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The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy
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Journal of Pediatric Urology (2018) xx, 1.e1e1.e7
The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy Heather N. Di Carlo, Mahir Maruf, John Jayman, Karl Benz, Mathew Kasprenski, John P. Gearhart Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children’s Hospital, Baltimore, MD, USA Correspondence to: John P. Gearhart, The Johns Hopkins University School of Medicine, The James Buchanan Brady Urological Institute, Robert D. Jeffs Division of Pediatric Urology, Charlotte Bloomberg Children’s Hospital, 1800 Orleans St, Suite 7304, Baltimore, MD 21287, USA. [email protected] (J.P. Gearhart) Keywords Bladder exstrophy; Bladder template; Delayed closure; Bladder capacity; Urinary continence Received 16 January 2018 Accepted 23 March 2018 Available online xxx
Summary Introduction Newborns with classic bladder exstrophy (CBE) may present with a bladder template that is inadequate for closure in the neonatal period (figure). In these cases, a delayed primary closure (DPC) is conducted to permit growth of the bladder template. This study reports the surgical and long-term urinary continence outcomes of poor template CBE patients undergoing DPC and compares them to patients who underwent DPC for reasons unrelated to bladder quality (i.e., prematurity, comorbidities, or a late referral). Methods An institutionally approved, prospectively maintained database of 1330 exstrophyeepispadias complex patients was reviewed for CBE patients who underwent DPC at the authors’ institution. A bladder template was considered inadequate for neonatal closure if found to be inelastic, <3 cm in diameter, and/or covered in hamartomatous polyps. Results In total, 63 patients (53 male and 10 female) undergoing DPC were identified. Of these, 36 had poor bladder templates (group 1). The remaining 27 patients (group 2) had adequate templates and their bladder closure was delayed for reasons unrelated to bladder quality. At the time of DPC, those in group 1 were relatively than those in group 2 (median of 229 vs. 128 days, p Z 0.094). All 36 group 1 patients and 26 (96%) group 2 patients underwent pelvic osteotomy during DPC (p Z 0.429). All patients in this study had a successful primary closure. There was little difference in longitudinal bladder capacities between group 1 and group 2 (p Z 0.518). Also, there was minimal difference in the median number of continence procedures between groups, with both groups having 1 (IQR 1e1) continence procedure (p Z 0.880). Eight patients in group 1, and three patients in group 2 underwent a bladder neck transection with urinary diversion. Of the 13 and 16 patients who have undergone a continence procedure in group 1 and 2, respectively, 11 (84.6%) and 13 (81.3%) are continent of urine. The age of first continence procedure was different between
groups 1 and 2 at 8.0 years (5.8e9.9 years) and 4.8 (3.5e6.0 years), respectively p Z 0.009. The majority of patients in group 1 established continence at a relatively later age when compared to those in group 2, at 11.4 (8.0e14.8) years and 7.9 (2.6e13.2) years of age respectively p Z 0.087. Discussion In the authors’ view, neonatal bladder closure is ideal for CBE patients as it minimizes potential damage to exposed bladder mucosa. However, prior studies indicate that the rate of bladder growth for patients undergoing a delayed primary closure does not differ from patients with a neonatal closure. Results from this study show continued evidence that patients with poor templates who undergo delayed closure have excellent primary closure outcomes, which is critical for further management. Furthermore, this study shows that an inadequate bladder does not affect DPC outcomes or the continence outcomes in DPC patients. However, the inadequate template does affect the type of continence procedure available to a DPC patient, the age of first continence procedure, and the age of continence. Conclusions DPC of the exstrophic bladder has a high rate of success when pelvic osteotomy is utilized as an adjunct. Patients having a DPC for reasons of an inadequate bladder template have comparable rates of bladder growth when compared to DPC of an adequate bladder template. The inadequate bladder template affects the type of continence procedure, with the majority of patients requiring urinary diversion for continence. Patients with an inadequate bladder template have a later age of first continence procedure and a relatively later age of continence, because of an inherently smaller bladder template at birth. The inadequate bladder template patients require a longer period of surveillance to access bladder growth and capacity in preparation of a continence procedure. Furthermore, as the majority of inadequate bladder template patients require a catheterizable channel for continence, the age of continence is also likely influenced by the patient’s preparation as they transition from volitional voiding to catheterization.
https://doi.org/10.1016/j.jpurol.2018.03.023 1477-5131/ª 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023
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Introduction The clinical course of the classic bladder exstrophy (CBE) patient depends upon a successful initial closure for optimal long-term outcomes [1,2]. A secure newborn closure within 30 days of birth, with or without pelvic osteotomy, is ideal in most situations. Occasionally, however, the newborn exstrophic bladder template is too small, is covered with multiple polyps, is inelastic and fibrous, or a combination of the above-mentioned characteristics precluding a successful tension-free primary closure. If the bladder template is deemed inadequate for a newborn period closure, options include cystectomy with urinary diversion or delayed primary closure (DPC) [3]. These patients typically undergo bladder closure after 6 months after monitoring somatic growth of the bladder template. The authors have previously reported on their early success with the latter approach [4e7]. The objective of the current study is to analyze the success of DPC in patients with and without an inadequate bladder template. The authors compare both the closure and continence outcomes of children with poor bladder templates to those with adequate templates who underwent DPC (due to either late referral, scheduling issues, insurance difficulties, prematurity, or other comorbidities). This study provides additional information on the effect inadequate bladder templates may have on DPC outcomes, if any, through its presentation of a large cohort of DPC patients. This study doubles the amount of DPC patients from prior series. Also, it examines the age of first continence procedure and age of continence in patients with an inadequate and inadequate bladder templates at birth. The authors’ hypothesize that the inadequate bladder template will have no effect on DPC outcomes or eventual continence of DPC patients, because delaying the bladder closure will allow the bladder to grow to a size more amenable to a successful closure. However, inadequate bladder template patients will have a later age of continence and more often require urinary diversion and augmentation for continence.
H.N. Di Carlo et al. as being difficult to manipulate manually into the pelvis. Patients with polyps were considered to have poor quality bladders if the bladder either remained too small for closure (diameter of <3 cm) or if the bladder was difficult to manipulate into the pelvis following polyp removal [7]. Group 2 consisted of patients who had a satisfactory bladder template and underwent DPC due to reasons unrelated to bladder quality (i.e. prematurity, comorbidity, insurance, late referral or scheduling reasons). Fig. 1 shows a diagram of patient selection.
Bladder closure Patients with more than 6 months of follow-up from the time of bladder closure were included in the assessment of bladder closure outcomes. A successful bladder closure was defined as a repair that remained intact with no evidence of bladder prolapse, dehiscence, vesicocutaneous fistula formation, or bladder outlet obstruction [8]. Given that all patients undergoing delayed primary closure where greater than 30 days of age, all patients underwent osteotomy to prevent significant abdominal tension with the closure. A pediatric orthopedic surgeon experienced with exstrophy repair determined the type of osteotomy, and the type of osteotomy was either anterior innominate, posterior iliac, or a combination of anterior innominate and posterior iliac given patients’ unique pubic bone anatomy. To secure the
Methods Patient selection An institutionally approved bladder exstrophy database was used to identify CBE patients who underwent DPC at the authors’ institution. All patients were evaluated after they were transferred to authors’ institution by one of two senior surgeons experienced with the reconstruction of exstrophy. The characteristics of the patients’ bladder template were analyzed and patients’ placed into groups through retrospective review. The patients were then classified into two groups to assess the effect an inadequate bladder template may have on DPC outcomes and continence. Group 1 consisted of patients with a poor bladder template that would be inadequate for immediate closure. A poor bladder template was defined as having at least one or a combination of the following characteristics: (1) being fibrous or inelastic, (2) having a diameter of <3 cm [3], or (3) being covered with hamartomatous polyps. A fibrous or inelastic bladder template was defined
Figure 1 Flow diagram of patient selection. Patients included in the study were those with a delayed primary closure. Group 1 consisted of patients whose bladder closure was delayed due to a bladder template with one of the following qualities: (1) fibrous or inelastic, (2) diameter of <3 cm, or (3) covered with hamartomatous polyps. Group 2 consisted of patients with an adequate template for neonatal closure, but whose closure was delayed due to late referral, scheduling, insurance reasons, or other patient factors.
Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023
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The inadequate bladder template bladder closure, immobilization with modified Buck’s traction and external fixation was utilized in patients with an osteotomy. The few DPC patients without an osteotomy were immobilized by modified Bryant’s traction. Epidural pain management was used to minimize discomfort and movement post closure.
Outcomes assessment The authors collected data on bladder template size, template character, reason for DPC, timing of surgery, type of reconstruction utilized, the use of pelvic osteotomy, postoperative outcomes, bladder capacities, use of augmentation cystoplasty, and long-term urinary continence. Bladder capacities were measured by yearly cystograms beginning 1 year after the patients’ primary closure, and cystoscopy was used to assess overall bladder health. The results were analyzed to identify DPC outcomes as well as long-term outcomes focusing on urinary continence. For the purpose of this study, continence was defined as dry intervals of 3 h or more during the day and completely dry at night. Patients who have undergone a continence procedure (defined as bladder neck reconstruction or augmentation, continent stoma creation, and bladder neck transection) were assessed for continence rates and need for augmentation cystoplasty. Continence was evaluated during follow-up visits to the authors’ clinics. Patients who void via urethra and patients who catheterize through a continent stoma were both considered continent. Comparisons between patients in group 1 and group 2 were performed using Fisher’s exact test tests for categorical data, and the Wilcoxon rank sum test for continuous data. The Wilcoxon rank sum test was used to compare populations that did not follow a normal distribution. A linear regression model was used to compare the rate of bladder growth between the two groups. A linear regression model was chosen to account for the many variables that may contribute to bladder capacities between the groups. In the linear regression model, all patients were included with bladder capacity measurements before their continence procedure. A continence procedure was defined as a bladder neck reconstruction or bladder neck transection with augmentation and continent stoma creation. Furthermore, a cumulative incidence curve with the logrank test was used to assess differences in the age of continence between the groups. The start point for the cumulative incidence curve was the patients’ date of birth. The end point was continence using the date of the last successful continence procedure. Censoring occurred at the date of last follow-up. The significance level was set at 0.05 for all comparisons. Statistical analyses were performed using R version 3.4.1 (R Foundation for Statistical Computing, Vienna, Austria).
Results Patient demographics and closure characteristics A total of 63 CBE patients (53 male and 10 female) undergoing DPC were identified. Thirty-six patients were in group 1, which consisted of patients with bladder templates
1.e3 unsuitable for newborn closure, while 27 patients were in group 2, which consisted of patients who otherwise had sufficient bladder templates for bladder closure, but whose closures were delayed. Fig. 2 shows examples of bladder templates that would be considered inadequate for closure in the newborn period. Patients in the group 1 were older at the time of closure when compared to patients in group 2 (229 vs. 128 days, p Z 0.094). The definitions of a poor bladder templates in group 1 were not mutually exclusive. Consequently, 31 bladders (86%) were considered small, 19 (53%) had numerous hamartomatous polyps, and two (6%) were considered inelastic or fibrous. During the primary closure, all 36 patients in group 1, and 26 patients (96%) in group 2 underwent pelvic osteotomy (p Z 0.429). Subsequently, 100% of patients in both groups were successfully closed. Forty patients (19 in group 1 and 21 in group 2) underwent epispadias repair. Demographic and surgical characteristics can be viewed in Table 1. The median follow-up time from closure was 9.6 years (range 0.2e36.4).
Bladder growth Bladder capacities prior to any continence procedure were measured annually in both groups following the successful DPC. The average initial bladder capacity measurements for patients in group 1 and group 2 at roughly 1 year of age were 42.9 mL and 54.8 mL, respectively (p Z 0.391). Fig. 3 shows the bladder capacities and a predicted linear model. The bladder capacity growth rate was 7.2 mL/year (95% CI 5.4e9.1) in group 1 and 6.3 mL/year (95% CI 0.2e12.4) in group 2. The difference between these growth rates was not statistically significant (p Z 0.518). Eight patients (62%) in group 1 and 5 patients (33%) in group 2 underwent bladder augmentation.
Urinary continence outcomes In total, 29 patients, 13 in group 1 and 16 in group 2, underwent a continence procedure. The median number of procedures was similar between these groups, both groups having a median of 1 (IQR 1e1) procedure, p Z 0.880. However, the median age of first continence procedure was different between groups 1 and 2 at 8.0 years (5.8e9.9) and 4.8 years (3.5e6.0) respectively, p Z 0.009 Table 1. Furthermore, the median age of continence was relatively different between the two patient groups, 11.4 years (95% CI 8.0e14.8) versus 7.9 years (95% CI 2.6e13.2), p Z 0.087 (Fig. 4). In group 1, three patients have undergone a Bladder Neck Reconstruction (BNR), all of which are continent and voiding per urethra. Two patients have undergone a BNR with concomitant stoma creation, and 8 have undergone a bladder neck transection with stoma creation. All eight of these patients required bladder augmentation with bowel. In total, 11 (84.6%) of the 13 patients in group 1 who had a continence procedure are continent for urine. Of the patients continent of urine, 3 (27.3%) are voiding from their urethra, whereas 8 (72.7%) are catheterizing through a continent stoma. Of the two patients incontinent of urine, both leak from their stoma.
Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023
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Figure 2 Examples of inadequate classic bladder exstrophy templates for newborn primary closure. A small bladder template with a few hamartomatous polyps (A). Moderate sized bladder covered in small polyps (B). Small fibrous template with polyps (C). Exceptionally small template barely visible below the umbilicus (D).
Table 1
Patient demographics.
Preoperative covariates
Group 1 (n Z 36)
Group 2 (n Z 27)
Sex, n (%) Female Male Median age at closure, days (IQR) Pelvic osteotomy, n (%) Successful outcome, n (%) Median number of continence Procedures, n (IQR) Age of first continence procedure, years (IQR)
6 (16.7%) 30 (83.3%) 228.5 (147.8e316) 36 (100%) 36 (100%) 1 (1e1) 8.0 (5.8e9.9)
4 (14.8%) 23 (85.2%) 128 (57e409) 26 (96.3%) 27 (100%) 1 (1e1) 4.8 (3.5e6.0)
For comparison, 11 patients in group 2 have undergone a BNR, of which 8 (73%) are continent and voiding per urethra. One patient had a BNR with a stoma and augmentation, and three patients had a bladder neck transection with Mitrofanoff appendicovesicostomy. One patient in group 2 is continent and voiding per urethra without a continence procedure. As such, 13 out of 16 patients (81.3% %) in group 2 are continent for urine. Of the patients continent of urine in group 2, 10 (76.9%) are voiding from below and 3 (23.1%) are catheterizing via a catheterizable channel. Of the patients who remain incontinent, two patients void from their urethra and one patient empties via a catheterizable stoma. Table 2 shows the rates of continence in each group.
p 0.999
0.094 0.429 e 0.880 0.009
Discussion The applicability of delayed primary closure has been a topic of debate in previous reports and in ongoing studies. Prior literature has shown the timing of closure does not affect the rate of bladder growth [6,9]. While other reports claim that early closure, allows for early bladder cycling, and carries the advantage of faster bladder growth in patients [10,11]. The inadequate bladder template has been theorized as a potential reason for diminished rates of bladder growth in exstrophy children, although a prior report showed it had no effect on growth [6]. This study validates findings from the previous study to show
Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023
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Figure 3 Bladder growth rate of delayed bladder exstrophy closures due to poor template (Group 1) and late referral or scheduling issues (Group 2). The difference in growth was not statistically significant (p Z 0.518).
Figure 4 Cumulative incidence curve of the age of continence in DPC patients with an inadequate bladder (Group 1) and with a late referral (Group 2). The median age of continence was 11.4 years for Group 1 and 7.9 years for Group 2.
definitively that the quality of the bladder template has no effect on the bladder growth after a delayed primary closure. Furthermore, the delayed primary closure of an exstrophic bladder is theorized to provide better primary closure outcomes for CBE patients with an inadequate bladder template [5,12]. Given the disastrous
consequences after a failed bladder closure, a successful primary closure is the ultimate goal of closure [13,14]. Arena et al. [15] even established that despite a small bladder template at birth, patients could develop normal bladder capacities after a successful closure. The delayed primary closure has previously described advantages of allowing somatic growth of the bladder plate [5]. Results
Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023
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Table 2 Continence rates for patients undergoing delayed bladder closure. Patient group
Continence procedure
N
Continent
Augmentation
Group 1
BNR BNR þ BNT þ Total BNR BNR þ BNT þ Total
3 2 8 13 11 1 3 15
3 (100%) 0 (0%) 5 (62.5%) 8 (61.5%) 8 (72.7%) 1 (100%) 2 (66.7%) 12a (80%)
0 (0%) 0 (0%) 8 (100%) 8 (61.5%) 0 (0%) 1 (100%) 3 (100%) 5a (33.3%)
Group 2
stoma diversion
stoma diversion
a
One patient was continent for urine without a continence procedure.
from this study show continued evidence that delaying bladder closure leads to better primary closure outcomes. Of the 63 patients with a DPC, all of the closures were successful. Osteotomy was utilized as an adjunct in 62 (98.4%) of these cases. While delaying the bladder closure leads to excellent bladder closure outcomes in patients with an inadequate bladder template, these are smaller in size, which may affect the procedure available to a patient for the establishment of continence. In a study by Dodson et al. [4], 47% of patients in the inadequate bladder template group attained continence through bladder neck reconstruction. Likewise, this study shows the majority of inadequate bladder patients obtain continence through a continent urinary diversion. This information is crucial in the early counseling of patients and parents about eventual continence. The present study further adds to the information available for patients with an inadequate bladder template through assessing the age of first continence procedure and the age of continence in DPC patients. The median age of the first continence procedure is different between DPC patients with and without an inadequate bladder template, 8.0 years (IQR 5.8e9.9) versus 4.8 years (IQR 3.5e6.0), respectively, p Z 0.009. The median age of continence for patients with an inadequate bladder was 11.4 years (95% CI 8.0e14.8) compared to 7.9 years (95% CI 2.6e13.2) for patients with an adequate bladder, p Z 0.087. The relative difference in the age of continence is attributable to longer periods of monitoring bladder growth through yearly cystograms, because of an inherently smaller bladder template at birth. This is exemplified in the later age of the first continence procedure for patients with an inadequate bladder template. As the majority of inadequate bladder template patients require catheterization from a continent urinary stoma, the later age of first continence procedure and established continence may also be influenced by the psychological preparation of patients transitioning from volitional voiding of the urethra to catheterizing a channel. Limitations of this study should be considered. First, this is a retrospective study and thus may be subject to selection biases. Some of the records for patients may be unavailable for review as this study extends over a 40-year period. Additionally, it is not possible to determine the causality of successful DPCs or subsequent continence
outcomes. Second, the sample size may be small, resulting in potential restrictions of comparisons. Another limitation is that information on anticholinergic medications in patients was difficult to find. Furthermore, yearly cystograms and measurements of bladder capacities at birth were at times difficult to obtain from paper records for the linear regression model. Another important limitation of this study to consider is that continence was assessed based on patient reported dry intervals of greater than 3 h. As future directions, we will analyze urodynamic parameters in these patient groups. Also, one could investigate the effect delaying bladder closure has on preservation of the upper tract. Still, the incidence of bladder exstrophy is low, making large cohorts, even from a referral center such at the authors’ difficult to obtain. Despite these potential limitations, investigation into the clinical outcomes of patients with poor bladder exstrophy templates is paramount for assessing management strategies.
Summary and guidelines for clinical decision making in cases of an inadequate bladder In summary, the inadequate bladder template can have impacts on surgical outcomes in CBE. DPC is the optimal option for patients with an inadequate bladder template, and leads to superb primary closure outcomes. When comparing DPC patients with and without inadequate bladders templates, the bladder growth rates remains similar. The inadequate bladder template group undergoes their first continence procedure at a later age and establishes continence relatively later when compared to patients with an adequate bladder template. This is likely because of an inherently smaller bladder. These patients require longer periods of monitoring bladder growth to determine the appropriate continence procedure in inadequate bladder patients and the time required for psychological readiness of continent catheterization for these patients.
Evaluation of patients for delayed primary bladder closure At the authors’ institution, the newborn with bladder exstrophy preferentially undergoes a primary closure within the first 48e72 h of life without osteotomy if the bladder template is a good size, the pubic diastasis is less than 4 cm, the pelvis is malleable under anesthesia, the infant’s overall health is acceptable, and scheduling permits [16]. If any of these factors are absent, strong consideration is given to performing the closure later with a bilateral anterior innominate and vertical iliac osteotomy. In rare cases, the bladder template is very small from slow growth of the infant (e.g., low birth weight or twin gestation), multiple polyps, or fibrosis of the bladder template. The fibrosis causes inelasticity that makes an adequate tension free primary closure extremely difficult. In such situations, DPC needs to be considered. If there is any doubt in the newborn period about the suitability of the bladder for closure, further assessment is made under anesthesia to allow a good appraisal of the bladder template and mobility of the pelvic bones when the child is relaxed. If the bladder is deemed to be unsuitable for closure secondary to the
Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023
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The inadequate bladder template presence of multiple hamartomatous polyps, the polyps are removed during the exam under anesthesia, and the bladder closure is delayed if the bladder template is small <3 cm in diameter or difficult to distend into the pelvis. . A bladder template is amendable for immediate closure in patients with a bladder >3 cm in diameter and in patients with a bladder that is easy to distend into the pelvis. Patients with polyps undergo an immediate bladder closure procedure after the polyps are removed, if the bladder is of an adequate size >3 cm and non-fibrotic [7].
Criteria for bladder neck reconstruction or bladder neck transection At the authors’ institution, the decision for patients to undergo either BNR or BNT is determined through a multitude of factors. BNR is recommended in patients who are maturationally ready (4e8 years of age) and who have a sufficient bladder capacity (85e100 mL). Furthermore, these patients are without contraindications such as a failed exstrophy closure or unsatisfactory urodynamic studies (i.e., poor compliance, elevated detrusor pressure, or low contractility) [14,17] If any contraindications are indicated, BNT with continent stoma and augmentation cystoplasty is considered as the first continence procedure.
Conclusions In the CBE child born with an inadequate bladder template, DPC is a reasonable management strategy. The surgeon should consider delaying the closure of inadequate bladders until the template becomes more amenable for reconstruction. Also, this study shows that select patients with poor bladder templates develop sufficient capacity to be continent and void per their urethra after successful BNR, but most are continent by catheterization. Patients with an inadequate bladder undergo a continence procedure at a later age and establish continence at a relatively later when compared to patients with an adequate bladder template at birth. Patients with an inadequate bladder template require longer periods of monitoring bladder growth to assess the appropriate continence procedure. As the majority of these inadequate bladder template patients require catheterization from a continent channel to establish continence, the later age of continence is further influenced by patients transitioning from volitional voiding to catheterizing from a continent channel.
Conflict of interest None.
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Acknowledgment The Kwok Family Foundation of Hong Kong supports the exstrophy database and laboratory research.
References [1] Baradaran N, et al. Effect of failed initial closure on bladder growth in children with bladder exstrophy. J Urol 2011;186: 1450e4. [2] Schaeffer AJ, et al. Complications of primary closure of classic bladder exstrophy. J Urol 2008;180(4 Suppl.):1671e4. discussion 1674. [3] Ko J, et al. Cystectomy in the pediatric exstrophy population: indications and outcomes. Urology 2017. pii: S0090-4295(17) 30990e30991. [4] Dodson JL, et al. The newborn exstrophy bladder inadequate for primary closure: evaluation, management and outcome. J Urol 2001;165(5):1656e9. [5] Baradaran N, et al. Delayed primary closure of bladder exstrophy: immediate postoperative management leading to successful outcomes. Urology 2012;79(2):415e9. [6] Baradaran N, et al. Delayed primary repair of bladder exstrophy: ultimate effect on growth. J Urol 2012;188(6): 2336e41. [7] Jayman J, et al. The surgical management of bladder polyps in the setting of exstrophy epispadias complex. Urology 2017; 109:171e4. [8] Stec AA, et al. The modern staged repair of classic bladder exstrophy: a detailed postoperative management strategy for primary bladder closure. J Pediatr Urol 2012;8(5):549e55. [9] Ferrara F, et al. Delayed exstrophy repair (DER) does not compromise initial bladder development. J Pediatr Urol 2014; 10(3):506e10. [10] Ellison JS, et al. A critical appraisal of continence in bladder exstrophy: long-term outcomes of the complete primary repair. J Pediatr Urol 2016;12:205.e1e7. [11] Grady RW, Mitchell ME. Complete primary repair of exstrophy. J Urol 1999;162(4):1415e20. [12] Dickson AP. The management of bladder exstrophy: the Manchester experience. J Pediatr Surg 2014;49(2):244e50. [13] Novak TE, et al. Failed exstrophy closure: management and outcome. J Pediatr Urol 2010;6(4):381e4. [14] Novak TE, et al. Bladder neck transection for intractable pediatric urinary incontinence. J Urol 2009;181(1):310e4. discussion 314. [15] Arena S, Dickson AP, Cervellione RM. Relationship between the size of the bladder template and the subsequent bladder capacity in bladder exstrophy. J Pediatr Surg 2012;47(2): 380e2. [16] Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 2007;3(4):311e5. [17] Purves T, et al. Modified Young-Dees-Leadbetter bladder neck reconstruction after exstrophy repair. J Urol 2009;182(4 Suppl.):1813e7.
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The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy Heather N. Di Carlo, Mahir Maruf, John Jayman, Karl Benz, Mathew Kasprenski, John P. Gearhart Robert D. Jeffs Division of Pediatric Urology, James Buchanan Brady Urological Institutions, Johns Hopkins Hospital, Johns Hopkins Medical Institutions, Charlotte Bloomberg Children’s Hospital, Baltimore, MD, USA Correspondence to: John P. Gearhart, The Johns Hopkins University School of Medicine, The James Buchanan Brady Urological Institute, Robert D. Jeffs Division of Pediatric Urology, Charlotte Bloomberg Children’s Hospital, 1800 Orleans St, Suite 7304, Baltimore, MD 21287, USA. [email protected] (J.P. Gearhart) Keywords Bladder exstrophy; Bladder template; Delayed closure; Bladder capacity; Urinary continence Received 16 January 2018 Accepted 23 March 2018 Available online xxx
Summary Introduction Newborns with classic bladder exstrophy (CBE) may present with a bladder template that is inadequate for closure in the neonatal period (figure). In these cases, a delayed primary closure (DPC) is conducted to permit growth of the bladder template. This study reports the surgical and long-term urinary continence outcomes of poor template CBE patients undergoing DPC and compares them to patients who underwent DPC for reasons unrelated to bladder quality (i.e., prematurity, comorbidities, or a late referral). Methods An institutionally approved, prospectively maintained database of 1330 exstrophyeepispadias complex patients was reviewed for CBE patients who underwent DPC at the authors’ institution. A bladder template was considered inadequate for neonatal closure if found to be inelastic, <3 cm in diameter, and/or covered in hamartomatous polyps. Results In total, 63 patients (53 male and 10 female) undergoing DPC were identified. Of these, 36 had poor bladder templates (group 1). The remaining 27 patients (group 2) had adequate templates and their bladder closure was delayed for reasons unrelated to bladder quality. At the time of DPC, those in group 1 were relatively than those in group 2 (median of 229 vs. 128 days, p Z 0.094). All 36 group 1 patients and 26 (96%) group 2 patients underwent pelvic osteotomy during DPC (p Z 0.429). All patients in this study had a successful primary closure. There was little difference in longitudinal bladder capacities between group 1 and group 2 (p Z 0.518). Also, there was minimal difference in the median number of continence procedures between groups, with both groups having 1 (IQR 1e1) continence procedure (p Z 0.880). Eight patients in group 1, and three patients in group 2 underwent a bladder neck transection with urinary diversion. Of the 13 and 16 patients who have undergone a continence procedure in group 1 and 2, respectively, 11 (84.6%) and 13 (81.3%) are continent of urine. The age of first continence procedure was different between
groups 1 and 2 at 8.0 years (5.8e9.9 years) and 4.8 (3.5e6.0 years), respectively p Z 0.009. The majority of patients in group 1 established continence at a relatively later age when compared to those in group 2, at 11.4 (8.0e14.8) years and 7.9 (2.6e13.2) years of age respectively p Z 0.087. Discussion In the authors’ view, neonatal bladder closure is ideal for CBE patients as it minimizes potential damage to exposed bladder mucosa. However, prior studies indicate that the rate of bladder growth for patients undergoing a delayed primary closure does not differ from patients with a neonatal closure. Results from this study show continued evidence that patients with poor templates who undergo delayed closure have excellent primary closure outcomes, which is critical for further management. Furthermore, this study shows that an inadequate bladder does not affect DPC outcomes or the continence outcomes in DPC patients. However, the inadequate template does affect the type of continence procedure available to a DPC patient, the age of first continence procedure, and the age of continence. Conclusions DPC of the exstrophic bladder has a high rate of success when pelvic osteotomy is utilized as an adjunct. Patients having a DPC for reasons of an inadequate bladder template have comparable rates of bladder growth when compared to DPC of an adequate bladder template. The inadequate bladder template affects the type of continence procedure, with the majority of patients requiring urinary diversion for continence. Patients with an inadequate bladder template have a later age of first continence procedure and a relatively later age of continence, because of an inherently smaller bladder template at birth. The inadequate bladder template patients require a longer period of surveillance to access bladder growth and capacity in preparation of a continence procedure. Furthermore, as the majority of inadequate bladder template patients require a catheterizable channel for continence, the age of continence is also likely influenced by the patient’s preparation as they transition from volitional voiding to catheterization.
https://doi.org/10.1016/j.jpurol.2018.03.023 1477-5131/ª 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
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Introduction The clinical course of the classic bladder exstrophy (CBE) patient depends upon a successful initial closure for optimal long-term outcomes [1,2]. A secure newborn closure within 30 days of birth, with or without pelvic osteotomy, is ideal in most situations. Occasionally, however, the newborn exstrophic bladder template is too small, is covered with multiple polyps, is inelastic and fibrous, or a combination of the above-mentioned characteristics precluding a successful tension-free primary closure. If the bladder template is deemed inadequate for a newborn period closure, options include cystectomy with urinary diversion or delayed primary closure (DPC) [3]. These patients typically undergo bladder closure after 6 months after monitoring somatic growth of the bladder template. The authors have previously reported on their early success with the latter approach [4e7]. The objective of the current study is to analyze the success of DPC in patients with and without an inadequate bladder template. The authors compare both the closure and continence outcomes of children with poor bladder templates to those with adequate templates who underwent DPC (due to either late referral, scheduling issues, insurance difficulties, prematurity, or other comorbidities). This study provides additional information on the effect inadequate bladder templates may have on DPC outcomes, if any, through its presentation of a large cohort of DPC patients. This study doubles the amount of DPC patients from prior series. Also, it examines the age of first continence procedure and age of continence in patients with an inadequate and inadequate bladder templates at birth. The authors’ hypothesize that the inadequate bladder template will have no effect on DPC outcomes or eventual continence of DPC patients, because delaying the bladder closure will allow the bladder to grow to a size more amenable to a successful closure. However, inadequate bladder template patients will have a later age of continence and more often require urinary diversion and augmentation for continence.
H.N. Di Carlo et al. as being difficult to manipulate manually into the pelvis. Patients with polyps were considered to have poor quality bladders if the bladder either remained too small for closure (diameter of <3 cm) or if the bladder was difficult to manipulate into the pelvis following polyp removal [7]. Group 2 consisted of patients who had a satisfactory bladder template and underwent DPC due to reasons unrelated to bladder quality (i.e. prematurity, comorbidity, insurance, late referral or scheduling reasons). Fig. 1 shows a diagram of patient selection.
Bladder closure Patients with more than 6 months of follow-up from the time of bladder closure were included in the assessment of bladder closure outcomes. A successful bladder closure was defined as a repair that remained intact with no evidence of bladder prolapse, dehiscence, vesicocutaneous fistula formation, or bladder outlet obstruction [8]. Given that all patients undergoing delayed primary closure where greater than 30 days of age, all patients underwent osteotomy to prevent significant abdominal tension with the closure. A pediatric orthopedic surgeon experienced with exstrophy repair determined the type of osteotomy, and the type of osteotomy was either anterior innominate, posterior iliac, or a combination of anterior innominate and posterior iliac given patients’ unique pubic bone anatomy. To secure the
Methods Patient selection An institutionally approved bladder exstrophy database was used to identify CBE patients who underwent DPC at the authors’ institution. All patients were evaluated after they were transferred to authors’ institution by one of two senior surgeons experienced with the reconstruction of exstrophy. The characteristics of the patients’ bladder template were analyzed and patients’ placed into groups through retrospective review. The patients were then classified into two groups to assess the effect an inadequate bladder template may have on DPC outcomes and continence. Group 1 consisted of patients with a poor bladder template that would be inadequate for immediate closure. A poor bladder template was defined as having at least one or a combination of the following characteristics: (1) being fibrous or inelastic, (2) having a diameter of <3 cm [3], or (3) being covered with hamartomatous polyps. A fibrous or inelastic bladder template was defined
Figure 1 Flow diagram of patient selection. Patients included in the study were those with a delayed primary closure. Group 1 consisted of patients whose bladder closure was delayed due to a bladder template with one of the following qualities: (1) fibrous or inelastic, (2) diameter of <3 cm, or (3) covered with hamartomatous polyps. Group 2 consisted of patients with an adequate template for neonatal closure, but whose closure was delayed due to late referral, scheduling, insurance reasons, or other patient factors.
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The inadequate bladder template bladder closure, immobilization with modified Buck’s traction and external fixation was utilized in patients with an osteotomy. The few DPC patients without an osteotomy were immobilized by modified Bryant’s traction. Epidural pain management was used to minimize discomfort and movement post closure.
Outcomes assessment The authors collected data on bladder template size, template character, reason for DPC, timing of surgery, type of reconstruction utilized, the use of pelvic osteotomy, postoperative outcomes, bladder capacities, use of augmentation cystoplasty, and long-term urinary continence. Bladder capacities were measured by yearly cystograms beginning 1 year after the patients’ primary closure, and cystoscopy was used to assess overall bladder health. The results were analyzed to identify DPC outcomes as well as long-term outcomes focusing on urinary continence. For the purpose of this study, continence was defined as dry intervals of 3 h or more during the day and completely dry at night. Patients who have undergone a continence procedure (defined as bladder neck reconstruction or augmentation, continent stoma creation, and bladder neck transection) were assessed for continence rates and need for augmentation cystoplasty. Continence was evaluated during follow-up visits to the authors’ clinics. Patients who void via urethra and patients who catheterize through a continent stoma were both considered continent. Comparisons between patients in group 1 and group 2 were performed using Fisher’s exact test tests for categorical data, and the Wilcoxon rank sum test for continuous data. The Wilcoxon rank sum test was used to compare populations that did not follow a normal distribution. A linear regression model was used to compare the rate of bladder growth between the two groups. A linear regression model was chosen to account for the many variables that may contribute to bladder capacities between the groups. In the linear regression model, all patients were included with bladder capacity measurements before their continence procedure. A continence procedure was defined as a bladder neck reconstruction or bladder neck transection with augmentation and continent stoma creation. Furthermore, a cumulative incidence curve with the logrank test was used to assess differences in the age of continence between the groups. The start point for the cumulative incidence curve was the patients’ date of birth. The end point was continence using the date of the last successful continence procedure. Censoring occurred at the date of last follow-up. The significance level was set at 0.05 for all comparisons. Statistical analyses were performed using R version 3.4.1 (R Foundation for Statistical Computing, Vienna, Austria).
Results Patient demographics and closure characteristics A total of 63 CBE patients (53 male and 10 female) undergoing DPC were identified. Thirty-six patients were in group 1, which consisted of patients with bladder templates
1.e3 unsuitable for newborn closure, while 27 patients were in group 2, which consisted of patients who otherwise had sufficient bladder templates for bladder closure, but whose closures were delayed. Fig. 2 shows examples of bladder templates that would be considered inadequate for closure in the newborn period. Patients in the group 1 were older at the time of closure when compared to patients in group 2 (229 vs. 128 days, p Z 0.094). The definitions of a poor bladder templates in group 1 were not mutually exclusive. Consequently, 31 bladders (86%) were considered small, 19 (53%) had numerous hamartomatous polyps, and two (6%) were considered inelastic or fibrous. During the primary closure, all 36 patients in group 1, and 26 patients (96%) in group 2 underwent pelvic osteotomy (p Z 0.429). Subsequently, 100% of patients in both groups were successfully closed. Forty patients (19 in group 1 and 21 in group 2) underwent epispadias repair. Demographic and surgical characteristics can be viewed in Table 1. The median follow-up time from closure was 9.6 years (range 0.2e36.4).
Bladder growth Bladder capacities prior to any continence procedure were measured annually in both groups following the successful DPC. The average initial bladder capacity measurements for patients in group 1 and group 2 at roughly 1 year of age were 42.9 mL and 54.8 mL, respectively (p Z 0.391). Fig. 3 shows the bladder capacities and a predicted linear model. The bladder capacity growth rate was 7.2 mL/year (95% CI 5.4e9.1) in group 1 and 6.3 mL/year (95% CI 0.2e12.4) in group 2. The difference between these growth rates was not statistically significant (p Z 0.518). Eight patients (62%) in group 1 and 5 patients (33%) in group 2 underwent bladder augmentation.
Urinary continence outcomes In total, 29 patients, 13 in group 1 and 16 in group 2, underwent a continence procedure. The median number of procedures was similar between these groups, both groups having a median of 1 (IQR 1e1) procedure, p Z 0.880. However, the median age of first continence procedure was different between groups 1 and 2 at 8.0 years (5.8e9.9) and 4.8 years (3.5e6.0) respectively, p Z 0.009 Table 1. Furthermore, the median age of continence was relatively different between the two patient groups, 11.4 years (95% CI 8.0e14.8) versus 7.9 years (95% CI 2.6e13.2), p Z 0.087 (Fig. 4). In group 1, three patients have undergone a Bladder Neck Reconstruction (BNR), all of which are continent and voiding per urethra. Two patients have undergone a BNR with concomitant stoma creation, and 8 have undergone a bladder neck transection with stoma creation. All eight of these patients required bladder augmentation with bowel. In total, 11 (84.6%) of the 13 patients in group 1 who had a continence procedure are continent for urine. Of the patients continent of urine, 3 (27.3%) are voiding from their urethra, whereas 8 (72.7%) are catheterizing through a continent stoma. Of the two patients incontinent of urine, both leak from their stoma.
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Figure 2 Examples of inadequate classic bladder exstrophy templates for newborn primary closure. A small bladder template with a few hamartomatous polyps (A). Moderate sized bladder covered in small polyps (B). Small fibrous template with polyps (C). Exceptionally small template barely visible below the umbilicus (D).
Table 1
Patient demographics.
Preoperative covariates
Group 1 (n Z 36)
Group 2 (n Z 27)
Sex, n (%) Female Male Median age at closure, days (IQR) Pelvic osteotomy, n (%) Successful outcome, n (%) Median number of continence Procedures, n (IQR) Age of first continence procedure, years (IQR)
6 (16.7%) 30 (83.3%) 228.5 (147.8e316) 36 (100%) 36 (100%) 1 (1e1) 8.0 (5.8e9.9)
4 (14.8%) 23 (85.2%) 128 (57e409) 26 (96.3%) 27 (100%) 1 (1e1) 4.8 (3.5e6.0)
For comparison, 11 patients in group 2 have undergone a BNR, of which 8 (73%) are continent and voiding per urethra. One patient had a BNR with a stoma and augmentation, and three patients had a bladder neck transection with Mitrofanoff appendicovesicostomy. One patient in group 2 is continent and voiding per urethra without a continence procedure. As such, 13 out of 16 patients (81.3% %) in group 2 are continent for urine. Of the patients continent of urine in group 2, 10 (76.9%) are voiding from below and 3 (23.1%) are catheterizing via a catheterizable channel. Of the patients who remain incontinent, two patients void from their urethra and one patient empties via a catheterizable stoma. Table 2 shows the rates of continence in each group.
p 0.999
0.094 0.429 e 0.880 0.009
Discussion The applicability of delayed primary closure has been a topic of debate in previous reports and in ongoing studies. Prior literature has shown the timing of closure does not affect the rate of bladder growth [6,9]. While other reports claim that early closure, allows for early bladder cycling, and carries the advantage of faster bladder growth in patients [10,11]. The inadequate bladder template has been theorized as a potential reason for diminished rates of bladder growth in exstrophy children, although a prior report showed it had no effect on growth [6]. This study validates findings from the previous study to show
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Figure 3 Bladder growth rate of delayed bladder exstrophy closures due to poor template (Group 1) and late referral or scheduling issues (Group 2). The difference in growth was not statistically significant (p Z 0.518).
Figure 4 Cumulative incidence curve of the age of continence in DPC patients with an inadequate bladder (Group 1) and with a late referral (Group 2). The median age of continence was 11.4 years for Group 1 and 7.9 years for Group 2.
definitively that the quality of the bladder template has no effect on the bladder growth after a delayed primary closure. Furthermore, the delayed primary closure of an exstrophic bladder is theorized to provide better primary closure outcomes for CBE patients with an inadequate bladder template [5,12]. Given the disastrous
consequences after a failed bladder closure, a successful primary closure is the ultimate goal of closure [13,14]. Arena et al. [15] even established that despite a small bladder template at birth, patients could develop normal bladder capacities after a successful closure. The delayed primary closure has previously described advantages of allowing somatic growth of the bladder plate [5]. Results
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Table 2 Continence rates for patients undergoing delayed bladder closure. Patient group
Continence procedure
N
Continent
Augmentation
Group 1
BNR BNR þ BNT þ Total BNR BNR þ BNT þ Total
3 2 8 13 11 1 3 15
3 (100%) 0 (0%) 5 (62.5%) 8 (61.5%) 8 (72.7%) 1 (100%) 2 (66.7%) 12a (80%)
0 (0%) 0 (0%) 8 (100%) 8 (61.5%) 0 (0%) 1 (100%) 3 (100%) 5a (33.3%)
Group 2
stoma diversion
stoma diversion
a
One patient was continent for urine without a continence procedure.
from this study show continued evidence that delaying bladder closure leads to better primary closure outcomes. Of the 63 patients with a DPC, all of the closures were successful. Osteotomy was utilized as an adjunct in 62 (98.4%) of these cases. While delaying the bladder closure leads to excellent bladder closure outcomes in patients with an inadequate bladder template, these are smaller in size, which may affect the procedure available to a patient for the establishment of continence. In a study by Dodson et al. [4], 47% of patients in the inadequate bladder template group attained continence through bladder neck reconstruction. Likewise, this study shows the majority of inadequate bladder patients obtain continence through a continent urinary diversion. This information is crucial in the early counseling of patients and parents about eventual continence. The present study further adds to the information available for patients with an inadequate bladder template through assessing the age of first continence procedure and the age of continence in DPC patients. The median age of the first continence procedure is different between DPC patients with and without an inadequate bladder template, 8.0 years (IQR 5.8e9.9) versus 4.8 years (IQR 3.5e6.0), respectively, p Z 0.009. The median age of continence for patients with an inadequate bladder was 11.4 years (95% CI 8.0e14.8) compared to 7.9 years (95% CI 2.6e13.2) for patients with an adequate bladder, p Z 0.087. The relative difference in the age of continence is attributable to longer periods of monitoring bladder growth through yearly cystograms, because of an inherently smaller bladder template at birth. This is exemplified in the later age of the first continence procedure for patients with an inadequate bladder template. As the majority of inadequate bladder template patients require catheterization from a continent urinary stoma, the later age of first continence procedure and established continence may also be influenced by the psychological preparation of patients transitioning from volitional voiding of the urethra to catheterizing a channel. Limitations of this study should be considered. First, this is a retrospective study and thus may be subject to selection biases. Some of the records for patients may be unavailable for review as this study extends over a 40-year period. Additionally, it is not possible to determine the causality of successful DPCs or subsequent continence
outcomes. Second, the sample size may be small, resulting in potential restrictions of comparisons. Another limitation is that information on anticholinergic medications in patients was difficult to find. Furthermore, yearly cystograms and measurements of bladder capacities at birth were at times difficult to obtain from paper records for the linear regression model. Another important limitation of this study to consider is that continence was assessed based on patient reported dry intervals of greater than 3 h. As future directions, we will analyze urodynamic parameters in these patient groups. Also, one could investigate the effect delaying bladder closure has on preservation of the upper tract. Still, the incidence of bladder exstrophy is low, making large cohorts, even from a referral center such at the authors’ difficult to obtain. Despite these potential limitations, investigation into the clinical outcomes of patients with poor bladder exstrophy templates is paramount for assessing management strategies.
Summary and guidelines for clinical decision making in cases of an inadequate bladder In summary, the inadequate bladder template can have impacts on surgical outcomes in CBE. DPC is the optimal option for patients with an inadequate bladder template, and leads to superb primary closure outcomes. When comparing DPC patients with and without inadequate bladders templates, the bladder growth rates remains similar. The inadequate bladder template group undergoes their first continence procedure at a later age and establishes continence relatively later when compared to patients with an adequate bladder template. This is likely because of an inherently smaller bladder. These patients require longer periods of monitoring bladder growth to determine the appropriate continence procedure in inadequate bladder patients and the time required for psychological readiness of continent catheterization for these patients.
Evaluation of patients for delayed primary bladder closure At the authors’ institution, the newborn with bladder exstrophy preferentially undergoes a primary closure within the first 48e72 h of life without osteotomy if the bladder template is a good size, the pubic diastasis is less than 4 cm, the pelvis is malleable under anesthesia, the infant’s overall health is acceptable, and scheduling permits [16]. If any of these factors are absent, strong consideration is given to performing the closure later with a bilateral anterior innominate and vertical iliac osteotomy. In rare cases, the bladder template is very small from slow growth of the infant (e.g., low birth weight or twin gestation), multiple polyps, or fibrosis of the bladder template. The fibrosis causes inelasticity that makes an adequate tension free primary closure extremely difficult. In such situations, DPC needs to be considered. If there is any doubt in the newborn period about the suitability of the bladder for closure, further assessment is made under anesthesia to allow a good appraisal of the bladder template and mobility of the pelvic bones when the child is relaxed. If the bladder is deemed to be unsuitable for closure secondary to the
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The inadequate bladder template presence of multiple hamartomatous polyps, the polyps are removed during the exam under anesthesia, and the bladder closure is delayed if the bladder template is small <3 cm in diameter or difficult to distend into the pelvis. . A bladder template is amendable for immediate closure in patients with a bladder >3 cm in diameter and in patients with a bladder that is easy to distend into the pelvis. Patients with polyps undergo an immediate bladder closure procedure after the polyps are removed, if the bladder is of an adequate size >3 cm and non-fibrotic [7].
Criteria for bladder neck reconstruction or bladder neck transection At the authors’ institution, the decision for patients to undergo either BNR or BNT is determined through a multitude of factors. BNR is recommended in patients who are maturationally ready (4e8 years of age) and who have a sufficient bladder capacity (85e100 mL). Furthermore, these patients are without contraindications such as a failed exstrophy closure or unsatisfactory urodynamic studies (i.e., poor compliance, elevated detrusor pressure, or low contractility) [14,17] If any contraindications are indicated, BNT with continent stoma and augmentation cystoplasty is considered as the first continence procedure.
Conclusions In the CBE child born with an inadequate bladder template, DPC is a reasonable management strategy. The surgeon should consider delaying the closure of inadequate bladders until the template becomes more amenable for reconstruction. Also, this study shows that select patients with poor bladder templates develop sufficient capacity to be continent and void per their urethra after successful BNR, but most are continent by catheterization. Patients with an inadequate bladder undergo a continence procedure at a later age and establish continence at a relatively later when compared to patients with an adequate bladder template at birth. Patients with an inadequate bladder template require longer periods of monitoring bladder growth to assess the appropriate continence procedure. As the majority of these inadequate bladder template patients require catheterization from a continent channel to establish continence, the later age of continence is further influenced by patients transitioning from volitional voiding to catheterizing from a continent channel.
Conflict of interest None.
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Acknowledgment The Kwok Family Foundation of Hong Kong supports the exstrophy database and laboratory research.
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Please cite this article in press as: Di Carlo HN, et al., The inadequate bladder template: Its effect on outcomes in classic bladder exstrophy, Journal of Pediatric Urology (2018), https://doi.org/10.1016/j.jpurol.2018.03.023