Search

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

Introduction: Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies., Objectives: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias., Methods: IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded., Results: A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness., Discussion: This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR., Conclusion: Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence., Competing Interests: Conflicts of interest None., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: Pelvic osteotomies relieve tension of the bladder and fascial closures during bladder exstrophy repair. Multiple techniques for postoperative immobilization of the pelvis and lower extremities have been described. The primary aim of this study was to assess differences in short and long-term changes in pubic rami diastasis when comparing Bryant traction to spica cast immobilization. Secondary aims included a comparison of length of stay, skin-related complications, and urologic outcomes., Methods: We performed a single-institutional retrospective review of bladder exstrophy patients younger than 18 months of age who underwent posterior pelvic osteotomy and bladder exstrophy closure from April 2005 to April 2020. Short-term and long-term pubic rami diastasis were defined as postoperative measurements ≤6 months and ≥12 months, respectively. Secondary outcomes included length of stay, pressure ulcer, skin rash/abrasion, urethrocutaneous fistula, and bladder or fascial dehiscence rates. Multivariable logistic regression assessed for an association between immobilization type and degree of diastasis while controlling for age at the time of diastasis measurement and sex., Results: Fifteen patients underwent Bryant traction and 36 patients underwent spica cast immobilization. In both the short-term and long-term, there was a greater reduction in pubic diastasis in the spica cast group ( P = 0.002 and P = 0.05, respectively). After adjustments, there were higher odds of having a greater reduction in pubic rami diastasis in both the short-term (odds ratio: 2.71, 95% CI: 1.52-4.86, P = 0.001) and long-term (odds ratio: 2.41, 95% CI: 1.00-5.80, P = 0.05). Length of stay was significantly higher in Bryant's traction group (26 vs 19 d, P < 0.001). Rates of pressure ulcers were higher in the Bryant traction group (26.7% vs 0%, P = 0.005). Rates of skin rash/abrasions, urethrocutaneous fistula, and bladder/fascial dehiscence did not differ., Conclusions: Spica cast immobilization is a safe and effective immobilization method. Compared with Bryant traction, spica cast immobilization was associated with a greater reduction in postoperative pubic diastasis both short and long-term, along with a shorter length of hospitalization and reduced rate of pressure ulcers., Level of Evidence: Level III-therapeutic study., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Individuals with bladder exstrophy-epispadias complex (EEC) need long-term integrated medical/surgical and psychosocial care. These individuals are at risk for medical and surgical complications and experience social and psychological obstacles related to their genitourinary anomaly. This care needs to be accessible, comprehensive, and coordinated. Multiple surgical interventions, reoccurring hospitalizations, urinary and fecal incontinence, extensive treatment regimens for continent diversions, genital differences, and sexual health implications affect the quality of life for the EEC patient. Interventions must include psychosocial support, medical literacy initiatives, behavioral health services, school and educational consultation, peer-to-peer opportunities, referrals to disease-specific camps, mitigation of adverse childhood events (ACEs), formal transition of care to adult providers, family and teen advisory opportunities, and clinical care coordination. The priority of long-term kidney health will necessitate strong collaboration among urology and nephrology teams. Given the rarity of these conditions, multi-center and global efforts are paramount in the trajectory of improving care for the EEC population. To achieve the highest standards of care and ensure that individuals with EEC can thrive in their environment, multidisciplinary and integrated medical/surgical and psychosocial services are imperative., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Introduction: Detrusor contraction in bladder exstrophy (BE) patients following reconstruction is poorly understood as there are few published studies assessing urodynamic findings in this population. Understanding the ability of the detrusor to contract in BE patients early after closure may be able to inform the longer-term management and potential for the development of future continence in this population., Objective: We sought to evaluate early detrusor contraction using urodynamic studies (UDS) in children who had previously undergone complete primary repair of bladder exstrophy (CPRE). We hypothesized that a majority of children with BE would display the presence of normal detrusor contractile function after CPRE., Study Design: A retrospective review of our prospectively collected database was performed for all patients with a diagnosis of classic BE who underwent primary CPRE between 2013 and 2017. From this cohort we identified patients with at least one post-operative UDS at 3 years of age or older who had undergone an initial CPRE. Our primary outcome was the presence of a detrusor contraction demonstrated on UDS., Results: There were 50 children (31 male, 19 female) with CBE who underwent CPRE between 2013 and 2017.There were 26 (13 male, 13 female) who met inclusion criteria. Median age was 3.5 (IQR: 3.2-4.7) years at the time of UDS Sixteen of the 26 (61.5%) generated a sustained detrusor contraction generating a void, with a median peak voiding pressure of 38 cm H20 (IQR: 28-51). The median bladder capacity reached was 48 ml, which represented a median of 30% of expected bladder capacity. The median post void residual (PVR) for the entire cohort was 26 ml (IQR: 9, 47) or 51% (IQR: 20%-98%) of their actual bladder capacity, while the median PVR for those children with a sustained detrusor contraction was 18 ml (IQR: 5, 46) or 33% (IQR: 27%, 98%) of their actual bladder capacity. Intraoperative bladder width and bladder dome to bladder neck length did not correlate with the presence of voiding via a detrusor contraction (p = 0.64)., Discussion: We present the first study assessing early UDS finding of detrusor contraction in BE patients after CPRE. In our cohort, 61.5% of patients were able to generate a sustained detrusor contraction on UDS which is a higher percentage than has been reported in previous series. A difference in initial surgical management may account for these findings., Conclusion: At short term follow up, the majority of children in our cohort were able to produce sustained detrusor contractions sufficient to generate a void per urethra with a modest post void residual volume. Long-term follow-up and repeated UDS will be needed to track detrusor contractility rates, bladder capacities, compliance, post void residuals and ultimately continence rates over time., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: ERAS (enhanced recovery after surgery) protocols are designed to optimize perioperative care and expedite recovery. Historically, complete primary repair of bladder exstrophy has included postoperative recovery in the intensive care unit and extended length of stay. We hypothesized that instituting ERAS principles would benefit children undergoing complete primary repair of bladder exstrophy, decreasing length of stay. We describe implementation of a complete primary repair of bladder exstrophy-ERAS pathway at a single, freestanding children's hospital., Materials and Methods: A multidisciplinary team developed an ERAS pathway for complete primary repair of bladder exstrophy, which launched in June 2020 and included a new surgical approach that divided the lengthy procedure into 2 consecutive operative days. The complete primary repair of bladder exstrophy-ERAS pathway was continuously refined, and the final pathway went into effect in May 2021. Post-ERAS patient outcomes were compared with a pre-ERAS historical cohort (2013-2020)., Results: A total of 30 historical and 10 post-ERAS patients were included. All post-ERAS patients had immediate extubation ( P = .04) and 90% received early feeding ( P < .001). The median intensive care unit and overall length of stay decreased from 2.5 to 1 days ( P = .005) and from 14.5 to 7.5 days ( P < .001), respectively. After final pathway implementation, there was no intensive care unit use (n=4). Postoperatively, no ERAS patient required escalation of care, and there was no difference in emergency department visits or readmissions., Conclusions: Applying ERAS principles to complete primary repair of bladder exstrophy was associated with decreased variations in care, improved patient outcomes, and effective resource utilization. Although ERAS has typically been utilized for high-volume procedures, our study highlights that an enhanced recovery pathway is both feasible and adaptable to less common urological surgeries.

Purpose: False-positive urine pregnancy screening tests (UPST) have been reported among patients with bowel-containing urinary reconstruction (BCUR). However, the true frequency of such inaccurate results, which have been attributed to urinary mucous or other proteins interfering with or mimicking the binding of beta-HCG in the assay, is unknown in this population. We sought to determine the incidence of false-positive pregnancy screening tests among this patient population at our institution., Materials and Methods: Using existing databases of patients with spina bifida, bladder exstrophy, and genitourinary rhabdomyosarcoma, we identified female patients with BCUR who had UPST over a 10-year period as screening prior to procedures or imaging. Patient and test result information was recorded., Results: A total of 120 patients with a history of BCUR were identified: 33 with spina bifida, 73 within the exstrophy-epispadias complex (EEC), and 14 with genitourinary rhabdomyosarcoma. Of this group, 46 patients (38%) had at least one UPST during the study period; 15 had 1 UPST, 6 had 2 UPSTs, 4 had 3 UPSTs, and 21 had greater than 3 UPSTs, for a total of 244 UPST in this cohort. UPSTs used at our institution included Sure-Vue brand and Alere brand (HCG sensitivity 20 mIU/ml). Types of BCUR included ileal enterocystoplasty in 25 patients, colon enterocystoplasty in 6, stomach enterocystoplasty in 5, composite enterocystoplasty in 7, and continent catheterizable channel alone (e.g. Yang-Monti, appendicovesicostomy) in 3 patients. Of the 244 UPSTs in patients with BCUR, zero (0%) were positive., Conclusions: Despite reports in the literature that false-positive UPST are common among patients with bowel-containing urinary diversions, we found no positive UPST among patients with BCUR in the healthcare setting. False-positive UPST in the home setting may be due to variability in sensitivity thresholds, binding agents, technical errors in test technique, kit quality control, or other factors., Competing Interests: Conflicts of interest The authors have no financial interests to disclose., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: To examine the prenatal diagnosis rates of bladder exstrophy (BE) and Omphalocele-Exstrophy-Imperforate anus-Spinal Defect Syndrome (OEIS) in a large cohort of patients over a 20-year period. We hypothesized that prenatal diagnosis rates improved over time due to evolving techniques in fetal imaging., Methods: A multi-institutional database was queried to identify BE or OEIS patients who underwent primary closure between 2000 and 2020. We retrospectively determined prenatal or postnatal diagnosis. Those with unknown prenatal history were excluded. Multivariable logistic regression was used to investigate temporal pattern in rate of prenatal diagnosis while adjusting for sex and treating institution., Results: Among 197 BE and 52 OEIS patients, 155 BE and 45 OEIS patients had known prenatal history. Overall prenatal diagnosis rates of BE and OEIS were 47.1% (73/155) and 82.2% (37/45), respectively. Prenatal diagnosis rate was significantly lower in BE compared to OEIS (P <.0001). The prenatal diagnosis rate for BE significantly increased over time (OR 1.10; [95%CI: 1.03-1.17]; P = .003). Between 2000 and 2005, the prenatal diagnosis rate of BE was 30.3% (10/33). Between 2015 and 2020, prenatal diagnosis rate of BE was 61.1% (33/54). Prenatal diagnosis rate for OEIS did not change over time. Rates of prenatal diagnosis did not differ by sex or treating institution., Conclusion: Rates of prenatal diagnosis of BE and OEIS are higher than previously reported. Prenatal diagnosis rate of BE doubled in the last 5 years compared to the first 5 years of the study period. Nonetheless, a significant proportion of both BE and OEIS patients remain undiagnosed prior to delivery., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Introduction: The complete primary repair of bladder exstrophy (CPRE) aims to restore normal anatomy through complete mobilization and reapproximation of the bladder neck and proximal urethra., Methods: The Peña stimulator has previously been used to identify musculature in the pelvis. The device is now used to distinguish intersymphyseal bands from pelvic floor and urethral sphincteric musculature during CPRE. The ability to distinguish the levator ani muscles from fibrotic bands assists the surgeon in identifying the appropriate extent of deep pelvic dissection during CPRE., Results: During the anatomic bladder neck recreation, the muscle stimulator demonstrated the striated muscle at the level of the bladder neck and urethral sphincter., Conclusion: Localization of the muscles at the bladder neck and proximal urethra corroborates the concept that pelvic floor physical therapy could enhance the strength of the pelvic floor in children with bladder exstrophy., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: To determine the prevalence of prostatic metaplasia in an expanded cohort of transmasculine individuals undergoing gender-affirming resection of vaginal tissue., Methods: Institutional Review Board approval was obtained. Clinical records were reviewed for all transmasculine individuals undergoing vaginal tissue resection at our institution between January 2018 and July 2021. Corresponding pathology specimens were examined grossly and microscopically, including immunohistochemical stains for NKX3.1, prostate-specific antigen (PSA), and androgen receptor (AR). Vaginal specimens from three patients without androgen supplementation were used as controls., Results: Twenty-one patients met inclusion criteria. The median age at surgery was 26.4 years (range 20.6-34.5 years). All patients had been assigned female gender at birth and lacked endocrine or genetic abnormalities. All were on testosterone therapy; median duration of therapy at surgery was 4.4 years (range 1.4-12.1 years). In the transmasculine group, no gross lesions were identified. Microscopically, all specimens demonstrated patchy intraepithelial glandular proliferation along the basement membrane and/or nodular proliferation of prostate-type tissue within the subepithelial stroma. On immunohistochemical staining, performed for a subset of cases, the glandular proliferation was positive for NKX3.1 (16/16 cases; 100%), PSA (12/14 cases; 85.7%), and AR (8/8 cases; 100%). Controls showed no evidence of prostatic metaplasia., Conclusion: One hundred percent of vaginal specimens obtained from transmasculine individuals on testosterone therapy (21/21 cases) demonstrated prostatic metaplasia. Further investigation is warranted to characterize the natural history and clinical significance of these changes. Patients seeking hormone therapy and/or gender-affirming surgery should be counseled on the findings and their yet-undetermined significance., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background and Study Objective: 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., Study Design: 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., Results: 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)., Discussion: 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., Conclusions: 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., Competing Interests: Conflicts of interest None., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Classic bladder exstrophy in the female results in an exstrophic bladder and urethra, an anterior introitus with a bifid clitoris and short labia minora. During closure, the lower abdominal wall is closed and the bifid clitori are brought into close apposition, but are often not completely closed to prevent injury to the clitoral bodies, thus leaving a persistent gap between the clitoral bodies that grows over time. We demonstrate a vertical z plasty closure to provide a 2 layer closure of the mons that decreases tension and improves cosmetic appearance by recreating a clitoral hood that provides a more normal appearance of the external genitalia for girls with bladder exstrophy., Competing Interests: Conflicts of interest There are no conflicts of interest., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction/background: Bladder exstrophy patients have a high prevalence of inguinal hernia that often become clinically evident following bladder closure. Understanding when the bladder exstrophy patient is under greatest risk of developing an inguinal hernia following bladder closure is important, since incarceration resulting in strangulation of intra-abdominal contents can lead to significant morbidity if not addressed in a timely fashion. Although the incidence and risk factors of inguinal hernia have been reported, the timing of occurrence is not well understood., Objective: The primary objective of this study was to assess the timing of inguinal hernia following complete primary repair of bladder exstrophy (CPRE). In addition, we aimed to evaluate possible risk factors associated with inguinal hernia, including sex, age at bladder closure and iliac osteotomy status., Study Design: A multi-institutional retrospective review identified patients with bladder exstrophy repaired by CPRE under 6 months of age while excluding those who underwent inguinal hernia repair before or during bladder closure. Timing of inguinal hernia following bladder closure was evaluated using Kaplan-Meier methods. Cox proportional hazards model was used to investigate association of sex, age at bladder closure, and osteotomy on the risk of developing of inguinal hernia while clustering for institution., Results: 91 subjects were included in our analysis with median follow-up time of 6.5 years. 34 of 53 males (64.2%) and 2 of 38 females (5.3%) underwent inguinal hernia repair. The median time to inguinal hernia was 4.7 months following closure. The greatest hazard of inguinal hernia was within the first six months following closure. In multivariate analysis, male sex was strongly associated with inguinal hernia (HR = 19.00, p = 0.0038). Osteotomy and delay in closure were not significantly associated with inguinal hernia. 7 of 36 patients (19.4%) who underwent inguinal hernia repair presented with recurrence on the ipsilateral side., Discussion: Our results suggest that the greatest risk of inguinal hernia is within the first six months following bladder closure. The decreased risk of inguinal hernia after one year of follow-up may reflect anatomic stability that is reached following major reconstruction of the pelvis. While male bladder exstrophy patients are significantly more susceptible to inguinal hernias following CPRE, osteotomy and delayed bladder closure do not appear to be protective factors for inguinal hernia development following initial bladder closure., Conclusions: There is a heightened risk of inguinal hernia in the first six months following closure. The rate of recurrence following inguinal hernia repair is significantly elevated compared to the general pediatric population., Competing Interests: Conflicts of interest The authors have no conflicts of interest relevant to this article to disclose., (Published by Elsevier Ltd.)

Purpose: Management of infants with OEIS complex is challenging and not standardized. Expeditious surgery after birth has been recommended to limit soilage of the urinary tract and optimize intestinal function. However, clinical instability secondary to comorbidities is common in this population and early operation carries risk. We sought to define the risk/benefit profile of delaying repair., Methods: All newborn patients with OEIS managed by our institution between Sep 2017 and Oct 2019 were reviewed. Comorbidities were evaluated, including cardiopulmonary pathologies and associated malformations., Results: Ten patients with OEIS were managed. Patients underwent early (2 patients, repair at 0-2 days) or delayed (6 patients, repair at 6-87 days) first-stage exstrophy repair. Two patients died prior to repair (progressive respiratory failure, severe genetic anomalies). Repairs were delayed secondary to cardiac conditions, neurosurgical interventions, medical disease, and/or delayed transfer. Delayed repair patients had longer lengths of stay and use of parenteral nutrition. No patients experienced urinary tract infections prior to repair., Conclusions: Delaying first-stage exstrophy repair to allow physiologic optimization is safe. All repaired patients were discharged home, without parenteral nutrition or supplemental oxygen.

Introduction: Cloaca malformation repair strategy is strongly dictated by common channel and urethral lengths. Mid to long common channel cloacas are challenging and often require laparotomy for dissection of pelvic structures. The balance of common channel and urethral lengths often dictates the approach for reconstruction. Laparoscopy has been utilized for rectal dissection but not for management of the urogenital (UG) structures. We hypothesized that laparoscopy could be applied to UG separation in reconstruction of cloaca malformations. Methods: Records were reviewed for 9 children with cloaca who underwent laparoscopic rectal mobilization and UG separation. Clinical parameters reviewed included demographics, relevant anatomic lengths, operative duration, transfusion requirements, and perioperative complications. Results: Repair was perfomed at a median (interquartile range) age of 12 (7, 15) months. Common channel length as measured by cystoscopy was 3.5 (3.3, 4.5) cm. There were no intraoperative complications. Transfusion requirements were minimal. Postoperative length of stay was 6 (5, 11) days. One patient developed a urethral web and 2 developed vaginal stenosis. One patient later underwent a laparotomy for obstruction due to a twisted rectal pull-through. Conclusions: Laparoscopic rectal mobilization and UG separation in long common channel cloaca are safe and well tolerated. Laparoscopy affords full evaluation of Mullerian structures and enables separation of the common UG wall, which may ultimately enhance long-term urinary continence.

Morbidity and mortality associated with pediatric necrotizing fasciitis are strongly dependent on early diagnosis and timely intervention. Yet, the lack of early cutaneous findings and nonspecific symptoms may result in initial delayed diagnosis or misdiagnosis. Infants may be particularly at risk of missed or delayed diagnosis due to inherent barriers in communication and rarity of the condition, especially among healthy patients. We describe 2 cases of necrotizing fasciitis following routine genitourinary surgery in healthy infants., (Published by Elsevier Inc.)

Introduction: Prenatal determination of bladder exstrophy (BE) or cloacal exstrophy (CE), known also as the omphalocele-exstrophy-imperforate anus-spinal anomaly complex (OEIS), is challenging. Distinguishing between BE and CE is important because children with CE have many more challenges initially and during their lifetime. An accurate diagnosis is critical when counselling expectant parents. We hypothesized that there are key imaging features that can distinguish BE from CE, and that there are areas of diagnostic concordance and discordance between fetal ultrasound (fUS) and fetal MRI (fMRI) among these entities., Materials and Methods: We queried a single institutional IRB-approved registry of children with BE and CE to identify those with accessible fetal imaging from 2000 to 2018, and formal interpretations were collected. Two pediatric radiologists performed independent retrospective blinded review of the images. Criteria evaluated included: genitalia, kidneys, bowel appearance, presence of anal dimple, location of insertion of umbilical cord into the abdomen relative to the abdominal wall defect, umbilical vessels, bladder protuberance, presence of omphalocele, and spine/neural cord abnormalities. We evaluated concordance between radiologic interpretations and postnatal diagnosis, as well as between specific findings in the two diagnostic modalities., Results: Twenty-one infants born between 2000 and 2018 with BE or CE had fetal imaging for review: 15 had both fUS and fMRI, 2 had fUS alone, and 4 fMRI alone. There was 100% concordance between fUS and fMRI in evaluating kidneys, presence of anal dimple, location of abdominal insertion of umbilical cord relative to the defect, number of umbilical vessels, and spine abnormalities/level of neural cord termination. The following discrepancies were observed: 1) genitalia and bowel appearance, and bladder protuberance in 1/15 (6.7%); 2) presence of an omphalocele in 2/15 (13.3%). Of the initial radiologic interpretations, 4/17 (23.5%) of fUS and 2/19 (10.5%) of fMRI erroneously were interpreted as on the OEIS spectrum when the post-natal diagnosis was BE. Errors in diagnosis were due to a protuberant bladder plate extending beyond the plane of the abdominal wall with bowel loops posteriorly mimicking an omphalocele. In all of these BE cases, the abdominal wall defect was located inferior to the umbilical cord insertion on the abdominal wall., Conclusion: An everting bladder plate with bowel loops posterior to the plate in classic BE may be misdiagnosed as CE. Identification of the location of umbilical cord insertion relative to the abdominal wall defect, with fetal US or MRI, results in the correct differentiation between BE and CE., Competing Interests: Conflicts of interest There are no conflicts of interest, (Copyright © 2020 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: The Multi-Institutional Bladder Exstrophy Consortium (MIBEC) was established in 2013 to refine technical aspects of the complete primary repair of bladder exstrophy (CPRE), to decrease complications, and to improve outcomes. In order to place outcomes from the consortium into context of historic outcomes, we evaluated continence and dry intervals in children who were repaired prior to the beginning of the consortium at these institutions. We hypothesized that continence (voiding with dryness) is rarely achieved after primary CPRE and surgery following CPRE improves dryness but may hinder voiding per urethra., Materials and Methods: We reviewed prospectively maintained IRB approved databases of children who underwent CPRE for classic bladder exstrophy (BE) between 5/1993 and 1/2013 at 3 institutions. Exclusion criteria included: lack of continence documentation, and lack of follow up subsequent to January 2014. We recorded age at closure, method of bladder emptying, bladder capacity and surgical history. We used a 3 part dryness scale for both patients who void volitionally and those on CIC. Children were considered dry if they could hold urine for over 3 h. An intermediate group was defined as having a dry interval of 2-3 h, with minimal dampness in between voids. If dry intervals were <2 h with frequent leakage, children were considered wet., Results: A total of 54 of 73 (38 M) children met inclusion criteria. 35 of 54 (64.8%) children void per urethra, while 18 (33.3%) perform clean intermittent catheterization (CIC) and 1 underwent a vesicostomy due to incomplete emptying and UTI's. 25/35 (71.4%) of those voiding per urethra underwent CPRE only, while the remaining 10 underwent secondary continence procedures. In total, 26 of 54 (48.1%) are dry, that is either continent or not wet for > 3 h, while only 11/54 (20.4%) are truly continent, i.e., voiding per urethra. Only 9/54 (16.7%) are continent after a single surgery (CPRE). 14/54 children who are dry (25.9%) underwent a bladder neck procedure with or without augmentation and empty with CIC., Discussion and Conclusion: Granular detail about the specifics of emptying, surgical history, and dry intervals is crucial to understand the true outcomes from the repair of BE. Children with BE undergoing CPRE prior to the institution of MIBEC experienced variable results, with only 17% achieving continence while spontaneously voiding per urethra without additional reconstruction. We are now engaged in MIBEC to identify factors that contribute to continence and to attempt to render such findings reproducible., (Copyright © 2020 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Historically after complete primary repair of exstrophy (CPRE) in girls, it had been more likely to observe urinary incontinence than retention. Following recent technical modifications of elongating the urethra and narrowing the bladder neck, the authors have observed a high rate of urinary retention in girls after CPRE., Objective: The aim was to identify factors that may be responsible for this observation by reviewing historical and current outcomes. The authors hypothesized that differences in anatomic dimensions at the time of CPRE may contribute to urinary retention., Study Design: A retrospective review of girls who underwent CPRE from December 1998 through September 2016 from a single institution was performed. Patients were deemed in retention if their clinical course was consistent with such, required a procedure to relieve urinary retention, and/or required clean intermittent catheterization., Results: Nineteen girls underwent CPRE during this period. In 2012, a change to delaying CPRE to approximately 2 months of age was made, and this led the authors to divide their experience into CPRE performed as a newborn (<72 h of age, 8 patients) versus delayed (>72 h, 11 patients) subgroups. There were no girls with retention in the newborn group and three (38%) girls with retention in the delayed group. In the delayed group, girls had a longer urethral plate and narrower bladder neck compared with the newborn group. Long-term outcomes greater than 9 years are available for six girls in the newborn group and two (33%) required bladder neck procedures for incontinence. None in the delayed group have required incontinence procedures; however, follow-up is limited at 25 months., Discussion: The absence of retention in the newborn group is concerning for the delayed group incurring a higher risk of retention after CPRE. This may be secondary to excessive compression of the urethra at the time of pubic symphysis approximation potentially leading to urethral ischemia. Different from the newborn CPRE girls, additional technical revision of CPRE, namely, elongation of the urethra and the dissection it involves and narrowing of the bladder neck, may increase the risk for retention., Conclusions: The multiple factors that were identified as potential contributors to post-CPRE urinary retention should result in a cautious reevaluation of female bladder exstrophy management at the time of CPRE. The authors now create a gradual tapered transition at the bladder neck and, similar to their previous experience, a more generous (wider) bladder neck and a shorter length for urethral plate., (Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Pre-operative physical examination of male epispadias allows for classification of epispadias level as glanular (GE), penile (PE) or penopubic (PPE), and for delineation of anatomic anomalies. The incidence of associated extragenital abnormalities, such as vesicoureteral reflux (VUR), bladder neck (BN) abnormality and abnormal pubic diastasis (PD), and their impact on urinary continence has not yet been systematically studied., Objective: The goal of this study was to evaluate whether the more proximal level of epispadias correlated with associated extragenital anatomic anomalies seen on initial imaging or endoscopic evaluation, and whether these pre-operative findings contributed to subsequent surgical management and impacted on achieving urinary continence. It was hypothesized that the more severe forms of epispadias may be associated with a higher frequency of associated anomalies., Study Design: The study was an IRB-approved, retrospective case study of all male patients treated initially for isolated epispadias at the current institution between 1994 and 2011. Data collection was achieved by chart and radiology review evaluating PD, BN appearance, presence of VUR, surgical treatment, and urinary continence., Results: A total of 26 patients were identified and divided into three groups based on appearance at physical examination: four glanular (GE), eight penile (PE), and 14 penopubic (PPE); 17 patients had an abnormal BN. Reflux was noted in nine of 20 patients who had a voiding cystourethrogram (VCUG), two of which had an episode of pyelonephritis. Of the 22 patients past the age of toilet training, 17 were continent (64% (9/14) penopubic, 63% (5/8) penile, and 75% (3/4) glanular)., Discussion: Anatomic classification for male epispadias did not provide sufficient information regarding extragenital findings. This study provided new information regarding PD, BN appearance, presence of reflux, and ultimate urinary continence. Pubic diastasis and BN abnormalities were more frequently seen in more severe forms of epispadias, whereas VUR seemed more prevalent in less severe forms. A template for pre-operative evaluation was outlined. Limitations of the study were its retrospective design and relatively small cohort of patients, which reflected the rarity of the condition., Conclusion: Based on the information generated, additional anatomic information was generated regarding boys with epispadias. This information will help guide the evaluation and the management of these patients in the future., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction/background: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care., Objective: Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term., Study Design: Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures., Results: From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below., Discussion: Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence., Conclusion: This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing., (Copyright © 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management., Objective: This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function., Study Design: A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Children's Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion., Results: Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis (Summary Table). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering., Discussion: Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis., Conclusions: This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD., (Copyright © 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: For bladder exstrophy repair it is universally accepted that successful initial surgery is paramount to achieve the optimal outcome. Gaining the necessary surgical experience is challenging due to the rarity of bladder exstrophy. We report preliminary findings of a multi-institutional collaboration created to increase experience and proficiency with the care of bladder exstrophy., Materials and Methods: Our 3 institutions alternatively served as the host site for scheduled surgeries with observation, commentary and critique by visitors from the other sites. The technique was complete primary repair with bilateral iliac osteotomy. The timing of complete primary repair at age 1 to 3 months facilitated collaboration. We recorded patient demographics and outcomes, and the impact of this collaboration on our technique and experience. Video recording was used for real-time observation and teaching, and future analysis, editing and review., Results: A total of 16 site visits occurred from February 2013 through May 2014. Complete primary repair was performed in 9 males and 7 females with bladder exstrophy. Median age at complete primary repair was 2 months (range 0.1 to 28.8). Median followup was 8.9 months (range 2.8 to 18.2). All closures were successful with no dehiscence. Complications included urethrocutaneous fistula in 2 patients, 1 episode of pyelonephritis in 3 each and urethral obstruction in 2 females, of whom 1 required clean intermittent catheterization., Conclusions: We report a multi-institutional collaboration to standardize the surgical management of bladder exstrophy. This effort increased the annual experience of each institution involved from threefold to ninefold and it has accelerated the physician knowledge base to ultimately benefit patient care., (Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: We evaluated the relationship between testicular volume differential, total testis volume and total motile sperm count in adolescents with varicocele. Prior reports have been inconclusive regarding the association of testis volume with semen analysis parameters., Materials and Methods: We retrospectively reviewed a single institution database of Tanner 5 adolescents with nonoperated left varicocele with at least 1 semen analysis available. Patients were included in analysis if they had clinical left varicocele, ultrasound measurement of testis volume and no prior inguinal or scrotal surgery., Results: We analyzed the records of 100 patients. Mean ± SD age at presentation was 15.2 ± 3.5 years. The mean testicular volume differential was 8% ± 23% and 31 patients (31%) had a greater than 20% testicular volume differential. Mean total testis volume was 30.4 ± 13.3 cc. Varicocele was grade 3 in 39 patients, grade 2 in 50, grade 1 in 8 and ungraded in 3. Median total motile sperm count in the cohort was 42.0 (IQR 9.2, 105.8). ROC analysis with the Youden index indicated an optimal total testis volume cutoff of 30 cc to predict the total motile sperm count. In a multivariable logistic model accounting for age and varicocele grade a testicular volume differential of greater than 20% was associated with twice the odds of a total motile sperm count of less than 20 million per cc (OR 2.1, 95% CI 1.02-4.12, p = 0.04). Total testis volume less than 30 cc more than quadrupled the odds of a total motile sperm count of less than 20 million per cc (OR 4.2, 95% CI 1.8-9.7, p <0.001)., Conclusions: Total testis volume and the testicular volume differential are associated with semen analysis outcomes in adolescents with varicocele. A testicular volume differential of greater than 20% doubles the odds of a low total motile sperm count. A total testis volume of less than 30 cc quadruples the odds of a low total motile sperm count., (Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Augmentation cystoplasty using gastrointestinal segments in children/adolescents with medically refractory neurogenic bladder is associated with significant complications. We evaluated an autologous cell seeded biodegradable scaffold (Tengion®) for bladder augmentation as an alternative to traditional enterocystoplasty in this population., Materials and Methods: A phase II prospective study was performed in children with neurogenic bladder due to spina bifida requiring enterocystoplasty for detrusor pressure 40 cm H2O or greater despite maximum antimuscarinic medication. Following open bladder biopsy, urothelial and smooth muscle cells were grown ex vivo and seeded onto a biodegradable scaffold to form a regenerative augment as the foundation for bladder tissue regeneration. Bladder neck sling was the only concomitant surgical procedure permitted. Bladders were cycled postoperatively to promote regeneration. Primary and secondary outcomes at 12 months included change in bladder compliance, bladder capacity and safety. Long-term assessment was done with similar outcomes at 36 months., Results: Compliance improved in 4 patients at 12 months and in 5 patients at 36 months, although the difference was not clinically or statistically significant. There was no clinical or statistical improvement in bladder capacity at 12 or 36 months in any patient. Adverse events occurred in all patients, and most were easily treated. Two patients had low cell growth following bladder biopsy, of whom 1 withdrew from the study and 1 underwent a second biopsy. Serious adverse events of bowel obstruction and/or bladder rupture occurred in 4 patients., Conclusions: Our autologous cell seeded biodegradable scaffold did not improve bladder compliance or capacity, and our serious adverse events surpassed an acceptable safety standard., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Concern in patients with bladder exstrophy after reconstruction regarding potential injury to pelvic neurourological anatomy and a resultant functional deficit prompted combined (simultaneous) cystometrography and electromyography after complete primary repair of bladder exstrophy. We determined whether complete primary repair of bladder exstrophy would adversely affect the innervation controlling bladder and external urethral sphincter function., Materials and Methods: Complete primary repair of bladder exstrophy was performed via a modified Mitchell technique in newborns without osteotomy. Postoperative evaluation included combined cystometrography and needle electrode electromyography via the perineum, approximating the external urethral sphincter muscle complex. Electromyography was done to evaluate the external urethral sphincter response to sacral reflex stimulation and during voiding., Results: Nine boys and 4 girls underwent combined cystometrography/electromyography after complete primary repair of bladder exstrophy. Age at study and time after complete primary repair of bladder exstrophy was 3 months to 10 years (median 11.5 months). Cystometrography revealed absent detrusor overactivity and the presence of a sustained detrusor voiding contraction in all cases. Electromyography showed universally normal individual motor unit action potentials of biphasic pattern, amplitude and duration. The external urethral sphincter sacral reflex response was intact with a normal caliber with respect to Valsalva, Credé, bulbocavernosus and anocutaneous (bilateral) stimulation. Synergy was documented by abrupt silencing of external urethral sphincter electromyography activity during voiding., Conclusions: After complete primary repair of bladder exstrophy combined cystometrography/electromyography in patients with bladder exstrophy showed normal neurourological findings, including sacral reflex responses, sustained detrusor voiding contraction and synergic voiding, in all patients postoperatively. These findings confirm the safety of complete primary repair of bladder exstrophy. Based on our results we have discontinued routine electromyography in these patients., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Research on health related quality of life in patients with bladder exstrophy has demonstrated mixed results compared to population samples. Few studies have had a comparison group with a urological disorder and none correlated body image to health related quality of life. We compared health related quality of life in patients with bladder exstrophy to that in patients with kidney stones and correlated body image to health related quality of life in the bladder exstrophy population., Materials and Methods: Participants included 24 patients with kidney stones and 24 with bladder exstrophy. Patient age was 8 to 25 years. Participants completed the PedsQL (Pediatric Quality of Life Inventory) and the 25-item multiple choice UBIQ (Urological Body Image Questionnaire), which was created for the current study., Results: Compared to patients with kidney stones those with bladder exstrophy indicated significantly higher scores on physical functioning (p = 0.0005) and overall health related quality of life (p = 0.02) in the 8 to 17-year-old cohort. When health related quality of life was compared to body image in patients with bladder exstrophy, lower social scores were associated with those who worried about friends finding out about the condition (p = 0.01) and about having romantic relationships (p = 0.003). Lower social (p = 0.006) and emotional (p = 0.009) functioning scores were associated with patients who thought that they were less likely to have romantic relationships., Conclusions: The finding that patients with bladder exstrophy had a better physical functioning score than those with kidney stones is not surprising, given the morbidity of active stone disease. The negative correlations with body image in the bladder exstrophy population may be due to the possibility of others discovering the condition and the perceived likelihood of not having romantic relationships., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Substantial variability exists in bladder exstrophy care, and little is known about costs associated with the condition. We define the care patterns and first year cost for patients with bladder exstrophy at select freestanding pediatric hospitals in the United States., Materials and Methods: We used the Pediatric Health Information System database to identify patients with bladder exstrophy born between January 1999 and December 2010 who underwent primary closure in the first 120 days of life. Demographic, surgical, postoperative and cost data for all encounters were assessed. Multivariate linear regression was used to examine the association between patient, surgeon and hospital characteristics and costs., Results: Of the 381 patients who underwent primary closure within the first 120 days of life 279 (73%) did so within the first 3 days of life. A total of 119 patients (31%) underwent pelvic osteotomy, including 51 of 279 (18%) who underwent closure within the first 3 days of life, 38 of 67 (56%) who underwent closure between 4 and 30 days of life, and 30 of 35 (86%) who underwent closure between 31 and 120 days of life (p = 0.0017). Median inflation adjusted, first year cost in United States dollars per patient was $66,577 (IQR $45,335 to $102,398). Presence of nonrenal comorbidity and completion of primary closure after 30 days of life increased first year costs by 24% and 53%, respectively. Increased post-closure length of stay was associated with greater costs., Conclusions: At select freestanding United States pediatric hospitals the majority of bladder exstrophy closures are performed within the first 3 days of life. Most, but not all, patients undergoing closure after the neonatal period undergo osteotomy. The presence of nonrenal comorbidity and increased postoperative length of stay are associated with greater costs., (Copyright © 2014 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Anorectal malformation (ARM) is a common birth defect but the developmental history and the underlying molecular mechanism are poorly understood. Using murine genetic models, we report here that a signaling molecule Dickkopf-1 (Dkk1) is a critical regulator. The anorectal and genitourinary tracts are major derivatives of caudal hindgut, or the cloaca.Dkk1 is highly expressed in the dorsal peri-cloacal mesenchymal (dPCM) progenitors. We show that the deletion of Dkk1 causes the imperforate anus with rectourinary fistula. Mutant genital tubercles exhibit a preputial hypospadias phenotype and premature urethral canalization.Dkk1 mutants have an ectopic expansion of the dPCM tissue, which correlates with an aberrant increase of cell proliferation and survival. This ectopic tissue is detectable before the earliest sign of the anus formation, suggesting that it is most likely the primary or early cause of the defect. Deletion of Dkk1 results in an elevation of the Wnt/ß-catenin activity. Signaling molecules Shh, Fgf8 and Bmp4 are also upregulated. Furthermore, genetic hyperactivation of Wnt/ß-catenin signal pathway in the cloacal mesenchyme partially recapitulates Dkk1 mutant phenotypes. Together, these findings underscore the importance ofDKK1 in regulating behavior of dPCM progenitors, and suggest that formation of anus and urethral depends on Dkk1-mediated dynamic inhibition of the canonical Wnt/ß-catenin signal pathway., (© 2013 Published by Elsevier Inc.)

Purpose: We evaluated post-operative bleeding complications in non-newborns following use of monopolar versus bipolar electrocautery for circumcision or revision circumcision., Materials and Methods: We retrospectively reviewed sequentially performed cases of circumcision and revision circumcision performed by nine pediatric urologists at our institution from 2005 to 2010. In order to incorporate both the monopolar and bipolar electrocautery experience for a single surgeon employing bipolar technique, sequential cases from 2002 to 2010 were reviewed. Variables assessed included age, procedure, method of electrocautery, skin approximation and dressing, and bleeding complications., Results: 1810 patients that underwent either circumcision or revision circumcision were reviewed. Complete data was available for 1617 patients. Age at operation was a mean 3.7 ± 4.9 yrs and median 1.5 yrs. Return for bleeding complication for all surgeons, was 2/336 (0.6%) for bipolar and 28/1281 (2.2%) for monopolar (p = 0.0545). For the single surgeon using bipolar technique, returns were 2/336 (0.6%) for bipolar and 5/309 (1.6%) for monopolar (p = 0.2133). Returns per procedure type were 1/200 (0.5%) bipolar and 24/844 (2.8%) monopolar for primary circumcision (p = 0.0513), and 1/136 (0.7%) bipolar and 4/437 (0.9%) monopolar (p = 0.84) for revision. Four of 1617 (0.2%) patients returned to the operating room [4/1281 (0.3%) monopolar (p = 0.31)]. There was no difference in return to the operating room for circumcision versus revision., Conclusion: Return for bleeding complications after circumcision and revision circumcision occurred more frequently after monopolar electrocautery compared to bipolar. However, there was no significant difference between the two electrocautery methods. Either form of electrocautery appears to be effective for this common pediatric urologic procedure., (Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Although research in patients with urological conditions indicates that functional limitations, pain, limited mobility and social restrictions can impact self-image, to our knowledge the influence of the visibility of the condition has not been examined. We assessed body image and self-image at school age through young adulthood in patients with bladder exstrophy compared to patients with kidney stones., Materials and Methods: Patients diagnosed with bladder exstrophy and kidney stones completed a 25-item multiple choice Urological Body Image Questionnaire for ages 13 to 25 years, which was created for the current study. It consists of questions on physical appearance, avoidance behaviors, urological condition disclosure, romantic relationships and future expectations. A modified Urological Body Image Questionnaire for patients 8 to 12 years old was created by removing questions on relationships and future expectations., Results: A total of 24 patients with bladder exstrophy and 24 with kidney stones 8 to 25 years old completed the questionnaire. Compared to patients with kidney stones, patients with bladder exstrophy were significantly more likely to avoid changing clothes in front of peers and to not tell friends about the condition (each p = 0.0001). Patients with bladder exstrophy were also significantly more likely to think that the condition would prevent having future romantic relationships (p = 0.002) and children (p = 0.003)., Conclusions: Differences in the visibility and privacy of the 2 conditions likely explain the discrepancy between the groups. Findings may call for an individual approach to assessing issues related to self-esteem and identity in patients with bladder exstrophy and may highlight the importance of receiving psychosocial support., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Ureteral complications of renal transplantation can dramatically impact renal outcomes. We studied whether complications are associated with preexisting genitourinary pathology or transplant using a deceased donor allograft., Materials and Methods: We retrospectively reviewed all patients undergoing renal transplantation at our institution between 2000 and 2010. We abstracted patient demographic details, donor type (living vs deceased), end-stage renal disease etiology, reimplant technique, stent use, preoperative and postoperative imaging, history of lower genitourinary pathology and postoperative complication management., Results: A total of 211 kidneys were transplanted into 206 patients (mean age 13.7 years, mean followup 4.6 years). Most patients (89%) underwent extravesical ureteroneocystostomy without stenting (97%), with roughly half (47%) of transplants being from living donors. Preexisting urological pathology was present in 34% of cases. Postoperative obstruction or extravasation occurred in 16 cases (7.6%), of which 15 were acute. Complications were not associated with donor type, preexisting urological pathology other than posterior urethral valves, surgical technique, etiology of end-stage renal disease or patient age. However, posterior urethral valves or other preexisting genitourinary pathology was not associated with an increased likelihood of genitourinary complications. Posterior urethral valves were associated with development of postoperative vesicoureteral reflux (OR 6.7, p = 0.004) but were not associated with stent placement, surgical technique, donor type or etiology of end-stage renal disease., Conclusions: Patients with posterior urethral valves undergoing renal transplantation are at increased risk for postoperative vesicoureteral reflux but not for other acute surgical complications. There is no association between donor type, etiology of end-stage renal disease, surgical technique or patient age and increased complications., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Separating digestive and urinary outlets is a critical step during mammalian embryogenesis. However, the natural history of these structures is poorly studied, and little is known about their embryonic origin. Here, we show that peri-cloacal mesenchymal (PCM) progenitors are the major source of these structures. Surprisingly, PCM progenitors also contribute to perineum, a structural barrier separating the urinary and digestive tracts, suggesting a potential role of PCM progenitors in establishing independent urinary and digestive outlets. We demonstrate that Six1 and Six2 are complementarily but asymmetrically expressed in the PCM progenitors. Deletion of these genes results in decreased cell survival and proliferation, and consequently in agenesis of the perineum and severe hypoplasia of the genital tubercle. Together, these findings suggest that PCM progenitors are the unexpected source of perineum and genital tubercle, and establish a basic framework for investigating normal and abnormal development of anorectal and genitourinary structures.

Purpose: Currarino syndrome is an inherited disorder consisting of a triad of anorectal anomaly, sacrococcygeal defect and presacral mass. We evaluated the urological issues in patients with Currarino syndrome and sought to determine whether spinal cord detethering improves urinary tract function., Materials and Methods: We retrospectively reviewed 14 patients diagnosed with Currarino syndrome. We evaluated urinary signs/symptoms and urodynamic findings before and after spinal cord detethering., Results: All patients with Currarino syndrome having a sacral defect and presacral mass were diagnosed between birth and 6 years. Of the patients 86% had a tethered spinal cord that was surgically detethered between 8 months and 6 years (average 3 years). Overall 10 of 12 children who underwent surgery had voiding complaints postoperatively, including urgency, frequency and incontinence. Five patients had recurrent urinary tract infections, of whom 3 had vesicoureteral reflux that resolved spontaneously. Three patients had mild unilateral hydronephrosis without reflux. Ten of 12 patients who underwent spinal cord detethering underwent comprehensive urodynamic evaluation. Of the 5 patients who underwent preoperative and postoperative urodynamic evaluation 3 showed improvement with resolution of detrusor overactivity or dyssynergia postoperatively, and 2 demonstrated no change. Of the 5 patients who underwent only postoperative urodynamic evaluation 4 had abnormal findings, including small capacity, poor compliance, detrusor overactivity, detrusor sphincter dyssynergia and/or high voiding pressure. No progressive denervation was seen on electromyography preoperatively or postoperatively., Conclusions: Currarino syndrome is a rare congenital disorder with few published reports regarding the long-term implications. Although no solid conclusions could be drawn regarding urodynamic improvement postoperatively due to our small sample size, spinal cord detethering did not lessen ongoing voiding complaints in the study patients., (Copyright © 2012 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: Augmentation cystoplasty is the mainstay of surgical treatment for medically refractory neurogenic bladder in patients with spina bifida. Concerns regarding an increased risk of malignancy have prompted many centers to consider routine postoperative screening. We examine the potential cost-effectiveness of such screening., Materials and Methods: A Markov model was used to compare 2 screening strategies among patients with spina bifida after cystoplasty, namely annual screening cystoscopy and cytology and usual care. Model parameters were informed via a systematic review of post-augmentation malignancy and cost estimates from published reports or government sources., Results: In a hypothetical cohort the individual increase in life expectancy for the entire cohort was 2.3 months with an average lifetime cost of $55,200 per capita, for an incremental cost-effectiveness ratio of $273,718 per life-year gained. One-way and two-way sensitivity analyses suggest the screening strategy could be cost effective if the annual rate of cancer development were more than 0.26% (12.8% lifetime risk) or there were a greater than 50% increase in screening effectiveness and cancer risk after augmentation. After adjusting for multiple levels of uncertainty the screening strategy had only an 11% chance of being cost effective at a $100,000 per life-year threshold or a less than 3% chance of being cost effective at $100,000 per quality adjusted life-year., Conclusions: Annual screening for malignancy among patients with spina bifida with cystoplasty using cystoscopy and cytology is unlikely to be cost effective at commonly accepted willingness to pay thresholds. This conclusion is sensitive to a higher than expected risk of malignancy and to highly optimistic estimates of screening effectiveness., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: A major goal of bladder exstrophy management is urinary continence, often using bladder neck reconstruction. We report our experience with bladder neck reconstruction after complete primary repair of exstrophy., Materials and Methods: Patient history, ultrasound, voiding cystourethrogram, examination using anesthesia and urodynamics were performed during a prospective evaluation. Continence was assessed using the International Children's Continence Society classification and the dry interval. Bladder capacity was measured by examination using anesthesia, voiding cystourethrogram and/or urodynamics. Urodynamics were also done to assess bladder compliance and detrusor muscle function., Results: From 1994 to 2010 we treated 31 male and 15 female patients with bladder exstrophy after complete primary repair of exstrophy. Of patients 5 years old or older bladder neck reconstruction was performed after complete primary repair in 9 of 21 males (43%) and in 3 of 11 females (27%) at a mean age of 6.3 and 8.1 years, respectively. By the International Children's Continence Society classification 6 of 12 patients (50%) were continent less than 1.5 years after bladder neck reconstruction and 2 of 9 (23%) were evaluable 1.5 years or greater after reconstruction. Median bladder capacity was 100 ml before, 50 ml less than 1.5 years after and 123 ml 1.5 years or greater after bladder neck reconstruction. Three males and 2 females emptied via an appendicovesicostomy. Two boys underwent augmentation., Conclusions: In our experience most patients with bladder exstrophy require bladder neck reconstruction after complete primary repair of exstrophy. The need for reconstruction is more common in males. Our rates of bladder neck reconstruction after complete primary repair of exstrophy and of continence after bladder neck reconstruction are similar to those in other reports., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Purpose: We investigated associated urodynamic abnormalities in toilet trained children with vesicoureteral reflux., Materials and Methods: A total of 298 toilet trained children with primary vesicoureteral reflux underwent urodynamic evaluation. Urodynamic parameters were reviewed and correlated with age, gender, presence of lower urinary tract symptoms and reflux severity., Results: Symptomatic lower urinary tract symptoms were present in 111 children (37.2%, group 1). Children with lower urinary tract symptoms had significantly decreased severity of vesicoureteral reflux compared to children without these symptoms (187 patients, group 2). The majority of the patients had normal early bladder compliance regardless of presence of lower urinary tract symptoms or reflux grade. On the other hand, decreased late bladder compliance was more common in group 1 vs group 2. Ratio of cystometric bladder capacity to expected bladder capacity was higher in group 2. Detrusor overactivity was observed in 28.5% of the children, and the incidence was significantly higher in group 1 vs group 2, and in mild vs moderate or severe reflux. Dysfunctional voiding from bladder sphincter dyscoordination was seen in 32% of children 2.5 to 4 years old with vesicoureteral reflux and lower urinary tract symptoms, compared to 8% in children 5 to 16 years old., Conclusions: The presence of lower urinary tract symptoms in children with vesicoureteral reflux correlated well with some urodynamic findings suggestive of overactive bladder and negatively correlated with reflux severity. In contrast, dysfunctional voiding was more common in younger children with reflux and lower urinary tract symptoms. These findings suggest that treatment of voiding dysfunction should be directed toward the specific type of abnormality in children with vesicoureteral reflux., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

A major goal of bladder exstrophy (BE) management is achieving urinary continence, most commonly with surgical bladder neck reconstruction (BNR). This is a report of outcome of BNR after complete primary repair of exstrophy (CPRE). At our institution, patient history, ultrasound, cystogram (VCUG) and urodynamic study (UDS) were performed during a prospective evaluation of patients with BE. Dry interval of >3 hours was used as the definition of continence and dry interval <1-hour incontinence. Bladder capacity was measured at VCUG and/or UDS. UDS was also used to assess bladder compliance. From 1994 to 2010, we cared for 47 BE patients (31 male, 15 female) after CPRE. For patients ≥3 years after CPRE, BNR was performed in 9 of 22 (41%) male and 3 of 11 (27%) female patients. Mean age at BNR was 6.3 and 5.9 years for male and female patients, respectively. The mean (±SD) bladder capacity pre-BNR was 104.8 (±20.4 mL). There was a significant increase in capacity from pre-BNR to ≥1.5 years post-BNR (P = 0.013) and from <1.5 and ≥1.5 years post-BNR (P = 0.002). In conclusion, most patients with BE require BNR after CPRE. The need for BNR is more common in male patients., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: Laparoscopic pyeloplasty is one of the more common robotic assisted procedures performed in children. However, data regarding long-term experience and clinical outcomes for this procedure are limited. We evaluated the long-term outcomes in a large series of patients undergoing robotic assisted laparoscopic pyeloplasty at a teaching institution, and the effect of a collaborative program between the robotic surgeons, surgical nurses and anesthesiologists on overall operative time., Materials and Methods: We retrospectively reviewed 155 patients who underwent robotic assisted laparoscopic pyeloplasty between 2002 and 2009. Operative data, including surgical approach, type of procedure, total and specific operative times and placement of ureteral stents, were determined. Postoperative outcome measurements, including duration of hospital stay, duration of Foley catheter drainage, radiological findings and any subsequent complications, were assessed., Results: Mean operative time and length of hospitalization decreased significantly by the end of the study. At a mean followup of 31.7 months the primary success rate was 96% (hydronephrosis was improved in 85% of patients and stable in 11%). The complication rate was 11%, and recurrent obstruction requiring redo robotic assisted laparoscopic pyeloplasty or open pyeloplasty developed in 3% of patients. Success rate and complication rate were unchanged during the study period., Conclusions: This study confirms that even within the confines of a pediatric urology training program successful collaboration between robotic surgeons, surgical nurses and anesthesiologists can lead to shorter operative times and hospital stays. Long-term surgical success and complication rates were comparable to open surgery., (Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Objective: Urethral strictures are among the most common complications after hypospadias repair. We report our 10-year experience with endoscopic incision or dilation of urethral strictures after hypospadias repair, to determine the best management technique., Methods: All cases of urethral strictures after hypospadias repair treated with direct vision internal urethrotomy (DVIU), dilation or urethroplasty at our institution from 1997 to 2007 were included. Records were reviewed and clinical parameters analyzed. Data were statistically analyzed to identify risk factors for stricture recurrence after initial or subsequent treatment(s)., Results: Of 2273 patients, 73 were treated for a postoperative urethral stricture and 15 others were referred for stricture treatment. Of these 88 patients, 39 were treated with initial dilation or DVIU and 49 underwent urethroplasty or reoperative hypospadias repair. Fifteen (38%) of the patients treated with initial DVIU or dilation showed no recurrence. Of the patients that did have a recurrence, a repeat DVIU or dilation had a success rate of 17% with no difference in success between these two groups. Choice of therapy between repeat dilation/DVIU and urethroplasty at the second procedure showed a statistically significant higher success rate in the urethroplasty group (67% vs 17%, P=0.03)., Conclusion: Although numbers are small, our data suggest that if there is recurrent stricture after initial DVIU/dilation then a formal urethroplasty has a significantly higher success rate than repeat DVIU/dilation., (Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: Urinary tract infection will develop in 40% of children who undergo renal transplantation. Post-transplant urinary tract infection is associated with earlier graft loss in adults. However, the impact on graft function in the pediatric population is less well-known. Additionally the risk factors for post-transplant urinary tract infection in children have not been well elucidated. The purpose of this study was to assess the relationship between pre-transplant and post-transplant urinary tract infections on graft outcome, and the risk factors for post-transplant urinary tract infection., Materials and Methods: A total of 87 patients underwent renal transplantation between July 2001 and July 2006. Patient demographics, cause of renal failure, graft outcome, and presence of pre-transplant and post-transplant urinary tract infections were recorded. Graft outcome was based on last creatinine and nephrological assessment., Results: Median followup was 3.12 years. Of the patients 15% had pre-transplant and 32% had post-transplant urinary tract infections. Good graft function was seen in 60% of the patients and 21% had failed function. Graft function did not correlate with a history of pre-transplant or post-transplant urinary tract infection (p >0.2). Of transplanted patients with urological causes of renal failure 57% had post-transplant urinary tract infection, compared to only 20% of those with a medical etiology of renal failure (p <0.001)., Conclusions: In this study there was no correlation between a history of urinary tract infection (either before or after transplant) and decreased graft function. History of pre-transplant urinary tract infection was suggestive of urinary tract infection after transplant. Patients with urological causes of renal failure may be at increased risk for post-transplant urinary tract infection., (Copyright © 2010 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)