- Email: [email protected]
The use of bladder neck reconstruction in bladder exstrophy
Seminars in Pediatric Surgery (2011) 20, 91-96
The use of bladder neck reconstruction in bladder exstrophy Joseph G. Borer, MD From the Department of Surgery (Urology), Harvard Medical School and Assistant in Urology, Children’s Hospital Boston, Boston, Massachusetts. KEYWORDS Bladder exstrophy; Bladder neck reconstruction; Urinary continence
A major goal of bladder exstrophy (BE) management is achieving urinary continence, most commonly with surgical bladder neck reconstruction (BNR). This is a report of outcome of BNR after complete primary repair of exstrophy (CPRE). At our institution, patient history, ultrasound, cystogram (VCUG) and urodynamic study (UDS) were performed during a prospective evaluation of patients with BE. Dry interval of ⬎3 hours was used as the definition of continence and dry interval ⬍1-hour incontinence. Bladder capacity was measured at VCUG and/or UDS. UDS was also used to assess bladder compliance. From 1994 to 2010, we cared for 47 BE patients (31 male, 15 female) after CPRE. For patients ⱖ3 years after CPRE, BNR was performed in 9 of 22 (41%) male and 3 of 11 (27%) female patients. Mean age at BNR was 6.3 and 5.9 years for male and female patients, respectively. The mean (⫾SD) bladder capacity pre-BNR was 104.8 (⫾20.4 mL). There was a significant increase in capacity from pre-BNR to ⱖ1.5 years post-BNR (P ⫽ 0.013) and from ⬍1.5 and ⱖ1.5 years post-BNR (P ⫽ 0.002). In conclusion, most patients with BE require BNR after CPRE. The need for BNR is more common in male patients. © 2011 Elsevier Inc. All rights reserved.
The goal of initial surgical management of the patient with bladder exstrophy (BE) is successful closure of the bladder and proximal urethra (ie, modern staged repair [MSRE])1 or successful bladder closure and epispadias repair (complete primary repair, ie, complete primary repair of exstrophy [CPRE]).2 Elimination of vesicoureteral reflux, thus decreasing the risk of reflux nephropathy, is also a priority in the management of BE, with antireflux surgery potentially at the time of initial closure when anatomy permits. Theoretically, one potential advantage of CPRE over MSRE is earlier provision of increased bladder outlet resistance resulting in bladder cycling and optimal bladder growth. It has been shown that, at early follow-up and irrespective of gender or management (MSRE post-bladder neck reconstruction [BNR] vs CPRE), percent predicted bladder capacity was equivalent.3 Address reprint requests and correspondence: Joseph G. Borer, MD Children’s Hospital Boston, 300 Longwood Ave, Boston, MA 02115. E-mail: [email protected].
1055-8586/$ -see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1053/j.sempedsurg.2010.12.003
Urinary continence with ability to void spontaneously and efficiently are major goals for management of BE. For MSRE, BNR, the third stage of the staged approach, is performed in all patients. BNR is performed in only those CPRE patients who require such. In one series, it has been shown that BNR was necessary in 80% of male and 57% of female patients after CPRE.4 Experience from other centers has also been reported showing that almost all patients may require BNR.5,6 The purpose of the present study was to examine the need for and outcome of BNR following CPRE in patients at our institution.
Methods A retrospective review of BE patients through use of our Institutional Review Board approved exstrophy database was performed. BE patients who had undergone CPRE were
92 identified. Early outcome in much of this patient population has been reported.7 Patient history, physical observations, renal and bladder ultrasound, voiding cystourethrogram (VCUG), and urodynamic study (UDS) were used during prospective evaluation of patients with BE.8 These observations and data were used as means to determine the need for BNR and to evaluate outcome afterward. Urinary continence was assessed in those patients ⱖ3 years post-CPRE and recorded as a dry interval. Dry interval was reported on a scale of ⬍1 hour 1-2 hours, ⬎2-3, or ⬎3 hours, and was recorded from patient and/or caregiver history and, at times, physician observation in the clinic. Urinary incontinence was defined as a dry interval ⬍1 hour and continence was defined as a dry interval ⬎3 hours. Bladder capacity was measured by gravity fill at examination under anesthetic and VCUG (saline or fluoroscopic contrast height at 40 cm above the bladder) and during UDS with body temperature saline instillation. Bladder capacity was recorded as the volume of fluid instilled or infused at either the onset of leaking or voiding, or at the report of sensation of bladder fullness by the patient. Specifics of technique for urodynamic assessment have been published.3 A patient was considered an appropriate candidate for BNR if all the following applied; (1) incontinent (dry interval ⬍1 h), (2) ⱖ5 years of age, (3) patient and caregiver(s) interested in attaining continence, and (4) patient and caregiver(s) acceptance of potential impaired bladder emptying and possible need for clean intermittent catheterization (CIC). Adjunctive catheterizable conduit creation was offered as an option to obviate the need for urethral catheterization in the case that CIC should be necessary following BNR. CPRE was performed as previously described.7 In those cases in which anatomy was amenable (adequate size of bladder plate), antireflux surgery either via cross-trigone or cephalotrigonal technique was performed at CPRE. BNR was performed via the method of Young,9 Dees,10 and Leadbetter.11 The strip of tissue to be tubularized was measured at approximately 13-15 mm in width and approximately 2.5-3 cm in length beginning from the bladder outlet. The reconstructed bladder neck tube was closed over either a 6-Fr or 8-Fr catheter (without balloon) and remained in place for approximately 5-7 days postoperatively. Urinary diversion was via bilateral ureteral stent and suprapubic cystostomy tube. Bilateral cephalotrigonal ureteral reimplantation was performed as previously described.12 Catheterizable conduit, such as appendicovesicostomy13 or reconfigured bowel tube14,15 was performed as previously described. SPSS 18.0 software was used for database management and statistical analysis. T test for independent samples and a paired t tests were used to compare bladder capacity relative to BNR status and timing. Unpaired t test was used to compare pre-BNR versus post-BNR bladder compliance. Statistical significance was considered P ⬍ 0.05.
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011
Results During the period 1994-2010, we cared for 47 BE patients (32 male, 15 female) after CPRE. Six had initial closure at an outside institution. Forty-one patients had CPRE at Children’s Hospital Boston. Thirty-two of these 41 patients underwent CPRE within 72 hours of birth. Outcome from initial closure (CPRE) was assessed by clinical parameters, physical examination, and VCUG. Early outcome was marked by successful closure in all but one female patient that suffered dehiscence. Thirty-two of our patients were operated as newborns and 3 underwent bilateral ureteral reimplantation at CPRE. Success rate for those 3 patients was 100%. Flow charts that designate patient progress and outcome during the course of care are shown in Figure 1A for male patients and Figure 1B for female patients ⱖ3 years of age and/or ⱖ3 years postCPRE. Regarding the specifics of BNR, of those patients ⱖ3 years of age, BNR was performed in 9 of 22 (41%) male and 3 of 11 (27%) female patients. Mean age at BNR was 6.3 and 5.9 years for male and female patients, respectively. All 12 patients were incontinent before BNR. Post-BNR dry interval was assessed at various points after the surgery, at 1 year (range of 3 months to 1 year) and ⱖ1.5 years after BNR. There was improvement in dry interval for 8 of 12 after the surgery. Three patients were continent 1 year post-BNR, and although some had a dry interval of ⬎2-3 hours, none were continent (by stringent definition of dry interval ⬎3 hours) at ⱖ1.5 years. Five of the 12 patients were on a trajectory trending toward continence at last follow-up. These results are before bladder augmentation that was eventually performed in 2 of the 12 patients, both male.
Bladder capacity Bladder capacity obtained at examination under anesthesia, VCUG, and UDS (Figure 2, Table 1). Pre-BNR bladder capacity was available for 11 of 12 patients who underwent BNR. The mean (⫾SD) bladder capacity pre-BNR was 104.8 ⫾ 20.4 mL. Mean time for first postoperative measure (⬍1.5 years post-BNR; n ⫽ 12) was 4.6 ⫾ 4.8 months with a range of 0.39-14.7 months. Mean time for latest post-BNR measure (ⱖ1.5 years post-BNR; n ⫽ 7) is 3.5 ⫾ 1.7 years with a range of 1.5-6.3 years. Assessment of bladder capacity post-BNR revealed that mean (⫾SD) capacity ⬍1.5 (n ⫽ 12) and ⱖ1.5 (n ⫽ 7) years post-BNR was 65 ⫾ 43.3 mL and 198 ⫾ 113 mL, respectively. There was a significant increase in capacity from pre-BNR to ⱖ1.5 years post-BNR (P ⫽ 0.013) and from ⬍1.5 and ⱖ1.5 years post-BNR (P ⫽ 0.002). There was a significant increase in capacity ⱖ1.5 years post-BNR relative to pre-BNR capacity (P ⫽ 0.005). For the 7 patients with data at both ⬍1.5 and ⱖ1.5 years after BNR, there was a significant increase in bladder capacity (P ⫽ 0.005) for these times. Results are before bladder augmentation that was eventually performed in 2 boys.
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BNR in Bladder Exstrophy
Figure 1
93
Flow chart of outcome; A. Males ⬎ years post-CPRE (n ⫽ 22) and B. Females ⬎3 years post-CPRE (n ⫽ 11).
Urodynamic study Table 2 shows detrusor pressure at cystometric capacity and compliance from UDS. Eight patients had UDS data available pre-BNR and 4 at ⱖ1.5 years post-BNR. Bladder compliance was significantly greater (P ⫽ 0.003) when we compared pre-BNR (n ⫽ 8) versus ⱖ1.5 years post-BNR for 2 male patients who did not go on to augment.
Discussion There are many goals and challenges apparent in the care of patients with BE. Initial repair may take the form of bladder
and posterior urethral closure up onto the penis as the first stage of the MSRE approach, or CPRE in which epispadias repair, or penile disassembly with resultant hypospadias, is performed with bladder closure. Although successful initial closure and early satisfactory cosmetic and functional results are gratifying for the family and health care team, this is only the beginning of the lifelong care necessary for the BE patient. When performing CPRE, it is indeed a challenge to close the bladder neck area in a way that will provide the appropriate amount of resistance to facilitate bladder cycling yet not result in urinary retention. This impacts growth and development of the bladder and, among other outcomes, directly impacts later urinary continence status in these
94
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Figure 2
Bladder capacity pre- and post-BNR for males and females. (Color version of figure is available online.)
individuals. Realizing adequate urinary continence and maintaining efficient voiding ability are, perhaps, the most difficult goals of care. Bladder functions of storage and emptying are influenced greatly by several bladder qualities and characteristics, such as capacity, compliance, contractility, and coordination. Ultimately, when normal, these characteristics help provide urinary continence. In the absence of normal continence, medical, endoscopic, or surgical management may be indicated. With regard to surgical intervention in the patient with BE and persistent incontinence secondary to inadequate bladder outlet resistance, this has typically taken the form of BNR. Again, with BNR, the surgeon seeks to create a degree of bladder outlet resistance that strikes the perfect and delicate balance between extreme outcomes of persistent urinary incontinence and retention, thus creating adequate outlet resistance that pro-
vides urinary continence without retention, while allowing the individual to void spontaneously and efficiently. Published urinary continence rates after staged repair of BE are variable and range from 12% to 83%.16-20 There are various confounding factors to these results, including the patient’s age at bladder closure, the type of closure performed, the number and type of procedures required to establish continence, the need for concomitant bladder augmentation, and the need for CIC. Further confusing the situation is that there is no standard definition for continence used in reports on this subject. Traditionally, in the exstrophy literature continence has been defined as maintaining
Table 2 Detrusor pressure and compliance at cystometric capacity for bladder exstrophy patients before and after bladder neck reconstruction
Table 1 Bladder capacity in bladder exstrophy patients pre- and post-BNR Pre-BNR capacity ⱕ19 months (n ⫽ 11) Mean: 104 ⫾ 20.4 mL Min: 78 mL /Max: 140 mL Post-BNR capacity ⬍1.5 years (n ⫽ 12) Mean: 65 ⫾ 43.3 mL Min: 15 mL /Max: 175 mL Post-BNR capacity ⱖ1.5 years (n ⫽ 7) Mean: 198 ⫾ 113 mL Min: 94 mL/Max: 350 mL Range in months ⬍1.5 years pre-BNR ⫽ 1-14 months. Mean time for first post-op measure (n⫽12) was 4.6 ⫾ 4.8 months, range 0.3914.7 months. Mean time for latest post op measure (n ⫽ 7) was 3.5 ⫾ 1.7 years; range 1.5-6.3 years. BNR, bladder neck reconstruction.
Pre-BNR (n ⫽ 8) ⬍1.5 years post-BNR (n ⫽ 6) ⱖ1.5 years post-BNR n⫽2 n ⫽ 2*
Detrusor pressure @ cystometric capacity (cm H2O)
Compliance @ cystometric capacity (mL/cm H2O)
9.8 ⫾ 7.1 Range: 1-25 14.8 ⫾ 12.5 Range: 5-38
11.4 ⫾ 3.6 Range: 5.6-14.40 8.8 ⫾ 9.7 Range: 1.18-28
8.0 ⫾ 2.8 Range: 6-10 17.5 ⫾ 3.4 Range: 13-22
23.2 ⫾ 3.8 Range: 20.5-25.9 5.9 ⫾ 3.1 Range: 3.7-8.1
Data reported post-BNR is before augmentation cystoplasty. BNR, bladder neck reconstruction. *Two go on to augment.
Borer
BNR in Bladder Exstrophy
dry periods of 3 hours or greater during the day with no stress incontinence. However, this is not the definition of continence established by the International Children’s Continence Society.21 The rate of urinary continence after BNR is not exceptional in most published series. In a retrospective review by Capolicchio et al16 of 43 patients with BE and cloacal exstrophy they found that only 5 patients were dry after BNR alone. 88 percent of their patients required further operations to achieve continence, including redo BNR (30%), bladder augmentation (42%), and bladder neck closure in 44%. Lottmann et al.18 provide a long-term (mean 12 years) analysis of a cohort of 57 patients with BE who underwent staged repair. Their continence rate is low, with only 21% of their patients achieving their definition of “good” continence after BNR. Although these are BE patients undergoing staged repair it is important to underscore that the result of urinary continence seems to be similar for both techniques after BNR although clearly further studies are warranted. In addition to clinical data (primarily dry interval), imaging and endoscopic assessment and data obtained from urodynamic studies were helpful in management of our patients. Although bladder neck anatomy was subjectively assessed at cystoscopy for appearance and ability for coaptation, transversely oriented oval shape of the bladder neck was the typical appearance in the incontinent patient. This appearance may be a result of lateral drift from midline of both pubic bones and attached soft tissues, including those tissues that comprise the bladder neck. This consistent observation at endoscopy may be one component responsible for the incontinence that is noted in most patients postCPRE. Subjective assessment of bladder neck anatomy at endoscopy or by fluoroscopic VCUG for ability to close did not have a significant impact on the decision-making process regarding need for BNR. Although several variables were used to help determine which of our patients were in need of and were appropriate candidates for BNR, perhaps, the most important of these variables was clinical observation of dry-interval either by caregiver(s) and/or physician. Family and patient interest in attaining continence also played an important role in the decision to proceed to BNR with an understanding and acceptance of other possible outcomes, such as the potential need for CIC. Urinary continence at 1 year post-BNR was observed in 3 of 12 (25%) patients. Over time, it appeared as that there was an inability for this initial level of success to be maintained as there were no patients with a dry interval of ⬎3 hours at ⱖ1.5 years post-BNR. However, 5 of the 12 patients were on a trajectory trending toward continence at last follow up. Time will tell if the bladder of these patients will continue to respond in a positive manner. Potentially, decompensation of the bladder wall in the face of increased bladder-outlet resistance is responsible for this phenomenon. For example, patient Number 9 (Figure 2) in this report underwent BNR and appendicovesicostomy that was initially successful, 5 months post-BNR, at a time
95 that the patient experienced a brief period of continence and increased capacity (200 mL every 3 hours CIC), but then at 18 months post-BNR returned to persistent incontinence and decreased capacity, and eventually went on to augmentation ileocystoplasty. This course and outcome may be evidence that this patient’s bladder was able to compensate and grow to a certain extent in the early stages post-BNR because of increased outlet resistance from BNR. However, over time, its ability to respond favorably to increased outlet resistance reached its limits, and there was not only no further growth or expansion, but perhaps, the bladder decompensated, and the continence mechanism provided by BNR was overwhelmed. Unfortunately, this was the outcome even with the use of maximum dose anticholinergic medication, and eventually this patient required bladder augmentation. In our patients, compared with pre-BNR bladder capacity, there was an initial decrease in capacity post-BNR, followed by significant increase beyond 1.5 years post-BNR. Overall, there was a significant increase in bladder capacity after BNR relative to pre-BNR values. Although the numbers were few, an associated improvement in bladder compliance appeared to be an indicator of long-term success of BNR alone versus decreased compliance and eventual need for bladder augmentation. Concerns after BNR include voiding pressures elevated greater than normal and inefficient emptying, and increased/ supranormal outlet resistance (“hypercontinence”). Mouriquand et al22 reported a series of BE patients undergoing BNR after MSRE. Although not CPRE patients, they found a 45% overall continence rate, 65% rate of recurrent urinary tract infections, 24% rate of urinary stones, 26% incidence of hydronephrosis, and a 16% rate of bladder perforation. Forty-eight percent of their patients required further surgery. They conclude that most complications encountered were related to the obstructive pattern of bladder emptying and the abnormal bladder urodynamic behavior caused by BNR. In our series, one 14-year-old patient post-CPRE and post-BNR had elevated voiding pressures, incomplete emptying, hydronephrosis (unilateral moderate), and eventually experienced bladder rupture after blunt abdominal trauma. This anecdotal case data reported by Mouriquand et al22 highlights the possibility that the bladder of the BE patient is not “normal” no matter how successful we are at achieving “optimal” outlet resistance and cycling with CPRE or MSRE. Electronmicroscopy studies of the newborn exstrophy bladder smooth muscle show a decrease in smooth muscle, increase in collagen and decrease in small nerve muscle innervations.23 With regard to BNR following CPRE, the authors of one series have shown that BNR may be necessary in as many as 80% of male and 57% of female patients.4 This finding is consistent with our data in that 41% of male and 27% of female patients have undergone BNR but 82% of males and 55% of females will likely need BNR (Figure 1A, B). It is interesting to note that both the current study and others16,22 have found overall greater continence rates for females than
96 males. Another single-center experience has also been reported by Shoukry and colleagues6 who reported 37% continence, but only after augmentation 1cystoplasty. All continent patients required CIC. This last study, along with our experience and others,22,24 emphasizes that it is likely that there is inefficient voiding and incomplete emptying in these patients and that the exstrophy bladder may not compensate after a bladder outlet procedure with increased growth in all cases. Finally, in a recent large study from a center that only does MSRE repairs, 34 patients (31 male, 3 female) were reported who underwent modified Young Dees Leadbetter repair after CPRE in the newborn period. The voided day and night continence rate was 55%. However, the most interesting finding was that of the dry group, all had pelvic osteotomy at closure, all had ahypospadias repair before 1 year and none had a ureteral reimplantation before BNR.25 Clearly, from all of these studies it is evident that these complex patients require close observation and follow up evaluation for upper urinary tract health and for bladder storage ability and emptying efficiency and careful evaluation for BNR. We realize that there are various limitations to the current study. It is a retrospective study of a small cohort of patients. Furthermore, continence history was obtained from parents and patients without the use of a validated urinary continence questionnaire. Finally, our data still represent relatively short-term follow-up. However, we believe that a significant strength is that all of our BE patients are followed on a prospective protocol that allow us to gather standardized clinical, radiographic and urodynamic data.
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011
4.
5.
6.
7.
8.
9. 10. 11. 12.
13.
14. 15.
16.
Conclusions On the basis of our findings we conclude that most patients with BE require BNR after CPRE. This need for BNR is greater in male patients. Relative to pre-BNR capacity, there is a significant increase in bladder capacity ⱖ1.5 years post-BNR. Some patients require adjunctive procedures to assure bladder emptying. The rate of BNR in our patients after CPRE is similar to that of other published reports. Our results have strengthened our view that the complex reconstructive surgery necessary in management of this rare entity, BE should be performed only by surgeons with extensive experience and at centers with multidisciplinary approach to care.
References 1. Baker LA, Gearhart JP. The staged approach to bladder exstrophy closure and the role of osteotomies. World J Urol 1998;16:205-11. 2. Grady RW, Mitchell ME. Newborn exstrophy closure and epispadias repair. World J Urol 1998;16:200-4. 3. Borer JG, Gargollo PC, Kinnamon DD, et al. Bladder growth and development after complete primary repair of bladder exstrophy in the
17. 18.
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24. 25.
newborn with comparison to staged approach. J Urol 2005;174: 1553-7; discussion:7-8. Shnorhavorian M, Grady RW, Andersen A, et al. Long-term followup of complete primary repair of exstrophy: The Seattle experience. J Urol 2008;180(4 suppl):1615-9; discussion: 9-20. Gearhart JP, Baird A, Nelson CP. Results of bladder neck reconstruction after newborn complete primary repair of exstrophy. J Urol 2007;178):1619-22; discussion: 22. Shoukry AI, Ziada AM, Morsi HA, et al. Outcome of complete primary bladder exstrophy repair: Single-center experience. J Pediatr Urol 2009;5:496-9. Borer JG, Gargollo PC, Hendren WH, et al. Early outcome following complete primary repair of bladder exstrophy in the newborn. J Urol 2005;174:1674-8; discussion: 8-9. Gargollo PC, Borer JG, Diamond DA, et al. Prospective followup in patients after complete primary repair of bladder exstrophy. J Urol 2008;180(4 suppl):1665-70; discussion: 70. Young HH. An operation for the cure of incontinence associated with epispadias. J Urol 1922;7:1-32. Dees JE. Congenital epispadias with incontinence. J Urol 1949;62: 513-22. Leadbetter GW. Surgical correction of total urinary incontinence. J Urol 1964;91:261-6. Canning DA, Gearhart JP, Peppas DS, et al. The cephalotrigonal reimplant in bladder neck reconstruction for patients with exstrophy or epispadias. J Urol 1993;150:156-8. Mitrofanoff P. Trans-appendicular continent cystostomy in the management of the neurogenic bladder [in French]. Chir Pediatr 1980;21: 297-305. Yang WH. Yang needle tunneling technique in creating antireflux and continent mechanisms. J Urol 1993;150:830-4. Monti PR, Lara RC, Dutra MA, et al. New techniques for construction of efferent conduits based on the Mitrofanoff principle. Urology 1997; 49:112-5. Capolicchio G, McLorie GA, Farhat W, et al. A population based analysis of continence outcomes and bladder exstrophy. J Urol 2001; 165:2418-21. Hollowell JG, Ransley PG. Surgical management of incontinence in bladder exstrophy. Br J Urol 1991;68:543-8. Lottmann HB, Melin Y, Cendron M, et al. Bladder exstrophy: Evaluation of factors leading to continence with spontaneous voiding after staged reconstruction. J Urol 1997;158:1041-4. Surer I, Baker LA, Jeffs RD, et al. Combined bladder neck reconstruction and epispadias repair for exstrophy-epispadias complex. J Urol 2001;165:2425-7. Shaw MB, Rink RC, Kaefer M, et al. Continence and classic bladder exstrophy treated with staged repair. J Urol 2004;172:1450-3; discussion: 3. Neveus T, von Gontard A, Hoebeke P, et al. The standardization of terminology of lower urinary tract function in children and adolescents: Report from the Standardisation Committee of the International Children’s Continence Society. J Urol 2006;176:314-24. Mouriquand PD, Bubanj T, Feyaerts A, et al. Long-term results of bladder neck reconstruction for incontinence in children with classical bladder exstrophy or incontinent epispadias. BJU Int 2003;92:9971001; discussion: 2. Mathews R, Wills M, Perlman E, et al. Neural innervation of the newborn exstrophic bladder: An immunohistochemical study. J Urol 1999;162:506-8. Yerkes EB, Adams MC, Rink RC, et al. How well do patients with exstrophy actually void? J Urol 2000;164:1044-7. Purves T, Novak T, King J, et al. Modified Young Dees Leadbetter bladder neck reconstruction after exstrophy repair. J Urol 2009;182: 1813-7.
The use of bladder neck reconstruction in bladder exstrophy Joseph G. Borer, MD From the Department of Surgery (Urology), Harvard Medical School and Assistant in Urology, Children’s Hospital Boston, Boston, Massachusetts. KEYWORDS Bladder exstrophy; Bladder neck reconstruction; Urinary continence
A major goal of bladder exstrophy (BE) management is achieving urinary continence, most commonly with surgical bladder neck reconstruction (BNR). This is a report of outcome of BNR after complete primary repair of exstrophy (CPRE). At our institution, patient history, ultrasound, cystogram (VCUG) and urodynamic study (UDS) were performed during a prospective evaluation of patients with BE. Dry interval of ⬎3 hours was used as the definition of continence and dry interval ⬍1-hour incontinence. Bladder capacity was measured at VCUG and/or UDS. UDS was also used to assess bladder compliance. From 1994 to 2010, we cared for 47 BE patients (31 male, 15 female) after CPRE. For patients ⱖ3 years after CPRE, BNR was performed in 9 of 22 (41%) male and 3 of 11 (27%) female patients. Mean age at BNR was 6.3 and 5.9 years for male and female patients, respectively. The mean (⫾SD) bladder capacity pre-BNR was 104.8 (⫾20.4 mL). There was a significant increase in capacity from pre-BNR to ⱖ1.5 years post-BNR (P ⫽ 0.013) and from ⬍1.5 and ⱖ1.5 years post-BNR (P ⫽ 0.002). In conclusion, most patients with BE require BNR after CPRE. The need for BNR is more common in male patients. © 2011 Elsevier Inc. All rights reserved.
The goal of initial surgical management of the patient with bladder exstrophy (BE) is successful closure of the bladder and proximal urethra (ie, modern staged repair [MSRE])1 or successful bladder closure and epispadias repair (complete primary repair, ie, complete primary repair of exstrophy [CPRE]).2 Elimination of vesicoureteral reflux, thus decreasing the risk of reflux nephropathy, is also a priority in the management of BE, with antireflux surgery potentially at the time of initial closure when anatomy permits. Theoretically, one potential advantage of CPRE over MSRE is earlier provision of increased bladder outlet resistance resulting in bladder cycling and optimal bladder growth. It has been shown that, at early follow-up and irrespective of gender or management (MSRE post-bladder neck reconstruction [BNR] vs CPRE), percent predicted bladder capacity was equivalent.3 Address reprint requests and correspondence: Joseph G. Borer, MD Children’s Hospital Boston, 300 Longwood Ave, Boston, MA 02115. E-mail: [email protected].
1055-8586/$ -see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1053/j.sempedsurg.2010.12.003
Urinary continence with ability to void spontaneously and efficiently are major goals for management of BE. For MSRE, BNR, the third stage of the staged approach, is performed in all patients. BNR is performed in only those CPRE patients who require such. In one series, it has been shown that BNR was necessary in 80% of male and 57% of female patients after CPRE.4 Experience from other centers has also been reported showing that almost all patients may require BNR.5,6 The purpose of the present study was to examine the need for and outcome of BNR following CPRE in patients at our institution.
Methods A retrospective review of BE patients through use of our Institutional Review Board approved exstrophy database was performed. BE patients who had undergone CPRE were
92 identified. Early outcome in much of this patient population has been reported.7 Patient history, physical observations, renal and bladder ultrasound, voiding cystourethrogram (VCUG), and urodynamic study (UDS) were used during prospective evaluation of patients with BE.8 These observations and data were used as means to determine the need for BNR and to evaluate outcome afterward. Urinary continence was assessed in those patients ⱖ3 years post-CPRE and recorded as a dry interval. Dry interval was reported on a scale of ⬍1 hour 1-2 hours, ⬎2-3, or ⬎3 hours, and was recorded from patient and/or caregiver history and, at times, physician observation in the clinic. Urinary incontinence was defined as a dry interval ⬍1 hour and continence was defined as a dry interval ⬎3 hours. Bladder capacity was measured by gravity fill at examination under anesthetic and VCUG (saline or fluoroscopic contrast height at 40 cm above the bladder) and during UDS with body temperature saline instillation. Bladder capacity was recorded as the volume of fluid instilled or infused at either the onset of leaking or voiding, or at the report of sensation of bladder fullness by the patient. Specifics of technique for urodynamic assessment have been published.3 A patient was considered an appropriate candidate for BNR if all the following applied; (1) incontinent (dry interval ⬍1 h), (2) ⱖ5 years of age, (3) patient and caregiver(s) interested in attaining continence, and (4) patient and caregiver(s) acceptance of potential impaired bladder emptying and possible need for clean intermittent catheterization (CIC). Adjunctive catheterizable conduit creation was offered as an option to obviate the need for urethral catheterization in the case that CIC should be necessary following BNR. CPRE was performed as previously described.7 In those cases in which anatomy was amenable (adequate size of bladder plate), antireflux surgery either via cross-trigone or cephalotrigonal technique was performed at CPRE. BNR was performed via the method of Young,9 Dees,10 and Leadbetter.11 The strip of tissue to be tubularized was measured at approximately 13-15 mm in width and approximately 2.5-3 cm in length beginning from the bladder outlet. The reconstructed bladder neck tube was closed over either a 6-Fr or 8-Fr catheter (without balloon) and remained in place for approximately 5-7 days postoperatively. Urinary diversion was via bilateral ureteral stent and suprapubic cystostomy tube. Bilateral cephalotrigonal ureteral reimplantation was performed as previously described.12 Catheterizable conduit, such as appendicovesicostomy13 or reconfigured bowel tube14,15 was performed as previously described. SPSS 18.0 software was used for database management and statistical analysis. T test for independent samples and a paired t tests were used to compare bladder capacity relative to BNR status and timing. Unpaired t test was used to compare pre-BNR versus post-BNR bladder compliance. Statistical significance was considered P ⬍ 0.05.
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011
Results During the period 1994-2010, we cared for 47 BE patients (32 male, 15 female) after CPRE. Six had initial closure at an outside institution. Forty-one patients had CPRE at Children’s Hospital Boston. Thirty-two of these 41 patients underwent CPRE within 72 hours of birth. Outcome from initial closure (CPRE) was assessed by clinical parameters, physical examination, and VCUG. Early outcome was marked by successful closure in all but one female patient that suffered dehiscence. Thirty-two of our patients were operated as newborns and 3 underwent bilateral ureteral reimplantation at CPRE. Success rate for those 3 patients was 100%. Flow charts that designate patient progress and outcome during the course of care are shown in Figure 1A for male patients and Figure 1B for female patients ⱖ3 years of age and/or ⱖ3 years postCPRE. Regarding the specifics of BNR, of those patients ⱖ3 years of age, BNR was performed in 9 of 22 (41%) male and 3 of 11 (27%) female patients. Mean age at BNR was 6.3 and 5.9 years for male and female patients, respectively. All 12 patients were incontinent before BNR. Post-BNR dry interval was assessed at various points after the surgery, at 1 year (range of 3 months to 1 year) and ⱖ1.5 years after BNR. There was improvement in dry interval for 8 of 12 after the surgery. Three patients were continent 1 year post-BNR, and although some had a dry interval of ⬎2-3 hours, none were continent (by stringent definition of dry interval ⬎3 hours) at ⱖ1.5 years. Five of the 12 patients were on a trajectory trending toward continence at last follow-up. These results are before bladder augmentation that was eventually performed in 2 of the 12 patients, both male.
Bladder capacity Bladder capacity obtained at examination under anesthesia, VCUG, and UDS (Figure 2, Table 1). Pre-BNR bladder capacity was available for 11 of 12 patients who underwent BNR. The mean (⫾SD) bladder capacity pre-BNR was 104.8 ⫾ 20.4 mL. Mean time for first postoperative measure (⬍1.5 years post-BNR; n ⫽ 12) was 4.6 ⫾ 4.8 months with a range of 0.39-14.7 months. Mean time for latest post-BNR measure (ⱖ1.5 years post-BNR; n ⫽ 7) is 3.5 ⫾ 1.7 years with a range of 1.5-6.3 years. Assessment of bladder capacity post-BNR revealed that mean (⫾SD) capacity ⬍1.5 (n ⫽ 12) and ⱖ1.5 (n ⫽ 7) years post-BNR was 65 ⫾ 43.3 mL and 198 ⫾ 113 mL, respectively. There was a significant increase in capacity from pre-BNR to ⱖ1.5 years post-BNR (P ⫽ 0.013) and from ⬍1.5 and ⱖ1.5 years post-BNR (P ⫽ 0.002). There was a significant increase in capacity ⱖ1.5 years post-BNR relative to pre-BNR capacity (P ⫽ 0.005). For the 7 patients with data at both ⬍1.5 and ⱖ1.5 years after BNR, there was a significant increase in bladder capacity (P ⫽ 0.005) for these times. Results are before bladder augmentation that was eventually performed in 2 boys.
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Figure 1
93
Flow chart of outcome; A. Males ⬎ years post-CPRE (n ⫽ 22) and B. Females ⬎3 years post-CPRE (n ⫽ 11).
Urodynamic study Table 2 shows detrusor pressure at cystometric capacity and compliance from UDS. Eight patients had UDS data available pre-BNR and 4 at ⱖ1.5 years post-BNR. Bladder compliance was significantly greater (P ⫽ 0.003) when we compared pre-BNR (n ⫽ 8) versus ⱖ1.5 years post-BNR for 2 male patients who did not go on to augment.
Discussion There are many goals and challenges apparent in the care of patients with BE. Initial repair may take the form of bladder
and posterior urethral closure up onto the penis as the first stage of the MSRE approach, or CPRE in which epispadias repair, or penile disassembly with resultant hypospadias, is performed with bladder closure. Although successful initial closure and early satisfactory cosmetic and functional results are gratifying for the family and health care team, this is only the beginning of the lifelong care necessary for the BE patient. When performing CPRE, it is indeed a challenge to close the bladder neck area in a way that will provide the appropriate amount of resistance to facilitate bladder cycling yet not result in urinary retention. This impacts growth and development of the bladder and, among other outcomes, directly impacts later urinary continence status in these
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Figure 2
Bladder capacity pre- and post-BNR for males and females. (Color version of figure is available online.)
individuals. Realizing adequate urinary continence and maintaining efficient voiding ability are, perhaps, the most difficult goals of care. Bladder functions of storage and emptying are influenced greatly by several bladder qualities and characteristics, such as capacity, compliance, contractility, and coordination. Ultimately, when normal, these characteristics help provide urinary continence. In the absence of normal continence, medical, endoscopic, or surgical management may be indicated. With regard to surgical intervention in the patient with BE and persistent incontinence secondary to inadequate bladder outlet resistance, this has typically taken the form of BNR. Again, with BNR, the surgeon seeks to create a degree of bladder outlet resistance that strikes the perfect and delicate balance between extreme outcomes of persistent urinary incontinence and retention, thus creating adequate outlet resistance that pro-
vides urinary continence without retention, while allowing the individual to void spontaneously and efficiently. Published urinary continence rates after staged repair of BE are variable and range from 12% to 83%.16-20 There are various confounding factors to these results, including the patient’s age at bladder closure, the type of closure performed, the number and type of procedures required to establish continence, the need for concomitant bladder augmentation, and the need for CIC. Further confusing the situation is that there is no standard definition for continence used in reports on this subject. Traditionally, in the exstrophy literature continence has been defined as maintaining
Table 2 Detrusor pressure and compliance at cystometric capacity for bladder exstrophy patients before and after bladder neck reconstruction
Table 1 Bladder capacity in bladder exstrophy patients pre- and post-BNR Pre-BNR capacity ⱕ19 months (n ⫽ 11) Mean: 104 ⫾ 20.4 mL Min: 78 mL /Max: 140 mL Post-BNR capacity ⬍1.5 years (n ⫽ 12) Mean: 65 ⫾ 43.3 mL Min: 15 mL /Max: 175 mL Post-BNR capacity ⱖ1.5 years (n ⫽ 7) Mean: 198 ⫾ 113 mL Min: 94 mL/Max: 350 mL Range in months ⬍1.5 years pre-BNR ⫽ 1-14 months. Mean time for first post-op measure (n⫽12) was 4.6 ⫾ 4.8 months, range 0.3914.7 months. Mean time for latest post op measure (n ⫽ 7) was 3.5 ⫾ 1.7 years; range 1.5-6.3 years. BNR, bladder neck reconstruction.
Pre-BNR (n ⫽ 8) ⬍1.5 years post-BNR (n ⫽ 6) ⱖ1.5 years post-BNR n⫽2 n ⫽ 2*
Detrusor pressure @ cystometric capacity (cm H2O)
Compliance @ cystometric capacity (mL/cm H2O)
9.8 ⫾ 7.1 Range: 1-25 14.8 ⫾ 12.5 Range: 5-38
11.4 ⫾ 3.6 Range: 5.6-14.40 8.8 ⫾ 9.7 Range: 1.18-28
8.0 ⫾ 2.8 Range: 6-10 17.5 ⫾ 3.4 Range: 13-22
23.2 ⫾ 3.8 Range: 20.5-25.9 5.9 ⫾ 3.1 Range: 3.7-8.1
Data reported post-BNR is before augmentation cystoplasty. BNR, bladder neck reconstruction. *Two go on to augment.
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dry periods of 3 hours or greater during the day with no stress incontinence. However, this is not the definition of continence established by the International Children’s Continence Society.21 The rate of urinary continence after BNR is not exceptional in most published series. In a retrospective review by Capolicchio et al16 of 43 patients with BE and cloacal exstrophy they found that only 5 patients were dry after BNR alone. 88 percent of their patients required further operations to achieve continence, including redo BNR (30%), bladder augmentation (42%), and bladder neck closure in 44%. Lottmann et al.18 provide a long-term (mean 12 years) analysis of a cohort of 57 patients with BE who underwent staged repair. Their continence rate is low, with only 21% of their patients achieving their definition of “good” continence after BNR. Although these are BE patients undergoing staged repair it is important to underscore that the result of urinary continence seems to be similar for both techniques after BNR although clearly further studies are warranted. In addition to clinical data (primarily dry interval), imaging and endoscopic assessment and data obtained from urodynamic studies were helpful in management of our patients. Although bladder neck anatomy was subjectively assessed at cystoscopy for appearance and ability for coaptation, transversely oriented oval shape of the bladder neck was the typical appearance in the incontinent patient. This appearance may be a result of lateral drift from midline of both pubic bones and attached soft tissues, including those tissues that comprise the bladder neck. This consistent observation at endoscopy may be one component responsible for the incontinence that is noted in most patients postCPRE. Subjective assessment of bladder neck anatomy at endoscopy or by fluoroscopic VCUG for ability to close did not have a significant impact on the decision-making process regarding need for BNR. Although several variables were used to help determine which of our patients were in need of and were appropriate candidates for BNR, perhaps, the most important of these variables was clinical observation of dry-interval either by caregiver(s) and/or physician. Family and patient interest in attaining continence also played an important role in the decision to proceed to BNR with an understanding and acceptance of other possible outcomes, such as the potential need for CIC. Urinary continence at 1 year post-BNR was observed in 3 of 12 (25%) patients. Over time, it appeared as that there was an inability for this initial level of success to be maintained as there were no patients with a dry interval of ⬎3 hours at ⱖ1.5 years post-BNR. However, 5 of the 12 patients were on a trajectory trending toward continence at last follow up. Time will tell if the bladder of these patients will continue to respond in a positive manner. Potentially, decompensation of the bladder wall in the face of increased bladder-outlet resistance is responsible for this phenomenon. For example, patient Number 9 (Figure 2) in this report underwent BNR and appendicovesicostomy that was initially successful, 5 months post-BNR, at a time
95 that the patient experienced a brief period of continence and increased capacity (200 mL every 3 hours CIC), but then at 18 months post-BNR returned to persistent incontinence and decreased capacity, and eventually went on to augmentation ileocystoplasty. This course and outcome may be evidence that this patient’s bladder was able to compensate and grow to a certain extent in the early stages post-BNR because of increased outlet resistance from BNR. However, over time, its ability to respond favorably to increased outlet resistance reached its limits, and there was not only no further growth or expansion, but perhaps, the bladder decompensated, and the continence mechanism provided by BNR was overwhelmed. Unfortunately, this was the outcome even with the use of maximum dose anticholinergic medication, and eventually this patient required bladder augmentation. In our patients, compared with pre-BNR bladder capacity, there was an initial decrease in capacity post-BNR, followed by significant increase beyond 1.5 years post-BNR. Overall, there was a significant increase in bladder capacity after BNR relative to pre-BNR values. Although the numbers were few, an associated improvement in bladder compliance appeared to be an indicator of long-term success of BNR alone versus decreased compliance and eventual need for bladder augmentation. Concerns after BNR include voiding pressures elevated greater than normal and inefficient emptying, and increased/ supranormal outlet resistance (“hypercontinence”). Mouriquand et al22 reported a series of BE patients undergoing BNR after MSRE. Although not CPRE patients, they found a 45% overall continence rate, 65% rate of recurrent urinary tract infections, 24% rate of urinary stones, 26% incidence of hydronephrosis, and a 16% rate of bladder perforation. Forty-eight percent of their patients required further surgery. They conclude that most complications encountered were related to the obstructive pattern of bladder emptying and the abnormal bladder urodynamic behavior caused by BNR. In our series, one 14-year-old patient post-CPRE and post-BNR had elevated voiding pressures, incomplete emptying, hydronephrosis (unilateral moderate), and eventually experienced bladder rupture after blunt abdominal trauma. This anecdotal case data reported by Mouriquand et al22 highlights the possibility that the bladder of the BE patient is not “normal” no matter how successful we are at achieving “optimal” outlet resistance and cycling with CPRE or MSRE. Electronmicroscopy studies of the newborn exstrophy bladder smooth muscle show a decrease in smooth muscle, increase in collagen and decrease in small nerve muscle innervations.23 With regard to BNR following CPRE, the authors of one series have shown that BNR may be necessary in as many as 80% of male and 57% of female patients.4 This finding is consistent with our data in that 41% of male and 27% of female patients have undergone BNR but 82% of males and 55% of females will likely need BNR (Figure 1A, B). It is interesting to note that both the current study and others16,22 have found overall greater continence rates for females than
96 males. Another single-center experience has also been reported by Shoukry and colleagues6 who reported 37% continence, but only after augmentation 1cystoplasty. All continent patients required CIC. This last study, along with our experience and others,22,24 emphasizes that it is likely that there is inefficient voiding and incomplete emptying in these patients and that the exstrophy bladder may not compensate after a bladder outlet procedure with increased growth in all cases. Finally, in a recent large study from a center that only does MSRE repairs, 34 patients (31 male, 3 female) were reported who underwent modified Young Dees Leadbetter repair after CPRE in the newborn period. The voided day and night continence rate was 55%. However, the most interesting finding was that of the dry group, all had pelvic osteotomy at closure, all had ahypospadias repair before 1 year and none had a ureteral reimplantation before BNR.25 Clearly, from all of these studies it is evident that these complex patients require close observation and follow up evaluation for upper urinary tract health and for bladder storage ability and emptying efficiency and careful evaluation for BNR. We realize that there are various limitations to the current study. It is a retrospective study of a small cohort of patients. Furthermore, continence history was obtained from parents and patients without the use of a validated urinary continence questionnaire. Finally, our data still represent relatively short-term follow-up. However, we believe that a significant strength is that all of our BE patients are followed on a prospective protocol that allow us to gather standardized clinical, radiographic and urodynamic data.
Seminars in Pediatric Surgery, Vol 20, No 2, May 2011
4.
5.
6.
7.
8.
9. 10. 11. 12.
13.
14. 15.
16.
Conclusions On the basis of our findings we conclude that most patients with BE require BNR after CPRE. This need for BNR is greater in male patients. Relative to pre-BNR capacity, there is a significant increase in bladder capacity ⱖ1.5 years post-BNR. Some patients require adjunctive procedures to assure bladder emptying. The rate of BNR in our patients after CPRE is similar to that of other published reports. Our results have strengthened our view that the complex reconstructive surgery necessary in management of this rare entity, BE should be performed only by surgeons with extensive experience and at centers with multidisciplinary approach to care.
References 1. Baker LA, Gearhart JP. The staged approach to bladder exstrophy closure and the role of osteotomies. World J Urol 1998;16:205-11. 2. Grady RW, Mitchell ME. Newborn exstrophy closure and epispadias repair. World J Urol 1998;16:200-4. 3. Borer JG, Gargollo PC, Kinnamon DD, et al. Bladder growth and development after complete primary repair of bladder exstrophy in the
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