacity of 75 cc at 1 year (range 60 to 90). The 5 older children had a mean bladder capacity of 120 cc (range 70 to 150) at 6 months, of whom 2 are completely continent and 3 have 1 to 3 hours of dry intervals. Complete repair of bladder exstrophy is feasible in neonates and children after failed initial closure with minimal morbidity. There is no short-term evidence of worsening reflux or hydronephrosis. Longitudinal followup with adequate recording is required for continence evaluation. This technique may minimize the future need of bladder neck reconstruction and augmentation in patients with exstrophy.

COMPLETE PRIMARY REPAIR OF EXSTROPHY

The surgical correction of bladder exstrophy to achieve continence with voiding remains a challenging problem for the urologist. Since 1989 we have performed complete primary repair for exstrophy based on the concept that the primary defect of bladder and cloacal exstrophy is anterior herniation. Thus, the bladder and urethra must be treated as a single unit to move them posteriorly into the pelvis. We present this technique. From 1989 to 1997, 18 patients with bladder exstrophy and 6 with cloacal exstrophy underwent complete primary repair of exstrophy. This procedure was done on day 1 of life in 18 patients. Mean followup is 44 months (range 4 months to 8 years). At a median followup of 48 months 4 boys and 4 girls have volitional voiding after complete primary repair of bladder exstrophy, 21 patients have continent intervals and 2 boys void with continent intervals after complete primary repair of cloacal exstrophy. No patient has had a loss of renal function in this series. Postoperative complications included urethrocutaneous fistula formation in 2 cases. No patient had primary closure dehiscence. The rate of urinary continence achieved with complete primary repair compares favorably to that of staged repair for exstrophy. Complete primary repair also minimizes the number of surgical procedures required to achieve urinary continence and potentiates bladder neck function that permits bladder cycling in year 1 of life. The complication rates of these techniques are significantly lower than those reported in previous series of primary closure of exstrophy.