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Single-stage repair of bladder exstrophy in older children and children with failed previous repair
Journal of Pediatric Urology (2007) 3, 391e394
Single-stage repair of bladder exstrophy in older children and children with failed previous repair Mohamed Youssif*, Haytham Badawy, Ashraf Saad, Ahmed Hanno, Ibrahim Mokhless Section of Pediatric Urology, Department of Urology, University of Alexandria School of Medicine, Alexandria, Egypt Received 4 September 2006; accepted 7 January 2007 Available online 26 March 2007
KEYWORDS Exstrophyeepispadias; Single stage repair; Bladder reconstruction; Osteotomy
Abstract Aim: Evaluation of cosmetic and functional outcome of single-stage exstrophye epispadias complex repair in older children and those with previously failed repair. Materials and methods: This study comprised 15 children (12 boys and 3 girls) with classic bladder exstrophy and a mean age at repair of 8.6 months (range 2e24 months). Eight children had a previously failed repair. All children underwent complete primary repair using the singlestage Mitchell technique. Half of the boys had complete penile disassembly, while in the others a modified CantwelleRansley technique for epispadias repair was used. Anterior iliac osteotomy was performed and hip spica used for immobilization in all children. Results: One child had urethral stricture treated by endoscopic visual urethrotomy. Three children had penopubic fistulae that closed spontaneously. No bladder dehiscence or prolapse was encountered. Vesicoureteral reflux was present in 20 renal units but ureteral reimplantation was not performed. Average bladder capacity after closure was 134 cm3 (range 110e 160 cm3) with only two partially continent and six incontinent children. Mean follow-up period is 2 years (range 1e3 years). Conclusions: Single-stage repair was performed in children with previously failed repair and those presenting at an older age with satisfactory results. Acceptable bladder and genital anatomy and function were achieved together with preservation of renal function. The impact of this technique on continence is not encouraging, but needs to be determined in a longer follow-up period. ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Reconstruction of exstrophyeepispadias complex represents a challenge to pediatric urologists. Repair of classic
* Corresponding author. Tel./fax: þ20 3 4860029. E-mail address: [email protected] (M. Youssif).
bladder exstrophy aims at successful bladder and abdominal wall closure with cosmetically and functionally acceptable external genitalia. Achieving continence and preservation of renal function are considered the most difficult aspect of the repair [1]. The repair of bladder exstrophy has evolved during the last 150 years [2]. Initially, repair was directed at reconstruction of the abdominal wall defect using different forms
1477-5131/$30 ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2007.01.193
392 of flaps and full-thickness skin grafts [3]. The major drawback of these techniques was that they did not address any functional aspect of the repair. In 1942, Young presented the first successful primary closure of bladder exstrophy [4]. Since then, all the techniques used have been aimed to address functional as well as anatomical aspects of the anomaly. Many reports of single-stage repair of bladder exstrophy have been presented but with disappointing results concerning urinary continence and renal function [5]. The staged repair proposed by Jeffs and co-workers has been adopted and accepted as the standard treatment of bladder exstrophy. The good results presented in his study were based on highly select groups [6e8]. In the staged technique, the child is exposed to multiple and repeated operations to achieve the ultimate goal of the repair. This drawback stimulated pediatric urologists to reconsider the single-stage repair [9]. Complete primary repair of exstrophy (CPRE) has been addressed in the neonatal period with many reports in the literature [10]. The aim was to optimize the chance of early bladder cycling and to potentiate bladder development. Here, we present our experience in performing this technique in older children and children with failed primary closure. Achieving a satisfactory outcome regarding successful closure, continence, preservation of renal function and cosmetically acceptable genitalia in such a group was evaluated [9].
Materials and methods Between January 2000 and December 2004, 15 children with classic bladder exstrophy underwent complete primary repair using Mitchell’s technique (CPRE). There were 12 boys and three girls. Seven children had been referred to our department after failed initial bladder closure. This had involved simple closure of the bladder without epispadias or bladder neck reconstruction and without performing an osteotomy. One child had failed single-stage Mitchell’s technique (CPRE) performed during the neonatal period in our department. The remaining seven patients were referred for consultation later in life with no previous surgery. Plain X-ray of abdomen and pelvis was performed in all children to assess the degree of pubic diastases. Ultrasound of the urinary tract was performed before the operation to detect any associated anomaly of the upper urinary tract and to serve as a baseline for follow up of renal growth. In the female patients, single-stage repair was performed with complete mobilization of the urethra and vagina as a single unit and repositioning of them deep in the pelvis using a Y-V advancement incision to restore the anatomical relationship of the vaginal and urethral complex with the pelvic diaphragm [10]. In the male patients, we used the standard single-stage Mitchell technique with complete penile disassembly in six boys, while the other six boys were repaired with a modified CantwelleRansley technique where the urethral plate is left attached distally to the glans and the distal corporal bodies [9,11,12]. In 10 boys we were able to identify the perineal urethral complex using low-current bipolar diathermy. We approximated the muscle complex around the urethra in the midline [13].
M. Youssif et al. Preoperative testosterone cream was used in all boys to enhance the growth of the urethral plate and improve the vascularity of the penile tissues, especially in redo cases [14]. Bilateral anterior oblique iliac osteotomies via separate incisions were performed in all cases. The bladder was closed over a 12-Fr suprapubic tube which was left for 2 weeks together with 3.8-Fr ureteral stents left for 10 days and a 6-Fr urethral catheter left for 1 week. Postoperative immobilization was assured by hip spica cast for all patients for 4 weeks. Postoperative antibiotics were administered for 5 days and then oral suppressive therapy in the form of trimethoprim 2 mg/kg per day was given up until the time of cystourethrogram. Repair of indirect inguinal hernia was performed in the same sitting in eight children and after 6 months in the remaining children. Ultrasound of the urinary tract was performed regularly every 3 months during the first year postoperatively and every 6 months thereafter. Cystourethrogram was obtained 6 months after the repair.
Results Mean age at surgery was 8.6 months (range 2e24 months). The average operative time was 6.5 h (range 6e8 h). The average length of pubic diastasis measured in plain X-ray was 5.4 cm (range 4e6 cm). Of the six boys in whom standard Mitchell’s technique with complete penile disassembly was performed, two had no previous repair and four had previous failed bladder closure performed at other institutions. None had glans or corporal atrophy or urethral loss. Hypospadias was the outcome in all of these children, proximal in three and midpenile in three. Successful repair of the resulting hypospadias was performed 6 months following the operation. Mild dorsal chordee was noticed in one child and repaired thereafter by simple dorsal multiple Z-plasty. No urethral fistulae were encountered. One child had a urethral stricture successfully treated by endoscopic visual urethrotomy. Of the other six boys, who had single-stage repair combined with modified CantwelleRansley technique for epispadias repair, there were three new and three redo cases. There was an orthotopic meatus in all cases, and dorsal penile chordee was present in four patients. Penopubic fistulae were detected in three patients but they were small and closed spontaneously without surgical intervention. No urethral stricture or bladder dehiscence was encountered. Of the female patients, we had two new cases and one redo. We did not encounter any dehiscence, prolapse or bladder fistula. Prolonged leak was encountered in one girl. She was closed primarily without bladder augmentation in spite of a small bladder plate. Augmentation cystoplasty was not performed in any of our children at time of repair or in the follow-up period. VUR ranging from grade II to IV was encountered in 20 out of 30 renal units. Pyelonephritis was reported in three children and managed conservatively, and none needed ureteral reimplantation with an average follow-up period of 2 years (range 1e3 years). Bilateral hydroureteronephrosis grade III was observed in one child with urethral stricture and improved after endoscopic visual urethrotomy. Renal
Single-stage repair in older/previously failed exstrophy patients growth observed by serial ultrasound was normal for the age and weight of these children. Average bladder capacity of 134 cm3 (range 110e160 cm3) was achieved after closure. Children treated for exstrophy are classified regarding continence into three categories, namely: continent children who are able to hold urine for 3 h with no leak day or night, partially continent who have intervals ranging from 1 to 3 h but with some wet nights and or stress incontinence, and incontinent children who cannot hold urine for a period of 1 h [14]. Of the eight children older than 2.5 years in this series, there were two partially continent and six incontinent children.
Discussion Primary repair of the exstrophyeepispadias anomaly is challenging and several procedures may be needed. We can imagine the extreme difficulty facing surgeons in treating children with previous failed repair [15]. We have aimed here to present our experience in the management of these failed repairs and older children using the singlestage Mitchell’s technique, and to illustrate the possibility of solving this problem with the least number of operations. Long-term follow up will determine the benefit of the single-stage repair in such a group of children concerning the most important issue: continence. During the last 4 years, we have managed 15 children with classic bladder exstrophy presenting with failed primary repair or at an older age, and the procedure was performed by two experienced surgeons. There was no dehiscence among our cases, which may be because we performed an anterior iliac osteotomy as a routine part of the repair. Trendelenberg (see ref. [2]) addressed the importance of pubic bone approximation in front of the bladder to prevent dehiscence. This fact was also emphasized by Gearhart who addressed the importance of osteotomy in any child after the age of 72 h, and in the neonatal period if there is an intersymphyseal distance of more than 4 cm or if there is any difficulty in approximating the pubic symphysis at the end of the operation without tension [16]. Postoperative immobilization by either hip spica or external fixators was also emphasized [9,16]. El-Sherbiny et al. have presented their experience with 19 children, among whom four had been repaired using the Mitchell technique in the neonatal period with no osteotomy. The remaining children, including seven with primary failed repairs, were operated upon using osteotomies with no case of dehiscence of the bladder [15]. The average duration of the operation in this study was 6.5 h which is longer than in other series [9]. This could be explained by adding the time for osteotomy, the simultaneous repair of inguinal hernia and the nature of our cohort of children having previous failed repair compared to the new neonatal cases presented by Grady and Mitchell [9]. We operated upon 12 boys using the single-stage repair. In the first 6 boys, complete penile disassembly was performed with a satisfactory genital anatomy except in one who had mild dorsal chordee corrected by Z-plasty. We did not encounter any fistulae but hypospadias was encountered in all boys necessitating another procedure. In the following 6 boys, the modified CantwelleRansley
393
technique was used. There was an orthotopic meatus in all of them but the major drawback was the higher incidence of dorsal chordee and the relatively shorter penis. This drew our attention to an important observation that leaving the short urethral plate attached to the glans together with repositioning the bladder deep in the pelvic ring may be responsible for such chordee. So, in spite of the hypospadias resulting from complete penile disassembly, we advise using it as described in the series of Hafez et al. [1], Grady and Mitchell [9], and El-Sherbiny et al. [15]. We had no glans or corporal atrophy owing to the careful dissection of the laterally placed neurovascular bundles and the preservation of the ventral urethral wedge, which represents the principal blood supply of the urethra. In contrast, Husmann and Gearhart [17] reported a high incidence of glans atrophy, corporeal loss and urethral stricture among their children using complete penile disassembly. We believe that careful dissection and preservation of the blood supply are the key to successful repair. We did not perform any augmentation cystoplasty contrary to the series of Hafez et al. [1], where this was performed in two girls owing to small bladder plates. Surer et al. [14] has addressed the fact that with an increased number of failed repairs and with increase in the age of the child at the time of first repair, there is a decreased chance of having a bladder with good capacity. In reporting the results of repair of the exstrophye epispadias complex, we are deeply concerned with the achievement of continence together with the preservation of the upper urinary tract. In our series we had two partially continent and six incontinent children. Grady and Mitchell [9] reported 80% continence among their children managed in the neonatal period, an early result depending on dryinterval criteria. This might shed light upon the importance of neonatal rather than delayed repair. At longer follow up, Grady et al. [18] reported that bladder neck reconstruction was needed in 72% of females and 86% of males to achieve continence. Borer et al. [19] showed that 63% of their patients needed bladder neck reconstruction at 4 years following CPRE. El-Sherbiny et al. [15] reported only 30e 90 min of dry intervals with regular voiding in only 5 children out of 15 repaired either at an older age or as redo cases. Baird et al. [20] reported that when combined exstrophy and epispadias closure is applied to a failed previous repair one can expect at best a 50% continence rate. Whether greater continence is achieved by single-stage repair than the modern staged approach, this seems to be a multifactorial outcome in which many variables are implicated, and long-term follow up will be needed to clarify this issue. Preservation of the upper tract is as important as achieving continence. The most deleterious factor is VUR encountered after bladder closure. In our practice, suppressive therapy was given to all children until performing cystogram and identifying children with no reflux. We reported reflux in 20 renal units out of 30. Only three children had a single attack of pyelonephritis treated medically with no further need to perform ureteral reimplantation. Grady and Mitchell [9] reported reflux in 12 out of 18 children; nine of them needed reimplantation due to repeated pyelonephritis. The high incidence of ureteral reimplantation was a major reason for criticism of the
394 Mitchell technique. This high incidence of reflux stimulated others to perform a Gilvernet ureteral advancement with the primary repair to prevent future need of reimplantation [21]. In our series we had optimum renal growth and no indication to perform ureteral reimplantation. In general, the final cosmetic appearance was satisfactory based on the opinion of the parents and observation of the genitalia.
Conclusion Single-stage exstrophy repair of older children and those with previous failed repair is feasible with a low incidence of complications. We strongly recommend complete penile disassembly for concomitant repair of the epispadias, in spite of the resulting hypospadias in most of these children. The presence of VUR necessitates the use of postoperative suppressive therapy with no increased need to perform ureteral reimplantation. The results concerning continence are not satisfactory and a bladder neck reconstruction procedure will be needed in many of these children to achieve dryness.
References [1] Hafez AT, Elsherbiny MT, Ghoneim MA. Complete repair of bladder exstrophy: preliminary experience with neonates and children with failed initial closure. J Urol 2001;156:2428e30. [2] Kulkarni B, Jiwane A, Tarun K. Anatomical repair of exstrophy bladder. Pediatr Surg Int 2004;20:455e9. [3] Grady R, Mitchell M. Newborn exstrophy closure and epispadias repair. World J Urol 1998;16:200e4. [4] Young H. Exstrophy of the bladder: the first case in which a normal bladder and urinary control have been obtained by a plastic operation. Surg Gynecol Obstet 1942;774: 729e19. [5] Megalli M, Lattimer J. Review of the management of 140 cases of exstrophy of the bladder. J Urol 1973;109:264e8. [6] Jeff R, Charrois R, Many M. Primary closure of the exstrophy bladder. In: Scott Jr R, Gordon H, Carlton C, et al., editors. Current controversies in urologic management. Philadelphia: W.B. Saunders; 1972. p. 235e43. [7] Jeffs R, Guice S, Oesch I. The factors in successful exstrophy closure. J Urol 1982;127:974e6.
M. Youssif et al. [8] Lepor H, Jeffs R. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J Urol 1983; 130:1142e5. [9] Grady R, Mitchell M. Complete primary repair of exstrophy. J Urol 1999;162:1415e20. [10] Grady R, Carr M, Mitchell M. Complete primary closure of bladder exstrophy. Epispadias and bladder exstrophy repair. Urol Clin North Am 1999;26:95e109. [11] Surer I, Baker I, Jeffs R, Gearhart J. Modified Cantwell-Ransley repair for exstrophy and epispadias: 10 year experience. J Urol 2000;164:1040e2. [12] Gearhart J, Sciortino C, Ben Chaim J, Poppas D, Jeffs R. The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned. Urology 1995;64:92e5. [13] Caione P, Capozza N, Lais A, Matarrazo E. Periurethral muscle complex reassembly for exstrophy epispadias repair. J Urol 200;164:2062e6. [14] Surer I, Baker I, Jeffs R, Gearhart J. Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex. J Urol 2001;165:2425e7. [15] El-Sherbiny MT, Hafez AT, Ghoneim MA. Complete repair of exstrophy: further experience with neonates and children after failed initial closure. J Urol 2002;168:1692e4. [16] Gearhart J. Complete repair of the bladder exstrophy in the newborn: complications and management. J Urol 2001;165: 2431e3. [17] Husmann D, Gearhart J. Loss of penile glans and corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J Urol 2004;172:1696e700. [18] Grady R, Joyner B, Feng W, Casale P, Mitchell M. The complete primary repair of exstrophy (CPRE) technique for repair of bladder exstrophy and epispadias: Long term follow up. Presented at the Section on Urology, American Academy of Pediatrics, New Orleans, LA, USA, 1e3 November 2003. [19] Borer JG, Gargollo PC, Hendren WH, Diamond DA, Peters CA, Atala A, et al. Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 2005;174: 1674e8 [discussion 1678e9]. [20] Baird AD, Mathews RI, Gearhart JP. The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences. J Urol 2005; 174:1421e4. [21] Garat J, De La Pena E, Caffaratti J, Villavicencio H. Prevention of vesicoureteral reflux at the time of complete repair of the exstrophy-epispadias complex. Int Urol Nephrol 2004;36: 211e2.
Single-stage repair of bladder exstrophy in older children and children with failed previous repair Mohamed Youssif*, Haytham Badawy, Ashraf Saad, Ahmed Hanno, Ibrahim Mokhless Section of Pediatric Urology, Department of Urology, University of Alexandria School of Medicine, Alexandria, Egypt Received 4 September 2006; accepted 7 January 2007 Available online 26 March 2007
KEYWORDS Exstrophyeepispadias; Single stage repair; Bladder reconstruction; Osteotomy
Abstract Aim: Evaluation of cosmetic and functional outcome of single-stage exstrophye epispadias complex repair in older children and those with previously failed repair. Materials and methods: This study comprised 15 children (12 boys and 3 girls) with classic bladder exstrophy and a mean age at repair of 8.6 months (range 2e24 months). Eight children had a previously failed repair. All children underwent complete primary repair using the singlestage Mitchell technique. Half of the boys had complete penile disassembly, while in the others a modified CantwelleRansley technique for epispadias repair was used. Anterior iliac osteotomy was performed and hip spica used for immobilization in all children. Results: One child had urethral stricture treated by endoscopic visual urethrotomy. Three children had penopubic fistulae that closed spontaneously. No bladder dehiscence or prolapse was encountered. Vesicoureteral reflux was present in 20 renal units but ureteral reimplantation was not performed. Average bladder capacity after closure was 134 cm3 (range 110e 160 cm3) with only two partially continent and six incontinent children. Mean follow-up period is 2 years (range 1e3 years). Conclusions: Single-stage repair was performed in children with previously failed repair and those presenting at an older age with satisfactory results. Acceptable bladder and genital anatomy and function were achieved together with preservation of renal function. The impact of this technique on continence is not encouraging, but needs to be determined in a longer follow-up period. ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Introduction Reconstruction of exstrophyeepispadias complex represents a challenge to pediatric urologists. Repair of classic
* Corresponding author. Tel./fax: þ20 3 4860029. E-mail address: [email protected] (M. Youssif).
bladder exstrophy aims at successful bladder and abdominal wall closure with cosmetically and functionally acceptable external genitalia. Achieving continence and preservation of renal function are considered the most difficult aspect of the repair [1]. The repair of bladder exstrophy has evolved during the last 150 years [2]. Initially, repair was directed at reconstruction of the abdominal wall defect using different forms
1477-5131/$30 ª 2007 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jpurol.2007.01.193
392 of flaps and full-thickness skin grafts [3]. The major drawback of these techniques was that they did not address any functional aspect of the repair. In 1942, Young presented the first successful primary closure of bladder exstrophy [4]. Since then, all the techniques used have been aimed to address functional as well as anatomical aspects of the anomaly. Many reports of single-stage repair of bladder exstrophy have been presented but with disappointing results concerning urinary continence and renal function [5]. The staged repair proposed by Jeffs and co-workers has been adopted and accepted as the standard treatment of bladder exstrophy. The good results presented in his study were based on highly select groups [6e8]. In the staged technique, the child is exposed to multiple and repeated operations to achieve the ultimate goal of the repair. This drawback stimulated pediatric urologists to reconsider the single-stage repair [9]. Complete primary repair of exstrophy (CPRE) has been addressed in the neonatal period with many reports in the literature [10]. The aim was to optimize the chance of early bladder cycling and to potentiate bladder development. Here, we present our experience in performing this technique in older children and children with failed primary closure. Achieving a satisfactory outcome regarding successful closure, continence, preservation of renal function and cosmetically acceptable genitalia in such a group was evaluated [9].
Materials and methods Between January 2000 and December 2004, 15 children with classic bladder exstrophy underwent complete primary repair using Mitchell’s technique (CPRE). There were 12 boys and three girls. Seven children had been referred to our department after failed initial bladder closure. This had involved simple closure of the bladder without epispadias or bladder neck reconstruction and without performing an osteotomy. One child had failed single-stage Mitchell’s technique (CPRE) performed during the neonatal period in our department. The remaining seven patients were referred for consultation later in life with no previous surgery. Plain X-ray of abdomen and pelvis was performed in all children to assess the degree of pubic diastases. Ultrasound of the urinary tract was performed before the operation to detect any associated anomaly of the upper urinary tract and to serve as a baseline for follow up of renal growth. In the female patients, single-stage repair was performed with complete mobilization of the urethra and vagina as a single unit and repositioning of them deep in the pelvis using a Y-V advancement incision to restore the anatomical relationship of the vaginal and urethral complex with the pelvic diaphragm [10]. In the male patients, we used the standard single-stage Mitchell technique with complete penile disassembly in six boys, while the other six boys were repaired with a modified CantwelleRansley technique where the urethral plate is left attached distally to the glans and the distal corporal bodies [9,11,12]. In 10 boys we were able to identify the perineal urethral complex using low-current bipolar diathermy. We approximated the muscle complex around the urethra in the midline [13].
M. Youssif et al. Preoperative testosterone cream was used in all boys to enhance the growth of the urethral plate and improve the vascularity of the penile tissues, especially in redo cases [14]. Bilateral anterior oblique iliac osteotomies via separate incisions were performed in all cases. The bladder was closed over a 12-Fr suprapubic tube which was left for 2 weeks together with 3.8-Fr ureteral stents left for 10 days and a 6-Fr urethral catheter left for 1 week. Postoperative immobilization was assured by hip spica cast for all patients for 4 weeks. Postoperative antibiotics were administered for 5 days and then oral suppressive therapy in the form of trimethoprim 2 mg/kg per day was given up until the time of cystourethrogram. Repair of indirect inguinal hernia was performed in the same sitting in eight children and after 6 months in the remaining children. Ultrasound of the urinary tract was performed regularly every 3 months during the first year postoperatively and every 6 months thereafter. Cystourethrogram was obtained 6 months after the repair.
Results Mean age at surgery was 8.6 months (range 2e24 months). The average operative time was 6.5 h (range 6e8 h). The average length of pubic diastasis measured in plain X-ray was 5.4 cm (range 4e6 cm). Of the six boys in whom standard Mitchell’s technique with complete penile disassembly was performed, two had no previous repair and four had previous failed bladder closure performed at other institutions. None had glans or corporal atrophy or urethral loss. Hypospadias was the outcome in all of these children, proximal in three and midpenile in three. Successful repair of the resulting hypospadias was performed 6 months following the operation. Mild dorsal chordee was noticed in one child and repaired thereafter by simple dorsal multiple Z-plasty. No urethral fistulae were encountered. One child had a urethral stricture successfully treated by endoscopic visual urethrotomy. Of the other six boys, who had single-stage repair combined with modified CantwelleRansley technique for epispadias repair, there were three new and three redo cases. There was an orthotopic meatus in all cases, and dorsal penile chordee was present in four patients. Penopubic fistulae were detected in three patients but they were small and closed spontaneously without surgical intervention. No urethral stricture or bladder dehiscence was encountered. Of the female patients, we had two new cases and one redo. We did not encounter any dehiscence, prolapse or bladder fistula. Prolonged leak was encountered in one girl. She was closed primarily without bladder augmentation in spite of a small bladder plate. Augmentation cystoplasty was not performed in any of our children at time of repair or in the follow-up period. VUR ranging from grade II to IV was encountered in 20 out of 30 renal units. Pyelonephritis was reported in three children and managed conservatively, and none needed ureteral reimplantation with an average follow-up period of 2 years (range 1e3 years). Bilateral hydroureteronephrosis grade III was observed in one child with urethral stricture and improved after endoscopic visual urethrotomy. Renal
Single-stage repair in older/previously failed exstrophy patients growth observed by serial ultrasound was normal for the age and weight of these children. Average bladder capacity of 134 cm3 (range 110e160 cm3) was achieved after closure. Children treated for exstrophy are classified regarding continence into three categories, namely: continent children who are able to hold urine for 3 h with no leak day or night, partially continent who have intervals ranging from 1 to 3 h but with some wet nights and or stress incontinence, and incontinent children who cannot hold urine for a period of 1 h [14]. Of the eight children older than 2.5 years in this series, there were two partially continent and six incontinent children.
Discussion Primary repair of the exstrophyeepispadias anomaly is challenging and several procedures may be needed. We can imagine the extreme difficulty facing surgeons in treating children with previous failed repair [15]. We have aimed here to present our experience in the management of these failed repairs and older children using the singlestage Mitchell’s technique, and to illustrate the possibility of solving this problem with the least number of operations. Long-term follow up will determine the benefit of the single-stage repair in such a group of children concerning the most important issue: continence. During the last 4 years, we have managed 15 children with classic bladder exstrophy presenting with failed primary repair or at an older age, and the procedure was performed by two experienced surgeons. There was no dehiscence among our cases, which may be because we performed an anterior iliac osteotomy as a routine part of the repair. Trendelenberg (see ref. [2]) addressed the importance of pubic bone approximation in front of the bladder to prevent dehiscence. This fact was also emphasized by Gearhart who addressed the importance of osteotomy in any child after the age of 72 h, and in the neonatal period if there is an intersymphyseal distance of more than 4 cm or if there is any difficulty in approximating the pubic symphysis at the end of the operation without tension [16]. Postoperative immobilization by either hip spica or external fixators was also emphasized [9,16]. El-Sherbiny et al. have presented their experience with 19 children, among whom four had been repaired using the Mitchell technique in the neonatal period with no osteotomy. The remaining children, including seven with primary failed repairs, were operated upon using osteotomies with no case of dehiscence of the bladder [15]. The average duration of the operation in this study was 6.5 h which is longer than in other series [9]. This could be explained by adding the time for osteotomy, the simultaneous repair of inguinal hernia and the nature of our cohort of children having previous failed repair compared to the new neonatal cases presented by Grady and Mitchell [9]. We operated upon 12 boys using the single-stage repair. In the first 6 boys, complete penile disassembly was performed with a satisfactory genital anatomy except in one who had mild dorsal chordee corrected by Z-plasty. We did not encounter any fistulae but hypospadias was encountered in all boys necessitating another procedure. In the following 6 boys, the modified CantwelleRansley
393
technique was used. There was an orthotopic meatus in all of them but the major drawback was the higher incidence of dorsal chordee and the relatively shorter penis. This drew our attention to an important observation that leaving the short urethral plate attached to the glans together with repositioning the bladder deep in the pelvic ring may be responsible for such chordee. So, in spite of the hypospadias resulting from complete penile disassembly, we advise using it as described in the series of Hafez et al. [1], Grady and Mitchell [9], and El-Sherbiny et al. [15]. We had no glans or corporal atrophy owing to the careful dissection of the laterally placed neurovascular bundles and the preservation of the ventral urethral wedge, which represents the principal blood supply of the urethra. In contrast, Husmann and Gearhart [17] reported a high incidence of glans atrophy, corporeal loss and urethral stricture among their children using complete penile disassembly. We believe that careful dissection and preservation of the blood supply are the key to successful repair. We did not perform any augmentation cystoplasty contrary to the series of Hafez et al. [1], where this was performed in two girls owing to small bladder plates. Surer et al. [14] has addressed the fact that with an increased number of failed repairs and with increase in the age of the child at the time of first repair, there is a decreased chance of having a bladder with good capacity. In reporting the results of repair of the exstrophye epispadias complex, we are deeply concerned with the achievement of continence together with the preservation of the upper urinary tract. In our series we had two partially continent and six incontinent children. Grady and Mitchell [9] reported 80% continence among their children managed in the neonatal period, an early result depending on dryinterval criteria. This might shed light upon the importance of neonatal rather than delayed repair. At longer follow up, Grady et al. [18] reported that bladder neck reconstruction was needed in 72% of females and 86% of males to achieve continence. Borer et al. [19] showed that 63% of their patients needed bladder neck reconstruction at 4 years following CPRE. El-Sherbiny et al. [15] reported only 30e 90 min of dry intervals with regular voiding in only 5 children out of 15 repaired either at an older age or as redo cases. Baird et al. [20] reported that when combined exstrophy and epispadias closure is applied to a failed previous repair one can expect at best a 50% continence rate. Whether greater continence is achieved by single-stage repair than the modern staged approach, this seems to be a multifactorial outcome in which many variables are implicated, and long-term follow up will be needed to clarify this issue. Preservation of the upper tract is as important as achieving continence. The most deleterious factor is VUR encountered after bladder closure. In our practice, suppressive therapy was given to all children until performing cystogram and identifying children with no reflux. We reported reflux in 20 renal units out of 30. Only three children had a single attack of pyelonephritis treated medically with no further need to perform ureteral reimplantation. Grady and Mitchell [9] reported reflux in 12 out of 18 children; nine of them needed reimplantation due to repeated pyelonephritis. The high incidence of ureteral reimplantation was a major reason for criticism of the
394 Mitchell technique. This high incidence of reflux stimulated others to perform a Gilvernet ureteral advancement with the primary repair to prevent future need of reimplantation [21]. In our series we had optimum renal growth and no indication to perform ureteral reimplantation. In general, the final cosmetic appearance was satisfactory based on the opinion of the parents and observation of the genitalia.
Conclusion Single-stage exstrophy repair of older children and those with previous failed repair is feasible with a low incidence of complications. We strongly recommend complete penile disassembly for concomitant repair of the epispadias, in spite of the resulting hypospadias in most of these children. The presence of VUR necessitates the use of postoperative suppressive therapy with no increased need to perform ureteral reimplantation. The results concerning continence are not satisfactory and a bladder neck reconstruction procedure will be needed in many of these children to achieve dryness.
References [1] Hafez AT, Elsherbiny MT, Ghoneim MA. Complete repair of bladder exstrophy: preliminary experience with neonates and children with failed initial closure. J Urol 2001;156:2428e30. [2] Kulkarni B, Jiwane A, Tarun K. Anatomical repair of exstrophy bladder. Pediatr Surg Int 2004;20:455e9. [3] Grady R, Mitchell M. Newborn exstrophy closure and epispadias repair. World J Urol 1998;16:200e4. [4] Young H. Exstrophy of the bladder: the first case in which a normal bladder and urinary control have been obtained by a plastic operation. Surg Gynecol Obstet 1942;774: 729e19. [5] Megalli M, Lattimer J. Review of the management of 140 cases of exstrophy of the bladder. J Urol 1973;109:264e8. [6] Jeff R, Charrois R, Many M. Primary closure of the exstrophy bladder. In: Scott Jr R, Gordon H, Carlton C, et al., editors. Current controversies in urologic management. Philadelphia: W.B. Saunders; 1972. p. 235e43. [7] Jeffs R, Guice S, Oesch I. The factors in successful exstrophy closure. J Urol 1982;127:974e6.
M. Youssif et al. [8] Lepor H, Jeffs R. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J Urol 1983; 130:1142e5. [9] Grady R, Mitchell M. Complete primary repair of exstrophy. J Urol 1999;162:1415e20. [10] Grady R, Carr M, Mitchell M. Complete primary closure of bladder exstrophy. Epispadias and bladder exstrophy repair. Urol Clin North Am 1999;26:95e109. [11] Surer I, Baker I, Jeffs R, Gearhart J. Modified Cantwell-Ransley repair for exstrophy and epispadias: 10 year experience. J Urol 2000;164:1040e2. [12] Gearhart J, Sciortino C, Ben Chaim J, Poppas D, Jeffs R. The Cantwell-Ransley epispadias repair in exstrophy and epispadias: lessons learned. Urology 1995;64:92e5. [13] Caione P, Capozza N, Lais A, Matarrazo E. Periurethral muscle complex reassembly for exstrophy epispadias repair. J Urol 200;164:2062e6. [14] Surer I, Baker I, Jeffs R, Gearhart J. Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex. J Urol 2001;165:2425e7. [15] El-Sherbiny MT, Hafez AT, Ghoneim MA. Complete repair of exstrophy: further experience with neonates and children after failed initial closure. J Urol 2002;168:1692e4. [16] Gearhart J. Complete repair of the bladder exstrophy in the newborn: complications and management. J Urol 2001;165: 2431e3. [17] Husmann D, Gearhart J. Loss of penile glans and corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J Urol 2004;172:1696e700. [18] Grady R, Joyner B, Feng W, Casale P, Mitchell M. The complete primary repair of exstrophy (CPRE) technique for repair of bladder exstrophy and epispadias: Long term follow up. Presented at the Section on Urology, American Academy of Pediatrics, New Orleans, LA, USA, 1e3 November 2003. [19] Borer JG, Gargollo PC, Hendren WH, Diamond DA, Peters CA, Atala A, et al. Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 2005;174: 1674e8 [discussion 1678e9]. [20] Baird AD, Mathews RI, Gearhart JP. The use of combined bladder and epispadias repair in boys with classic bladder exstrophy: outcomes, complications and consequences. J Urol 2005; 174:1421e4. [21] Garat J, De La Pena E, Caffaratti J, Villavicencio H. Prevention of vesicoureteral reflux at the time of complete repair of the exstrophy-epispadias complex. Int Urol Nephrol 2004;36: 211e2.