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.

Enhanced Recovery After Surgery for an Uncommon Complex Urological Procedure: The Complete Primary Repair of Bladder Exstrophy.

ERAS (enhanced recovery after surgery) protocols are designed to optimize perioperative care and expedite recovery. Historically, complete primary repair of bladder exstrophy has included postoperative recovery in the intensive care unit and extended length of stay. We hypothesized that instituting ERAS principles would benefit children undergoing complete primary repair of bladder exstrophy, decreasing length of stay. We describe implementation of a complete primary repair of bladder exstrophy-ERAS pathway at a single, freestanding children's hospital. A multidisciplinary team developed an ERAS pathway for complete primary repair of bladder exstrophy, which launched in June 2020 and included a new surgical approach that divided the lengthy procedure into 2 consecutive operative days. The complete primary repair of bladder exstrophy-ERAS pathway was continuously refined, and the final pathway went into effect in May 2021. Post-ERAS patient outcomes were compared with a pre-ERAS historical cohort (2013-2020). A total of 30 historical and 10 post-ERAS patients were included. All post-ERAS patients had immediate extubation (P = .04) and 90% received early feeding (P < .001). The median intensive care unit and overall length of stay decreased from 2.5 to 1 days (P = .005) and from 14.5 to 7.5 days (P < .001), respectively. After final pathway implementation, there was no intensive care unit use (n=4). Postoperatively, no ERAS patient required escalation of care, and there was no difference in emergency department visits or readmissions. Applying ERAS principles to complete primary repair of bladder exstrophy was associated with decreased variations in care, improved patient outcomes, and effective resource utilization. Although ERAS has typically been utilized for high-volume procedures, our study highlights that an enhanced recovery pathway is both feasible and adaptable to less common urological surgeries.

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.

V08-05 THE ANATOMIC BLADDER NECK TAILORING IN CPRE: THE PATH TO NORMAL ANATOMY

You have accessJournal of UrologyCME1 May 2022V08-05 THE ANATOMIC BLADDER NECK TAILORING IN CPRE: THE PATH TO NORMAL ANATOMY Dana Weiss, Elizabeth Roth, Travis Groth, John Kryger, Richard Lee, Aseem Shukla, Joseph G., Michael Mitchell, and Douglas Canning Dana WeissDana Weiss More articles by this author , Elizabeth RothElizabeth Roth More articles by this author , Travis GrothTravis Groth More articles by this author , John KrygerJohn Kryger More articles by this author , Richard LeeRichard Lee More articles by this author , Aseem ShuklaAseem Shukla More articles by this author , Joseph G.Joseph G. More articles by this author , Michael MitchellMichael Mitchell More articles by this author , and Douglas CanningDouglas Canning More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002606.05AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: The goals of the complete primary repair of bladder exstrophy include the anatomic closure of the bladder, bladder neck and urethra. During this reconstruction, the bladder, bladder neck and urethral complex are placed into the deep pelvis to allow for optimal function and successful closure. METHODS: Over time, the bladder neck reconstruction has evolved with the identification of the continence zone – the area between the verumontanum and the midpoint of the bladder neck in males, and between the anterior edge of the vaginal orifice and the midpoint of the bladder neck in females. The bladder neck reconstruction involves careful measurements and then tailoring of the proximal urethra, which allows for gentle outlet resistance without outlet obstruction. RESULTS: Among all bladder phenotypes, the anatomy of the bladder neck area is consistent. The length of the verumontanum to the bladder neck is equivalent to the length from the vaginal orifice to the bladder neck. The distance of this continence zone has been consistently between 1-1.5cm in both males and females. Starting a gradual taper from the verumontanum to the bladder neck allows for a natural, funneled shape once healed. CONCLUSIONS: Through attention to detail and constant re-evaluation of game film, we have identified a consistent section of the proximal urethra and bladder neck that we believe is the continence zone. This section is readily identifiable based on fixed landmarks in the urethra, at the verumontanum or anterior vaginal opening, and the midpoint of bladder neck striations. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e715 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Dana Weiss More articles by this author Elizabeth Roth More articles by this author Travis Groth More articles by this author John Kryger More articles by this author Richard Lee More articles by this author Aseem Shukla More articles by this author Joseph G. More articles by this author Michael Mitchell More articles by this author Douglas Canning More articles by this author Expand All Advertisement PDF DownloadLoading ...

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium

Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3h, 9 (13%) patients have dry intervals of 1-3h and 44 (61%) patients had dry intervals <1h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p=0.3). In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

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.

Impact of bilateral ureteral reimplantation at the time of complete primary repair of bladder exstrophy on reflux rates, renogram abnormalities and bladder capacity

Bilateral ureteral reimplantation at the time of the complete primary repair of bladder exstrophy (BUR-CPRE) has been proposed and has demonstrated favorable outcomes in the past. However, the potential benefits, including prevention of vesicoureteral reflux (VUR) and renal scarring must be tempered with any risks of reimplantation, persistent VUR, and the potential for overtreatment. We aimed to determine the impact of BUR-CPRE on reflux rates, renogram findings and bladder capacity. An IRB approved registry of children treated for bladder exstrophy epispadias complex (BEEC) during a long-term international collaboration hosted in a region with high prevalence of BEEC was queried. Children undergoing primary CPRE for bladder exstrophy (BE) were identified. Surgical procedure and outcome measures nuclear medicine dimercaptosuccinic acid (DMSA) scintigraphy scans, voiding cystourethrogram (VCUG), and urodynamic study (UDS) were assessed for presence and degree of VUR, renogram abnormalities, and bladder capacity. A total cohort of 147 patients with BEEC was queried; 52 children (37 males, 71%) underwent primary CPRE for BE between 2009 and 2019at median age of 1.1 years (IQR 0.6-1.9 years) with median follow up 4.4 years (IQR 2.4-6.4 years). BUR-CPRE was performed in 22/52 (42%). After BUR-CPRE, children were less likely to have VUR (any VUR present in 9 of 20 with imaging (45%) compared to 23 of 26 with imaging (82%) in the CPRE alone group (p=0.007)). VUR in the BUR-CPRE group tended to be unilateral and lower grade in comparison to the CPRE alone group. DMSA abnormalities were less common in the BUR-CPRE group (4/19 (21%) vs.12/27 (44%)), although the difference did not reach statistical significance (p=0.1). At 4 years follow-up, the BUR-CPRE group had a larger bladder capacity (p=0.016). After BUR-CPRE, children had a lower rate of VUR, and when present, VUR was more often unilateral and lower grade compared to the CPRE alone group. Fewer numbers of children in the BUR-CPRE group depicted DMSA abnormalities. No children developed obstruction after BUR-CPRE and none have undergone repeat reimplantation. We documented a larger bladder capacity at the time of maximum follow-up available (4 years)-but further data are needed to confirm this observation. BUR-CPRE decreases the incidence and severity of VUR after CPRE, but the clinical significance of this remains unclear. We are encouraged by these initial results, but since BUR-CPRE does not uniformly eliminate VUR, we continue to proceed carefully in the well selected patient.

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.

Continence after BNR in the complete repair of bladder exstrophy (CPRE): A single institution expanded experience

Several surgical methods have been used for primary repair of bladder exstrophy in the newborn. Complete primary repair of exstrophy (CPRE) aims to prevent the need for surgeries beyond the newborn period. Due to the rarity of bladder exstrophy, it has proven difficult in the past to analyze whether use of this method of closure truly does confer acceptable continence outcomes and hence minimizes the requirement for additional surgeries later in life. To describe the continence outcomes of CPRE patients who went on to receive bladder neck reconstruction (BNR), and secondarily, to compare clinical features between those patients who were able to receive undergo a BNR compared to those who were not. An IRB approved database of 1330 exstrophy-epispadias patients was used to identify referred patients after successful CPRE for management of continued urinary incontinence. Urinary continence outcomes were assessed in those who underwent modified Young Dees Leadbetter BNR following CPRE. Sixty-one patients were referred for treatment after successful CPRE between 1996 and 2016. None developed continence or a dry interval after primary closure. Of these, forty-two (68.9%) underwent BNR by a single surgeon at a mean age of 5.8 years (range 5-8.4). The mean bladder capacity at BNR was 147mL (range 102-210mL). Twenty-five (59.5%) achieved day and night continence, 7 (16.7%) gained daytime continence with nocturnal leakage, and 10 (23.8%) remain totally incontinent. Mean follow-up after BNR was 5.9 years. Combined CPRE and pelvic osteotomy were performed in 100% of patients who were continent and 75% of those who were daytime dry. No continent patient had a ureteral reimplantation before BNR, whereas 4 patients with daytime continence and nocturnal leakage and 7 patients who remained continuously incontinent did. This is the largest known series of BNRs in exstrophy patients closed by CPRE. Previous smaller studies have demonstrated mild to moderate success rates of BNR after CPRE, with many patients still requiring additional continence surgeries. The present study found similar results, with additional indication that successful primary closure and use of pelvic osteotomies may correlate with enhanced continence. This study includes outcomes from a single surgeon, with a maximum length of follow up of 13 years. CPRE alone often does not render patients continent of urine, based on the authors' referral population. However, following BNR continence rates in this subgroup were found to reach 76%. Surgeons who treat this population should keep these factors in mind when planning continence surgeries.

Re: Complete Primary Repair of Bladder Exstrophy: Critical Analysis of the Long-Term Outcome.

Re: Complete Primary Repair of Bladder Exstrophy: Critical Analysis of the Long-Term Outcome.

Penile Disassembly in Complete Primary Repair of Bladder Exstrophy: Time for Re-evaluation?

To explore a series of classic bladder exstrophy (CBE) cases referred to the authors' institution where primary closure with penile disassembly epispadias repair was complicated by penile injury. The penile disassembly technique is frequently combined with bladder closure in patients with CBE undergoing the complete primary repair of exstrophy (CPRE). Penile disassembly has been posited as a risk for penile injury by ischemic mechanisms. A prospectively-maintained institutional database of 1337 exstrophy-epispadias complex patients was reviewed for CPRE cases referred to the authors' institution, and those with injury to the penis were identified. The location, extent of injury, and subsequent management is reported. One hundred and thirteen male CBE patients were referred after prior CPRE. Twenty-six (20%) were identified with penile loss and reviewed. Eighty-one percent were closed in the neonatal period, and 54% had a pelvic osteotomy. Median follow-up time was 9.9 years (range 0.6-21.3). Of 26 patients with penile loss, 77% had unilateral loss and in 23% had bilateral loss involving the glans and/or one or both corpora cavernosa. Three patients were successfully managed with myocutaneous neophalloplasty. Complete penile disassembly during bladder exstrophy closure may lead to penile injury. This major complication questions the continued application of complete penile disassembly in the reconstruction of bladder exstrophy.

A single-institution experience of complete primary repair of bladder exstrophy in girls: risk factors for urinary retention

Historically after complete primary repair of exstrophy (CPRE) in girls, it had been more likely to observe urinary incontinence than retention. Following recent technical modifications of elongating the urethra and narrowing the bladder neck, the authors have observed a high rate of urinary retention in girls after CPRE. The aim was to identify factors that may be responsible for this observation by reviewing historical and current outcomes. The authors hypothesized that differences in anatomic dimensions at the time of CPRE may contribute to urinary retention. A retrospective review of girls who underwent CPRE from December 1998 through September 2016 from a single institution was performed. Patients were deemed in retention if their clinical course was consistent with such, required a procedure to relieve urinary retention, and/or required clean intermittent catheterization. Nineteen girls underwent CPRE during this period. In 2012, a change to delaying CPRE to approximately 2 months of age was made, and this led the authors to divide their experience into CPRE performed as a newborn (<72h of age, 8 patients) versus delayed (>72h, 11 patients) subgroups. There were no girls with retention in the newborn group and three (38%) girls with retention in the delayed group. In the delayed group, girls had a longer urethral plate and narrower bladder neck compared with the newborn group. Long-term outcomes greater than 9 years are available for six girls in the newborn group and two (33%) required bladder neck procedures for incontinence. None in the delayed group have required incontinence procedures; however, follow-up is limited at 25 months. The absence of retention in the newborn group is concerning for the delayed group incurring a higher risk of retention after CPRE. This may be secondary to excessive compression of the urethra at the time of pubic symphysis approximation potentially leading to urethral ischemia. Different from the newborn CPRE girls, additional technical revision of CPRE, namely, elongation of the urethra and the dissection it involvesand narrowing of the bladder neck, may increase the risk for retention. The multiple factors that were identified as potential contributors to post-CPRE urinary retention should result in a cautious reevaluation of female bladder exstrophy management at the time of CPRE. The authors now create a gradual tapered transition at the bladder neckand, similar to their previous experience, a more generous (wider) bladder neckand a shorter length for urethral plate.

Complete penile disassembly in epispadias repair.

To report current results of complete penile disassembly technique in epispadias repair. In ten years, we have preformed 31 complete penile disassembly for proximal epispadias repair. Twenty-four patients had epispadias after primary repair of bladder exstrophy and 7 isolated penopubic epispadias. The age of the patients ranged from 10months to 6years (median 3years). The shortening of urethral plate was found in 30 patients (97% of cases), and this shortening varied between 6 and 16mm. However, in one patient we found a lengthening of the urethral plate of 8mm. The narrowing of urethral plate was found in all patients, and this narrowing varied between 30 and 50% of the width of the plate. Postoperative complications encountered in our patients were dominated by fistulas and dehiscence, particularly in patients who had bladder exstrophy-epispadias complex. After dehiscence and fistulas repair, the cosmetic results were satisfying in 25 patients (80.5% of cases) with conical glans and meatus in orthotopic position without any necrosis of the glans. However, the urinary continence ≥ 1h was observed in 6 patients (19% of cases) and only 3 patients (9.7% of cases) had a urinary continence ≥ 3h. The mean follow-up was 61 months. The complete penile disassembly remains one of the best techniques for epispadias repair. However, we noticed a reappearance of the dorsal curvature of the penis in a large number of patients treated for isolated epispadias and the impact of this technique on urinary incontinence remains uncertain.

Commentary to 'Modified staged repair of bladder exstrophy: a strategy to prevent penile ischemia while maintaining advantage of the complete primary repair of bladder exstrophy'

Commentary to 'Modified staged repair of bladder exstrophy: a strategy to prevent penile ischemia while maintaining advantage of the complete primary repair of bladder exstrophy'

Modified staged repair of bladder exstrophy: a strategy to prevent penile ischemia while maintaining advantage of the complete primary repair of bladder exstrophy

Penile ischemic injury is a reported catastrophic complication after complete primary repair of exstrophy (CPRE). Aiming to improve the bladder exstrophy-epispadias repair outcomes, the study institution adopted a modified staged exstrophy repair to incorporate the advantages of CPRE by avoiding concurrent epispadias repair and adding bilateral ureteral re-implantation and bladder neck tailoring (staged repair of bladder exstrophy with bilateral ureteral re-implantation [SRBE-BUR]) at the initial repair. It was hypothesized that such modifications minimize penile complications and prevent upper tract deterioration while enhancing bladder resistance and consequent capacity. Here, a comparative series of outcomes between CPRE and SRBE-BUR is reported. A retrospective cohort study including all exstrophy-epispadias male neonates managed in the study institution from January 2000 to December 2014 was performed. Patients were divided into those who underwent CPRE-BUR (group 1) and SRBE-BUR (group 2) (Figure). Baseline characteristics, peri-operative data, and long-term surgical outcomes were collected and analyzed for between-group comparison. Fisher exact and Mann-Whitney U tests were performed for statistical analysis. A total of 21 eligible patients were included: 10 in group 1 and 11 in group 2. Baseline characteristics were comparable. Two patients in group 1 had intra-operative penile ischemic injury (one with subsequent penile tissue loss), whereas none of the group 2 patients had intra-operative complications. No significant difference between the groups was noted for operative time; however, significantly lesser blood loss was noted in group 2. Comparable long-term surgical outcomes such as additional surgical intervention, urinary continence, bladder capacity, vesicoureteral reflux, hydronephrosis and recurrent urinary tract infections (UTIs) were noted. In addition, although subjective, better penile length and cosmesis were achieved by staging the repair (Figure). The SRBE with bilateral ureteral re-implantation is a safe alternative for the repair of the exstrophy-epispadias repair as it prevents the catastrophic complication of penile tissue loss, while having comparable long-term outcomes with the CPRE. Delaying epispadias repair avoids penile injury besides possible improvement of its overall cosmesis.

Is there a shortening of the urethral plate in complete penile disassembly used in epispadias repair, and what is its impact on the final outcomes?

PurposeTo assess the importance of shortening of the urethral plate that occurred with complete penile disassembly technique in epispadias repair and its impact on cosmetic and functional results (on urinary incontinence). MethodsFrom January 2009 to December 2016, 26 boys underwent complete penile disassembly technique for proximal epispadias repair. Twenty-one patients had epispadias after primary repair of bladder exstrophy, and 5 patients had isolated penopubic epispadias. The age of the patients ranged from 11 months to 6 years (median 3 years). ResultsAfter disassembling the penis in three parts, the shortening and narrowing of urethral plate were found in all patients; the shortening varied between 2 and 16 mm. However, in isolated epispadias, the urethral plate is easily extensible. The cosmetic results (after dehiscence and fistulas repair) were found to be satisfactory in 24 patients with conical glans and meatus in the orthotopic position without any necrosis of the glans. However, 18 patients (81.8% of cases) who initially had a bladder exstrophy presented a dehiscence or fistula. The urinary continence ≥ 1 h was observed in 5 patients (19% of cases), and only 3 patients (11.5% of cases) had a urinary continence ≥ 3 h. ConclusionsThe complete penile disassembly procedure restores the normal anatomy of the penis. Despite the shortening and narrowing of the urethral plate, the cosmetic results were good in the majority of patients. However, its functional outcomes on urinary incontinence, particularly for epispadias with bladder exstrophy, remain uncertain.