Complete Primary Repair Of Exstrophy Research Articles

Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes.

Bladder exstrophy (BE) poses challenges both during the surgical repair and throughout follow-up. In 2013, a multi-institutional BE consortium was initiated, which included utilization of unified surgical principles for the complete primary repair of exstrophy (CPRE), real-time coaching, ongoing video capture and review of video footage, prospective data collection, and routine patient data analysis, with the goal of optimizing the surgical procedure to minimize devastating complications such as glans ischemia and bladder dehiscence while maximizing the rate of volitional voiding with continence and long-term protection of the upper tracts. This study reports on our short-term complications and intermediate-term continence outcomes. A single prospective database for all patients undergoing surgery with a BE epispadias complex diagnosis at 3 institutions since February 2013 was used. For this study, data for children with a diagnosis of classic BE who underwent primary CPRE from February 2013 to February 2021 were collected. Data recorded included sex, age at CPRE, adjunct surgeries including ureteral reimplantations and hernia repairs at the time of CPRE, osteotomies, and immobilization techniques, and subsequent surgeries. Data on short-term postoperative outcomes, defined as those occurring within the first 90 days after surgery, were abstracted. In addition, intermediate-term outcomes were obtained for patients operated on between February 2013 and February 2017 to maintain a minimum follow-up of 4 years. Outcomes included upper tract dilation on renal and bladder ultrasound, presence of vesicoureteral reflux, cortical defects on nuclear scintigraphy, and continence status. Bladder emptying was assessed with respect to spontaneous voiding ability, need for clean intermittent catheterization, and duration of dry intervals. All operating room encounters that occurred subsequent to initial CPRE were recorded. CPRE was performed in 92 classic BE patients in the first 8 years of the collaboration (62 boys), including 46 (29 boys) during the first 4 years. In the complete cohort, the median (interquartile range) age at CPRE was 79 (50.3) days. Bilateral iliac osteotomies were performed in 89 (97%) patients (42 anterior and 47 posterior). Of those undergoing osteotomies 84 were immobilized in a spica cast (including the 3 patients who did not have an osteotomy), 6 in modified Bryant's traction, and 2 in external fixation with Buck's traction. Sixteen (17%) patients underwent bilateral ureteral reimplantations at the time of CPRE. Nineteen (21%) underwent hernia repair at the time of CPRE, 6 of which were associated with orchiopexy. Short-term complications within 90 days occurred in 31 (34%), and there were 13 subsequent surgeries within the first 90 days. Intermediate-term outcomes were available for 40 of the 46 patients, who have between 4 and 8 years of follow-up, at a median of 5.7 year old. Thirty-three patients void volitionally, with variable dry intervals. Cumulative efforts of prospective data collection have provided granular data for evaluation. Short-term outcomes demonstrate no devastating complications, that is, penile injury or bladder dehiscence, but there were other significant complications requiring further surgeries. Intermediate-term data show that boys in particular show encouraging spontaneous voiding and continence status post CPRE, while girls have required modification of the surgical technique over time to address concerns with urinary retention. Overall, 40% of children with at least 4 years of follow-up are voiding with dry intervals of > 1 hour.

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.

The Role of Bladder Neck Injection in the Quest for Continence

The Role of Bladder Neck Injection in the Quest for Continence

The use of a modified tanagho flap in the repair of posterior urethral stricture after primary exstrophy closure

In patients with classic bladder exstrophy (CBE), posterior urethral strictures after primary bladder closure can be difficult to manage and therefore necessitate alternative techniques in reconstruction of the proximal urethra. There is a paucity of literature describing treatment and management of posterior urethral stricture arising after repair of classic bladder exstrophy. To describe the technique of a bladder neck reconstruction (BNR) with a modified Tanagho anterior detrusor flap in the treatment of posterior urethral stricture arising after repair of classic bladder exstrophy. A prospectively maintained, IRB-approved database of 1401 exstrophy-epispadias patients was reviewed for patients who underwent bladder neck or proximal urethral reconstruction with modified Tanagho flap for proximal urethral stricture disease from the time period of 1975-2021. A total of 5 patients (1 female and 4 males) with classic bladder exstrophy who underwent proximal urethral reconstruction with modified Tanagho flap were identified. Of these 5 patients, the 1 female patient was treated for ongoing incontinence, not posterior urethral stricture, and was thus excluded from this report. Outcomes measured included post operative bladder capacity, post-operative continence status, upper urinary tract status, and stricture recurrence. Four male patients with CBE were treated for posterior urethral strictures and are reviewed here. Three patients underwent successful primary closure (1 via modern staged repair of exstrophy (MSRE) and 2 via complete primary repair of exstrophy (CPRE)), and 1 patient (MSRE) required repeat closure at the time of stricture repair. Mean age at BNR Tanagho flap was 3.8 (range 2.3-5.0) years. All patients remained stricture free with post-operative urinary incontinence; 2 patients underwent subsequent elective bladder neck transection to achieve continence. Mean follow up 9.9 (range 2.1-15.6) years. All 4 patients had excellent long term success rates, comparable to results reported in several adult studies utilizing a modified Tanagho flap in women with post-traumatic bladder neck contractures. In addition, our technique described here shares similarities with both the originally described Tanagho flap as well as with a dorsal onlay graft urethroplasty. In this limited series, modified Tanagho flap reconstruction is effective in treating posterior urethral stricture disease in CBE. This technique adds a valuable adjunct to the armamentarium of surgical options for this difficult to manage condition.

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.

How We Made Exstrophy Bladder Continent and Preserved the Renal Functions.

Crucial requirement of exstrophy bladder repair is to make patients continent as well as to preserve kidney functions. We analyzed our patients' data retrospectively to study their continence and to find out the justification behind continence and preservation of renal functions. We selected files of 18 fully continent patients from 52 patients operated. Eleven out of 18 patients were presented from beginning and 7 were referred after around 8 to 14 years, as incontinent bladder following good repair of bladder neck and posterior urethra. Eleven were operated with complete primary repair of exstrophy along with pubic osteotomy minimal and were kept on cystostomy track (CT) till augmentation to vent out vesical pressure. In seven patients, we did reduction of caliber of posterior urethra and bladder neck along with CT followed by augmentation after 6 months. All 18 patients are maintaining dry period for 24 h. Two patients had enuresis but are manageable with partial fluid restriction from evening. Seventeen out of 18 patients are maintaining their renal functions. No tension abdominal wall closure with rectus muscle apposition is essential to preserve repaired bladder exstrophy. Osteotomy prevents lateral drag to overcome failure of whole reconstruction. Increased "systolic" vesical pressure from contraction of small bladder might destroy the mechanism of continence and renal functions. Hence, venting of vesical pressure through CT is obligatory till augmentation which is of necessity to be done as early as possible to create a low-pressure continent system.

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.

Long-term urological and gynecological outcomes following complete primary repair in females with bladder exstrophy

Long-term continence outcomes in patients undergoing complete primary repair of exstrophy (CPRE) have shown that a subset of patients do not achieve continence until after puberty. We aim to update the continence outcomes as well as describe gynecological outcomes for females having undergone CPRE. This was a retrospective review between 1989 and 2019at a single institution. Inclusion criteria were females with classic bladder exstrophy who underwent CPRE. Exclusion criteria were variant diagnoses and age <4 years. Continence was defined as dry for ≥3h (volitional voiding or clean intermittent catheterization (CIC)). Partial continence was defined as minor stress incontinence or enuresis. Vaginal stenosis was evaluated in post-pubertal patients. Eighteen patients met inclusion criteria. Median age at last follow-up was 15.9 years (IQR 13.1, 18.4). All patients underwent primary closure <30 days of life (n=18). Subsequent procedures included bladder augmentation (n=4), continent catheterizable channel (n=7), bladder neck injections (n=12) and bladder neck reconstruction (n=12). Continence and partial continence were achieved in 6/18 (33.3%) and 9/18 (50.0%), respectively, with mean 3.2±2.5 continence procedures at 9.6 years (IQR 7.3, 15.2). Volitional voiding was seen in 11/18 (61.1%) and 7/18 (38.9%) performed CIC, with no significant difference in continence. Mean bladder capacity was 199ml±96 versus 90ml±29 in the volitional voiding versus CIC group (P=0.0047). Eleven women with median age of 18.0 years (IQR 15.2, 21.4) had recorded menarche: 6/11 (54.5%) patients reported painful/irregular menses, controlled with hormonal therapy. Six of 11 (54.5%) women had vaginal stenosis managed with vaginal dilation (n=2) or vaginoplasty (n=4). Three (27.3%) reported tampon use and penetrative intercourse. Overall, the majority of women who have undergone CPRE achieved complete or partial continence, though most required additional procedures and time to attain it. Additionally, volitional voiding was achievable. Bladder capacity was significantly lower in patients dependent on CIC. Most required medical or surgical interventions for gynecologic concerns post menarche. This study underscores the unique needs of girls and young women with bladder exstrophy and further supports the importance of close long-term urologic and gynecologic management throughout development.

Outcomes of inflatable penile prosthesis insertion using a neotunica allograft in neophalluses of patients on the bladder exstrophy–epispadias complex spectrum

Men born on the bladder exstrophy-epispadias complex (BEEC) spectrum may undergo neophallus reconstruction with a variety of free flaps, most commonly radial forearm. In order to achieve erectile function, a penile prosthesis may be inserted following neophallus construction. In this study, we sought to describe the perioperative and clinical outcomes of inflatable penile prosthesis (IPP) insertion into neophalluses of patients along the BEEC spectrum. Using a prospectively maintained institutional BEEC database, patients who had undergone both neophallus creation and IPP placement were identified for this study. Surgery was performed by two surgeons with expertise in IPP implantation. Operative details and postoperative outcomes and complications were collected with review of the medical record. Overall, there were a total of 13 men who underwent neophallus reconstruction with subsequent IPP placement. Of these men, 76.9% (10/13) had experienced successful primary closure in childhood. One patient had corporal loss secondary to complete primary repair of exstrophy (CPRE) following a failed primary attempt. The average age at IPP placement was 23.2 years (SD±5.06). Mean OR time (n=11) was 244.8min (SD±46.2). 76.9% (10/13) of the cases used AlloDerm™ as a neotunica albuginea for support of the IPP. Three patients (23.1%) experienced complications all of which required revision surgery. Two of these complications were erosions in patients for whom AlloDerm™ was not used in the initial IPP placement. The results of this study suggest that IPP placement in neophalluses of males on the BEEC spectrum have low perioperative and postoperative morbidity with successful outcomes. While the rate of complications is notably higher in this group compared to the native penis IPP literature, the use of AlloDerm™ or other allograft materials may help to narrow this gap. IPP placement in neophalluses of men on the BEEC spectrum is successful in the majority of cases. Complications may be reduced with the use of AlloDerm™ in initial placement.

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.

V09-05 ASSESSMENT OF TISSUE PERFUSION USING INTRAOPERATIVE LASER ANGIOGRAPHY IN BLADDER EXSTROPHY CLOSURE

INTRODUCTION AND OBJECTIVE: Penile reconstruction during exstrophy closure is fraught with the specter of penile loss. To date, no clear clinical method can reliably predict this rare and devastating outcome. To this end we have incorporated penile perfusion testing using intraoperative laser angiography with indocyanine green (ICG) in to our operative approach. METHODS: We describe our IRB-approved intraoperative technique of using ICG at key points during exstrophy closure. Perfusion measurements are taken at four times: 1) prior to dissection, 2) after dissection of the bladder plate and bladder neck 3) with the hips rotated inwards to approximate the pubis and 4) at the completion of the case. This inexpensive fluorescent agent provides real time measurement of tissue perfusion with the ability to redose every 15 minutes. Measurements are taken at 80 seconds post infusion and the medial thigh serves as the reference control. RESULTS: To date, six patients have been evaluated with this technique. After initial dissection blood flow does not change to any significant degree. Upon approximating the pubis, perfusion decreases by more than 50%. Patients who underwent complete primary repair of exstrophy (CPRE) experienced a greater reduction in perfusion than patients who underwent a staged approach. CONCLUSIONS: Intraoperative laser angiography is easy to employ and should be considered a reasonable adjunct to tissue assessment and operative decision making in patients undergoing closure of bladder exstrophy. Source of Funding: none

Practice patterns in classic bladder exstrophy: A global perspective

While evaluation and management options for classic bladder exstrophy (CBE) patients are numerous and varied, little is known regarding the relative utilization of these different methods throughout the world. A large group of exstrophy surgeons practicing globally was surveyed, seeking to document their methods of care. A list of international exstrophy surgeons' email addresses was compiled using professional contacts and referral networking. An online survey was sent to each email address. Surgeons who had not performed a CBE closure within the previous 5 years were excluded. Survey questions queried the respondents' surgical practice type, years since training, and their preferred methods of preoperative evaluation, operative management, and postoperative management. Survey invitations were sent out starting in December 2014 and responses were collected for approximately 6 months. A total of 1152 valid email addresses were invited, resulting in 293 respondents (25%) from 39 countries and every American Urological Association (AUA) section. Seventy-six were excluded, leaving 217 respondents (Table). Respondents reported a median of 17 years since finishing their surgical training (IQR 8-25 years). Practice types included pediatric urology (n=209), general urology (n=9), pediatric surgery (n=59), and other practice makeup (n=3). On subgroup analyses, there were no significant regional practice differences, with the exception of complete primary repair of exstrophy (CPRE) and oral opioid prescribing being significantly higher in North America compared to other regions. Findings indicate that there may be diversity in CBE practice patterns globally. While most responding surgeons from regions outside of North America indicated modern staged repair of exstrophy (MSRE) as their preferred closure technique, a relatively equal distribution of respondents from North America selected CPRE and MSRE. A majority of North American surgeons chose performing osteotomies for both newborn and delayed closures, while an appreciable number of respondents from other regions selected never using osteotomies in their closures. Limitations to this study include a low survey response rate, particularly from surgeons outside of the United States, which may have significantly impacted the ability to draw meaningful global comparisons. Global variation among practices of surgeons performing CBE closures may exist. The wide range of methods demonstrated by this survey suggests the need for more conclusive comparative studies to elucidate whether an optimal standard exists. Local social factors, access to surgical expertise and transportation to referral centers, and finances play a role in what constitutes the best operative approach.

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.

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.

How to close classic bladder exstrophy: Are subspecialty training and technique important?

Successful primary closure is one of the main factors for achieving continence in a classic bladder exstrophy (CBE) patient. Even with contemporary management, patients still have failed primary closures. We sought to understand the role of training, surgical technique, and their impacts on outcomes of CBE closure. A retrospective cohort study from the largest single-institution database of primary and re-closure CBE patients in the world was performed. Failed closure was defined as developing bladder outlet obstruction, wound dehiscence, bladder prolapse, or any need for a re-closure operation. Patient demographics and surgical factors were abstracted and analyzed. Multivariable analysis was performed to test for associations with successful exstrophy closure. Data from 722 patients were analyzed. On bivariate analysis, successful closure was associated with gestational age at presentation, time of closure, location of closure, credential of surgeon performing the closure, closure type, concomitant osteotomy, and type of immobilization. Multivariable analysis, adjusting for patient comorbidity and location of closure, demonstrated increased odds of failure for closure by pediatric surgeon compared with pediatric urologist (OR 4.32, 95% CI 1.98-9.43; p=0.0002), closure by unknown credentialed surgeon (OR 1.86, 95% CI 1.15-2.99; p=0.011), Complete Primary Repair of Exstrophy (CPRE) closure compared with Modern Staged Repair of Exstrophy (OR 2.05, 95% CI 1.29-2.99; p=0.0024), and unknown closure type (OR 4.81, 95% CI 2.94-7.86; p<0.0001) (Table). Many factors associated with failure on bivariate analysis can be explained by these patients presenting to a center of excellence or the selection bias of this cohort stemming from a single center database that have been previously published. However, the finding on adjusted multivariable logistic regression analysis that closure by a pediatric surgeon is associated with higher odds of failure is novel. The additional finding that CPRE closure is associated with failure is most likely secondary to these patients being referred to our institution after having been closed with CPRE which falsely increases its impact on closure failure. Nevertheless, as a center with a large exstrophy volume, this study draws from a cohort that is larger than any other. Classic bladder exstrophy closure should be performed at a center with pediatric urologists to ensure the best chance of a successful primary closure.

A Model for Sustained Collaboration to Address the Unmet Global Burden of Bladder Exstrophy-Epispadias Complex and Penopubic Epispadias

International collaboration to alleviate the massive burden of surgical disease is recognized by World Health Organization as an urgent need, yet the surgical mission model to treat reconstructive surgical challenges is often constrained in ensuring adequate patient follow-up, optimal outcomes, and sustainability. To determine whether a collaboration predicated on long-term commitment by surgeons returning to the same institution annually combined with an experienced host surgical team and infrastructure to ensure sustained patient follow-up could provide surgical care with acceptable outcomes to treat bladder exstrophy-epispadias complex (BE) and penopubic epispadias (PE). In this prospective, observational study, long-term collaboration was created and based at a public hospital in Ahmedabad, India, between January 2009 and January 2015. The entire postoperative cohort was recalled in January 2016 for comprehensive examination, measurement of continence outcomes, and assessment of surgical complications. Seventy-six percent of patients (n = 57) who underwent complete primary repair of exstrophy during the study interval returned for annual follow-up in 2016 and formed the study cohort: 23 patients with primary BE, 19 patients with redo BE, and 11 patients with PE repair. Demographics, operative techniques, and perioperative complications were recorded. A postoperative protocol outlining procedures to ensure monitoring of study participants was followed including removal of ureteral stents, urethral catheter, external fixators, imaging, and patient discharge. Of the 57 patients, 4 were excluded because they underwent ureterosigmoidostomy. Median age at time of surgery was 3 years (primary BE), 7 years (redo BE), and 10 years (PE), with median follow-up of 3 years, 5 years and 3 years, respectively; boys made up more than 70% of each cohort (n = 17 for primary BE, n = 15 for redo BE, and n = 9 for PE). All BE and 3 PE repairs (27%) were completed with concurrent anterior pubic osteotomies. Seventeen of 53 patients (32%) experienced complications. Only 1 patient with BE (4%) had a bladder dehiscence and was repaired the following year. A unique surgical mission model consisting of an international collaborative focused on treating the complex diagnoses of BE and PE offers outcomes comparable with those in high-income countries, demonstrating a significant patient retention rate and an opportunity to rigorously study outcomes over an accelerated interval owing to the high burden of disease in India. Postoperative care following a systematized algorithm and rigorous follow-up is mandatory to ensure safety and optimal outcomes.

A critical appraisal of continence in bladder exstrophy: Long-term outcomes of the complete primary repair

Long-term continence outcomes for patients with bladder exstrophy are lacking in the literature. The complete primary repair of exstrophy (CPRE) is a widely adopted approach that seeks to normalize anatomy at the initial repair, thereby allowing early bladder filling and cycling. Previous reports of continence following CPRE, however, are limited by variable follow-up duration and continence definitions. To assess continence and factors associated with a positive outcome in a cohort of patients following CPRE with long-term follow-up. A retrospective chart review was conducted of all patients with primary bladder closure using CPRE at the present institution for classic bladder exstrophy from 1990 to 2010. Patients <6 years of age or with an incomplete continence assessment at last follow-up were excluded. Continence was defined as voiding volitionally with dry intervals of ≥3h, which was consistent with other high-volume series in the literature. Twenty-nine of the 52 patients (56%) were eligible for inclusion. Twelve patients were continent at last follow-up, including seven boys and five girls. Mean follow-up for the entire cohort was 148 months. The Summary table displays comparisons between continent and incontinent patients. Of patients achieving continence, 42% did not require further continence operations, while 17% required only one additional continence operation. Long-term CPRE continence rates were lower than previously reported, but comparable to other series in the literature. Of those achieving continence, 59% will do so with zero or one additional operation. Sex and osteotomy status were not associated with continence outcomes, although age at follow-up was a predictor of continence, favoring older patients in the present series. The study was limited by lack of standardized, patient-reported continence outcomes and by the retrospective nature of the review. However, the data add to the literature of long-term continence outcomes and are important for counseling families both at initial repair and follow-up. Complete primary repair of exstrophy can achieve continence without additional operations in a subset of patients. Some patients, however, may not achieve continence until adolescence, underscoring the need for long-term urologic follow-up in patients with bladder exstrophy.

Clinical pathway for early discharge after complete primary repair of exstrophy and epispadias by using a spica cast

Secure closure of the pubic diastasis during bladder exstrophy and epispadias repair decreases the abdominal wall tension at the time of reconstruction. Pelvic osteotomies are routinely performed at the time of abdominal wall and bladder reconstruction in order to more easily facilitate pubic symphyseal diastasis approximation. Postoperative pelvic immobilization is performed by methods that include modified Buck's traction, modified Bryant's traction, and spica casting. People undergoing closure often require inpatient hospitalization for 2-8 weeks because of the pelvic immobilization. The present study examined the findings from a clinical pathway for early discharge after complete primary repair of exstrophy (CPRE) and proximal epispadias repair with spica casting. The present study is a retrospective review of patients that underwent pelvic osteotomies with spica casting at the time of CPRE or proximal epispadias repair from November 2006 to March 2013. All patients had anterior innominate osteotomies and spica cast pelvic immobilization. Pelvic osteotomies and spica cast pelvic immobilization were performed on 17 children. The median postoperative stay was 6.0 days and the subdivided results are in Table. No children experienced an abdominal or orthopedic complication. A few children required minor cast adjustments to relieve pressure. After cast removal, no skin breakdown, pressure necrosis, or nerve palsy were found. The median length of casting without pinning was 31 (26-48) days. The use of spica cast pelvic immobilization after exstrophy and epispadias repair is safe and allows for earlier discharge when compared to other methods of pelvic immobilization. However, although the family appreciates early discharge and additional bonding, the priority is the success of the closure. The present findings demonstrate, and are corroborated by other spica cast publications, that spica casting is as effective as modified Bryant's traction or modified Buck's traction. The success rates for CPRE with spica casting are similar to published staged repairs and have the benefit of allowing for bladder cycling, which potentially enables better bladder growth and development. If success and complication rates are comparable amongst the different pelvic immobilization groups, then variables including hospital length of stay and cost become appropriate comparisons. The shortened discharge time, along with a significant decrease in acuity of care leads to significant decreases in hospital costs. Additional hospital stay when using modified Bryant's traction or modified Buck's traction with external fixation will accrue significant hospital costs.

V7-04 MULTI-INSTITUTIONAL BLADDER EXSTROPHY CONSORTIUM: COMPLETE PRIMARY REPAIR OF EXSTROPHY

You have accessJournal of UrologyPediatrics1 Apr 2015V7-04 MULTI-INSTITUTIONAL BLADDER EXSTROPHY CONSORTIUM: COMPLETE PRIMARY REPAIR OF EXSTROPHY Joseph G. Borer, Evalynn Vasquez, Anthony J. Schaeffer, Douglas A. Canning, John V. Kryger, and Michael E. Mitchell Joseph G. BorerJoseph G. Borer More articles by this author , Evalynn VasquezEvalynn Vasquez More articles by this author , Anthony J. SchaefferAnthony J. Schaeffer More articles by this author , Douglas A. CanningDouglas A. Canning More articles by this author , John V. KrygerJohn V. Kryger More articles by this author , and Michael E. MitchellMichael E. Mitchell More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2015.02.1935AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES For bladder exstrophy (BE) repair, gaining necessary surgical experience is challenging due to the rarity of BE. We created a multi-institutional collaboration for the purposes of sharing collective expertise, to efficiently maximize surgical proficiency, and to standardize surgical technique and subsequent care of complete primary repair of exstrophy (CPRE). Our objective is to demonstrate the surgical technique adopted by this collaboration. METHODS Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as host site for scheduled surgeries, with observation, commentary and critique by surgeons from the other sites. Technique was CPRE with bilateral iliac osteotomy performed at 1-3 months of age. Video recording was used for real-time observation both locally and remotely, teaching, future analysis, editing, and review. RESULTS From February 2013 through September 2014, CPRE was performed in 23 patients: 12 boys and 8 girls with classic bladder exstrophy; 1 boy and 2 girls with epispadias. Three surgical modifications were adopted by the group. The first concerned approach to the urethral plate dissection in the boy. The current approach uses a ventral dissection along the medial aspect of the corpora cavernosa using bipolar electrocautery. The second was the deliberate attempt to form a bladder neck in an effort to increase continence by elongating the urethra and creating a more acutely angled transition from proximal urethra to bladder neck into bladder. The third was made after urethral obstruction was noted in 3 girls. Preplacement of perineal and urethral meatus sutures prior to approximation of the pubis allowed optimal exposure of the tissue for accurate and precise suture placement. Complications in girls included 4 episodes of pyelonephritis, 3 urethral obstructions (1 resulted in bladder rupture and 2 required temporary clean intermittent catheterization), and 1 partial labial separation. Complications in boys included 3 urethrocutaneous fistula, 1 hypospadias, return to operating room for 1 suprapubic tube removal, and 1 spica cast change. All closures were successful without dehiscence. CONCLUSIONS We report the surgical method adopted by a multi-institutional collaboration. The increased volume of patients, expert opinions shared, and proficiency gained were immediate benefits observed. This effort increased the annual experience of each institution involved from 3 to 9-fold to ultimately benefit patient care. © 2015 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 193Issue 4SApril 2015Page: e660 Advertisement Copyright & Permissions© 2015 by American Urological Association Education and Research, Inc.MetricsAuthor Information Joseph G. Borer More articles by this author Evalynn Vasquez More articles by this author Anthony J. Schaeffer More articles by this author Douglas A. Canning More articles by this author John V. Kryger More articles by this author Michael E. Mitchell More articles by this author Expand All Advertisement Advertisement PDF DownloadLoading ...