Complete Primary Repair Of Bladder Exstrophy Research Articles

Long term evaluation of continence after complete primary bladder exstrophy repair

Continence after bladder exstrophy (BE) repair remains a major debatable challenge to pediatric urologists, together with the lack of standard definitions and long-term results in large series. We assessed the long-term urinary continence in 142 toilet-trained cases after one (1-) stage of complete primary repair of bladder exstrophy (CPRE) and consequent procedures to achieve this goal in a single tertiary referral center. The current retrospective study included 123 boys and 19 girls with BE that were repaired by (1-) stage CPRE. The Mean age at (BE) repair was 9.5±2.6 weeks. Complete penile disassembly (CPD) was used for epispadias repair in 42 (34.1%) and modified Cantwell-Ransley repair (MCR) was used in 81 (65.9%) boys. Bilateral anterior transverse innominate osteotomies (ATIO) were applied in all. Urinary continence was expressed in terms of the dry interval (DI). Continence procedures were afforded if CPRE failed to achieve DI≥3h (hrs.), those were in the form of endoscopic bladder neck injection (BNI), bladder neck reconstruction (BNR), and bladder neck closure (BNC) with catheterizable stoma. The mean age at follow up was 12.1±5.2 years. DI≥3h was gained in 23 (16.2%) after CPRE alone, while complementary post-CPRE continence procedures were required to reach this goal in the remaining patients. Deflux injection was reported in 10 (7%), CIC in 8 (5.6%), BNR in 32 (22.5), and BNC with catheterizable stoma alone in 37 (26.1%), or with Charleston pouch in 32 (22.5%). We think that ≥3h DI with voiding represents an appropriate definition of continence after BE repair. According to the results in the current series, we think that successful anatomical closure of BE is achievable, but the functional outcome in terms of continence and its evaluation is tricky. Results of continence were reported to change with age of the child, and it is difficult to evaluate both before toilet training age and long-term follow up. Long-term follow up of CPRE with bilateral ATIO alone or with BNI results in ≥3h DI in a few cases; BNR after CPRE can provide a good chance for continence; otherwise, BNC with catheterizable stoma is a valid option.

Early urodynamic findings after complete primary repair of exstrophy

Detrusor contraction in bladder exstrophy (BE) patients following reconstruction is poorly understood as there are few published studies assessing urodynamic findings in this population. Understanding the ability of the detrusor to contract in BE patients early after closure may be able to inform the longer-term management and potential for the development of future continence in this population. We sought to evaluate early detrusor contraction using urodynamic studies (UDS) in children who had previously undergone complete primary repair of bladder exstrophy (CPRE). We hypothesized that a majority of children with BE would display the presence of normal detrusor contractile function after CPRE. A retrospective review of our prospectively collected database was performed for all patients with a diagnosis of classic BE who underwent primary CPRE between 2013 and 2017. From this cohort we identified patients with at least one post-operative UDS at 3 years of age or older who had undergone an initial CPRE. Our primary outcome was the presence of a detrusor contraction demonstrated on UDS. There were 50 children (31 male, 19 female) with CBE who underwent CPRE between 2013 and 2017.There were 26 (13 male, 13 female) who met inclusion criteria. Median age was 3.5 (IQR: 3.2-4.7) years at the time of UDS Sixteen of the 26 (61.5%) generated a sustained detrusor contraction generating a void, with a median peak voiding pressure of 38cm H20 (IQR: 28-51). The median bladder capacity reached was 48ml, which represented a median of 30% of expected bladder capacity. The median post void residual (PVR) for the entire cohort was 26ml (IQR: 9, 47) or 51% (IQR: 20%-98%) of their actual bladder capacity, while the median PVR for those children with a sustained detrusor contraction was 18ml (IQR: 5, 46) or 33% (IQR: 27%, 98%) of their actual bladder capacity. Intraoperative bladder width and bladder dome to bladder neck length did not correlate with the presence of voiding via a detrusor contraction (p=0.64). We present the first study assessing early UDS finding of detrusor contraction in BE patients after CPRE. In our cohort, 61.5% of patients were able to generate a sustained detrusor contraction on UDS which is a higher percentage than has been reported in previous series. A difference in initial surgical management may account for these findings. At short term follow up, the majority of children in our cohort were able to produce sustained detrusor contractions sufficient to generate a void per urethra with a modest post void residual volume. Long-term follow-up and repeated UDS will be needed to track detrusor contractility rates, bladder capacities, compliance, post void residuals and ultimately continence rates over time.

V04-01 NOVEL UTILIZATION OF INDOCYANINE GREEN TO ASSESS BLADDER PLATE AND PENILE PERFUSION DURING BLADDER EXSTROPHY CLOSURE

You have accessJournal of UrologyCME1 Apr 2023V04-01 NOVEL UTILIZATION OF INDOCYANINE GREEN TO ASSESS BLADDER PLATE AND PENILE PERFUSION DURING BLADDER EXSTROPHY CLOSURE Amanda Raines, Nicolas Fernandez, Jennifer Ahn, Margarett Shnorhavorian, Hailey Silverii, and Paul Merguerian Amanda RainesAmanda Raines More articles by this author , Nicolas FernandezNicolas Fernandez More articles by this author , Jennifer AhnJennifer Ahn More articles by this author , Margarett ShnorhavorianMargarett Shnorhavorian More articles by this author , Hailey SilveriiHailey Silverii More articles by this author , and Paul MerguerianPaul Merguerian More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003252.01AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Reconstruction of bladder exstrophy poses a unique challenge to urologic surgeons. At our institution the current practice is to perform complete primary repair of bladder exstrophy (CPRE). However, there have been concerns about compromise of penile perfusion using this technique with reports of penile or glans loss due to ischemia. Currently, one method of penile perfusion assessment after closure is completed is a needlestick to the glans to observe for bleeding, however, this provides limited and subjective information. Indocyanine green or ICG is a fluorescent dye which has been in use since the 1950s. With a short half-life and favorable safety profile, it is ideal for intraoperative use. It has been widely used throughout the surgical community to assess tissue perfusion, but its use within pediatric urology has been limited and not standardized. To our knowledge, ICG use in bladder exstrophy has not been previously described. We aim to describe our approach to CPRE with use of ICG and detail our intraoperative findings. METHODS: An otherwise healthy four month old male with classic bladder exstrophy was taken to the operating for bladder closure with CPRE. ICG was given in three doses first after complete penile degloving, next after complete mobilization of the corpora and take down of intersymphyseal bands, and finally after osteotomy and pubic symphysis approximation. Quantification software is used to measure ICG uptake at each stage. RESULTS: ICG uptake in the glans remained symmetric and around 100% at the time of the first and second doses. However, uptake on the third dose was significantly decreased and asymmetric with the right glans having an uptake of 20-30% and the left 40-50%. CONCLUSIONS: The current standard of penile perfusion assessment with a needle stick to the glans is subjective and limited in the detail it provides. More updated methods like ICG can generate an objective quantitative and more detailed assessment which could be useful in complex reconstructions such as bladder exstrophy closure. While more data is currently needed than this single experience, future applications of ICG may alter surgical decision making such as loosening of the symphyseal sutures to improve penile perfusion. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e338 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Amanda Raines More articles by this author Nicolas Fernandez More articles by this author Jennifer Ahn More articles by this author Margarett Shnorhavorian More articles by this author Hailey Silverii More articles by this author Paul Merguerian More articles by this author Expand All Advertisement PDF downloadLoading ...

Long term renal outcome and risk of elevated blood pressure in children undergoing complete primary repair of bladder exstrophy (CPRE)

Since the initiation of staged reconstruction for bladder exstrophy (BE), hypertension has been a known complication of the procedure. Hypertension is a well-established risk factor for chronic kidney disease (CKD) progression and associated with cardiovascular/cerebrovascular morbidity and mortality. Few studies exist evaluating the risk of developing hypertension among patients with bladder exstrophy who underwent CPRE. We hypothesized that long-term blood pressure levels may be elevated in males vs females, and may be correlated with presence of hydronephrosis, bladder neck reconstruction, or continence status. We sought to revisit our long-term experience with CPRE and determine factors associated with incidence of elevated blood pressures. We reviewed all BE patients undergoing CPRE at our institution from 1999 to 2019. Patients were considered eligible for inclusion if last renal ultrasound was obtained at least 5 years after repair. Upper tract outcomes based on imaging, history of pyelonephritis and renal function tests measured by serum creatinine and estimated glomerular filtration rate (eGFR, Schwartz formula) were reviewed. Systolic/diastolic blood pressures (SBP/DBP) from all encounters were captured. All blood pressure values were age adjusted by percentile. A total of 36 patients were considered eligible for review. Median follow-up of this cohort was 10.01 (5.16-21.47) years. The mean creatinine for the patients available was 0.58mg/dL (SD=0.20), at mean age of 8.90 years Neither SBP or DBP were significantly elevated in males vs females, but had lower odds of elevation >90th percentile for those with higher eGFR, lower renal length, and reimplantation. Pyelonephritis incidence was 38% (n = 14) with first episode at mean age of 8.8 years, and mean of 3.7 episodes per patient. At long term follow up, blood pressures following CPRE were not significantly elevated, despite the relatively frequent occurrence of CKD, and hydronephrosis. Male gender does appear to suggest higher risk for long-term deterioration in this regard. Higher eGFR, higher renal length, and presence of ureteral reimplantation were associated with lower likelihood of systolic/diastolic blood pressure elevation. Continence status and bladder neck reconstruction were not associated with likelihood of blood pressure elevation. Blood pressure and upper-tract outcomes for patients undergoing CPRE at birth are positive for the majority of patients. To avoid complications from hypertension, patients should be closely evaluated as the risks associated with elevated blood pressure are significant. Ultimately, larger-scale prospective and multi-institutional studies are further needed to characterize risks of hypertension in this complex patient population.

Repurposing tools: A peña stimulator as a tool to map the limits of dissection and continence zone during bladder exstrophy repair

The complete primary repair of bladder exstrophy (CPRE) aims to restore normal anatomy through complete mobilization and reapproximation of the bladder neck and proximal urethra. The Peña stimulator has previously been used to identify musculature in the pelvis. The device is now used to distinguish intersymphyseal bands from pelvic floor and urethral sphincteric musculature during CPRE. The ability to distinguish the levator ani muscles from fibrotic bands assists the surgeon in identifying the appropriate extent of deep pelvic dissection during CPRE. During the anatomic bladder neck recreation, the muscle stimulator demonstrated the striated muscle at the level of the bladder neck and urethral sphincter. Localization of the muscles at the bladder neck and proximal urethra corroborates the concept that pelvic floor physical therapy could enhance the strength of the pelvic floor in children with bladder exstrophy.

Bilateral ureteral reimplantation at complete primary repair of exstrophy: Post-operative outcomes

The value of bilateral ureteral reimplant (BUR) at the time of complete primary repair of bladder exstrophy (CPRE) has been suggested, however, outcomes are poorly characterized in current medical literature. We hypothesize that BUR at time of CPRE will decrease the rate of recurrent pyelonephritis, post-operative vesicoureteral reflux (VUR), and the need for subsequent ureteral surgery. We analyzed 64 consecutive patients with a diagnosis of classic bladder exstrophy (BE) who underwent CPRE at three institutions from 2013 to 2019.15 patients underwent cephalotrigonal BUR-CPRE and 49 patients underwent CPRE alone. Our primary outcome was >1 episode of pyelonephritis as documented in the medical record. Secondary outcomes were persistent vesicoureteral reflux (VUR), with a sub-analysis of number of refluxing renal units and presence of dilating VUR, and the need for subsequent ureteral surgery. Descriptive statistics in addition to standard, two tailed univariate statistics, were used to compare the groups where appropriate. BUR-CPRE was associated with a significant decrease in the rates of post-operative VUR, number of refluxing renal units, and need for subsequent ureteral surgery (p=0.002, p=0.001, and p=0.048 respectively). There was a reduction in the rates of recurrent pyelonephritis and dilating reflux in patients undergoing BUR-CPRE, though it did not reach significance. Female gender was significantly associated with recurrent pyelonephritis regardless of BUR-CPRE status (p=0.005). There were no reports of distal ureteral obstruction or other complications following BUR-CPRE. The mean post-operative follow up for the BUR-CPRE group was 46.33 (10.26) months vs. 53.76 (26.05) months for CPRE (p=0.11). Recurrent pyelonephritis following bladder closure in patients with BE is a well-documented surgical complication, with centers performing CPRE reporting rates of post-operative pyelonephritis from 22 to 50%. Our series demonstrates similar efficacy of BUR-CPRE compared to other contemporary series and provides additional detail about need for subsequent ureteral surgeries and increased long term follow-up of these complex patients. Limitations of the study include male predominance of the cohort and lack of randomization of BUR-CPRE. BUR-CPRE decreases postoperative VUR and the need for additional ureteral surgery in select BE patients; it should be considered when technically feasible. While results continue to suggest a trend toward decreased recurrent pyelonephritis and dilating reflux, further longitudinal follow-up in our cohort will be needed.

Timing of inguinal hernia following complete primary repair of bladder exstrophy

Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood. The primary objective of this study was to assess the timing of inguinal hernia following complete primary repair of bladder exstrophy (CPRE). In addition, we aimed to evaluate possible risk factors associated with inguinal hernia, including sex, age at bladder closure and iliac osteotomy status. A multi-institutional retrospective review identified patients with bladder exstrophy repaired by CPRE under 6 months of age while excluding those who underwent inguinal hernia repair before or during bladder closure. Timing of inguinal hernia following bladder closure was evaluated using Kaplan-Meier methods. Cox proportional hazards model was used to investigate association of sex, age at bladder closure, and osteotomy on the risk of developing of inguinal hernia while clustering for institution. 91 subjects were included in our analysis with median follow-up time of 6.5 years. 34 of 53 males (64.2%) and 2 of 38 females (5.3%) underwent inguinal hernia repair. The median time to inguinal hernia was 4.7 months following closure. The greatest hazard of inguinal hernia was within the first six months following closure. In multivariate analysis, male sex was strongly associated with inguinal hernia (HR=19.00, p=0.0038). Osteotomy and delay in closure were not significantly associated with inguinal hernia. 7 of 36 patients (19.4%) who underwent inguinal hernia repair presented with recurrence on the ipsilateral side. Our results suggest that the greatest risk of inguinal hernia is within the first six months following bladder closure. The decreased risk of inguinal hernia after one year of follow-up may reflect anatomic stability that is reached following major reconstruction of the pelvis. While male bladder exstrophy patients are significantly more susceptible to inguinal hernias following CPRE, osteotomy and delayed bladder closure do not appear to be protective factors for inguinal hernia development following initial bladder closure. There is a heightened risk of inguinal hernia in the first six months following closure. The rate of recurrence following inguinal hernia repair is significantly elevated compared to the general pediatric population.

Evaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model

The Multi-Institutional Bladder Exstrophy Consortium (MIBEC) was established in 2013 to refine technical aspects of the complete primary repair of bladder exstrophy (CPRE), to decrease complications, and to improve outcomes. In order to place outcomes from the consortium into context of historic outcomes, we evaluated continence and dry intervals in children who were repaired prior to the beginning of the consortium at these institutions. We hypothesized that continence (voiding with dryness) is rarely achieved after primary CPRE and surgery following CPRE improves dryness but may hinder voiding per urethra. We reviewed prospectively maintained IRB approved databases of children who underwent CPRE for classic bladder exstrophy (BE) between 5/1993 and 1/2013at 3 institutions. Exclusion criteria included: lack of continence documentation, and lack of follow up subsequent to January 2014. We recorded age at closure, method of bladder emptying, bladder capacity and surgical history. We used a 3 part dryness scale for both patients who void volitionally and those on CIC. Children were considered dry if they could hold urine for over 3h. An intermediate group was defined as having a dry interval of 2-3h, with minimal dampness in between voids. If dry intervals were <2h with frequent leakage, children were considered wet. A total of 54 of 73 (38M) children met inclusion criteria. 35 of 54 (64.8%) children void per urethra, while 18 (33.3%) perform clean intermittent catheterization (CIC) and 1 underwent a vesicostomy due to incomplete emptying and UTI's. 25/35 (71.4%) of those voiding per urethra underwent CPRE only, while the remaining 10 underwent secondary continence procedures. In total, 26 of 54 (48.1%) are dry, that is either continent or not wet for>3h, while only 11/54 (20.4%) are truly continent, i.e., voiding per urethra. Only 9/54 (16.7%) are continent after a single surgery (CPRE). 14/54 children who are dry (25.9%) underwent a bladder neck procedure with or without augmentation and empty with CIC. Granular detail about the specifics of emptying, surgical history, and dry intervals is crucial to understand the true outcomes from the repair of BE. Children with BE undergoing CPRE prior to the institution of MIBEC experienced variable results, with only 17% achieving continence while spontaneously voiding per urethra without additional reconstruction. We are now engaged in MIBEC to identify factors that contribute to continence and to attempt to render such findings reproducible.

Complete Primary Repair of Bladder Exstrophy: Critical Analysis of the Long-term Outcome

To demonstrate the long-term outcome of a contemporary series of 64 children who underwent complete primary repair of bladder exstrophy (CPRE) in a single tertiary referral center. Between 1998 and 2012, 64 children, 47 boys and 17 girls, were identified. Only 60 of the 64 cases were available for follow-up. The follow-up was done by renal bladder ultrasound and serum creatinine every 3 months and voiding cystourethrogram from 6 to 12 months postoperatively. Continence was defined as dryness ≥3 hours. Median (range) follow-up is 14 years (from 5 to 19 years). Voided continence was achieved in 14 children (23%) after CPRE only. Additionally, 6 children were continent after bladder neck reconstruction (BNR) and 2 after bladder neck injection (BNI), raising the percentage of voided continence to 36%. The remaining 38 (64%) patients were using clean intermittent catheterization. All cases were continent at last assessment. The results of BNR or BNI were better in de novo than in redo cases (P <.05). The percentage of cases that needed augmentation ileocystoplasty in combination with multiple bladder neck procedures was lower in both female and de novo cases (P <.05). The percentage of children with classic bladder exstrophy who underwent CPRE who will achieve continence with volitional voiding via the urethra is 36%. The continence results after BNR and BNI are better in de novo cases than in redo ones. Continence in female and de novo cases is more likely to be achieved with lower number of continence procedures.

Long-term fate of the upper tracts following complete primary repair of bladder exstrophy

Complete primary repair of bladder exstrophy (CPRE) is widely used for classic bladder exstrophy (CBE) closure. Long-term renal function with or without dilation in these patients is poorly characterized and may be impacted by bladder storage parameters or recurrent urinary tract infections (UTIs). We sought to assess our long-term experience with CPRE and investigate factors that may influence upper-tract deterioration. A retrospective review of patients at our institution with CBE undergoing CPRE from 1990 to 2015 was performed. Patients were considered included if age at the last renal ultrasound was at least 5 years. Renal imaging and renal function were reviewed. The Society of Fetal Urology (SFU) and Upper Tract Dilation (UTD) grades were retrospectively assigned to all available ultrasounds with hydronephrosis (HN). Additionally, outcomes related to vesicoureteral reflux and lower urinary tract function were assessed. Descriptive and comparative statistical analyses were performed to assess factors influencing HN and renal function at follow-up. Thirty patients (57% male) had a median follow-up of 9.7 (3.9-22.3) years. The table shows the HN status and grade for the entire group, stratified by gender, continence, and surgical reconstruction in. The mean creatinine was 0.50mg/dL (0.2-1.0) and the mean estimated glomerular filtration rate (eGFR) was 106.8mL/min/1.73m2. No patient had greater than stage 2 chronic kidney disease. Male gender was associated with worse renal outcomes, including overall rate of HN (p<0.001), severity of HN (p=0.004) and worse eGFR (p=0.05). Lower tract reconstruction, urodynamic parameters, and continence were not associated with differences in upper-tract outcomes. Ureteral reimplantation was performed in 22 patients (73%) at a mean age of 22 months for indications of persistent VUR (10), worsening HN (1), or recurrent UTI (11). HN is common following CPRE, although severe HN is seen infrequently. Overall long-term renal outcomes are similar to those of other techniques presented in the literature. However, male gender portends a higher risk for long-term upper-tract deterioration as measured by HN and eGFR. Ureteral reimplantation and the status of the lower urinary tract were not associated with differences in upper-tract outcomes. Prospective, standardized approaches to characterize upper-tract outcomes are needed to follow children with bladder exstrophy into adulthood. We believe higher-risk patients should be assessed early and managed more aggressively with attention paid towards accurately assessing renal function and lower tract anatomy.

Commentary to “Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration”

Summary Introduction/background Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. Objective Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term. Study design Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1–3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures. Results From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0–12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1–51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below. Discussion Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence. Conclusion This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing. Summary Table . Diagnosis, timing of CPRE and complications per gender. Gender Girls Boys Classic BE/epispadias 10/3 12/2 Median age at CPRE (range) 1.9 months (0.1–51.6) 2.9 months (0.4–28.8) Successes No dehiscence 7 without complications No dehiscence 11 without complication Complications 6 girls total 5 pyelonephritis 4 urinary retention (BOO) 2 temporary CIC 1 vesicostomy 1 bladder rupture 3 boys total 1 pyelonephritis 2 urethrocutaneous fistula

Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration

Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term. Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures. From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below. Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence. This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.

Complete Primary Repair of Bladder Exstrophy Is Associated With Detrusor Underactivity Type of Neurogenic Bladder

To test the hypothesis that complete primary repair of bladder exstrophy (CPRE) is associated with detrussor underactivity. For this purpose, we review (1) our experience, (2) the results of the published literature as it pertains to bladder function, and (3) the known anatomic basis on which the mechanism of the observed outcome can be understood. The medical records of all patients who underwent CPRE by the author between 2004 and 2010 were reviewed. Attention was focused on the clinical, imaging, and urodynamic findings. Four men and 2 women underwent CPRE. Follow-up ranges from 2 to 8 years. Four underwent bilateral ureteral reimplantation combined with bladder neck repair in 3. Detrusor activity (or overactivity) was not recorded in the 5 patients who underwent urodynamic studies. Four patients achieved short periods of urinary continence. The percent predicted bladder capacity, adjusted for age, ranged from 25 to 70, with a median of 60. Of the 68 publications on CPRE since 1999, none reports the presence of detrusor activity. A description of the pelvic plexus anatomy by Walsh and Donker provides a basis for the mechanism of injury resulting in the previously mentioned results: (1) complete penile disassembly eliminates the distal fixation point of the bladder-urethral plate, (2) the subsequent dissection and mobilization result in shearing injury to the microscopic pelvic plexus branches to the bladder, external sphincter, and prostatic urethra. CPRE results in disruption of the branches of the pelvic plexus and a neurogenic bladder (detrussor underactivity).

Does Bilateral Ureteral Reimplantation at the Time of Primary Bladder Exstrophy Closure (CPRE-BUR) Protect the Upper Tracts?

Purpose Recurrent febrile urinary tract infections (UTI) have been reported following complete primary repair of bladder exstrophy (CPRE), probably due to the association of vesico-ureteral reflux (VUR) and increased outlet resistance provided by CPRE. We compared outcomes of children who underwent CPRE-BUR to CPRE alone. Material and Methods CPRE-BUR was performed using a cephalo-trigonal technique in 13 pts with favorable bladder (smooth/elastic plate, group I) and compared to 23 pts who had CPRE alone (group II). Post-op assessment included ultrasound and voiding cystogram in all pts. Outcome measurements included: post-op hydronephrosis, febrile UTIs and presence of VUR. Results Mean follow-up was 21 m (6-42) and 58 m (11-105) for groups I and II, respectively. Median age at surgery was 3 days for both, ranging from 1 to 140 days. There were 9 boys and 4 girls in group I and 10 boys and 13 girls in group II. Outcomes are shown in the table below. There were no complications related to reimplantation. Variables CPRE-BUR CPRE n = 13 (%) n = 23(%) p value Post-op hydronephrosis 2 (15) ∗ 10 (43) 0.28 Post-op febrile UTIs 1 (8) ∗∗ 11 (48) 0.07 Vesico-ureteral reflux 0 (0) 17 (74) 0.004 ∗ Mild post-op hydronephrosis, resolved spontaneously. ∗∗ Febrile UTI after voiding cystogram. Conclusions CPRE-BUR can be safely performed in newborns with bladder exstrophy. It may prevent renal damage by early correction of VUR, and appears to reduce subsequent recurrent febrile UTIs.

GENDER-BASED OUTCOME ANALYSIS AFTER COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY (CPRE)

You have accessJournal of Urology1 Apr 2008GENDER-BASED OUTCOME ANALYSIS AFTER COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY (CPRE) Luis H Braga, Armando J Lorenzo, Ricky Jrearz, Darius J Bagli, Walid A Farhat, Joao L Pippi Salle, and Antoine E Khoury Luis H BragaLuis H Braga More articles by this author , Armando J LorenzoArmando J Lorenzo More articles by this author , Ricky JrearzRicky Jrearz More articles by this author , Darius J BagliDarius J Bagli More articles by this author , Walid A FarhatWalid A Farhat More articles by this author , Joao L Pippi SalleJoao L Pippi Salle More articles by this author , and Antoine E KhouryAntoine E Khoury More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(08)60278-5AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "GENDER-BASED OUTCOME ANALYSIS AFTER COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY (CPRE)." The Journal of Urology, 179(4S), p. 95 © 2008 by American Urological AssociationFiguresReferencesRelatedDetailsCited byBraga L, Lorenzo A, Jrearz R, Bagli D and Salle J (2010) Bilateral Ureteral Reimplantation at Primary Bladder Exstrophy ClosureJournal of Urology, VOL. 183, NO. 6, (2337-2341), Online publication date: 1-Jun-2010. Volume 179Issue 4SApril 2008Page: 95 Advertisement Copyright & Permissions© 2008 by American Urological AssociationMetricsAuthor Information Luis H Braga More articles by this author Armando J Lorenzo More articles by this author Ricky Jrearz More articles by this author Darius J Bagli More articles by this author Walid A Farhat More articles by this author Joao L Pippi Salle More articles by this author Antoine E Khoury More articles by this author Expand All Advertisement PDF downloadLoading ...

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

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

BLADDER GROWTH AND DEVELOPMENT AFTER COMPLETE PRIMARY REPAIR OF BLADDER EXSTROPHY IN THE NEWBORN WITH COMPARISON TO STAGED APPROACH

We assessed bladder growth and dynamics following complete primary repair of bladder exstrophy (CPRE) compared to the staged approach. We reviewed the records of 16 boys and 7 girls who underwent CPRE within 3 days of life from 1996 to 2004 and compared them to the records of 8 boys and 6 girls treated with a staged repair from 1979 to 1996. Screening methods included voiding cystourethrogram, radionuclide cystogram and urodynamic study. We estimated growth curves for bladder capacity following repair in each group, and compared percent predicted bladder capacity (PPBC), compliance and detrusor overactivity between the CPRE and staged repair groups following bladder neck reconstruction. Bladder capacity in the staged repair group was 69.8 ml (95% CI 46.7-104.4) immediately after bladder neck reconstruction and increased by 15.0% per year thereafter (95% CI 6.2-24.5, p = 0.002). In the CPRE group bladder capacity was 29.0 ml (95% CI 21.3-39.5) initially and increased by 28.9% per year thereafter (95% CI 17.4-41.5, p <0.001). PPBC started at 45.6% (95% CI 35.7-55.5) and increased 1.2% per year (95% CI -1.1-3.5, p = 0.29) following repair for all genders and surgery groups. Compliance was 124.4% (95% CI 22.6-310.7, p = 0.01) greater in the CPRE group at all times following repair. Detrusor overactivity was present in 0 of 19 patients in the CPRE group and 6 of 13 (46%) in the staged group (exact p = 0.002). Within the CPRE group bladder stability was universal, and sphincter electromyography was normal suggesting no neuromuscular compromise of the pelvic floor. At early followup, our results suggest that PPBC is equivalent irrespective of gender or management. Further objective evaluation is needed in both groups.

COMPLETE REPAIR OF BLADDER EXSTROPHY: MANAGEMENT OF RESULTANT HYPOSPADIAS

In our hands complete primary repair (CPR) of bladder exstrophy results in hypospadias in two-thirds of boys. To our knowledge hypospadias repair following CPR of bladder exstrophy has not been reported previously. We report our experience with hypospadias repair following CPR of bladder exstrophy. A total of 22 boys with bladder exstrophy underwent CPR using Mitchell's technique between November 1998 and January 2002. The procedure resulted in hypospadias in 15 boys (68%). The site of the meatus was distal penile in 5 patients, mid penile in 3 and proximal penile in 7. Hypospadias repair was performed in all 15 boys. The 5 patients with distal penile hypospadias underwent repair consisting of Thiersch-Duplay urethroplasty with dorsal incision in 3 and tubularized vertical island flap with glanular tunnel in 2. All patients with mid (3) or proximal (7) penile hypospadias underwent Mustarde repair with glanular tunnel. Followup ranged from 6 to 18 months (median 13). The Thiersch-Duplay procedure with dorsal incision resulted in fistula/stenosis in 2 of 3 boys. None of the remaining 12 boys with tubularized penile flap urethroplasty (tubularized vertical island flap or Mustarde) with glanular tunneling had development of fistula or stenosis. Complete degloving of the penis and penile skin redistribution were not necessary with the Mustarde technique. Thiersch-Duplay urethroplasty with dorsal incision does not seem to be a good option for hypospadias repair following CPR of bladder exstrophy. The use of penile skin as a tubularized flap with glanular tunneling seems to yield excellent functional/cosmetic results.