Search

Purpose: A pivotal factor in the success of bladder closure in patients with classic bladder exstrophy (CBE) is the postoperative immobilization of the pelvis and lower extremities after pelvic osteotomy. This study investigates the outcomes of closure among patients with lower limb immobilization using many techniques. The authors hypothesize that the addition of external fixation (pelvic immobilization) in patients with any form of limb immobilization will be associated with improved outcomes., Methods: A prospectively maintained institutional exstrophy-epispadias complex database of 1415 patients was reviewed for patients with CBE who had undergone closure with available immobilization and osteotomy data. Association between closure outcomes and immobilization techniques were determined. Univariate analysis was performed using Chi-Square or Fischer-Exact test as appropriate for categorical variables. Multivariate analysis via binomial logistic regression was used to identify factors leading to successful closure., Results: A total of 747 closure events matching the inclusion criteria were identified. Patients included 508 males and 239 females. There were 597 primary closures (79.9%) with 150 reclosure events (20.1%). Limb immobilization was used in 627 (83.9%) of closure events. Successful closures were associated with osteotomy use (p < 0.0001) and limb immobilization (p < 0.0001); specifically, the combined anterior innominate with posterior vertical iliac osteotomy and modified Buck's traction with external fixation (p < 0.0001, p < 0.0001). Among the group of 33 patients who received external fixation alone and no other type of immobilization, the failure rate was 33.3%, comparatively, patients with any form of combined immobilization (external fixation with lower limb immobilization) had a failure rate of 7.1% ( Table 1). Among patients immobilized with mummy wrap, spica casting, or knee immobilizers, external fixation was associated with 3.76 increased odds of successful closure (p = 0.0005, 95% CI 1.79-7.90). In a unique group of 67 patients without pelvic osteotomy or any form of pelvic or limb immobilization, the failure rate was 74.6%., Discussion: This study confirms, in a larger series, previous findings of improved outcomes when patients are immobilized with modified Buck's traction and external fixation. The authors apply this technique in most all closures and recommend this technique be utilized whenever feasible. However, regardless of the manner of lower limb immobilization, external fixation is a critical factor to optimize closures and ensure success., Conclusion: The results of this study clearly suggest the use of external fixation can be protective against bladder closure failure. The use of pelvic immobilization, in addition to post-operative lower limb immobilization should be strongly considered., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Closure of bladder exstrophy is one of the most complex reconstructive procedures. Basic concepts of these operations, as well as their subtle nuances, require years of experience. However, the volume of these cases is low. The authors describe the experience of a junior surgeon learning the details of exstrophy closure during fellowship and her initial career., Methods: Fellows graduating in 2014 were surveyed for their exstrophy experience during fellowship and the following four years. Operative logs of the junior faculty member were reviewed during the same time. Average operative times were calculated for each year., Results: Seventeen of the 25 fellows responded to the survey. Eight surgeons did one or fewer exstrophy closures during fellowship; 14 did one or fewer closures as an attending. The junior faculty member assisted in 18 closures as a fellow and 48 exstrophy closures during the next four years. Average operative time decreased from 485 to 309 min from attendingship year one to four., Conclusions: There are subtle details inside and outside of the operating room required to ensure a successful outcome in this important step of exstrophy reconstruction. Working alongside senior colleagues is essential to allow the junior surgeon to ascend this ladder of mastering exstrophy closure., Type of Study: Diagnostic., Level of Evidence: V., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: 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., Methods: 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., Results: 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., Conclusion: 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., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: We investigated surgical approaches to urinary incontinence and long-term continence outcomes after successful bladder reconstruction in a heterogeneous patient population with classic bladder exstrophy. We hypothesized that while most patients will achieve urinary continence after surgery, only a select group will void volitionally per urethra., Materials and Methods: An institutional database of 1,323 patients with exstrophy-epispadias complex was reviewed for patients with classic bladder exstrophy who underwent successful bladder closure and a subsequent continence procedure between 1975 and 2017. Procedures included bladder neck reconstruction, bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, and bladder neck closure with continent catheterizable stoma. Cloacal exstrophy, epispadias and variant exstrophy cases were excluded from analysis. Continence at last followup was defined as a dry interval of 3 or more hours without nighttime leakage. Those patients with more than 3 months of followup were assessed., Results: Overall 432 patients underwent successful bladder closure (primary 71.5%, repeat 28.5%) and a urinary continence procedure. At last followup 162 (37%) underwent bladder neck reconstruction, 76 (18%) underwent bladder neck reconstruction with augmentation cystoplasty or continent catheterizable stoma, 173 (40%) underwent bladder neck closure with continent catheterizable stoma and 18 underwent other procedures. Median followup from the first continence procedure was 7.2 years (IQR 2.3-13.7). Continence was assessed in 350 patients. After isolated bladder neck reconstruction 91 of 142 patients were continent (64%, 95% CI 56-72). After bladder neck closure with continent catheterizable stoma 124 of 133 patients evaluated were continent (93%, 95% CI 87-97)., Conclusions: Most patients with classic bladder exstrophy require multiple reconstructive procedures to achieve continence. Only about 25% of patients are expected to void normally per urethra without reliance on catheterization or urinary diversion.

Purpose: Cloacal exstrophy (CE) is the most severe presentation of the Exstrophy-Epispadias Complex (EEC) and is associated with an omphalocele, making the bladder and abdominal wall closure difficult. If the bladder closure fails, a secondary closure is necessary. The objective of this study is to identify patient or surgical factors associated with a successful secondary closure., Methods: The institution's EEC database was reviewed for CE patients between 1975 and 2015. Inclusion criteria included a failed primary bladder closure with a secondary closure. Patient demographics, surgical factors and outcomes of the secondary bladder closure were reviewed., Results: Twenty-four patients met inclusion criteria. 8/8 patients had a successful two-staged closure at the author's institution (100%); 2/16 patients had a successful closure at an outside institution (12.5%). Older median age at secondary closure was associated with outcome, p = 0.045. Pelvic osteotomy was associated with successful secondary closure, p = 0.013. Using Buck's immobilization with external fixation was associated with a higher proportion of successful secondary closures compared to Spica cast, p = 0.012., Conclusion: Successful reclosure in CE patients is associated with the use of osteotomy as well as Buck's immobilization with external fixation. While successful reclosure can be achieved, it is often at the cost of multiple procedures and, therefore, all efforts should be expended to achieve a successful primary closure., Type of Study: Prognostic., Level of Evidence: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: To compare the surgical subspecialties performing bladder exstrophy closures and characterize their practice patterns using both a national and institutional database., Methods: The National Surgical Quality Improvement Program Pediatric (NSQIPP) database was reviewed for all bladder exstrophy closures performed from 2012 to 2017. A single institutional exstrophy-epispadias complex database of 1337 patients was reviewed for patients with a bladder closure at a referring institution from 1975 to 2018. Patients with cloacal exstrophy were excluded. The subspecialties of the surgeons performing the closures were identified. Practice patterns such as the use of a pelvic osteotomy and postoperative immobilization, and perioperative outcomes were compared for each subspecialty group., Results: A total of 84 bladder exstrophy patients from NSQIPP and 263 from the author's institutional database met the inclusion criteria. From NSQIPP, 88% of closures were performed by pediatric urologists while 12% were done by other subspecialists. From the institutional database, 75% of referred bladder exstrophy closures were done by a pediatric urologist, and 25% by other services. Gender, race, operation time, length of stay, and postoperative complications were not significantly different between the groups. In one database, pediatric surgeons performed closures earlier, and in another database, pediatric urologists had greater utilization of osteotomy with different immobilization techniques. Pediatric urologists had a higher success rate., Conclusion: Pediatric urologists performed the most bladder exstrophy closures in both databases; they operated on more delayed closures with a greater use of adjunctive procedures and a higher success rate. Differences in surgical training may contribute to the differences in practice patterns., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: A successful abdominal wall and bladder closure is critical in the management of cloacal exstrophy (CE). This study examines closure outcomes and practices over the last 4 decades at the authors' institution. Beginning in 1995, the authors' institution standardized CE closure and management with the Dual-Staged Pathway (DSP). The DSP consists of a staged bladder closure, a staged or concurrent osteotomy, and postoperative immobilization with external fixation. The authors hypothesize that the DSP has provided better outcomes in CE closures., Methods: A prospective database of 1332 Exstrophy-Epispadias Complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. The DSP consists of a staged osteotomy and a staged bladder closure in CE patients with a diastasis greater than 4 cm. To evaluate the DSP, outcomes of closure at the authors' institution were compared between two equal, twenty-year periods before and after its implementation. Data on timing of closure, postoperative management, surgical complications, and outcomes were collected., Results: There are 142 CE patients in the database. In this study, 49 CE patients with 50 closures met inclusion criteria. The overall success rate of closures from 1975 to 1994 was 88% (14 of 16), while the success rate of the DSP was 100% (n = 34), p = 0.098. Twenty-two (65%) primary and 12 (35%) secondary closures were performed using the DSP. Overall complication rates of the DSP remained similar to previous closures, (29% vs 19%, p = 0.508). Since incorporation of the DSP, patients referred for closure have generally had a larger preclosure diastasis (7.2 cm vs 5.1 cm, p = 0.011)., Conclusion: The standardized DSP closure has proven successful in 34 primary and reoperative cloacal closures in the past 20 years. With this approach, the authors feel that the DSP offers greater patient safety and better outcomes., Level of Evidence: Level III, retrospective comparative study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To investigate the factors affecting primary bladder closure in cloacal exstrophy (CE). A successful primary closure is important for optimizing reconstructive outcomes, and it is a critical first-step in the reconstruction of CE. The authors' hypothesize that a smaller diastasis and use of an osteotomy are independent predictors of a successful closure., Methods: A prospectively maintained database of 1332 exstrophy-epispadias complex (EEC) patients was reviewed for CE patients closed between 1975 and 2015. Univariate and multivariable analyses were performed to identify significant factors associated with CE primary bladder closure., Results: Of 143 CE patients identified, 99 patients met inclusion criteria. Median follow-up time was 8.82 [IQR 5.43-14.26] years. In the multivariable model, the odds of having a successful closure are about 4 times greater for the staged cloacal approach compared to the 1-stage approach (OR, 3.7; 95% CI 1.2-11.5; p-value = 0.023). Also, having an osteotomy increases the chance of a successful closure by almost six-fold (OR, 5.8; 95% CI 1.7-19.6; p-value = 0.004)., Conclusions: Using the staged approach with a pelvic osteotomy is paramount to a successful primary closure in CE. The authors strongly recommend using the staged approach and osteotomy as these factors independently increase the chance for a successful primary bladder closure., Study Type: Therapeutic study., Level of Evidence: Level III, Retrospective comparative study., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: 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., Materials and Methods: 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., Results: 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)., Discussion: 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., Conclusion: Classic bladder exstrophy closure should be performed at a center with pediatric urologists to ensure the best chance of a successful primary closure., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Cloacal exstrophy (CE) is a severe midline congenital abnormality that requires numerous surgical corrections to achieve an acceptable quality of life. Candidates for urinary continence undergo multiple procedures, most often continent bladder diversions, to become socially dry. Here, the authors investigate the number of genitourinary interventions that patients with CE undergo to attain urinary continence., Materials and Methods: A retrospective review of a prospectively maintained database of 1311 exstrophy epispadias complex patients was performed. Patients with CE who have had at least one continence procedure were included. A continence procedure was defined as bladder neck reconstruction with or without augmentation, bladder neck transection with continent urinary diversion, augmentation cystoplasty, or use of injectable bulking agents. Continence was defined as a dry interval greater than 3 hours without leakage at night., Results: In total, 140 CE and CE variant patients have been managed at the authors' institution. Of the 116 CE patients, 59 received at least one continence procedure, 14 were excluded for incontinent diversion or cystectomy, and the remaining 43 patients are awaiting a continence procedure. At the time of analysis, 42 (71%) patients who underwent a continence procedure were dry. The median number of total urologic procedures to reach urinary continence was 4 (range 2-10). This included 1 bladder closure (range 1-3), 2 urinary continence procedures (range 1-4), and 1 (range 0-4) "other" genitourinary procedures. The median time to urinary continence was 11.0 years (95% CI [9.2-14.2])., Conclusions: A majority of CE patients who undergo a diversion procedure can achieve urinary continence. However multiple continence procedures are likely necessary. Of patients who are candidates for a continence procedure, half will be continent by the age of 11., Level of Evidence: Level IV, Case series with no comparison group., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Bladder exstrophy and cloacal exstrophy are rare congenital defects of the genitourinary tract that require complex surgical reconstruction. Malrotation of the bony pelvis causes a characteristic diastasis of the pubic symphysis, which is surgically reduced at the time of initial bladder closure. For a successful primary closure without tension such that the bladder can be placed deep within the pelvis, pelvic osteotomy is often used. However, alternative techniques have been utilized to bring the pubic rami into apposition. The authors present four bladder/cloacal exstrophy patients in which an intrapubic wire was used for pubic apposition, resulting in significant genitourinary complications.

Purpose: Failure of primary closure in classic bladder exstrophy (CBE) is a significant cause of morbidity, and yet its relative economic impact has not been well characterized. The authors aim to determine whether CBE patients who underwent failed primary closure incur greater economic burden in the year following their successful closure than those patients who underwent a successful primary closure., Materials and Methods: After institutional review board approval CBE patients who were successfully closed between 1993 and 2013 were identified in an institutional exstrophy-epispadias database. Patients who were never closed at the study institution and those who had no documented successful closure were excluded. Inpatient hospital charges, hospital costs, and professional fees were collected for the year following successful closure., Results: 162 patients met the inclusion and exclusion criteria and accounted for 312 inpatient admissions in the year following and including their respective successful bladder closures. 62 of the patients failed their primary closure and the remaining 100 succeeded. Adjusting for covariates, patients who underwent successful primary closure experienced a reduction in inpatient hospital charges of $8497, hospital costs of $9046 and professional fees of $11,180 in the year following their successful closure compared to those patients who failed their primary closure., Conclusion: Apart from the self-evident financial advantages of a successful primary closure, namely the avoidance of reclosure, there appears to be a lasting negative financial impact of failed primary closure even after these patients undergo successful reclosure at the study institution., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Introduction: Recent articles document successful classic bladder exstrophy (CBE) closure without osteotomy. Still, many patients require osteotomy if they have a large bladder template and pubic diastasis, or non-malleable pelvis., Objective: To understand the indications and outcomes of bladder closure with and without pelvic osteotomy in patients younger than 1 month of age., Methods: An institutional database of 1217 exstrophy-epispadias patients was reviewed for CBE patients closed at the authors' institution within the first month of life. Patient demographics, closure history, pubic diastasis distance, bladder capacity, and outcomes were recorded and compared using chi-square tests between osteotomy and non-osteotomy patients. Failure was defined as bladder dehiscence, prolapse, vesicocutaneous fistula, or bladder outlet obstruction requiring reoperation. Bladder capacity >100 mL was deemed sufficient for bladder neck reconstruction (BNR)., Results: One hundred CBE patients were included for analysis: 38 closed with osteotomy (26 male, 12 female), and 62 closed without osteotomy (42 male, 20 female). There were four failed closures in the osteotomy group (2 dehiscence, 2 prolapse) and four failed closures in the non-osteotomy group (2 dehiscence, 2 prolapse). This corresponded to statistically equivalent rates of failure between the osteotomy and non-osteotomy groups (10.5% vs. 6.5%, p = 0.466). There was no statistically significant difference between the groups' ability to achieve bladder capacity sufficient for BNR (82% vs. 71%, p = 0.234)., Discussion: A successful primary bladder closure, regardless of the use of osteotomy, has been shown to be the single most important predictor of eventual continence. Because of the complexity of exstrophy manifestations, a multidisciplinary team approach is of the utmost importance. Based on our institutional experience, closure without osteotomy is considered when patients are <72 h of life, have a pubic diastasis <4 cm, malleable pelvis, and pubic apposition without difficulty. Rates of successful closure and attaining sufficient capacity for BNR were both statistically equivalent across groups. This retrospective study is limited by selection bias and the significant difference in follow-up time between groups. Nevertheless, as a high-volume exstrophy center this study draws from one of the largest cohorts available., Conclusions: Regardless of the type of closure undertaken, there clearly is a role for newborn CBE closure without pelvic osteotomy in patients considered suitable for closure by both the pediatric urologist and orthopedic consultant. However, if there is any doubt concerning pubic diastasis width, pelvic malleability, or ease of pubic apposition, an osteotomy is highly recommended., (Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: This study examines a large single-institution experience with cloacal exstrophy patients, analyzing patient demographics and surgical strategies predictive of bladder closure outcomes., Methods: One hundred patients with cloacal exstrophy were identified. Complete closure history including demographics, operative history, and outcomes was available on 60 patients. Twenty-six patients with a history of failed initial bladder closure were compared to 34 with a history of successful initial bladder closure. Univariate logistic regression analysis was used to compare the two groups., Results: Median follow up time after initial closure was 9years (range: 13months-29years). A 1cm increase in pre-closure diastasis resulted in a 2.64 increase in the odds of initial closure failure (p=0.004). Protective strategies against failure included delaying closure (per month) (OR=0.894, p=0.009), employing pelvic osteotomies (OR=0.095, p<0.001), and applying external fixation (OR=0.024; p=0.001). Among patients who underwent osteotomy (31% of patients in the failed group, 82% in the successful group), a longer delay between osteotomy and closure (OR=0.033; p=0.005) was also protective against failure., Conclusion: Patients with a large diastasis are more likely to fail initial closure. Delaying initial closure for at least 3months, performing pelvic osteotomy, and using an external fixation device post-operatively are strategies that improve closure success., (Published by Elsevier Inc.)

Objective: To describe the potential predisposing clinical characteristics at initial closure, the treatments of and outcomes associated with vesicocutaneous fistula (VCF) after primary bladder closure., Materials and Methods: Eighteen patients were referred for VCF after primary bladder exstrophy closure. Aspects from the primary closure such as gender, timing of diagnosis and repair, osteotomies, pelvic immobilization, layers of closure, use of tissue adjuncts, and complications in addition to details from their required treatment of VCF were retrospectively extracted from the medical record., Results: A diagnosis of failed closure was made at the time of evaluation in 13 of 18 patients who presented with VCF. All 13 patients underwent delayed single-layer closures and had a widened pubic diastasis. Five patients with secure closures who underwent successful simple fistula repairs were all closed early in life, had multi-layered closures with pelvic osteotomies, and had minimal change in pubic diastasis., Conclusion: VCF may represent a failed bladder closure. Factors which may suggest failure are a fistula tract in the lower abdominal midline between the pubic rami, a pubic diastasis increased from pre-closure measurement, and cystoscopic evidence of an anteriorly positioned bladder., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Complete female epispadias, which occurs much more rarely than classic bladder exstrophy in females, is thought to have a more benign clinical course. We hypothesized that patients with complete female epispadias are more likely to have a larger bladder capacity and achieve voiding continence than females with classic bladder exstrophy., Materials and Methods: After obtaining institutional review board approval, females with complete female epispadias or classic bladder exstrophy were identified from an institutionally approved prospective database. We retrospectively reviewed the charts of 22 patients with complete female epispadias and 23 with female classic bladder exstrophy, including 3 with delayed primary closure., Results: Patients with complete female epispadias presented later and underwent the first reconstructive procedure at an older age than patients with classic bladder exstrophy. Patients with complete female epispadias had lower initial and final age adjusted bladder capacity than those with classic bladder exstrophy but the bladder growth rate did not differ between the groups. When patients with complete female epispadias were stratified by age at initial reconstruction, there was no difference in final age adjusted bladder capacity or the bladder growth rate. There was also no statistical difference between the groups in the number of surgeries, continence rate from initial reconstruction, bladder neck reconstruction success or need for a continent stoma., Conclusions: This study suggests that females with classic bladder exstrophy who undergo successful primary closure have higher initial and final age adjusted bladder capacity than females with complete female epispadias but with a similar growth rate. This may reflect the creation of outlet resistance at a younger age than in those with complete female epispadias. However, no difference was identified between patients with complete female epispadias who initially underwent repair before vs after age 1 year. Patients with complete female epispadias undergo procedures to achieve continence that are similar to those in patients with classic bladder exstrophy., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: To review the urological management and outcomes of patients with the OEIS (omphalocele, exstrophy of the bladder, imperforate anus, spinal abnormalities) complex., Patients and Methods: 80 patients with the OEIS complex managed at a single institution between 1974 and 2009 were reviewed., Results: 37 had initial closure at our institution (2 failed - 5%); 22 with successful closure were referred for incontinence; 15 failed closure at an outside institution (2 of whom are awaiting closure); 6 are skin-covered variants. Osteotomy was performed in 39/43 (91%) with successful closure versus 8/17 (47%) who failed initial bladder closure. 40 were dry (56%), but most needed additional urinary reconstruction: 2 had small bowel neobladders; 32 (84%) had augmentation cystoplasty; 30 (79%) had a continent catheterizable channel; only 9 (24%) were continent with an intact urethra. Bladder neck reconstruction allowed dryness in 7 (18%). 45 patients had XY genotype--19 had female gender assignment at birth. All patients with XX genotype had female gender assignment., Conclusions: Osteotomy improves success of initial bladder closure. A bladder neck procedure, catheterizable channel, and augmentation cystoplasty will be required in the majority of patients to attain urinary dryness., (Published by Elsevier Ltd.)

Introduction: As the primary practitioner managing patients with classic bladder exstrophy (CBE), it is incumbent upon the pediatric urologist to understand the associated orthopedic anomalies and their management., Methods: A Pubmed search was performed with the keyword exstrophy. Resulting literature pertaining to orthopedics and published references were reviewed., Results: Anatomic changes to the bony pelvis include outward rotation, acetabular retroversion with compensatory femoral anteversion, anterior pubic shortening, and pubic diastasis. Imaging options have improved, which impacts surgical planning. Surgical approach, including type of osteotomy and method of pubic approximation, is evolving. Most centers employ immobilization after surgery, with external fixation, Bryant's traction, Buck's traction, and spica casting being the most common methods. Orthopedic complications range from minor pin-site infections to neurologic and vascular compromise. Most experts agree osteotomy aids bladder closure beyond 72 h of life, but effect on continence remains controversial. Although no significant orthopedic benefit has been expounded, it may be too early to appreciate improvement in frequency or severity of osteoarthritis or hip dysplasia., Conclusion: While orthopedic surgeons remain vital to managing exstrophy patients, knowledge of the anatomy, imaging, surgical approaches, and immobilization enable effective communication with parents and other physicians, improving care for these complicated patients., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To compare the estimated glomerular filtration rate (eGFR) in bladder exstrophy patients with published normative GFR estimates., Patients and Methods: eGFR was calculated using the Schwartz formula at three timepoints, with mean eGFR at each timepoint compared to normative values., Results: At primary closure (n = 53) the mean eGFR (ml/min/1.73 m(2)) in exstrophy patients was similar to norms at 0-7 days (exstrophy vs norm: 42.5 vs 40.6, p > 0.05) and after 2 years of age (108.8 vs 133, p > 0.05). However, the mean eGFR in exstrophy patients was significantly lower than norms between 8 days (44.8 vs 65.8, p < 0.0001) and 2 years of life (68 vs 95.7, p = 0.01). At bladder neck reconstruction (n = 13) no statistically significant difference existed between the exstrophy and normative eGFR values (137.1 vs 133, p > 0.05). Similarly, among 27 patients with at least 1 year follow-up after bladder neck reconstruction, the mean exstrophy eGFR was no worse or higher than normative values (2-12 years: 124.5 vs 133, p > 0.05; males ≥13 years 175.6 vs 140, p = 0.04; females ≥13 years 128.8 vs 126, p > 0.05)., Conclusion: The staged reconstruction of exstrophy does not appear to negatively impact renal function in most patients. As eGFR detects only significant changes, surgical reconstruction may still cause more subtle renal damage., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Late referrals or unsuitable bladder templates often require delayed primary repair of bladder exstrophy. We investigated longitudinal bladder growth rates and eventual outcomes following this approach., Materials and Methods: After institutional review board approval, we reviewed the medical records of patients with classic bladder exstrophy who underwent neonatal or delayed (more than 30 days) primary closure at our institution between 1970 and 2006. Clinical characteristics and annual cystographic bladder capacity before the continence procedure were compared. Failed primary exstrophy repairs were excluded., Results: A total of 33 patients with available bladder capacity measurements underwent delayed exstrophy closure due to small bladder template in 18 (88% male) and late referral in 15 (80% male) at respective median ages of 305 days (range 86 to 981) and 172 days (31 to 676). They were compared to 82 patients (71% male) undergoing neonatal closure at a median of 2 days of life (range 0 to 27). Pelvic osteotomy was performed in 32 of 33 delayed closures. Longitudinal analysis of the bladder capacities demonstrated that, compared to neonatally closed cases, bladder capacities were on average 36 ml smaller in those with delayed repair due to small templates (p = 0.01) and 29 ml smaller in those with late referrals (p = 0.13). However, the rate of bladder growth did not differ significantly among the 3 groups., Conclusions: Delayed primary repair of exstrophy does not compromise the rate of bladder growth. However, children born with smaller templates will have overall smaller capacities and are less likely to undergo bladder neck reconstruction., (Copyright © 2012 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Successful primary bladder closure of classic bladder exstrophy sets the stage for development of adequate bladder capacity and eventual voided continence. The postoperative pathway following primary bladder closure at the authors' institution is quantitatively and qualitatively detailed., Materials and Methods: Sixty-five consecutive newborns (47 male) undergoing primary closure of classic bladder exstrophy were identified and data were extracted relating to immediate postoperative care. Overall success rate was utilized to validate the pathway., Results: Mean age at time of primary closure was 4.6 days and mean hospital stay was 35.8 days. Osteotomy was performed in 19 patients (mean age 8.8 days), and was not required in 39 infants (mean age 2.9 days). All patients were immobilized for 4 weeks. Tunneled epidural analgesia was employed in 61/65 patients. All patients had ureteral catheters and a suprapubic tube, along with a comprehensive antibiotic regimen. Postoperative total parenteral nutrition was commonly administered, and enteral feedings started around day 4.6. Our success rate of primary closure was 95.4%., Conclusions: A detailed and regimented plan for bladder drainage, immobilization, pain control, nutrition, antimicrobial prophylaxis, and adequate healing time is a cornerstone for the postoperative management of the primary closure of bladder exstrophy., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: Partial or complete penile loss following bladder exstrophy and/or epispadias repair has been reported in the literature progressively more frequently., Patients and Methods: The authors report new cases of penile injury following bladder exstrophy and/or epispadias repair referred to their centers and not previously published. They review the literature on this subject and offer an explanation as to the likely mechanism for the penile injury and recommendations to avoid this complication., Results: Seven new cases of partial or complete penile loss following bladder exstrophy or epispadias repair have been recently referred to the authors' institutions. Twenty-one patients have previously been reported in the literature. Altogether, 24 cases occurred after bladder exstrophy closure: 23 after complete primary repair of exstrophy (Mitchell repair) and one after first-stage radical soft-tissue mobilization (Kelly repair). Nineteen of 24 patients did not have a pelvic osteotomy at the time of primary closure. Four cases occurred after epispadias repair: two following the second-stage radical soft-tissue mobilization (Kelly repair) and two following penile disassembly epispadias repair (Mitchell repair)., Conclusion: Exstrophy closure combined with epispadias repair can be followed by ischemic penile injury, particularly when osteotomy is not performed. Compression of the pudendal vessels after pubic apposition and/or direct injury to the pudendal vessels play an important role in the pathogenesis of this complication., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

This review of the complications of all methods of modern treatment of bladder exstrophy emphasizes the complexity of reconstruction of the bladder exstrophy spectrum. The main complications of any method of primary bladder exstrophy closure are complete wound dehiscence, bladder prolapse and urethral outlet obstruction; others include bladder and renal calculi. These complications as well as methods to avoid them are discussed here. Evidence supporting the management strategy is presented where possible.

Purpose: We report the urological, orthopedic and neurological complications of primary closure of classic bladder exstrophy using modern staged repair of exstrophy., Materials and Methods: An approved database identified 137 males and 57 females with classic bladder exstrophy who underwent primary repair by 1 of 2 surgeons in 23 years. A total of 185 patients underwent primary closure using modern staged repair of exstrophy with or without osteotomies, whereas 9 underwent delayed primary closure with epispadias repair at age 12 months. Of the patients 63 received osteotomies. Mean age at closure was 60 days and mean followup was 9 years., Results: There were 14 major complications (11%) and 27 minor complications (14%). Major urological complications included bladder prolapse or dehiscence in 6 male patients (3%), which was successfully reclosed. Major orthopedic complications, including osteotomy nonunion in 2 cases, leg length inequality in 1 and persistent joint pain in 1, developed in 4 of the 63 patients (6%) who underwent osteotomy. Major neurological complications included femoral nerve palsy in 4 patients (2%). There were 21 minor urological complications (11%), including posterior bladder outlet obstruction in 4 cases, urethrocutaneous fistula in 2, suprapubic tube removal in 2, intrapubic stitch erosion in 4, febrile urinary tract infection in 6 and surgical site infection in 3. Six patients (3%) had minor orthopedic complications, including pelvic osteomyelitis in 1, pin site infection in 3 and a pressure sore from immobilization in 1., Conclusions: Closure of bladder exstrophy is a safe surgery with an acceptable risk of complications. A critical review of outcomes provides insight to further refine the technique and manage complications when they develop.

Purpose: We evaluated the current application of Duckett paraexstrophy skin flaps for bladder exstrophy reconstruction., Materials and Methods: We reviewed the records of cases of classic exstrophy treated at our institution between September 1993 and March 2007. A total of 21 patients were identified in whom modified Duckett paraexstrophy skin flaps were used in bladder closure at our institution. Another 12 patients were referred during the same time after closure with complications with flap use., Results: Of the 21 patients who underwent closure at our institution with our modified version of the Duckett flaps 1 (4%) had a complication, that is urethral stricture. This responded to internal urethrotomy and daily intermittent catheterization for 4 months, and the stricture stabilized. Four of the 12 referred patients responded to multiple urethral dilations, 3 underwent open repair with a buccal graft, 2 received a full-thickness skin graft and 3 underwent internal urethrotomy with daily intermittent catheterization for 4 months. Of our 21 patients with internal treatment 14 underwent bladder neck repair, 5 underwent epispadias repair and 2 were awaiting further reconstruction. Six of the 12 referred patients underwent bladder neck repair, 4 underwent epispadias repair and 2 were awaiting further reconstruction., Conclusions: By modifying our version of the Duckett paraexstrophy skin flaps we have lowered our complication rate significantly. While overall use of these flaps has continued to decrease, when they are required, these modifications help avoid complicating strictures and their sequelae in the bladder and upper urinary tract.

Objective: Many changes have occurred in the treatment of bladder exstrophy over the last few years and several repairs are now offered, but there is a lack of long-term follow-up data. The purpose of this study was to evaluate long-term outcomes in a select group of female patients in whom modern staged repair was undertaken., Patients and Methods: From an institutionally approved database 41 patients were identified. All had undergone primary bladder exstrophy closure in 1988-2005, at a mean age of 2 months (range 4h to 3 months), with or without an osteotomy by a single surgeon, and all were followed up for a minimum of 5 years. Twelve patients underwent osteotomy at the time of primary closure. Eight had a classic transverse innominate and vertical iliac osteotomy, and four a transverse innominate only. Mean age at the time of bladder neck repair (BNR) was 4.2 years (39-65 months). Mean measured bladder capacity under gravity cystograms at the time of repair was 109 cc (80-179 cc)., Results: Thirty patients (74%) were continent day and night, and voiding per urethra without augmentation or intermittent catheterization. Social continence, defined as dry for more than 3h during the day but damp at night, was found in a further four cases (10%). Seven patients are completely incontinent with dry intervals of less than 1h day and night. The mean time to daytime continence was 12 months (4-16 months) and to night-time continence was 19 months (10-28 months). Patients with a mean capacity greater than 100 cc had better outcomes. Six of the 30 patients achieved dryness after primary closure only, and all six had transverse innominate and vertical iliac osteotomy at the time of primary closure., Conclusions: Female classic exstrophy patients with a good template who develop adequate capacity after a successful primary closure can achieve acceptable continence without bladder augmentation and intermittent catheterization. A select group will develop continence with closure alone without the need for bladder neck repair.

Purpose: The radical soft-tissue mobilization procedure was developed as a component of the staged closure of classical bladder exstrophy to improve continency rates without having to perform pelvic osteotomies. The authors describe complications following this procedure and discuss possible etiologies and subsequent management., Materials and Methods: We extracted from an institutionally approved exstrophy database the records of patients evaluated for complications following radical soft-tissue mobilization repair from 1999 to 2002., Results: Four patients were referred to our institution following closure of exstrophy with the radical soft-tissue mobilization technique; two boys and two girls. Complications included ischemic penile injuries in both males, failed exstrophy closure in one female, incontinence with need for bladder neck transection and diversion in two patients, and upper tract deterioration in two patients of whom one required cystectomy and incontinent diversion., Conclusions: Omission of osteotomies when employing the radical soft-tissue mobilization repair appears to result in complications that could otherwise be prevented. Additionally, the complex dissection of the pelvic musculature, innervation and vasculature performed during radical mobilization has great potential to injure the pelvic structures and genitalia, as has been seen with the cases presented herein.

Purpose: We describe the results of modified Young-Dees-Leadbetter bladder neck reconstruction to achieve continence in patients who underwent complete primary repair of bladder exstrophy as newborns., Materials and Methods: Using the Johns Hopkins Exstrophy Database we identified patients who underwent bladder neck reconstruction after having undergone complete primary repair of bladder exstrophy as newborns. We determined patient characteristics and surgical outcomes., Results: A total of 30 males and 3 females were referred after complete primary repair of bladder exstrophy, of whom 26 underwent bladder neck reconstruction. Results of complete primary repair of bladder exstrophy were strongly associated with osteotomy use. Of the patients 19 (58%), including 16 males and 3 females, who underwent complete primary repair of bladder exstrophy without osteotomy had complications (dehiscence and bladder prolapse), while none of 14 male patients who underwent complete primary repair of bladder exstrophy with osteotomy had complications, although none were subsequently continent. Of the 19 patients who had complications after complete primary repair of bladder exstrophy 12 underwent bladder neck reconstruction and total continence was achieved in only 3 (25%). Of the 14 patients with successful complete primary repair of bladder exstrophy 8 (57%) are dry day and night, 4 (28%) are dry during the day and wet at night, and 2 (14%) are completely incontinent. Continent children underwent successful complete primary repair of bladder exstrophy with pelvic osteotomy, all underwent hypospadias repair before age 1 year and none required ureteral reimplantation before bladder neck reconstruction., Conclusions: Many patients who undergo newborn complete primary repair of bladder exstrophy will require bladder neck reconstruction. Bladder neck reconstruction is more successful in those in whom complete primary repair of bladder exstrophy was successful. As in all types of repair, failed initial closure usually results in a bladder that is unsuitable for bladder neck reconstruction. These patients often require bladder augmentation and a continent stoma to be dry.

Purpose: We describe the complications of complete repair and their management., Materials and Methods: A total of 19 patients were referred after failed complete repair. Total dehiscence occurred in 6 males, major bladder prolapse in 3, minor prolapse in 3, pubic separation in 1, impassable stricture in 1, and total hemiglans and corporal loss in 2. Overall, partial glans loss was seen in 7 patients, urethral loss in 5 and penile skin loss in 3. One female had complete dehiscence and 1 had major prolapse, both losing the urethrovaginal septum. One female had an impassable stricture., Results: Six males with dehiscence underwent re-closure with osteotomy. Urethral replacement was performed with full thickness skin graft (FTSG) in 3 and with buccal mucosa in 3. Five patients underwent a modified Cantwell-Ransley (C-R) epispadias repair after placement of skin expanders, and 1 awaits repair. The 3 patients with major prolapse underwent re-closure with osteotomy. A urethral buccal graft was used in 1 patient, FTSG was used in 2 at a later operation and all 3 underwent C-R epispadias repair. Of the 3 patients with minor prolapse 2 underwent re-closure with osteotomy using urethral buccal graft or FTSG followed later with a C-R repair. The final patient with minor prolapse underwent re-closure with osteotomy and C-R repair after testosterone stimulation. One patient with pubic separation and urethral and skin loss underwent re-closure with osteotomy, C-R repair after skin expanders and later bladder neck repair. In 1 case a ureteral graft replaced a posterior urethral stricture. Of the 2 patients with hemiglans and corporal loss 1 underwent penile torsion repair and later hypospadias repair, while the other is being observed. Two females underwent re-closure with osteotomy and urethral replacement with tubularized bladder. The case of stricture was managed endoscopically., Conclusions: Complications of complete repair are similar to those of other repairs but more serious if soft tissue loss occurs. Because of these increased risks, this procedure and its formidable complications are best managed by experienced exstrophy surgeons.

Purpose: Failed initial bladder exstrophy closure may hinder the natural course of bladder growth compared to successful primary reconstruction. By measuring successive bladder capacities within the first 5 years of life, we compared the rate of bladder growth in children with failed vs successful initial closure. Materials and Methods: We used an approved bladder exstrophy database to identify and review retrospectively patients with classic bladder exstrophy who underwent repeat cystograms between ages 1 and 6 years. Two groups of patients were identified—those with successful neonatal closure (group 1) and those with successful reclosure after an initial failed procedure (group 2). A generalized linear mixed model was fit to evaluate the impact of treatment group and age on bladder growth. Results: We identified 48 patients in group 1 (75% male) and 62 in group 2 (71% male). Initial pelvic osteotomy was done in 60% of group 1 and 34% of group 2. Patients in group 1 had significantly larger cystographic capacity at 2, 4, 5 and 6 years after successful bladder closure compared to those in group 2 (p <0.05). The bladder tended to grow at a significantly slower rate in group 2 (9.38 cc yearly) compared to group 1 (14.76 cc yearly, p = 0.005). Conclusions: Patients with initial failed bladder exstrophy closure showed significantly smaller cystographic capacities and slower bladder growth compared to those who underwent successful neonatal bladder closure. These data clearly underscore the importance of a secure, successful primary closure. [Copyright &y& Elsevier]

Purpose: The reasons for referral and treatment strategies in patients who underwent complete primary repair of bladder exstrophy elsewhere in the newborn period were evaluated. Materials and Methods: An institutionally approved database identified patients who underwent complete primary repair of exstrophy and were subsequently referred for continued care. Results: A total of 10 females and 55 males were referred for treatment between 1996 and 2010. Six females and 23 males were referred for complications following initial complete primary repair of exstrophy. Female complications included dehiscence (3 patients), prolapse (2) and stricture (1). Male complications included dehiscence (10 patients), prolapse (9), pubic separation (1) and stricture (3). Nine males had posterior urethral loss and 13 had major penile soft tissue injuries. Grade V vesicoureteral reflux and severe hydronephrosis were seen in the 4 patients with urethral strictures. Patients with dehiscence or prolapse underwent successful reclosure with osteotomy. Cases with posterior urethral loss or strictures were repaired with grafts. Four females and 32 males were referred for incontinence. Nine of these patients had poor bladder capacity, of whom 5 underwent bladder augmentation with continent stoma and 4 are awaiting further bladder growth. A total of 27 patients underwent bladder neck repair, with 15 (56%) subsequently continent, 5 (19%) daytime continent with nocturnal incontinence and 7 (26%) continuously incontinent. At initial closure osteotomy had been performed in all patients who were continent following bladder neck reconstruction and in 4 of 5 who were daytime continent. Conclusions: Complications of complete primary repair of bladder exstrophy can result in undesired upper urinary tract changes and penile soft tissue loss. Surgical reconstruction of such complications and acceptable continence are attainable in select cases. [ABSTRACT FROM AUTHOR]

Purpose: We report the indications, technique and outcome of a large series of children who underwent bladder neck transection for intractable urinary incontinence. Materials and Methods: We retrospectively reviewed demographics, operative details, complications and outcomes of 76 patients (47 males, 29 females) who underwent bladder neck closure at our institution between 1996 and 2006. Mean patient age at the time of the procedure was 12 years, 10 months. The most common diagnosis was bladder exstrophy. Of the patients 31 had undergone prior bladder neck reconstruction (30) or sling repair (1). All patients underwent concomitant augmentation and creation of a catheterizable stoma. Results: A total of 50 patients had more than 12 months of followup (mean 44, range 12 to 128). Continence was achieved initially in 86% of the patients (43 of 50). Of the 7 primary failures 2 leaked via the urethra and 5 via the stoma. Six of these patients achieved dryness with revision, for a final continence rate of 98%. A single renal unit suffered significant loss of function during this period. New, nonobstructive hydronephrosis developed in 8 additional renal units. Stones developed in 30% of the patients. There were no spontaneous bladder ruptures. Conclusions: Bladder neck transection in combination with enterocystoplasty and creation of a continent catheterizable stoma is an effective approach to incontinent cases with severely damaged bladder outlets and poor quality bladders in which other reconstructive approaches either have failed or are deemed likely to fail. Specific concerns regarding the risk of poor renal outcomes and perforation seem unwarranted at present. [Copyright &y& Elsevier]

Summary Purpose The authors have reviewed the use of muscle pedicle flaps for the treatment of failed bladder neck closure in exstrophy spectrum patients. Methods A retrospective review of all exstrophy spectrum patients who underwent continence procedures with the use of muscle pedicle flaps at our institution during the last 15 years was performed. Patient characteristics, surgical history, and outcomes, including complications, continence, morbidity, and infection, were assessed. The authors utilized muscle pedicle flaps in eight exstrophy patients, including four patients with classic bladder exstrophy and four patients with cloacal exstrophy. Seven of eight patients had failed at least one prior bladder neck closure, and they had undergone a median of three prior urologic procedures. To achieve continence, five rectus muscle flaps and three gracilis muscle flaps were utilized in combination with bladder neck closure. Results There were no major intraoperative or postoperative complications. All patients were initially continent, and after a median follow-up of 18.7 months seven of eight patients were continent. One patient required continent urinary stoma revision and one patient developed perineal incontinence after perineal trauma. No patients required revision of, or additional, continence procedures at the bladder neck. Discussion The use of pedicle muscle flaps appears to be a safe and feasible option for exstrophy spectrum patients with failed bladder neck closure. Although achieving continence can be difficult in this population, use of muscle flaps and bladder neck closure is a viable and effective option in this challenging subset of patients. Figure Rectus muscle flap with vascular pedicle via the inferior epigastrics. [ABSTRACT FROM AUTHOR]