Search

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: 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.)

Introduction and Objective: Staged pelvic osteotomy (SPO) prior to bladder closure has been shown to be a safe and effective method for achieving pubic approximation in cloacal exstrophy (CE) patients with extreme diastasis. However, SPO outcomes have never been compared to those for combined pelvic osteotomy (CPO) at the time of closure in CE patients., Methods: A prospectively maintained database of 1208 exstrophy-epispadias complex patients was reviewed for CE patients treated with pelvic osteotomies. Inclusion criteria were osteotomy at the authors' institution and closure within two months of osteotomy. After inclusion, patients were separated into four groups depending on osteotomy procedure (SPO vs. CPO) and whether their osteotomy occurred with primary closure or re-closure. Patient demographics, closure history, pre-operative diastasis measurement, most recent post-operative diastasis measurement, and outcomes were recorded and compared by chi-squared tests and ANOVA., Results: Among 116 CE patients reviewed, 46 met inclusion criteria. With primary closure or re-closure, 27 had SPO and 19 had CPO. No SPO re-closure patients had previous osteotomy; 4 CPO re-closure patients had a previous osteotomy with closure. Median time between osteotomy and closure in SPO patients was 14 days. Median follow-up after SPO and CPO were 4 and 11 years, respectively. SPO significantly reduced the pre-operative diastasis compared to CPO on most recent diastasis measurement (3.5 cm vs. 0.4 cm, p=0.003). There were no significant differences in the overall complication rate, or the rates of each specific complication, between the SPO and CPO groups. No patients had wound dehiscence or prolapse. One CPO patient was able to intermittently catheterize per urethra while all other patients required continent urinary diversion to achieve continence., Conclusions: To the authors' knowledge, this is the first study comparing SPO and CPO outcomes in CE patients. SPO reduces pre-operative diastasis more than CPO, and does not appear to incur increased rates of complication, closure failure, or incontinence. Due to its apparent safety and greater efficacy, SPO should be considered in all CE patients with extreme diastases undergoing primary closure or re-closure., (Copyright © 2014. Published by Elsevier Ltd.)

Objective: To present the authors' experiences with urologic complications associated with various techniques used to create a continent stoma (CS), augmentation cystoplasty (AC), and neobladder in the exstrophy-epispadias complex (EEC) population., Methods: Retrospective review of medical records of patients who underwent CS with or without bladder augmentation were identified from an institutional review board-approved database of 1208 EEC patients. Surgical indications, tissue type, length of hospital stay, age, preoperative bladder capacity, prior genitourinary surgeries, postoperative urological complications, and continence status were reviewed., Results: Among the EEC patients reviewed, 133 underwent CS (80 male, 53 female). Mean follow-up time after initial continent stoma was 5.31 years (range: 6 months to 20 years). Appendix and tapered ileum were the primary bowel segments used for the continent channel and stoma in the EEC population. The most common stomal complications in this population were stenosis, incontinence, and prolapse. Seventy-nine percent of EEC CS patients underwent AC primarily done with sigmoid colon or ileum. Eleven patients (8%) underwent neobladder creation with either colon or a combination of colon and ileum. Bladder calculi, vesicocutaneous fistula, and pyelonephritis were the most common non-stomal complications. Stomal ischemia was significantly increased in Monti ileovesicostomy compared to Mitrofanoff appendicovesicostomy in classic bladder exstrophy patients (p = 0.036). Furthermore, pyelonephritis was more than twice as likely in colonic neobladder than all other reservoir tissue types in the same cohort (OR = 2.53, 95% CI: 1.762-3.301, p < 0.001)., Conclusions: To the best of the authors' knowledge, this is the largest study examining catheterizable stomas in the exstrophy population. While Mitrofanoff appendicovesicostomy is preferred to Monti ileovesicostomy because it is technically less challenging, it may also confer a lower rate of stomal ischemia. Furthermore, even though ileum or colon can be used in AC with equally low complication rates, practitioners must be wary of potential urologic complications that should be primarily managed by an experienced reconstructive surgeon., (Copyright © 2014 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

The exstrophy-epispadias complex is a rare spectrum of anomalies affecting the genitourinary system, anterior abdominal wall, and pelvis. Recent advances in the repair of classic bladder exstrophy (CBE) and cloacal exstrophy (CE) have resulted in significant changes in outcomes of surgical management (including higher continence rate, fewer surgical complications, and better cosmesis) and health-related quality of life in these patients. These noteworthy changes resulted from advances in the pathophysiological and genetic backgrounds of this disease and better radiologic assessment of the three-dimensional anatomy of the bony pelvis and its musculature. A PubMed search was performed with the keyword exstrophy. The resulting literature pertaining to genetics, stem cells, imaging, tissue engineering, epidemiology, and endocrinology was reviewed. The following represents an overview of the advances in basic science understanding and imaging of the exstrophy-epispadias spectrum and discusses their possible and future effects on the management of CBE and CE., (Copyright © 2013. Published by Elsevier Ltd.)