Classic Bladder Exstrophy Research Articles

Application of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex

To describe long-term outcomes and experience with the tunica vaginalis flap (TVF) as a tissue augment for complex epispadias repair. A prospectively maintained institutional database was reviewed for patients who underwent epispadias repair with TVF from 2010 to 2021. Evaluation of patient characteristics between those who developed dehiscence or UCF and those who did not was performed via Mann-Whitney U, Kruskal-Wallis, or Fisher's exact test, as appropriate. A total of 47 male patients were identified, of whom, 1 (2.1%) had cloacal exstrophy, 38 (80.9%) had classic bladder exstrophy or a variant, and 8 (17.0%) had epispadias as their primary exstrophy-epispadias complex diagnosis. The median age at epispadias repair was 13 months (IQR 11 - 19). The overall rate of fistula or dehiscence development was 19.1% (9 patients), of whom, 7 developed urethrocutaneous fistulae, 1 dehiscence, and 1 both. Notably, 0 of the 5 patients who had undergone prior epispadias repair and 0 of the 8 patients with a diagnosis of isolated epispadias developed a UCF or dehiscence after repair with TVF. Differences in age at repair, primary EEC diagnosis, prior epispadias repair, pre-repair testosterone, fibrin sealant utilization, closure layers, stent removal time, and bladder capacity at repair were not statistically significant (P>.05) CONCLUSION: Our expanded evaluation indicates that utilization of the tunica vaginalis as an adjunct to epispadias repair may provide durable protection against fistula development in EEC patients who have undergone prior epispadias repair and in primary repair of patients with isolated epispadias.

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

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

Orthopedic complications after osteotomy in patients with classic bladder exstrophy and cloacal exstrophy: a comparative study

The addition of pelvic osteotomy to the armamentarium of tools for correction of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) has undeniably served as one of the most effective advancements in improving the likelihood of successful primary bladder closure. Osteotomy-related complications have been studied and documented extensively in patients with CBE, yet evaluation remains limited in CE concordant with its relative rarity. To compare orthopedic complications in patients with CBE and CE who underwent primary bladder closure with osteotomy. A prospectively maintained, IRB-approved database of 1401 exstrophy-epispadias patients was reviewed for patients with CBE or CE after primary closure and pelvic osteotomy performed at a single institution from 1975 to 2021. Failed closure was defined as dehiscence, bladder prolapse, or vesicocutaneous fistula at any point. Surgery or anesthesia-related complications were captured within 6 weeks of osteotomy or closure. A total of 146 patients were included in the analysis with 109 and 37 patients with CBE and CE, respectively. Between the CBE and CE cohorts, there were significant differences in median age at primary closure (68 days [IQR 10-260] vs 597 [448-734]; p<0.001), diastasis width (4cm IQR [3.8-4.6] vs 6.1 [5.0-7.2]; p<0.001), osteotomy at time of closure (99.1% vs 75.7%; p<0.001), and utilization of external hip fixation (67.9% vs 89.2%; p=0.011). There was no significant difference by gender, osteotomy technique, or hip immobilization technique. Regarding exstrophy closure outcomes, there were 5 failures in the CBE group and 1 in the CE group (p=1.000). Complications were experienced in 38.5% and 56.8% of CBE and CE patients (p=0.054) with a significant difference in orthopedic complications (primarily consisting of superficial pin-site infections) between the cohorts (4.6% vs 16.2%, p=0.031). There was no significant difference in grade 3 or higher complications between cohorts (5.5% vs 13.5%, p=0.147). This was the first study comparing orthopedic complications after osteotomy between CBE and CE, providing valuable insight into which factors vary among cohorts and which are associated with increased complication rates. Despite availability of high case numbers for these rare disorders, the analysis continued to be limited sample size and missing data for retrospective analysis. While exstrophy closure success and overall complications rates are similar in patients with CBE and CE, patients with CE experience more superficial pin-site infections after pelvic osteotomy. External hip fixation may be associated with the increase in orthopedic complications, though further research is required to elucidate the underlying cause of these complications.

Mini-open Anterior Pelvic Osteotomy and Spica Casting for Primary Closure of the Pelvis in Infants With Classic Bladder Exstrophy.

The objective of this study was to investigate the efficacy and safety of mini-open Salter-like anterior pelvic osteotomy followed by spica casting as an adjunct to urological reconstruction in infants with classic bladder exstrophy (CBE). Twenty-eight infants with CBE were operated upon according to the following protocol: initial bilateral mini-open Salter-like osteotomies followed by urological reconstruction and subsequent spica casting for 3 to 4 weeks. Postoperative clinical and radiologic assessments of wound complications and union were done, respectively. During subsequent follow-ups, the foot progression angle (FPA), the interpubic (IP) distance, interischial (IS) distance, and the IS/IP ratio were measured. The Pearson correlation was used to correlate between age at the time of last follow-up, the FPA, the IP and IS distances, and the IS/IP ratio. The mean age at the time of operation was 5.89±0.89 months. The operative procedure took 10 to 15 minutes (mean of 12.5±1.5min) for each side and the blood loss was negligible. Tension-free complete approximation of the symphysis was achieved in all cases. No wound complications were reported in any case. The total duration of the cast was 3.07±0.14 weeks. The mean duration of follow-up was 4.78±3.09 years. Strong positive correlation was found between the IP and IS distances ( r =0.833, P <0.0001) as well as the IS distance and age (r=0.455, P =0.015). Mini-open anterior pelvic osteotomy and spica casting may provide a minimally invasive and effective option for closure of the pelvis in infants with CBE. Level IV-case series.

Exstrophy-Epispadias Complex With Isolated Ectopic Bowel Segment: A Case Series and Literature Review

ObjectiveTo present a case series of the exstrophy-epispadias complex (EEC) with isolated ectopic bowel segment (IEBS) with the literature review, highlighting the clinical findings and treatments. Materials and MethodsWe present 3 cases of bladder exstrophy (BE) with IEBS in our institute and reviewed the literature in PubMed with the terms “(“bladder exstrophy” OR “epispadias”) AND (“visceral sequestration” OR “sequestered” OR “ectopic bowel”).” ResultsThere were 2 males and 1 female. The IEBS was detected by physical examination in 2 cases and by ultrasonography in another one. All cases were BE accompanying with lower abdominal mass which adhered to the bladder wall but was separated from the digestive system. All cases underwent the IEBS excision and BE repair simultaneously. Pathological result of IEBS suggested the histological structures of colon. There were totally 13 cases of EEC with IEBS reported in the literature, including 2 (15%) epispadias, 9 (69%) covered BE, 1 (8%) duplicate BE and 1 (8%) classic bladder exstrophy. Although their clinical manifestations were various, IEBS excision were safely conducted in all cases. ConclusionEEC with IEBS is an extremely rare congenital malformation. Physical and imaging examinations are important for diagnoses. Surgical excision is safe and effective for managing IEBS.

Classical bladder exstrophy in an adult female

Classical bladder exstrophy results from a defective lower abdominal wall with open bladder and urethra, and anteriorly opened bony pelvis [1]. Families with low socioeconomic status, lack of education and limited access to health care in developing countries often neglect treatment of such cases [2].

PD02-08 BLADDER CAPACITY AND GROWTH IN CLASSIC BLADDER EXSTROPHY: A NOVEL PREDICTIVE TOOL

You have accessJournal of UrologyCME1 May 2022PD02-08 BLADDER CAPACITY AND GROWTH IN CLASSIC BLADDER EXSTROPHY: A NOVEL PREDICTIVE TOOL Tamir Sholklapper, Mahir Maruf, Jiafeng Zhu, Jason Michaud, Mohammad Zaman, John Jayman, Roni Manyevitch, Rachel Davis, Wayland Wu, Thomas Harris, Heather DiCarlo, Gayane Yenokyan, and John Gearhart Tamir SholklapperTamir Sholklapper More articles by this author , Mahir MarufMahir Maruf More articles by this author , Jiafeng ZhuJiafeng Zhu More articles by this author , Jason MichaudJason Michaud More articles by this author , Mohammad ZamanMohammad Zaman More articles by this author , John JaymanJohn Jayman More articles by this author , Roni ManyevitchRoni Manyevitch More articles by this author , Rachel DavisRachel Davis More articles by this author , Wayland WuWayland Wu More articles by this author , Thomas HarrisThomas Harris More articles by this author , Heather DiCarloHeather DiCarlo More articles by this author , Gayane YenokyanGayane Yenokyan More articles by this author , and John GearhartJohn Gearhart More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002517.08AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Bladder capacity (BC) is an important metric in the management of patients with classic bladder exstrophy (CBE) and is used to clinically estimate likelihood of continence and determine eligibility for continence procedures, such as bladder neck reconstruction. The Koff equation, which estimates BC from patient’s age has limited applicability in the exstrophy population. We sought to develop a model of maximum bladder capacities for those with CBE using readily available clinical variables. METHODS: An institutional database of CBE patients was reviewed for those who have undergone annual gravity cystogram 6 months after bladder closure. Linear mixed effects models with random intercept and slope were used to construct models predicting log transformed BC and were compared with adjusted R2, Akaike Information Criterion, and cross-validated mean square error. The final model includes outcome of primary closure, sex, log-transformed age at successful closure, time from successful closure, and interaction between outcome of primary closure and log-transformed age at successful closure as the fixed effects with random effect for patient and random slope for time since successful closure. RESULTS: In total, 369 patients (107 female, 262 male) with CBE had at least one BC measurement after bladder closure. Patients had a median of 3 annual measurements (range 1-10). The log bladder capacity increases with the age at cystographic evaluation. Other factors associate with capacity include sex, outcome of primary bladder closure, age at successful bladder closure and age at evaluation. A web based interactive CBE bladder growth nomogram (Figure 1) may be found at https://exstrophybladdergrowth.shinyapps.io/be_app/. CONCLUSIONS: Using readily accessible patient and disease related information, the bladder capacity model in this study provides an accurate prediction of bladder capacity. Using clinical factors specific for the exstrophy population should be considered over age-based Koff equation estimates. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e38 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Tamir Sholklapper More articles by this author Mahir Maruf More articles by this author Jiafeng Zhu More articles by this author Jason Michaud More articles by this author Mohammad Zaman More articles by this author John Jayman More articles by this author Roni Manyevitch More articles by this author Rachel Davis More articles by this author Wayland Wu More articles by this author Thomas Harris More articles by this author Heather DiCarlo More articles by this author Gayane Yenokyan More articles by this author John Gearhart More articles by this author Expand All Advertisement PDF DownloadLoading ...

MP11-14 ACHIEVING GOAL CAPACITY FOR CONTINENCE SURGERY: A CUMULATIVE EVENT ANALYSIS OF BLADDER EXSTROPHY PATIENTS

You have accessJournal of UrologyCME1 May 2022MP11-14 ACHIEVING GOAL CAPACITY FOR CONTINENCE SURGERY: A CUMULATIVE EVENT ANALYSIS OF BLADDER EXSTROPHY PATIENTS Preeya Khandge, Kelly Harris, Wayland Wu, Mahir Maruf, Hiten Patel, Heather Di Carlo, and John Gearhart Preeya KhandgePreeya Khandge More articles by this author , Kelly HarrisKelly Harris More articles by this author , Wayland WuWayland Wu More articles by this author , Mahir MarufMahir Maruf More articles by this author , Hiten PatelHiten Patel More articles by this author , Heather Di CarloHeather Di Carlo More articles by this author , and John GearhartJohn Gearhart More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000002533.14AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Following successful closure of patients with classic bladder exstrophy (CBE), the next major milestone is the establishment of urinary continence. However, prior to determining the appropriate continence surgery, it is imperative to reach an adequate bladder capacity of 100 cc minimum in order to make the decision between bladder neck reconstruction (BNR) and continent stoma with or without augmentation cystoplasty (AC). The authors sought to examine the timing of when patients achieve this threshold bladder capacity to be eligible for BNR. METHODS: An institutional database of 1388 exstrophy patients was retrospectively reviewed for CBE patients after successful primary bladder closure. Bladder capacities was measured via gravity cystography. The cohort was stratified by location, neonatal (≤28 days) or delayed closure period and osteotomy status. The bladder capacities were categorized to either reaching goal or not and a cumulative event analysis was performed. The event being reaching 100cc capacity or greater and time being the number of years between bladder closure and attainment of goal capacity. RESULTS: Two hundred fifty-three patients met inclusion criteria from 1982 to 2019. The majority were of male gender (72.9%), had their closure performed at the authors’ institution (52.5%), within the neonatal period (80.7%), and without an osteotomy (51.7%). 64.9% of patients reached goal bladder capacity. There were no significant differences in those who did or did not achieve goal except for clinical follow up. The cumulative event analysis demonstrated that a median time of 5.73 years (95% CI 5.2 – 6.20) corresponded with a 50% event probability of reaching goal capacity. Cox-proportional hazards showed location of closure was significant associated with hazards of reaching goal bladder capacity (HR = 0.58, CI 0.40 – 0.85, p = 0.005). Based on this model, the median time to event would be 5.20 years (95% CI 4.76 – 5.80) for cases done at the authors’ hospital and 6.26 years for those performed at an outside (95% CI 5.77 – 7.24). CONCLUSIONS: These findings help counsel families appropriately on the odds of attaining goal capacity at various ages. For those who do not reach 100cc by 5 years of age, it helps further characterize the odds of requiring bladder augmentation and the best timing for reconstructive surgery in order to safely gain urinary continence. Families may also be assured that most patients would have the breadth of options when it comes to continence surgery as more than half of patients reached the bladder capacity threshold. Source of Funding: None © 2022 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 207Issue Supplement 5May 2022Page: e162 Advertisement Copyright & Permissions© 2022 by American Urological Association Education and Research, Inc.MetricsAuthor Information Preeya Khandge More articles by this author Kelly Harris More articles by this author Wayland Wu More articles by this author Mahir Maruf More articles by this author Hiten Patel More articles by this author Heather Di Carlo More articles by this author John Gearhart More articles by this author Expand All Advertisement PDF DownloadLoading ...

Insights and outcomes of single-staged repair of female bladder exstrophy–epispadias complex without osteotomy: 15 Years experience of a single institution

Female exstrophy-epispadias complex (BEEC) has been considered as a rare malformation of the genito-urinary tract affecting. Combining procedures during the reconstruction of bladder exstrophy-epispadias complex to reduce the number of procedures and improve the outcomes has evoked great interest. we tried to describe the application and results of a single-stage approach for reconstruction of female BEEC during initial reconstruction or following prior failed bladder closure (FBC). The records of 37 female patients referred for the repair of BEEC without the application of pelvic osteotomies were extracted from an institutionally approved database from September 2002 to August 2018. The mean patient age was 7.24 and 26 patients had a prior FBC. All patients underwent pelvic floor electrical stimulation and toilet training for 1 year after the closure. Complete continence was defined as having the ability to stay dry for more than 3h without leakage during the day and night. Partial continence has traditionally been defined as retaining urine for 1-3h or having some stress incontinence. Incontinence was defined as a continence interval of less than 1h. None of the patients presented bladder prolapse or dehiscence on follow-up; while stricture developed in 2 patients (5.4%). A total of 25 (67.6%) children were dry during the day and night. However, 9 (24.3%) were dry during the day but wet at night; while 3 (8.1%) were totally incontinent. The patients were followed up for a mean of 112.56 months. Although earlier reports of this technique seem encouraging, it should be mentioned that postoperative complications are possible and difficult to manage. However, none of our patients were presented with severe postoperative complications in the follow-ups. The single-stage technique provides satisfactory outcomes in selected patients with classic bladder exstrophy. The majority of patients attained social dryness without bladder augmentation and intermittent catheterization accompanied with minimum complication rate and best cosmetic results.

An evaluation of perioperative surgical procedures and complications in classic bladder exstrophy patients Using the National Surgical Quality Improvement Program-Pediatric (NSQIP-P)

Classic bladder exstrophy (CBE) repair report wide variation in success. Given the complexity of CBE care, benefit would be derived from validation of reported outcomes. Using the National Surgical Quality Improvement Program-Pediatric (NSQIP-P) data, this manuscript evaluates surgical complications for bladder closure and advanced urologic reconstruction in CBE patients. The primary aim of this study was to determine complication rates in the CBE population for bladder closure and advanced urologic reconstruction in national studies compared to single-institutional studies. Pediatric cases and complications were identified in the 2012-2019 NSQIP-P database in CBE patients who had either bladder closure or advanced urologic reconstruction. Bladder closure was further defined as early (<7 days) or delayed (>7 days). Differences were assessed using Fisher's exact test and analysis was conducted using SPSS with significance defined as p-value <0.05. 302 patients were included; 152 patients underwent bladder closure, and 150 patients underwent advanced urologic reconstruction. The 30-day complication rate for bladder closure is 30.3% and for advanced urologic reconstruction is 24.0% in the CBC cohort. No differences were found in the rates of NSQIP complications between early and delayed bladder closure, though significant differences (p<0.001) were found in the rates of blood transfusion (17.9 vs 65.3%). This may be due to the different rates of osteotomy (25.0 vs 48.3%) between early and delayed bladder closure. Rates of readmission are 14.7% and rates of reoperation are 8.0% for advanced urologic reconstruction procedures. Both bladder closure and advanced urologic reconstruction had infectious issues in greater than 10% of the population. CBE surgeries nationally carry a higher risk of complications than is reported in most institutional studies. Infectious complications occur greater than 10% of the time in both bladder closure and advanced urologic reconstruction, which should be the source of additional study given the inverse relationship infections pose to surgical success in BE patients. A limitation of this study is that the data is derived from Children's hospitals that elect to participate and includes only data from 30 days after a procedure. CBE complication data for both bladder closure and advanced urologic reconstruction may be underrepresented in the literature.

Sexual health outcomes after penile reconstruction in the exstrophy–epispadias complex

The penis in exstrophy-epispadias complex (EEC) males is diminutive and patients are often dissatisfied with appearance and length. Due to an increased risk of psychosexual dysfunction, patients desire penile reconstruction. Surgical options include penile lengthening with a full thickness skin graft (SG) or tissue expansion (TE) used for cutaneous coverage or neophalloplasty using a radial forearm free flap or pedicled antero-lateral thigh flap. The aim of this study was to assess sexual health outcomes before and after SG or TE assisted lengthening and neophalloplasty. Patients aged 18 years or older, who underwent penile reconstruction were identified using an institutionally approved database of EEC patients. Patient perception of penile appearance, sexual function, their frequency of sexual intercourse and overall satisfaction with reconstruction were assessed using a survey consisting of validated and non-validated questions. Eighty-three patients underwent penile reconstruction, 57 met the inclusion criteria and 28 responded to the survey (49.1%). Fourteen were reconstructed using a SG, 6 with TE and 8 underwent neophalloplasty. Median time from reconstruction to survey completion was 4.4 years (range 1.2-13.2) Four patients had a diagnosis of epispadias, 1 cloacal exstrophy, 23 classic bladder exstrophy (CBE). Before reconstruction patients were dissatisfied with their penile appearance with a median penile perception score of 4.5 compared to 7.5 after surgery (p=0.0034, Fig.1). Twenty-three patients were dissatisfied with penile length, with 18 reporting an improvement following reconstruction (p=0.0002). There was no correlation in time after reconstruction with PPS or satisfaction with penile length (p=0.86 and p=0.55, respectively). Overall, the median Sexual Health Inventory for Men (SHIM) score was 19, with no difference between the surgical groups (p=0.33). Nine patients engaged in sexual intercourse before reconstruction which increased to 17 afterwards. All three surgical methods improved patient perception of penile appearance and length. Patients who previously never engaged in intercourse were able to and those who had were doing so more frequently, of which a greater proportion were in a relationship. The results are encouraging though must be interpreted with caution due to the small number of responders and possible risk of bias. These findings provide vital information for future patients and routine psychological assessment will help manage patient expectations to improve satisfaction.

Pelvic Floor Anatomical Variations in Children With Exstrophy-Epispadias Complex Using Magnetic Resonance Imaging

ObjectiveTo quantitatively measure the anatomical variations of the pelvic floor in children with exstrophy-epispadias complex using magnetic resonance imaging. Materials and MethodsSix cases of classic bladder exstrophy (CBE), 5 cases of penile epispadias (PE) and 11 cases of penopubic epispadias (PPE) were included. Another 8 cases with the testicular tumor were taken as the controls. A series of measurements obtained from the pelvic floor magnetic resonance imaging were analyzed, and the measurements with significant differences were obtained by ANOVA. ResultsThe pelvic floor of the CBE was significantly different from that of controls in measurements including wider pubic diastasis (P <.001), greater posterior anal distance (P = .019), greater posterior bladder neck distance (P = .004), larger iliac wing angle (P <.001), diminutive ischial angle (P <.001), bigger puborectalis angle (P <.001), larger ileococcygeous angle (P = .002) and shortened anterior corporal length (P <.001). For the PE, the posterior bladder neck distance (P = .038) was greater than that of controls. In the PPE, the posterior bladder neck distance (P = .001) and puborectalis angle (P = .026) was greater than that of controls, respectively. ConclusionCBE shows severe anatomical variations of the pelvic floor. The bladder neck moves more anteriorly both in PE and PPE than the control. The enlarged puborectalis angle resulting from wider pubic diastasis and more anterior position of the anorectal canal is also noticed in PPE.

Bladder exstrophy: Modern staged repair experience in our institution.

Introduction:Bladder exstrophy is a major malformation in paediatric urology. The treatment results are not still completely satisfactory, and their management is an enormous problem in Sub-Saharan Africa. While outlining our challenges, we report our management experience to improve our results.Subjects and Methods:We retrospectively reviewed the records of patients undergoing surgical repair of classic bladder exstrophy at our department between January 2010 and December 2019 (10 years). Epidemiological, clinical, therapeutic and evolution data were analysed.Results:Twenty-five children with classic bladder exstrophy were treated. Our series included 16 boys and 9 girls with a sex ratio of 1.7. Age ranged from 0 day to 6 years. Twenty-five bladder closures were performed, associated to pelvic osteotomy in 11 cases. Epispadias repair was performed on nine boys. Eight cases of bladder neck reconstruction and three cases of bladder enlargement were performed. We observed six bladder fistulas, four wound dehiscence, of which three partial, two parietal suppurations and six cases of urinary tract infection. Eight children had a continence of 1–2 h.Conclusion:The treatment of bladder exstrophy in our context is still limited because of financial difficulties encountered by the population and the insufficient technical platform in our country.

Delayed primary closure of bladder exstrophy without osteotomy: 12 year experience in a safe and gentle alternative to neonatal surgery

BackgroundSuccessful primary closure of bladder exstrophy is of utmost importance for bladder capacity and urinary continence. We evaluated our concept of delayed primary closure that challenges the role of neonatal surgery, pelvic osteotomy, and perioperative pain management. Material and MethodsWe reviewed the medical records of patients with classic bladder exstrophy (CBE) who had undergone delayed primary closure without osteotomy at our institution between January 2008 and May 2020. Data to be analyzed included patient demographics, intraoperative pelvic laxity, blood transfusion, postoperative ventilation time, requirement of pain medication, time to full feeds, length of ICU stay, postoperative complications, and total hospital stay. Results66 patients (44 boys) met the inclusion criteria. Mean age at surgery was 64.8 days (SD±24.7). Pelvic approximation < 5 mm was possible in 66 (100%) patients. Blood transfusion was required by 31 (47%) patients. 14 (21.2%) patients needed postoperative ventilation for a mean time of 2.7 h. 45 (68.2%) children required intravenous opioids in addition to an epidural catheter. Oral feeding started on average 17.6 h after surgery. Mean ICU stay was 1.3 day. The initial success rate of delayed closure was 93.9%. None of the patients had bladder dehiscence. Girls developed more often minor postoperative complications than boys (m/f: 12 [27.3%] vs. 8 [36.4%]. Mean overall time of hospitalization was 19 days (13–34 d). ConclusionDelayed primary closure of CBE without osteotomy but with continuous epidural blockage is a safe and promising procedure that has crucial advantages in the pre- and postoperative management of CBE. Level of EvidenceLevel III.

Delayed surgical treatment of children with exstrophy without approximation of the pubic bones, the use of displaced flaps and immobilization of the patient

BACKGROUND: Bladder exstrophy is one of the most difficult for the reconstruction of malformations.&#x0D; AIM: This study aimed to evaluate the outcomes of delayed bladder closure in exstrophy in comparison with procedures in newborns and to test the hypothesis that the reliability of bladder closure does not decrease without closing the fascial defect between the rectus abdominis muscles and that the postoperative period is more favorable.&#x0D; MATERIALS AND METHODS: Delayed closure of exstrophy without osteotomy, convergence of pubic bones, or flap movement was performed in 46 patients with classic bladder exstrophy. Among them, 34 were primary patients, and the remaining patients, who had a complete relapse of exstrophy as a result of suture divergence, had undergone unsuccessful surgery in other clinics 14 times (n = 12). In total, 25 were boys and 21 were girls who underwent surgery from 2006 to 2021.&#x0D; RESULTS: Delayed bladder closure was successful in all 46 children (100%). No signs of acute pain were noted. Serious complications such as dehiscence of the sutures in the bladder and proximal urethra were not observed. Minor complications occurred in five patients, which resolved promptly.&#x0D; DISCUSSION: In the treatment of exstrophy, successful primary bladder closure is critical to achieving future urinary continence. The elimination of the defect between the rectus abdominis muscles with the convergence of the pubic bones has significant drawbacks, including an increase in the operating time and trauma, need for blood transfusion, risk of orthopedic and neurological complications, and a more difficult postoperative period. The vast majority of the patients develop rediastasis. The authors proposed a more reliable method of delayed closure of exstrophy.&#x0D; CONCLUSIONS: The presented method of bladder closure was 100% successful. The procedure was simplified technically, the surgery time was reduced, and the postoperative period proceeded more easily without prolonged pain syndrome, prolonged use of analgesics, and, in most cases, without blood transfusions.

Rare Variants of Bladder Exstrophy-Epispadias Complex: A Report of Two Cases

Exstrophy-epispadias complex encompasses a spectrum of pathologies ranging from isolated epispadias to classical bladder exstrophy to cloacal exstrophy as the most severe and the rarest variant, are quite uncommon and constitute about 8% of all the cases. The treating surgeon should be aware of all the variants, their diagnosis and modalities of treatment for the same. We present two such unique variants and successful surgical management of the same.

Rare Variants of Bladder Exstrophy-Epispadias Complex: A Report of Two Cases

Exstrophy-epispadias complex encompasses a spectrum of pathologies ranging from isolated epispadias to classical bladder exstrophy to cloacal exstrophy as the most severe and the rarest variant, are quite uncommon and constitute about 8% of all the cases. The treating surgeon should be aware of all the variants, their diagnosis and modalities of treatment for the same. We present two such unique variants and successful surgical management of the same.

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

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

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

OEIS Syndrome: Omphalocele, Exstrophy of the Cloaca, Imperforate Anus, and Spinal Defects

EDITORIAL COMMENT

This is an interesting offering that adds to our knowledge of the problems that can be incurred in managing an exstrophy female during pregnancy. The most interesting vignette is in the lack of uterine prolapse in those patients who had an old fashioned posterior iliac osteotomy. The newer combined transverse innominate and anterior iliac osteotomies with pelvic fixation do a much better job of transferring the major pelvic musculature from behind the anus to a more anterior position to better support the anterior pelvic compartment seen on three-dimensional magnetic resonance imaging (3D MRI). 1 Stec AA Tekes A Ertan G et al. Evaluation of pelvic floor muscular redistribution after primary closure of classic bladder exstrophy by 3-dimensional magnetic resonance imaging. J Urol. 2012; 188: 1535-1542 Crossref PubMed Scopus (23) Google Scholar Also, we have seen very few post Cesarean section prolapses in this group regardless of whether they void from below or have a continent reservoir. We tell these ladies they usually can have one delivery without prolapse but afterwards the risk does increase regardless of having a prior osteotomy. That being said we have performed sacroculpopexies for these women to have subsequent pregnancies if they desire. Although pelvic osteotomies is a morbid procedure in adults, the authors have performed pelvic osteotomies to facilitate uterine suspension in patients with failed prior attempts at repairing uterine prolapse. 2 Ansari MS Gearhart JP Cervellione RM Sponseller PD. The application of pelvic osteotomy in adult female patients with exstrophy: applications and outcomes. BJU Int. 2011; 108: 908-912 PubMed Google Scholar Recently reported 3D MRI-guided surgery combined with pelvic osteotomy may allow for better recapitulation of the pelvic floor thus decreasing the rate or prolapse in adult life. 3 Di Carlo HN Maruf M Massanyi EZ Shah B Tekes A Gearhart JP. 3-Dimensional magnetic resonance imaging guided pelvic floor dissection for bladder exstrophy: a single arm trial. J Urol. 2019; 202: 406-412 Crossref PubMed Scopus (5) Google Scholar More data in the future like this paper is needed as modern exstrophy surgery along with better pelvic reconstructive osteotomies will hopefully decrease the complication rate in this interesting group of patients.