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Introduction: 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., Objective: 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., Patients and Methods: 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., Results: 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., Discussion: 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., Conclusion: 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., Competing Interests: Conflicts of interest The authors have no financial or personal relationships with other people or organizations that could inappropriately influence their work., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: 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., Objective: 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., Study Design: 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., Results: 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 147 mL (range 102-210 mL). 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., Discussion: 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., Conclusions: 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., (Copyright © 2020. Published by Elsevier Ltd.)

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.

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.)

Objective: To explore the long-term outcomes and indications for cystectomy in patients with bladder exstrophy. Although rare, cystectomy is the final surgical alternative to bladder repair among these patients with a poor quality bladder template., Methods: A prospectively maintained database of 1298 patients with exstrophy-epispadias complex was reviewed for patients who underwent cystectomy between 1970 and 2015 at the authors' institution. Demographic data, indication for cystectomy, surgical history, postoperative outcomes, and continence status were collected., Results: Eighteen (6 male; 12 female) patients with exstrophy (15 classic bladder exstrophy; 2 bladder exstrophy variants; 1 cloacal exstrophy) underwent cystectomy at a median age of 3.8 years. Six patients (33.3%) underwent primary cystectomy without attempted bladder closure. Eight patients (44.4%) had a history of failed primary closure with loss of capacity or inadequate growth after closure. Four patients (22.2%) had successful primary closure but underwent cystectomy secondary to poor bladder compliance with declining renal function or poor bladder growth or quality. Urinary diversion included 6 cutaneous ureterostomies, 4 bowel conduits (1 ileal; 3 colon), 6 continent urinary diversions with ileosigmoid reservoir, and 1 ureterosigmoidostomy. Of 8 patients who underwent a continence procedure, all were dry at a median of 25.3 months after cystectomy., Conclusion: Cystectomy was most commonly indicated in intrinsically diseased bladder templates that remained too small despite permitting time for interval growth. These bladders often were of poor quality and compliance and did not reduce into the pelvis on examination. It was, however, possible to achieve urinary continence in these patients with cystectomy and urinary diversion., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function., Design: Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire., Setting: Maryland, United States., Participants: Review of a single-institution exstrophy-epispadias complex database resulted in 25 adult female patients with a history of POP treated at the authors' institution. Eleven patients participated and were included in the analysis., Main Outcome Measures: Urinary continence and sexual function., Results: All participants underwent surgical repair for prolapse, with 7 (63.6%) experiencing unsuccessful initial repair and subsequent recurrence. Median total number of POP repairs was 2.5 (1-4). After correction of POP, patients reported a median improvement in International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form scores of 11 (21 to -1) of 21 and in POP-Urinary Incontinence Sexual Questionnaire scores of 9.5 (6.5-33.0) of 48.0. With regard to urinary continence, 6 (54.5%) patients presently reported no incontinence, 3 (27.3%) reported mild incontinence, and 2 (18.2%) reported continuous incontinence., Conclusions: Pelvic organ prolapse poses significant reductions in quality of life for women born with exstrophy, with effects on urinary continence and sexual function. Identification and correction of prolapse seems to result in notable improvements in the lives of these patients.

Background: Surgical advancements have made cloacal exstrophy (CE) a survivable condition, though management remains complex. Urologic, orthopedic, colorectal and gynecologic interventions are not standardized, and the cost of this care is high. While the importance of a successful primary closure in terms of outcomes is known, the economic consequences of failure remain uncharacterized., Methods: A prospectively maintained institutional database of epispadias-exstrophy complex patients was reviewed for continent CE patients. Hospital charges for all inpatient admissions prior to achieving urinary continence were inflation-adjusted to year 2013 values using Consumer Price Index for medical care published by the United States Bureau of Labor Statistics. Records for which charge data were incomplete were completed by using single mean imputation, also inflation-adjusted. Descriptive data are presented as mean±standard deviation (SD)., Results: Of 102 CE patients, 35 had available hospital charge data: 15 who underwent successful primary closure at the authors' institution and 20 who presented after previously failed primary closures at referring institutions. The mean±SD hospital charges for primary closure in the success group were $136,201±$48,920. These patients then underwent subsequent additional surgeries that accrued charges of $59,549±$25,189 in order to achieve continence. Overall, successful primary closures accumulated hospital charges of $200,366±$40,071. In comparison, patients referred after prior failure required significantly more hospital admissions and additional charges of $207,674±$65,820 were required to achieve continence (p<0.001). Patients who failed primary closure are estimated to accumulate 70% more total health care charges compared to the group following successful primary closure., Conclusion: The cost of CE management until urinary continence is high, averaging more than $200,000 in inpatient hospital charges alone. Initial success is desirable from both an outcomes and economic perspective, as the cost of salvaging a failed primary closure at our institution is similar to the overall costs of a successful closure; this is in addition to the cost of any previous failed closures. Further studies will be required to determine the optimal timing of surgical management in terms of both patient outcomes and financial consequences., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Injection of urethral bulking agents (UBA) has been used to increase bladder capacity prior to bladder neck reconstruction (BNR) or as an adjuvant therapy following BNR to improve continence. The purpose of this study was to determine the effectiveness of urethral injections in the exstrophy population., Materials and Methods: A review was performed of patient characteristics, bladder capacity, and continence status of all patients with the exstrophy-epispadias complex who underwent injection of UBA between 1980 and 2008., Results: Among 66 patients with a median follow-up of 8 years, 41 underwent injections prior to BNR, and 25 had injections after BNR. Only 24% of patients who underwent injections prior to BNR were continent on last follow-up. Among 25 patients who underwent BNR prior to injection(s), 16 were partially continent and nine were incontinent prior to first injection. Patients who were partially continent attained social continence (dry interval greater than 3 h) at a significantly higher rate than those who were incontinent (63% vs. 13%, p = 0.047). No patient with cloacal exstrophy in either group attained urethral continence., Conclusion: UBAs do not appear to have a role prior to BNR. However, they may provide benefit when given adjunctively following BNR in patients who are partially continent., (Published by Elsevier Ltd.)

Purpose: We describe the application and results of modified Young-Dees-Leadbetter bladder neck reconstruction after successful complete primary repair in the newborn period., Materials and Methods: The records of 34 patients referred for a continence procedure after successful exstrophy closure were extracted from an institutionally approved database. Patient characteristics and surgical outcomes were assessed., Results: A total of 31 male and 3 female patients were identified, of whom 27 and 1, respectively, underwent osteotomy at initial closure. No patients attained urinary continence and so they were referred for a continence procedure. Nine patients did not have adequate bladder capacity for bladder neck repair (mean bladder capacity 63 ml, range 20 to 80). In those with suitable capacity mean capacity was 119 ml (range 85 to 180) and they underwent bladder neck reconstruction at a mean age of 4.9 years. Of the 25 patients who underwent bladder neck repair 14 (56%) were dry during the day and night, 5 (20%) were dry during the day but wet at night and 6 (24%) were totally incontinent. Pelvic osteotomies were performed at initial closure in 14 totally continent patients (100%) and in 4 (80%) with daytime continence but in no totally incontinent patients. All continent patients underwent hypospadias repair before age 1 year and none required ureteral reimplantation before bladder neck repair., Conclusions: A number of patients require bladder neck reconstruction to achieve continence after successful initial closure with complete primary repair. The modified Young-Dees-Leadbetter technique provides reasonable results with daytime and nighttime dryness attained by more than half of the patients.

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.

Purpose: Successful initial surgical management of bladder exstrophy does not always lead to continence. We evaluated the ultrastructure of the exstrophic bladder using electron microscopy (EM) at various stages of reconstruction to determine whether morphology could correlate with the potential for continence., Materials and Methods: Bladder specimens obtained from 32 patients undergoing various stages of exstrophy reconstruction were evaluated by EM. Specimens were obtained at primary newborn closure (group 1-10), reclosure following failure (group 2-2), bladder neck reconstruction (group 3-9) and augmentation cystoplasty (group 4-11). Evaluation was performed by a single anatomist with experience with EM. Biopsies were separated into those with good, intermediate or poor ultrastructural parameters and then correlated clinically., Results: In group 1, 4 children had good and 2 had intermediate parameters. All showed increased bladder volumes at followup. Four patients had poor parameters and poor bladder growth. The 2 group 2 children had poor parameters and bladder growth. In group 3, 7 of 9 patients had good and 1 had intermediate parameters. Eight of the 9 patients are doing well. Only 3 of the 11 group 4 children had good parameters and an additional 2 had intermediate parameters., Conclusions: Ultrastructural evaluation can identify changes in the bladder that may portend a poor prognosis for eventual continence. Although the correlation was good at bladder closure, some patients with good parameters still had failed reconstruction. Since this is a small study, we continue to recommend reconstruction in all patients who have an adequate bladder template.

Purpose: Bladder exstrophy is a rare malformation affecting only 1 female out of every 5 patients. In the female initial closure is combined with reconstruction of the outer genitalia, and urinary continence can be achieved by some girls without the need for later bladder neck reconstruction. We evaluated the management and outcome of failed initial closures in the female exstrophy population., Materials and Methods: We performed a retrospective database review of patients with the exstrophy complex. Females with classic bladder exstrophy with failure of initial closure were identified. Age at initial closure, use of osteotomies, reasons for failure and number of closures, as well as definitive treatment and long-term outcomes were evaluated., Results: Of 71 females with classic bladder exstrophy 14 had failure of initial closure. Of these patients 1 had undergone initial closure at our institution and 13 were referred for reclosure. Mean followup was 6.5 years (range 3 to 12) and mean age was 10 years (4 to 14). The patients underwent a maximum of 3 closures (mean 2.4). Initial osteotomies were performed in 4 patients, no osteotomy in 8 and status was unknown in 2. Reason for initial failure was dehiscence in 11 patients and prolapse in 3. Five patients underwent a second closure elsewhere. On referral reclosure was successful using osteotomies in all patients. Bladder neck reconstruction was done in 5 patients (3 are daytime continent) and continent diversion in 4 (all are dry). The other patients are awaiting final treatment., Conclusions: The single most important step to achieve urinary continence is successful initial bladder and posterior urethral closure. Pelvic osteotomies ensure a tension-free closure and enhance bladder outlet resistance. Radical mobilization of the vesicourethral complex allows placement of the bladder deep within the pelvis. Failure of the initial closure in the female exstrophy population has a severe impact on long-term outcome and quality of life.

What's known on the subject? and What does the study add? Continent urinary diversion with bladder augmentation is an established method of providing urinary continence in children with bladder exstrophy, who are not suitable candidates or have a failed bladder neck reconstruction. Sub-mucosal implantation of the tubularized catheterizable stoma (usually the appendix) into the reservoir, with backing typically provided by either the bladder musculature or colonic taenia, is safe and highly effective in these children., In some cases of classic bladder exstrophy and in the majority of patients with cloacal exstrophy, the ileum is used for enterocystoplasty and therefore there is no taenia to back the implanted catheterizable channel. This study describes the steps for providing a reliable flap-valve mechanism for the continent catheterizable channel using the serosal trough technique., Objectives To evaluate the efficacy and potential complications of the serosal-trough ( ST) technique for the implantation of a continent catheterizable stoma ( CCS) during enterocystoplasty., To describe the surgical technique and provide detailed illustrations., Patients and Methods Using an institutional review board-approved departmental database, children with bladder exstrophy, born after 1990, were selected, and patients who had undergone urinary diversion with a CCS created using the ST technique were identified., Demographic and technical characteristics, as well as the eventual clinical outcomes, were retrospectively reviewed., Results A total of 135 patients with urinary diversion were identified, of whom 26 (13 males) had undergone CCS implantation using the ST technique. Patients included 14 classic exstrophies, 10 cloacal exstrophies, and two epispadias., The appendix and tapered ileum were used for the creation of a CCS in 11 and 15 patients, respectively. The median (range) age at creation of a CCS was 10.7 (4.4-17.4) years. At the time of CCS creation, 21 patients underwent initial enterocystoplasty, four had repeat augmentations, and one had a CCS on a previously augmented bladder., Ileum (mean length 18 cm) was used in 24/25 augmentations and was selected owing to lack of redundant sigmoid in 52% of patients and intraoperative surgeon preference in the remaining cases. In one case of cloacal exstrophy, a hindgut remnant was used., In 24 (92%) cases, initial CCS resulted in complete continence of the catheterizable channel. After a median (range) of 2.5 (0.2-7.5) years' follow-up all patients were dry via intermittent catheterization. The CCS failed at postoperative months 6 and 21 and required complete revision in two cases., Conclusions Using a ST to provide a strong backing for a catheterizable channel is an excellent option when a channel must be placed in the ileum, hindgut, or in an area of augmentation where muscular backing is not available., The ST technique provides a reliably catheterizable tunnel, durable continence mechanism and a good success rate when creating a CCS in combination with a urinary diversion. [ABSTRACT FROM AUTHOR]

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]

Abstract: Objective: In a series of failed exstrophy closures, to identify determinants of successful repeat closure and the impact of failed closure on the fate of the lower urinary tract and continence status. Patients and methods: We performed a retrospective review of operative notes and medical records of patients with a history of one or more failed exstrophy closures in 1978–2007. The primary surgical endpoints were failure rate of repeat closure attempts, mode of continence surgery and continence outcome. Continence was defined as achieving a dry interval of >3h and voiding through the urethra. Results: We identified 122 patients (85 male/37 female) who had undergone repeat closure following failure. The success rate of repeat closure attempts at our institution was 98%. Of the 94 patients who had undergone successful repeat closure, definitive continence management and had their dryness evaluated, 38 were candidates for bladder neck reconstruction and 17(18%) were continent. Of the remaining patients, 90% were able to attain dryness, but at the expense of clean intermittent catheterization and continent urinary diversion. Conclusion: A failed exstrophy closure has significant implications for long-term surgical outcome. Reclosure can be accomplished in the majority of cases. In comparison to patients with successful primary closure, the rates of urethral continence following successful repeat closure were lower. [Copyright &y& Elsevier]

Purpose: The complications of lower urinary tract reconstruction have been well documented in children with neurogenic bladders. While most series include small numbers of nonneurogenic diagnoses, this group is typically underrepresented. Despite a number of fundamental anatomical and functional differences, a direct comparison of surgical complications of lower urinary tract reconstruction in patients with neurogenic vs nonneurogenic bladders has not been performed. Materials and Methods: We identified patients undergoing lower urinary tract reconstruction incorporating enterocystoplasty from 1996 to 2006. We performed a retrospective review of operative notes and medical records of patients who met inclusion criteria. Patients were divided into a neurogenic group and a nonneurogenic group based on the underlying diagnosis. The 2 groups were compared with respect to demographics, historical data, operative techniques, perioperative morbidity, long-term complications and need for surgical revision. Results: Of the 127 patients who met inclusion criteria 72 were assigned to the nonneurogenic group and 55 to the neurogenic group. Overall the rates of significant perioperative morbidity (39%), long-term complications (54%) and need for surgical revision (39%) were substantial. The rates of catheter related complications, rehospitalization for dehydration and spontaneous bladder rupture were higher in the neurogenic group (p <0.05). Conclusions: Reconstruction of the lower urinary tract in children is associated with a considerable rate of complications and need for surgical revision regardless of whether the bladder is neurogenic or nonneurogenic. Children with neurogenic bladders are more prone to spontaneous rupture, catheter mishaps and early rehospitalization for dehydration. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

Classic bladder exstrophy is characterized by displaced pelvic floor musculature and significant skeletal and genitourinary defects. A paucity of data exist evaluating long-term pelvic floor function in exstrophy patients after ureterosigmoidostomy. This study is an initial attempt to evaluate the prevalence of urofecal incontinence, pelvic organ prolapse, and overall quality of life in patients who have had ureterosigmoidostomies. Fifty-two individuals who underwent ureterosigmoidostomy between 1937 and 1990 were identified through the Ureterosigmoidostomy Association and the Johns Hopkins bladder exstrophy database and mailed questionnaires approved by the Institutional Review Board (Johns Hopkins). Data were analyzed with SigmaStat 3.0 (SPSS, Inc., Chicago, IL). Eighty-three percent of the subjects responded, with a mean age of 44.4 years (range, 14–73 years) and mean of 40.9 years (range, 14–65 years) after ureterosigmoidostomy. Prevalence of daily urinary and fecal incontinence was 48% (n = 20) and 26% (n = 11), respectively, whereas the prevalence of weekly combined urofecal incontinence was 63% (n = 27). The incidence of pelvic organ prolapse in this cohort was 48% (n = 20). In these patients, a significant risk of urofecal incontinence and pelvic organ prolapse exists. Long-term follow-up studies are needed to understand the role of pelvic floor musculature in this complex birth defect. [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]

Introduction: Failed primary closure of the exstrophic bladder is a serious complication with significant impact on the continence outcomes and stability of the urinary tract. Pelvic ostetomy is a critical component of repeat closure as it facilitates a tension-free approximation of the pubis and abdominal wall, and gives the best chance for a successful outcome. This study examines the success rates and complications of repeat pelvic osteotomy(RPO) following failed closure of patients with classic bladder and cloacal exstrophy. Methods: The authors analyzed patient data from our institutionally-approved exstrophy database and identified all bladder and cloacal exstrophy patients who have undergone RPO in conjuction with repeat exstrophy closure. Within this select group we analyzed patient history, complications, and orthopedic outcomes. Results: 75 patients who underwent RPO were identified from the database, 56% male and 44% female. In 83% of patients, RPO was performed prior to 36 months of age. Mean time from initial to repeat osteotomy was 20 months. Anterior inominate osteotomy was utilized in 41% of cases, while combined transverse-anterior inominate/vertical iliac comprised 40% of cases. All cloacal exstrophy patients remain closed, while 5(7%) of the bladder exstrophy patients required repeat bladder closure. Minor complications, such as pin site infections or loose hardware, were observed in 10 patients. 3 patients had major infections of the hardware, and 3 patients had non-union of their pelvic bones after osteotomy. 63 patients now walk with a normal gait, 10 ambulate with assistance or with a limp, while only 2 are non-ambulatory. Conclusions: A failed exstrophy closure is a serious complication which significantly affects the functional outcome of the child. When performed in conjunction with repeat pelvic osteotomy and proper immobilization, secondary closure can be successful and safely performed, and major post-osteotomy complications are uncommon. [Copyright &y& Elsevier]

: ObjectivesAfter initial closure, all exstrophy patients have vesicoureteral reflux. This reflux is usually managed with antimicrobial prophylaxis, surveillance, and ureteral reimplantation concurrent with bladder neck plasty. Patients with recurrent urinary tract infections or worsening hydronephrosis may require earlier correction of reflux. This subset of patients was reviewed to determine the ability to correct reflux adequately, the difficulty with subsequent bladder neck plasty, and the long-term continence results.: MethodsWe reviewed an exstrophy database of more than 700 patients and identified 19 who underwent ureteral reimplantation independent of bladder neck plasty. All had undergone bladder closure in infancy. Eleven had since undergone modified Young-Dees-Leadbetter bladder neck plasty. Five patients were awaiting bladder neck reconstruction, 2 girls were continent without bladder neck plasty, and one had undergone augmentation.: ResultsIndications for early ureteral reimplant were recurrent febrile infections despite adequate prophylaxis (n = 15) or worsening hydronephrosis on follow-up (n = 4). In the 11 patients who had undergone subsequent bladder neck repair, prior reimplantation did not increase the operative difficulty or complications. The continence results among these 11 were comparable with those in patients without prior reimplantation. In 1 patient, reflux recurred and was corrected at the time of bladder neck plasty. None had further urinary tract infections, and hydronephrosis improved in three and stabilized in one.: ConclusionsIn patients who present with recurrent infections or worsening hydronephrosis after initial exstrophy closure, early reimplantation can be undertaken. If performed with later bladder neck plasty in mind, the surgical treatment of incontinence is not compromised and recurrent infections and upper tract changes will be abated. [Copyright &y& Elsevier]