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Anterior Perineal Reconstruction in Exstrophy-epispadias Complex
European Urology
European Urology 47 (2005) 872–878
Anterior Perineal Reconstruction in Exstrophy-epispadias Complex Paolo Caione*, Nicola Capozza, Daniela Zavaglia, Mauro De Dominicis Division of Pediatric Urology, Department of Nephrology and Urology, ‘‘Bambino Gesu`’’ Children’s Hospital, Rome, Italy Received 30 December 2004; accepted 10 February 2005 Available online 9 March 2005
Abstract Objectives: To assess the role of correct anatomical reconfiguration of the anterior perineal musculature in exstrophy-epispadias (E-E) patients. To stress the use of a bipolar stimulator to detect the perineal muscular complex intraoperatively, and to increase the functional results of reconstruction in E-E patients. Methods: A total of 22 patients with E-E complex were treated in a 7-year period: 17 patients presenting classic bladder extrophy (aged 3 days to 6 years) and 5 incontinent male epispadias (aged 9 months to 16 years). An electric bipolar stimulator was used to identify and reapproximate at the midline the muscular fibers that constitute the periurethral muscular complex, as a part of the anterior perineal membrane. Outcome was evaluated at 24 months from surgery, considering bladder capacity, dry intervals, urinary infections (UTI’s), upper tract deterioration and surgical complications (fistula, obstruction, dehiscence). Results were compared with a matched group of 19 E-E patients treated in the previous 5-year period, without the presented technique (control group). Student T-test was used for statistical analysis, considering p 0.05 as significant. Results: No bladder neck or urethral dehiscence was observed. Mean bladder capacity at 2-year follow-up was 80 cc in the exstrophic patients and 120 cc in the male epispadias. Mean dry interval increased to 75 minutes in the exstrophy patients and to 130 minutes in the male epispadias. Full daytime continence was achieved in 3 exstrophic and in all the epispadic patients. The difference with the control group of patients was significant (p < 0.05). Conclusions: Proper identification of the anterior perineal muscular complex, using a bipolar stimulator, and its reapproximation at the posterior urethra on the midline was demonstrated to be effective in increasing bladder cycling and in developing adequate bladder volume, anticipating coordinated micturition. # 2005 Elsevier B.V. All rights reserved. Keywords: Bladder Exstrophy; Epispadias; Perineum; Urinary continence; Urethra
1. Introduction Surgical reconstruction of the E-E complex in newborns or in young infants represents one of the most challenging tasks in any Pediatric Urology center. The goal is to achieve a low urinary tract compliance and continence, with volitional voiding preventing renal damage. Urethral and genitalia reconstruction is not a * Corresponding author. Pressent address: Division of Pediatric Urology ‘‘Bambino Gesu`’’ Children’s Hospital Piazza S. Onofrio, 4 - 00165 Rome, Italy. Tel.: +39 06 68592337; Fax: +39 06 68592849. E-mail address: [email protected] (P. Caione).
secondary point, that should be achieved before the memory is fully established in young children. To date, the goals of functional reconstruction in E-E have not yet been adequately achieved, as shown by different newly proposed surgical techniques and several advances made in our knowledge of the principles for surgical repair [1]. Complete penile disassembly technique for epispadia repair [2] may be associated with the primary closure of the bladder exstrophy in a single-stage procedure [3]. The technique offers potential advantages in terms of anatomical reconstruction of the male external genitalia and in terms of positioning of the
0302-2838/$ – see front matter # 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.eururo.2005.02.005
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bladder neck and the urethra within the pelvis. We developed an extention of this technique that allows a more anatomical anterior perineal reconstruction, based on the identification of the perineal musculature using an electric stimulator during surgery, with its reapproximation on the sagittal line in periurethral position [4]. The outcomes of our 7-year experience using this technique are presented and discussed, comparing these with the results of the previous 5-years. 2. Materials and methods Bladder exstrophy closure and epispadias repair was performed in 17 consecutive patients, 13 males and 4 females, from March 1995 to February 2002. A single-stage procedure in neonatal age (within 72 hours of age) was performed in 5 cases, 2 males and 3 females with classic bladder exstrophy. The remaining 12 patients underwent a 2-stage repair: 11 exstrophic males and 1 female presenting cloacal exstrophy. Bilateral posterior iliac osteotomy was performed in all patients, excluding 3 newborns operated on within 48 hours of life for classic bladder exstrophy. During the same 7-year period, a total number of 5 primary penopubic male epispadias were treated, using the modified disassembly technique [5] (age 11 months to 5 years, mean age: 2.3 years), associated with the periurethral musculature reapproximation and anterior perineal reconfiguration. 2.1. Technique After bilateral posterior iliac osteotomy, the exstrophic bladder is closed with a suprapubic tube and the urethral plate is fully mobilized from the corpora in the male patient. The bladder neck tissue is freeded from the inner surface of the symphysis [4]. The prostate must stay joint with the urethral spongiosum tissue. The corporeal bodies and the glans are split on the sagittal plane according to Grady and Mitchell [3]. The midline incision is made in the intersymphyseal tissue and along the infrapubic space. It is mandatory to remain strictly on the midline, and the frequent use of bipolar stimulation helps to identify any striated muscular tissue and to maintain a symmetrical position. This tissue is usually present in the E-E complex, although it is displaced laterally due to pelvic dysmorphism. The use of the electrical stimulator enables us to observe small contractions of this muscular structures. Any available muscle is preserved and the perineal tissue are strictly divided on the midline [4]. No attempt is made to perform any plasty of the bladder neck, such as the Young-Dees or similar procedures. The exstrophic bladder neck groove is elongated with multiple incisions on both lateral aspects and tubulized on a 8 F silicon catheter, which is removed on the 4th to 5th postoperative day. The tubularized bladder neck and posterior urethra are then placed deeply into the pelvis, just anteriorly to the anorectal canal and levator ani fibers. The ‘‘anterior muscular complex’’, which is part of the pelvic floor musculature, is then reapproximated at the midline by 4/0 or 5/0 tiny sutures, closely to the tubularized posterior urethra (Fig. 1 A and B). The penile disassembly procedure with anterior epispadic male urethra repair [2,5] is carried out at the same time or as a second stage within 6 months. In the female patient, the bladder neck and urethral plate are mobilized together with the vagina, similarly to the posterior
Fig. 1. Periurethral musculature reapproximation. (A) The thiny anterior perineum musculature is identified using a bipolar electric stimulator, through a deep midline incision on the intersymphyseal space. (B) The periurethral muscular complex is gently reapproximated at the tubularized membranous urethra in a U-shaped fashion, restoring the anterior perineum anatomy; Suprapubic tube; Closed exstrophied bladder; Mobilized urethral plate; Corporeal bodies; Pubis bone; Midline intersymphyseal incision; Scrotum; Electric stimulator; Anterior perineal muscular complex and periurethral musculature reapproximation.
urethra and prostatic bundle in males. The urethral and vaginal structures are positioned ‘‘en block’’ posteriorly through a deep sagittal incision on the perineal body [6]. Vulvoplasty and clitoroplasty are accomplished. Patient outcome was evaluated at 24 months from the initial reconstructive surgery. Bladder capacity at 40 cm H2O pressure under anesthesia, dry intervals and day-time continence achievement were recorded, as well as any febrile UTI episodes, upper tract dilatation and surgical complications (male urethra fistula or obstruction, bladder neck/posterior urethra dehiscence, female uterine cervix procidentia). The study group results were compared with the outcomes of a population of 19 E-E patients (15 bladder exstrophy and 4 male epispadias), age and sex matched, who had been treated in the same institution by the some surgeon (PC) during the previous 5-year period, without adopting the surgical techniques of anterior perineal reconstruction with periurethral muscular reapproximation (‘‘Control group’’). For statistical analysis, the Student-T test was used to compare means (p < 0.05 was considered significant) and Wilcoxon/Mann Whitney to compare medians. Count data were analyzed by the Chi square Test or Fisher exact test, where appropriate.
3. Results In the study group, all patients underwent 1 or 2 calibrations of the urethra and bladder neck during the
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Table 1 Surgical outcomes at 24 months from anterior perineal reconstruction in e-e complex
STUDY GROUP Bladder Exstrophy Males Females Male Epispadias CONTROL GROUP Bladder Exstrophy Males Females Male Epispadias P (t- Student test)
Patients
Febrile UTIs
Upper tract dilatation
Bladder neck dehiscence
Male urethral fistula/stenosis
Female uterus procidentia
22 17 11 6 5 19 15 7 4 4
2 2* 1 1 0 6 6* 5 1 0 * < 0.05
8 7* 6 1 1 6 6* 4 2 0 *NS
0* 0
3* 3 3 – 0 9* 6 6 – 3 *0.05
0* 0
first 2 to 6 months after surgery. Low dose antibacterial prophylaxis was administered for at least 24 months after surgery with periodical urinalysis. Renal and bladder ultrasound scans were performed at 6-month intervals and cystogram under anesthesia with bladder capacity assessment was carried out at 2-year intervals from surgery. No bladder neck, urethral dehiscence or exstrophy relapses were observed. No uterine cervix procidentia occurred in female patients. A male newborn, who underwent bladder and penile reconstruction in a single stage at the beginning of this study, developed distal urethra stenosis with partial glans disrupture, as consequence of the island flap urethroplasty, performed to increase the distal length of the native urethra. Two urethral fistulas were observed at the coronal and mid-shaft position, which required surgical fermature. Mild upper tract dilatation was detected respectively in 7 patients (31.8%) of the exstrophic series and in 1 pure epispadias. Febrile UTIs were experienced in 2 patients with refluxes (Table 1). Bladder capacity increased mostly in the first 12–18 months after surgery and it was larger in the pure epispadic patients (range 50 to 180 mls, mean
0 3* 3 1 2 0 * < 0.05
0 2* 2 – 2 – *0.05
123 mls). A mean bladder capacity of 78 mls (30 to 110 mls) was achieved in the exstrophic patients (Table 2). No significant difference was observed between male and female patients (75 mls in males, 82 mls in females) or in the 3 newborns without osteotomies. Unilateral or bilateral vesico-ureteral refluxes were observed in 16 of 17 exstrophic bladders and in 2 of 5 penopubic epispadias. No ureteral reimplantation or bladder neck plasty was performed in the study group of patients within the follow-up period. Concerning bladder activity, cyclic voiding with urinary stream, associated with 30 to 120 minutes dry intervals were present in 11 of the 17 exstrophic patients at the 24 month follow-up. No patient achieved full urinary continence during the night-time. Four exstrophic patients (23.5%) experienced persistent daytime and night-time incontinence, with continuous urinary dribbling and poor functional bladder capacity. Day-time continence with volitionary micturition was observed in all 5 penopubic epispadic boys (Table 2). Spontaneous penile erections were observed in all the male patients (11 exstrophy and 5 primary penopubic epispadias) treated by our technique [5].
Table 2 Anterior perineal reconstruction in e-e complex: functional bladder outcomes at 24 months follow-up
BLADDER EXSTROPHY Study Group Control Group P (t- Student test) MALE EPISPADIAS Study Group Control Group P (t- Student test)
Patients (#)
Mean (Range) Bl. Capacity (cc)
Dry intervals (# pts)
Mean (range) Dry interval (mins)
Day-time continence (# pts)
17 15
78 (30–110) 45 (10–65) <0.05
11 2
75 (30–120) 55 (35–75) <0.05
3 (17.6%) 0
5 4
123 (50–180) 95 (55–140) <0.05
5 3
130 (45–180) 90 (30–140) <0.05
5 (100.8%) 2 (50%)
# pts = number of patients; cc = milliliters; mins = minutes.
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In the control group of 15 exstrophies, 6 patients experienced febrile UTIs and 3 suffered from some degree of bladder neck dehiscence. In 9 boys (6 exstrophies and 3 epispadias), urethral fistulas with/ without urethral stenosis were observed. In 2 exstrophic females, some degree of uterine procidentia was present. The difference with the study group outcomes was significant (p < 0.05, Table 1). Mean bladder capacity assessed at the 24 month follow-up in the control group was lower both in the exstrophic (mean volume = 45 mls, range 10 to 65 mls) and in the male epispadic patients (mean volume = 95 mls, range 55 to 140 mls). The difference was statistically significant (p < 0.05, Table 2). Only 2 exstrophic children of 15 patients and 3 of 4 male epispadias achieved dry intervals (mean = 55 minutes in exstrophic and 90 minutes in epispadic patients). The number of patients with dry intervals and its length were significantly reduced, compared with the study group children (p < 0.05, Table 2). Concerning adjunctive surgical procedures, 15 of 17 patients with bladder exstrophy and 1 of 5 epispadias of the study group received 1 to 3 (mean 1.8) endoscopic treatments with submucosal injection of dextranomer 1% in jaluronic acid as bulking material at the bladder neck, to increase bladder outlet resistance [7]. No major surgery was required during the 24-month follow-up, but 3 of 4 incontinent exstrophic patients are scheduled for bladder neck plasty/fermature with continent appendicovesicostomy procedure and bladder augmentation. All the 15 exstrophic patients and 1 of the 4 male epispadias of the control group needed bladder neck plasty with ureteral reimplantation at the age of 4 to 9 years. Seven exstrophic patients required continent appendicovesicostomy for intermittent catheterization and 5 of them received bladder neck tightening or fermature with augmentation. The difference between the study and the control group of patients, concerning the number of major surgical procedures after primary reconstruction, was statistically significant (p < 0.05).
4. Discussion To enable a child or a teenager born with E-E to participate fully in modern society, without significant functional, cosmetic or psychological handicap is still difficult. The complexity and number of the required reconstructive procedures is usually very high, especially in the exstrophic population. Urinary reservoir reconstruction is nowadays easily achieved, through primary closure with/without augmentation of the
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bladder. External genitalia can be repaired effectively by using the Cantwell-Ransley procedure [8] or the Mitchell technique [9]. Full urinary continence, obtained by volitional urination, remains probably the most difficult result to achieve for the Pediatric Urologist but, at the same time, it is the most pressing need for these patients as they reach school-age or start social engagements. The standard Young-Dees bladder neck plasty and its variants [10,11,12] are still widely used to increase bladder outlet resistance and achieve urinary continence in patients affected by E-E complex. The results are controversial, although it is reported to achieve voiding control in a significant percentage of exstrophic patients [12,13,14]. More often, the result is not always easily predictable and bladder neck plasty has not provided constant results at long term followup [15]. Bladder neck reconstruction, performed by tubulizing the trigonal musculature, has been commonly associated with the use of the paraexstrophic skin flaps to elongate the posterior urethra during first stage bladder fermature [16]. In our experience, this procedure causes significant scarred tissue and could interfere with the neuromuscular maturation and the functional coordination of trigonal area. Thus, the Young-Dees bladder neck ‘‘reconstruction’’ often results in a malfunction of the cervico-urethral segment. Usual consequence is partially obstructed micturition with high intravesical pressure values, which can be increased by the Crede` maneuver. Significant urine residue and different degrees of urinary incontinence are commonly associated. The incontinence may depend on overflow or on inefficient outlet resistance. In these patients, the common position of the bladder neck and posterior urethra is very superficial, in the subcutaneous plane between the transsimphyseal space. The superficial position increases the reconstruction inefficacy, because the urinary tract lies in an inappropriate plane, outside the perineal musculature. Better results have been obtained when the primary bladder closure is performed early postnatally and if the posterior urethra with the bladder neck are deeply positioned within the pelvic diaphragm [3,17]. Our results demonstrated that, in the study group of 22 consecutive patients affected by E-E complex who underwent intraoperative identification and reconstruction of the anterior perineal musculature [4], the outcomes were significantly better than in the control group of patients, who received the standard 3-stage reconstruction with classic or modified Young-Dees bladder neck plasty [10–12]. Recurrence of bladder neck widening/dehiscence, or female uterine prolapse,
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as well as male urethral fistulas or stenosis were significantly reduced or absent in the study group patients (Table 1). We experienced a case of distal urethral stenosis with partial glans disrupture in a male newborn, who underwent a single stage repair, with adjunctive island flap preputial urethroplasty to increase the distal length of the native urethral plate, performed during the same procedure. Single-stage adjunctive urethroplasty was abandoned in our further cases. The functional bladder outcomes at 2-year followup from the E-E repair, with anterior perineal reconstruction and periurethral musculature reapproximation, were demonstrated to be significantly better in terms of mean bladder capacity, mean dry interval and achievement of day-time continence, compared with the control group patients. Bladder cycling restoration with volitional control of micturition was achieved, albeit not fully, in about 65% (11 out of 17) of exstrophic patients and in all the pure male epispadias (Table 2). Bladder neck plasty with ureteral reimplantation was required in all the control group exstrophic patients and in 1 epispadic boy, but major surgery for continence control (such as cutaneous appendicovesicostomy and bladder augmentation) was needed in 5 (33%) of them. The overall number of secondary procedures after the exstrophic bladder and epispadias fermature was significantly higher in the control group patients, but 3 of 4 persistent incontinent exstrophic children of the study group will require surgery to achieve continence control. We believe that proper identification and correct positioning of the muscular structures that normally constitute part of the anterior pelvic floor is a central point of E-E surgical repair. The main structure is represented by the two anterior branches of the levator ani muscle and its correlated muscles (ilio-coccygeus, pubo-coccyggeus, pubo-rectalis and ischio-cavernosum). Together, these components of the perineal membrane act as a ‘‘muscular complex’’ of the anterior
perineum and take an active part in urinary continence in both sexes, as well as in preventing bladder and genital procidentia in the exstrophic females [4,5]. The ‘‘muscular complex’’ extends from the anterior pubic branches to the sacrococcygeal bones and is in close relation with the bulbo-prostatic urethra. In the exstrophic perineum, the wide anterior opening of the pubic bones causes lateral dislocation of these muscular structures. Magnetic resonance imaging (MRI) has recently been used to study the exstrophic pelvic floor anatomy [18,19]. The bladder neck and the posterior urethra in males, as well as the hole urethra and the vagina in females are dislocated to a more anterior and superficial position, losing the normal relationship with the anterior perineum musculature, which contribute to define the external urethral sphincter [5].
5. Conclusion The deeper positioning of the bladder neck and posterior urethra within the midline anterior perineum, the proper identification by electric bipolar stimulator and reapproximation of the delicate muscular fibers that constitute the anterior perineal muscular complex, with appropriate pubic bone fermature, are considered cornerstones of the functional repair of the E-E complex. The anterior perineum reconfiguration with the periurethral musculature reapproximation seems to guarantee significant advantages in terms of surgical and functional outcomes in E-E reconstruction.
Acknowledgements The Authors would like to thank Dr. Lucilla Rava`, Unit of Epidemiology, Bambino Gesu` Children’s Hospital, for her cooperation in developing the statistical analysis.
References [1] Cheng EY, Kropp BP. New concepts in Bladder Exstrophy and Epispadias Reconstruction. Guest Editor’s Notes. Dialogues in Ped Urol 1999;22:1. [2] Grady RW, Mitchell ME. Complete primary repair for exstrophy. J Urol 1999;162:1415–20. [3] Grady RW, Mitchell ME. Total penile disassembly for epispadias and complete primary repair for exstrophy. The Seattle experience. Dialogues in Ped Urol 1999;22:3–4.
[4] Caione P, Capozza N, Lais A, Matarazzo E. Periurethral muscle complex reassembly for exstrophy-epispadias repair. J Urol 2000;164:2062–6. [5] Caione P, Capozza N. Evolution of male epispadias repair: 16-year experience. J Urol 2001;165:2410–3. [6] Kropp BP, Cheng EY. Total urogenital complex mobilization in female patients with bladder exstrophy. J Urol 2000;164: 1035–9.
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[7] Caione P, Lais A. The endoscopic treatment of incontinence in children. Current Urology Reports 2002;3:121–4. [8] Ransley PG, Woppin M. Bladder exstrophy closure and epispadias repair. In: Spitz L, editor. Operative Surgery – Paediatric Surgery. Edimburgh Butterworks; 1988. p. 620620. [9] Mitchell ME, Bagli DJ. Complete penile disassembly for epispadias repair: the Mitchell technique. J Urol 1996;155:300–4. [10] Perlmutter AD, Weinstein MD, Reitelman C. Vesical neck reconstruction in patients with epispadias exstrophy complex. J Urol 1991;146:613–5. [11] Jones JA, Mitchell ME, Rink RC. Improved results using a modification of the Young-Dees-Leadbetter bladder neck repair. Br J Urol 1993;71:555–7. [12] Ferrer FA, Tadras YE, Gearhart J. Modified Young-Dees-Leadbetter bladder neck reconstruction: new concepts about old ideas. Urology 2001;58:791–6. [13] Lottmann HB, Yaquoti M, Melin Y. Male epispadias repair: surgical and functional results with the Cantwell-Ransley procedure in 40 patients. J Urol 1999;162:1176–80.
[14] Ben Chaim J. Successfull primary exstrophy closure: a very important factor for achieving urinary continence. Dial Pediatr Urol 1999;22: 2–3. [15] Moriquand PD, Bubanj T, Feyaerts A, Jandric M, Timsit M, Mollard P, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int 2003;92:997–1002. [16] Duckett JW. Use of paraexstrophy skin pedicle grafts for correction of exstrophy and epispadias repair. Birth Defects 1977;13:175–9. [17] Grady RW, Mitchell M. Management of epispadias. Urol Clin N Amer 2002;29:349–60. [18] Halachmi S, Farhat W, Konen O, Khan A, Hodapp J, Bagli DJ, McLorie GA, Khoury AE. Pelvic floor magnetic resonance imaging after neonatal single stage reconstruction in male patients with classic bladder exstrophy. J Urol 2003;170:1505–9. [19] Williams AM, Solaiyappan M, Pannu HK, Bluemke D, Shechter G, Gearhart JP. 3-Dimensional magnetic resonance imaging modeling of the pelvic floor musculataure in classic bladder exstrophy before pelvic osteotomy. J Urol 2004;172:1702–5.
Editorial Comment Prof. John P. Gearhart, Baltimore, MD, USA [email protected]
level of the levator hiatus where one must go to allow deeper placement of the posterior vesicourethral unit. This allows lateral tension and brings this area anterior again when the pelvic bones are brought together. Fourthly, since the advent of the Cantwell-Ransley repair paraexstrophy flaps have become outdated and thus have no bearing on bladder neck repair. Lastly, these two groups cannot be compared because as the study group becomes older more major procedures will likely be requires. [2] The Mansoura group at the recent AAP meeting presented data that likely over 80% of their patients who underwent ‘‘Complete Repair’’ were going to require bladder neck reconstruction [3] This data falls almost in parallel to the authors data in Table 2 of 17.6% daytime dryness in their exstrophy group. In summary, the authors have shown nicely that their perineal dissection adds to bladder capacity and potentially to continence. However, only time will tell where the ‘‘Complete Repair’’ falls with or without anterior pelvic reconstruction in the armamentarium of exstrophy reconstructive procedures. Recent papers have shown severe complications with this procedure while luckily our authors did not experience these problems [4]. In conclusion, one might suggest that these little patients might be better served by anterior perineal reconstruction in all types of exstrophy repair with a better pelvic osteotomy and good postoperative immobilization to give them the best chance at obtaining a better capacity and better chance at eventual urinary continence.
This is an interesting and informative paper about a novel technique to increase outlet resistance during exstrophy closure and epispadias repair. The authors accomplish this and make a good argument for the use of this as an adjunctive procedure. However, they detract from the message of this paper by trying to present their data on the ‘‘Complete Repair’’ and using a control group which is actually simply a historical series. This can confuse the reader and takes away from the paper’s important message. The first point is that posterior iliac osteotomy has been discarded in most centers in favor of the combined anterior innominate and vertical iliac osteotomy because the latter allows easier and more precise pubic bone approximation. There is less ‘‘Drift’’ of the pelvic rami afterwards which keeps the bladder neck and posterior urethra in a pelvic position where the authors want it. Secondly, many of the study group patients had injection of ‘‘Deflux’’ into the bladder neck area. Was this because the bladders were not growing and the authors were trying to stimulate the bladders to grow? Thirdly, the authors imply that the ‘‘Complete Repair’’ allows one to position the bladder and posterior urethra deeper into the pelvis. As a surgeon who recloses 5–10 failed exstrophies per year I can say candidly in both failed ‘‘Complete Repairs’’ and ‘‘Modern Staged Repairs’’ the reason the bladder and posterior urethra become superficial is because of a timid dissection of the posterior vesicourethral unit at the time of primary closure and inadequate postoperative immobilization. [1] Oftentimes, the urogenital diaphragm fibers and symphyseal bands are still intact as surgeons who do these infrequently do not take the dissection down to the
References [1] Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder exstrophy closure: Complications and impact on success. Urology 2003;62:1109–13.
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[2] Borer J. et al. Early outcome following complete repair of bladder exstrophy in the newborn. Abstract 92, American Academy of Pediatrics, Urology Section, San Francisco, CA. October 11, 2004. [3] Hafez T, El-Sherbiny MT. Complete primary repair of bladder exstrophy in children presenting late and those with failed initial closure:
Single center experience with 30 children. Abstract #14. American Academy of Pediatrics, Urology Section, San Francisco, CA. October 11, 2004. [4] Gearhart JP, Baird AD. Complications of complete repair of bladder exstrophy in infancy: insights and outcomes. Abstract #91. American Academy of Pediatrics, San Francisco, CA. October 11, 2004.
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Anterior Perineal Reconstruction in Exstrophy-epispadias Complex Paolo Caione*, Nicola Capozza, Daniela Zavaglia, Mauro De Dominicis Division of Pediatric Urology, Department of Nephrology and Urology, ‘‘Bambino Gesu`’’ Children’s Hospital, Rome, Italy Received 30 December 2004; accepted 10 February 2005 Available online 9 March 2005
Abstract Objectives: To assess the role of correct anatomical reconfiguration of the anterior perineal musculature in exstrophy-epispadias (E-E) patients. To stress the use of a bipolar stimulator to detect the perineal muscular complex intraoperatively, and to increase the functional results of reconstruction in E-E patients. Methods: A total of 22 patients with E-E complex were treated in a 7-year period: 17 patients presenting classic bladder extrophy (aged 3 days to 6 years) and 5 incontinent male epispadias (aged 9 months to 16 years). An electric bipolar stimulator was used to identify and reapproximate at the midline the muscular fibers that constitute the periurethral muscular complex, as a part of the anterior perineal membrane. Outcome was evaluated at 24 months from surgery, considering bladder capacity, dry intervals, urinary infections (UTI’s), upper tract deterioration and surgical complications (fistula, obstruction, dehiscence). Results were compared with a matched group of 19 E-E patients treated in the previous 5-year period, without the presented technique (control group). Student T-test was used for statistical analysis, considering p 0.05 as significant. Results: No bladder neck or urethral dehiscence was observed. Mean bladder capacity at 2-year follow-up was 80 cc in the exstrophic patients and 120 cc in the male epispadias. Mean dry interval increased to 75 minutes in the exstrophy patients and to 130 minutes in the male epispadias. Full daytime continence was achieved in 3 exstrophic and in all the epispadic patients. The difference with the control group of patients was significant (p < 0.05). Conclusions: Proper identification of the anterior perineal muscular complex, using a bipolar stimulator, and its reapproximation at the posterior urethra on the midline was demonstrated to be effective in increasing bladder cycling and in developing adequate bladder volume, anticipating coordinated micturition. # 2005 Elsevier B.V. All rights reserved. Keywords: Bladder Exstrophy; Epispadias; Perineum; Urinary continence; Urethra
1. Introduction Surgical reconstruction of the E-E complex in newborns or in young infants represents one of the most challenging tasks in any Pediatric Urology center. The goal is to achieve a low urinary tract compliance and continence, with volitional voiding preventing renal damage. Urethral and genitalia reconstruction is not a * Corresponding author. Pressent address: Division of Pediatric Urology ‘‘Bambino Gesu`’’ Children’s Hospital Piazza S. Onofrio, 4 - 00165 Rome, Italy. Tel.: +39 06 68592337; Fax: +39 06 68592849. E-mail address: [email protected] (P. Caione).
secondary point, that should be achieved before the memory is fully established in young children. To date, the goals of functional reconstruction in E-E have not yet been adequately achieved, as shown by different newly proposed surgical techniques and several advances made in our knowledge of the principles for surgical repair [1]. Complete penile disassembly technique for epispadia repair [2] may be associated with the primary closure of the bladder exstrophy in a single-stage procedure [3]. The technique offers potential advantages in terms of anatomical reconstruction of the male external genitalia and in terms of positioning of the
0302-2838/$ – see front matter # 2005 Elsevier B.V. All rights reserved. doi:10.1016/j.eururo.2005.02.005
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bladder neck and the urethra within the pelvis. We developed an extention of this technique that allows a more anatomical anterior perineal reconstruction, based on the identification of the perineal musculature using an electric stimulator during surgery, with its reapproximation on the sagittal line in periurethral position [4]. The outcomes of our 7-year experience using this technique are presented and discussed, comparing these with the results of the previous 5-years. 2. Materials and methods Bladder exstrophy closure and epispadias repair was performed in 17 consecutive patients, 13 males and 4 females, from March 1995 to February 2002. A single-stage procedure in neonatal age (within 72 hours of age) was performed in 5 cases, 2 males and 3 females with classic bladder exstrophy. The remaining 12 patients underwent a 2-stage repair: 11 exstrophic males and 1 female presenting cloacal exstrophy. Bilateral posterior iliac osteotomy was performed in all patients, excluding 3 newborns operated on within 48 hours of life for classic bladder exstrophy. During the same 7-year period, a total number of 5 primary penopubic male epispadias were treated, using the modified disassembly technique [5] (age 11 months to 5 years, mean age: 2.3 years), associated with the periurethral musculature reapproximation and anterior perineal reconfiguration. 2.1. Technique After bilateral posterior iliac osteotomy, the exstrophic bladder is closed with a suprapubic tube and the urethral plate is fully mobilized from the corpora in the male patient. The bladder neck tissue is freeded from the inner surface of the symphysis [4]. The prostate must stay joint with the urethral spongiosum tissue. The corporeal bodies and the glans are split on the sagittal plane according to Grady and Mitchell [3]. The midline incision is made in the intersymphyseal tissue and along the infrapubic space. It is mandatory to remain strictly on the midline, and the frequent use of bipolar stimulation helps to identify any striated muscular tissue and to maintain a symmetrical position. This tissue is usually present in the E-E complex, although it is displaced laterally due to pelvic dysmorphism. The use of the electrical stimulator enables us to observe small contractions of this muscular structures. Any available muscle is preserved and the perineal tissue are strictly divided on the midline [4]. No attempt is made to perform any plasty of the bladder neck, such as the Young-Dees or similar procedures. The exstrophic bladder neck groove is elongated with multiple incisions on both lateral aspects and tubulized on a 8 F silicon catheter, which is removed on the 4th to 5th postoperative day. The tubularized bladder neck and posterior urethra are then placed deeply into the pelvis, just anteriorly to the anorectal canal and levator ani fibers. The ‘‘anterior muscular complex’’, which is part of the pelvic floor musculature, is then reapproximated at the midline by 4/0 or 5/0 tiny sutures, closely to the tubularized posterior urethra (Fig. 1 A and B). The penile disassembly procedure with anterior epispadic male urethra repair [2,5] is carried out at the same time or as a second stage within 6 months. In the female patient, the bladder neck and urethral plate are mobilized together with the vagina, similarly to the posterior
Fig. 1. Periurethral musculature reapproximation. (A) The thiny anterior perineum musculature is identified using a bipolar electric stimulator, through a deep midline incision on the intersymphyseal space. (B) The periurethral muscular complex is gently reapproximated at the tubularized membranous urethra in a U-shaped fashion, restoring the anterior perineum anatomy; Suprapubic tube; Closed exstrophied bladder; Mobilized urethral plate; Corporeal bodies; Pubis bone; Midline intersymphyseal incision; Scrotum; Electric stimulator; Anterior perineal muscular complex and periurethral musculature reapproximation.
urethra and prostatic bundle in males. The urethral and vaginal structures are positioned ‘‘en block’’ posteriorly through a deep sagittal incision on the perineal body [6]. Vulvoplasty and clitoroplasty are accomplished. Patient outcome was evaluated at 24 months from the initial reconstructive surgery. Bladder capacity at 40 cm H2O pressure under anesthesia, dry intervals and day-time continence achievement were recorded, as well as any febrile UTI episodes, upper tract dilatation and surgical complications (male urethra fistula or obstruction, bladder neck/posterior urethra dehiscence, female uterine cervix procidentia). The study group results were compared with the outcomes of a population of 19 E-E patients (15 bladder exstrophy and 4 male epispadias), age and sex matched, who had been treated in the same institution by the some surgeon (PC) during the previous 5-year period, without adopting the surgical techniques of anterior perineal reconstruction with periurethral muscular reapproximation (‘‘Control group’’). For statistical analysis, the Student-T test was used to compare means (p < 0.05 was considered significant) and Wilcoxon/Mann Whitney to compare medians. Count data were analyzed by the Chi square Test or Fisher exact test, where appropriate.
3. Results In the study group, all patients underwent 1 or 2 calibrations of the urethra and bladder neck during the
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Table 1 Surgical outcomes at 24 months from anterior perineal reconstruction in e-e complex
STUDY GROUP Bladder Exstrophy Males Females Male Epispadias CONTROL GROUP Bladder Exstrophy Males Females Male Epispadias P (t- Student test)
Patients
Febrile UTIs
Upper tract dilatation
Bladder neck dehiscence
Male urethral fistula/stenosis
Female uterus procidentia
22 17 11 6 5 19 15 7 4 4
2 2* 1 1 0 6 6* 5 1 0 * < 0.05
8 7* 6 1 1 6 6* 4 2 0 *NS
0* 0
3* 3 3 – 0 9* 6 6 – 3 *0.05
0* 0
first 2 to 6 months after surgery. Low dose antibacterial prophylaxis was administered for at least 24 months after surgery with periodical urinalysis. Renal and bladder ultrasound scans were performed at 6-month intervals and cystogram under anesthesia with bladder capacity assessment was carried out at 2-year intervals from surgery. No bladder neck, urethral dehiscence or exstrophy relapses were observed. No uterine cervix procidentia occurred in female patients. A male newborn, who underwent bladder and penile reconstruction in a single stage at the beginning of this study, developed distal urethra stenosis with partial glans disrupture, as consequence of the island flap urethroplasty, performed to increase the distal length of the native urethra. Two urethral fistulas were observed at the coronal and mid-shaft position, which required surgical fermature. Mild upper tract dilatation was detected respectively in 7 patients (31.8%) of the exstrophic series and in 1 pure epispadias. Febrile UTIs were experienced in 2 patients with refluxes (Table 1). Bladder capacity increased mostly in the first 12–18 months after surgery and it was larger in the pure epispadic patients (range 50 to 180 mls, mean
0 3* 3 1 2 0 * < 0.05
0 2* 2 – 2 – *0.05
123 mls). A mean bladder capacity of 78 mls (30 to 110 mls) was achieved in the exstrophic patients (Table 2). No significant difference was observed between male and female patients (75 mls in males, 82 mls in females) or in the 3 newborns without osteotomies. Unilateral or bilateral vesico-ureteral refluxes were observed in 16 of 17 exstrophic bladders and in 2 of 5 penopubic epispadias. No ureteral reimplantation or bladder neck plasty was performed in the study group of patients within the follow-up period. Concerning bladder activity, cyclic voiding with urinary stream, associated with 30 to 120 minutes dry intervals were present in 11 of the 17 exstrophic patients at the 24 month follow-up. No patient achieved full urinary continence during the night-time. Four exstrophic patients (23.5%) experienced persistent daytime and night-time incontinence, with continuous urinary dribbling and poor functional bladder capacity. Day-time continence with volitionary micturition was observed in all 5 penopubic epispadic boys (Table 2). Spontaneous penile erections were observed in all the male patients (11 exstrophy and 5 primary penopubic epispadias) treated by our technique [5].
Table 2 Anterior perineal reconstruction in e-e complex: functional bladder outcomes at 24 months follow-up
BLADDER EXSTROPHY Study Group Control Group P (t- Student test) MALE EPISPADIAS Study Group Control Group P (t- Student test)
Patients (#)
Mean (Range) Bl. Capacity (cc)
Dry intervals (# pts)
Mean (range) Dry interval (mins)
Day-time continence (# pts)
17 15
78 (30–110) 45 (10–65) <0.05
11 2
75 (30–120) 55 (35–75) <0.05
3 (17.6%) 0
5 4
123 (50–180) 95 (55–140) <0.05
5 3
130 (45–180) 90 (30–140) <0.05
5 (100.8%) 2 (50%)
# pts = number of patients; cc = milliliters; mins = minutes.
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In the control group of 15 exstrophies, 6 patients experienced febrile UTIs and 3 suffered from some degree of bladder neck dehiscence. In 9 boys (6 exstrophies and 3 epispadias), urethral fistulas with/ without urethral stenosis were observed. In 2 exstrophic females, some degree of uterine procidentia was present. The difference with the study group outcomes was significant (p < 0.05, Table 1). Mean bladder capacity assessed at the 24 month follow-up in the control group was lower both in the exstrophic (mean volume = 45 mls, range 10 to 65 mls) and in the male epispadic patients (mean volume = 95 mls, range 55 to 140 mls). The difference was statistically significant (p < 0.05, Table 2). Only 2 exstrophic children of 15 patients and 3 of 4 male epispadias achieved dry intervals (mean = 55 minutes in exstrophic and 90 minutes in epispadic patients). The number of patients with dry intervals and its length were significantly reduced, compared with the study group children (p < 0.05, Table 2). Concerning adjunctive surgical procedures, 15 of 17 patients with bladder exstrophy and 1 of 5 epispadias of the study group received 1 to 3 (mean 1.8) endoscopic treatments with submucosal injection of dextranomer 1% in jaluronic acid as bulking material at the bladder neck, to increase bladder outlet resistance [7]. No major surgery was required during the 24-month follow-up, but 3 of 4 incontinent exstrophic patients are scheduled for bladder neck plasty/fermature with continent appendicovesicostomy procedure and bladder augmentation. All the 15 exstrophic patients and 1 of the 4 male epispadias of the control group needed bladder neck plasty with ureteral reimplantation at the age of 4 to 9 years. Seven exstrophic patients required continent appendicovesicostomy for intermittent catheterization and 5 of them received bladder neck tightening or fermature with augmentation. The difference between the study and the control group of patients, concerning the number of major surgical procedures after primary reconstruction, was statistically significant (p < 0.05).
4. Discussion To enable a child or a teenager born with E-E to participate fully in modern society, without significant functional, cosmetic or psychological handicap is still difficult. The complexity and number of the required reconstructive procedures is usually very high, especially in the exstrophic population. Urinary reservoir reconstruction is nowadays easily achieved, through primary closure with/without augmentation of the
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bladder. External genitalia can be repaired effectively by using the Cantwell-Ransley procedure [8] or the Mitchell technique [9]. Full urinary continence, obtained by volitional urination, remains probably the most difficult result to achieve for the Pediatric Urologist but, at the same time, it is the most pressing need for these patients as they reach school-age or start social engagements. The standard Young-Dees bladder neck plasty and its variants [10,11,12] are still widely used to increase bladder outlet resistance and achieve urinary continence in patients affected by E-E complex. The results are controversial, although it is reported to achieve voiding control in a significant percentage of exstrophic patients [12,13,14]. More often, the result is not always easily predictable and bladder neck plasty has not provided constant results at long term followup [15]. Bladder neck reconstruction, performed by tubulizing the trigonal musculature, has been commonly associated with the use of the paraexstrophic skin flaps to elongate the posterior urethra during first stage bladder fermature [16]. In our experience, this procedure causes significant scarred tissue and could interfere with the neuromuscular maturation and the functional coordination of trigonal area. Thus, the Young-Dees bladder neck ‘‘reconstruction’’ often results in a malfunction of the cervico-urethral segment. Usual consequence is partially obstructed micturition with high intravesical pressure values, which can be increased by the Crede` maneuver. Significant urine residue and different degrees of urinary incontinence are commonly associated. The incontinence may depend on overflow or on inefficient outlet resistance. In these patients, the common position of the bladder neck and posterior urethra is very superficial, in the subcutaneous plane between the transsimphyseal space. The superficial position increases the reconstruction inefficacy, because the urinary tract lies in an inappropriate plane, outside the perineal musculature. Better results have been obtained when the primary bladder closure is performed early postnatally and if the posterior urethra with the bladder neck are deeply positioned within the pelvic diaphragm [3,17]. Our results demonstrated that, in the study group of 22 consecutive patients affected by E-E complex who underwent intraoperative identification and reconstruction of the anterior perineal musculature [4], the outcomes were significantly better than in the control group of patients, who received the standard 3-stage reconstruction with classic or modified Young-Dees bladder neck plasty [10–12]. Recurrence of bladder neck widening/dehiscence, or female uterine prolapse,
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as well as male urethral fistulas or stenosis were significantly reduced or absent in the study group patients (Table 1). We experienced a case of distal urethral stenosis with partial glans disrupture in a male newborn, who underwent a single stage repair, with adjunctive island flap preputial urethroplasty to increase the distal length of the native urethral plate, performed during the same procedure. Single-stage adjunctive urethroplasty was abandoned in our further cases. The functional bladder outcomes at 2-year followup from the E-E repair, with anterior perineal reconstruction and periurethral musculature reapproximation, were demonstrated to be significantly better in terms of mean bladder capacity, mean dry interval and achievement of day-time continence, compared with the control group patients. Bladder cycling restoration with volitional control of micturition was achieved, albeit not fully, in about 65% (11 out of 17) of exstrophic patients and in all the pure male epispadias (Table 2). Bladder neck plasty with ureteral reimplantation was required in all the control group exstrophic patients and in 1 epispadic boy, but major surgery for continence control (such as cutaneous appendicovesicostomy and bladder augmentation) was needed in 5 (33%) of them. The overall number of secondary procedures after the exstrophic bladder and epispadias fermature was significantly higher in the control group patients, but 3 of 4 persistent incontinent exstrophic children of the study group will require surgery to achieve continence control. We believe that proper identification and correct positioning of the muscular structures that normally constitute part of the anterior pelvic floor is a central point of E-E surgical repair. The main structure is represented by the two anterior branches of the levator ani muscle and its correlated muscles (ilio-coccygeus, pubo-coccyggeus, pubo-rectalis and ischio-cavernosum). Together, these components of the perineal membrane act as a ‘‘muscular complex’’ of the anterior
perineum and take an active part in urinary continence in both sexes, as well as in preventing bladder and genital procidentia in the exstrophic females [4,5]. The ‘‘muscular complex’’ extends from the anterior pubic branches to the sacrococcygeal bones and is in close relation with the bulbo-prostatic urethra. In the exstrophic perineum, the wide anterior opening of the pubic bones causes lateral dislocation of these muscular structures. Magnetic resonance imaging (MRI) has recently been used to study the exstrophic pelvic floor anatomy [18,19]. The bladder neck and the posterior urethra in males, as well as the hole urethra and the vagina in females are dislocated to a more anterior and superficial position, losing the normal relationship with the anterior perineum musculature, which contribute to define the external urethral sphincter [5].
5. Conclusion The deeper positioning of the bladder neck and posterior urethra within the midline anterior perineum, the proper identification by electric bipolar stimulator and reapproximation of the delicate muscular fibers that constitute the anterior perineal muscular complex, with appropriate pubic bone fermature, are considered cornerstones of the functional repair of the E-E complex. The anterior perineum reconfiguration with the periurethral musculature reapproximation seems to guarantee significant advantages in terms of surgical and functional outcomes in E-E reconstruction.
Acknowledgements The Authors would like to thank Dr. Lucilla Rava`, Unit of Epidemiology, Bambino Gesu` Children’s Hospital, for her cooperation in developing the statistical analysis.
References [1] Cheng EY, Kropp BP. New concepts in Bladder Exstrophy and Epispadias Reconstruction. Guest Editor’s Notes. Dialogues in Ped Urol 1999;22:1. [2] Grady RW, Mitchell ME. Complete primary repair for exstrophy. J Urol 1999;162:1415–20. [3] Grady RW, Mitchell ME. Total penile disassembly for epispadias and complete primary repair for exstrophy. The Seattle experience. Dialogues in Ped Urol 1999;22:3–4.
[4] Caione P, Capozza N, Lais A, Matarazzo E. Periurethral muscle complex reassembly for exstrophy-epispadias repair. J Urol 2000;164:2062–6. [5] Caione P, Capozza N. Evolution of male epispadias repair: 16-year experience. J Urol 2001;165:2410–3. [6] Kropp BP, Cheng EY. Total urogenital complex mobilization in female patients with bladder exstrophy. J Urol 2000;164: 1035–9.
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[7] Caione P, Lais A. The endoscopic treatment of incontinence in children. Current Urology Reports 2002;3:121–4. [8] Ransley PG, Woppin M. Bladder exstrophy closure and epispadias repair. In: Spitz L, editor. Operative Surgery – Paediatric Surgery. Edimburgh Butterworks; 1988. p. 620620. [9] Mitchell ME, Bagli DJ. Complete penile disassembly for epispadias repair: the Mitchell technique. J Urol 1996;155:300–4. [10] Perlmutter AD, Weinstein MD, Reitelman C. Vesical neck reconstruction in patients with epispadias exstrophy complex. J Urol 1991;146:613–5. [11] Jones JA, Mitchell ME, Rink RC. Improved results using a modification of the Young-Dees-Leadbetter bladder neck repair. Br J Urol 1993;71:555–7. [12] Ferrer FA, Tadras YE, Gearhart J. Modified Young-Dees-Leadbetter bladder neck reconstruction: new concepts about old ideas. Urology 2001;58:791–6. [13] Lottmann HB, Yaquoti M, Melin Y. Male epispadias repair: surgical and functional results with the Cantwell-Ransley procedure in 40 patients. J Urol 1999;162:1176–80.
[14] Ben Chaim J. Successfull primary exstrophy closure: a very important factor for achieving urinary continence. Dial Pediatr Urol 1999;22: 2–3. [15] Moriquand PD, Bubanj T, Feyaerts A, Jandric M, Timsit M, Mollard P, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int 2003;92:997–1002. [16] Duckett JW. Use of paraexstrophy skin pedicle grafts for correction of exstrophy and epispadias repair. Birth Defects 1977;13:175–9. [17] Grady RW, Mitchell M. Management of epispadias. Urol Clin N Amer 2002;29:349–60. [18] Halachmi S, Farhat W, Konen O, Khan A, Hodapp J, Bagli DJ, McLorie GA, Khoury AE. Pelvic floor magnetic resonance imaging after neonatal single stage reconstruction in male patients with classic bladder exstrophy. J Urol 2003;170:1505–9. [19] Williams AM, Solaiyappan M, Pannu HK, Bluemke D, Shechter G, Gearhart JP. 3-Dimensional magnetic resonance imaging modeling of the pelvic floor musculataure in classic bladder exstrophy before pelvic osteotomy. J Urol 2004;172:1702–5.
Editorial Comment Prof. John P. Gearhart, Baltimore, MD, USA [email protected]
level of the levator hiatus where one must go to allow deeper placement of the posterior vesicourethral unit. This allows lateral tension and brings this area anterior again when the pelvic bones are brought together. Fourthly, since the advent of the Cantwell-Ransley repair paraexstrophy flaps have become outdated and thus have no bearing on bladder neck repair. Lastly, these two groups cannot be compared because as the study group becomes older more major procedures will likely be requires. [2] The Mansoura group at the recent AAP meeting presented data that likely over 80% of their patients who underwent ‘‘Complete Repair’’ were going to require bladder neck reconstruction [3] This data falls almost in parallel to the authors data in Table 2 of 17.6% daytime dryness in their exstrophy group. In summary, the authors have shown nicely that their perineal dissection adds to bladder capacity and potentially to continence. However, only time will tell where the ‘‘Complete Repair’’ falls with or without anterior pelvic reconstruction in the armamentarium of exstrophy reconstructive procedures. Recent papers have shown severe complications with this procedure while luckily our authors did not experience these problems [4]. In conclusion, one might suggest that these little patients might be better served by anterior perineal reconstruction in all types of exstrophy repair with a better pelvic osteotomy and good postoperative immobilization to give them the best chance at obtaining a better capacity and better chance at eventual urinary continence.
This is an interesting and informative paper about a novel technique to increase outlet resistance during exstrophy closure and epispadias repair. The authors accomplish this and make a good argument for the use of this as an adjunctive procedure. However, they detract from the message of this paper by trying to present their data on the ‘‘Complete Repair’’ and using a control group which is actually simply a historical series. This can confuse the reader and takes away from the paper’s important message. The first point is that posterior iliac osteotomy has been discarded in most centers in favor of the combined anterior innominate and vertical iliac osteotomy because the latter allows easier and more precise pubic bone approximation. There is less ‘‘Drift’’ of the pelvic rami afterwards which keeps the bladder neck and posterior urethra in a pelvic position where the authors want it. Secondly, many of the study group patients had injection of ‘‘Deflux’’ into the bladder neck area. Was this because the bladders were not growing and the authors were trying to stimulate the bladders to grow? Thirdly, the authors imply that the ‘‘Complete Repair’’ allows one to position the bladder and posterior urethra deeper into the pelvis. As a surgeon who recloses 5–10 failed exstrophies per year I can say candidly in both failed ‘‘Complete Repairs’’ and ‘‘Modern Staged Repairs’’ the reason the bladder and posterior urethra become superficial is because of a timid dissection of the posterior vesicourethral unit at the time of primary closure and inadequate postoperative immobilization. [1] Oftentimes, the urogenital diaphragm fibers and symphyseal bands are still intact as surgeons who do these infrequently do not take the dissection down to the
References [1] Meldrum KK, Baird AD, Gearhart JP. Pelvic and extremity immobilization after bladder exstrophy closure: Complications and impact on success. Urology 2003;62:1109–13.
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[2] Borer J. et al. Early outcome following complete repair of bladder exstrophy in the newborn. Abstract 92, American Academy of Pediatrics, Urology Section, San Francisco, CA. October 11, 2004. [3] Hafez T, El-Sherbiny MT. Complete primary repair of bladder exstrophy in children presenting late and those with failed initial closure:
Single center experience with 30 children. Abstract #14. American Academy of Pediatrics, Urology Section, San Francisco, CA. October 11, 2004. [4] Gearhart JP, Baird AD. Complications of complete repair of bladder exstrophy in infancy: insights and outcomes. Abstract #91. American Academy of Pediatrics, San Francisco, CA. October 11, 2004.