Multi-Institutional Bladder Exstrophy Consortium After 8 Years: The Short- and Intermediate-Term Outcomes.

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.

Male epispadias repair: Outcomes at three sites prior to the establishment of a multi-institutional collaboration.

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.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

Caudal anesthesia is not associated with post-operative complications following distal hypospadias repair.

INTRODUCTION: The use of caudal anesthesia at the time of hypospadias repair remains controversial as some prior studies have reported increased postoperative complication rates associated with caudal nerve block. However, these results have been called into question due to confounding factors and heterogeneous study groups. OBJECTIVE: Given the importance of identifying true risk factors associated with increased hypospadias complication rate, we examined our experience with caudal anesthesia limiting our analysis to distal repairs. We hypothesized that caudal anesthesia would not be associated with increased postoperative complications. STUDY DESIGN: We retrospectively reviewed our institutional hypospadias database from June 2007 to January 2021. All boys who underwent single-stage distal hypospadias repair with either caudal or penile block with minimum 1 month follow up were included. Records were reviewed to determine the type of local anesthesia, type of hypospadias repair, all complications, and time to complication. Association between any complication and local anesthesia type was evaluated by univariate and multivariate logistic regression analysis controlling for age at surgery and type of repair. A sub-analysis was performed for complications occurring ≤30 days. RESULTS: Overall, 1008 boys, 832 (82.5%) who received caudal and 176 (17.5%) penile block, were included. Median age at surgery was 8.1 months and median follow up was 13 months. Overall complication rate was 16.4% with 13.8% of patients requiring repeat operation. Median time to complication was 10.59 months and was significantly shorter in the caudal group (8.45 vs. 25.2 months). Caudal anesthesia was associated with higher likelihood of complication on univariate analysis; however, this was not true on multivariate analysis when controlling for age and type of repair. Caudal anesthesia was not associated with increased likelihood of complication within 30 days. DISCUSSION: Since the association between caudal anesthesia and hypospadias complications was first suggested, several studies have tried to answer this question with variable results. Our findings add to the evidence that there is no association between caudal anesthesia and increased hypospadias complications in either the short or long term. The major strengths of our study are a large, homogenous study population, robust follow up and inclusion of data from 14 surgeons over 14 years. Limitations include the study's retrospective nature as well as lack of standardized follow up protocol throughout the study period. CONCLUSIONS: After controlling for possible confounders, caudal nerve block was not associated with increased risk of postoperative complications following distal hypospadias repair.

Does ketorolac administration at the time of hypospadias surgery increase unplanned encounters in the immediate postoperative period?

INTRODUCTION & OBJECTIVE: The opioid crisis has raised concerns for long-term sequela of routine administration of opioids to patients, particularly in the pediatric population. Nonsteroidal anti-inflammatory drug use is limited in hypospadias surgery due to concerns for post-operative bleeding, particularly with ketorolac. We hypothesize that ketorolac administration at the time of hypospadias repair is not associated with increased bleeding or immediate adverse events. METHODS: A retrospective single institution study included all patients undergoing hypospadias surgery from 2018 to 2021. Outcomes measured include peri-operative ketorolac administration, opioid prescriptions, and unplanned encounters (i.e., emergency department or office visits). Comparative statistics using non-parametric and binary/categorical tests and a logistic regression were performed. RESULTS: 1044 patients were included, among whom there were 562 distal, 278 proximal and 204 hypospadias complication repairs. Ketorolac was administered to 396 (37.9%) patients and its utilization increased during the study period [Summary Figure]. Patients receiving ketorolac were older (p = 0.002) and were prescribed opioids less often after surgery (2.0% vs 5.2%, p = 0.009). There was no difference in unplanned encounters across repair types (p = 0.1). Multivariate logistic regression showed ketorolac use was not associated with an increased likelihood of an unplanned encounter. DISCUSSION: The use of NSAIDs post-operatively has traditionally been limited due to concerns about bleeding risks, however the present study displayed no significant increases in unplanned patient encounters either in the ED or outpatient clinic after ketorolac administration. Our study has several limitations including its retrospective and single-institutional design, difficulties of pain assessment in pediatric population, and possibility of under estimation of unplanned encounters due to limited access to patients' records outside of our institution. CONCLUSIONS: The use of ketorolac is not associated with an increase in unplanned encounters in children undergoing hypospadias repair. It should be considered a safe agent for perioperative analgesia to decrease opioid utilization. Further studies will evaluate long-term surgical outcomes in children receiving ketorolac after hypospadias repair.

Retrograde urethrography in children: a decade of experience at a children's hospital.

BACKGROUND: Retrograde urethrography (RUG) is a radiologic procedure that optimizes imaging evaluation of the urethra, particularly in settings of difficulty with micturition or urethral injury. OBJECTIVE: To review our experience with RUG at a large pediatric radiology practice. MATERIALS AND METHODS: We conducted a retrospective review of medical records and fluoroscopic images of RUGs performed from January 2010 to December 2020. RESULTS: We identified 180 RUG exams (median frequency 17 exams per year), all in male children (median age 13 years). The most common indications were stricture (42%; n=76), postsurgical evaluation (34%; n=62) and trauma (16%; n=29). The most commonly used catheter was Foley (40%; n=72), with a median catheter size of 5 French (Fr) for infants younger than 1 year, 7 Fr for children ages 1-5 years and 8 Fr for children older than 5 years. About a third of the children (57; 32%) had combined voiding cystourethrography (VCUG)-RUG exams. Water-soluble contrast agent, either 17% or 43% Cysto-Conray, was used. Most RUG exams were normal (46%; n=83). The most common urethral pathology was stricture (30%; n=54), commonly involving a bulbar urethra (n=26). Urethral trauma was seen in 11 children (6%), 10 bulbar and 1 membranous. Most children with stricture were surgically treated (n=40; 74%), whereas most children with trauma were conservatively treated (n=8; 73%). The remaining diagnoses included diverticula, polyps, valves, fistulas and duplications, constituting <17% of our sample; most of these were surgically treated. Four exams (2%) were non-diagnostic. RUG showed 89% sensitivity and 97% specificity compared to cystourethroscopy/VCUG findings. Technical difficulties occurred in 14 (8%) children (e.g., pain or inappropriate catheter seal). CONCLUSION: Our experience indicates that when catheterization techniques are properly tailored, RUG provides a useful and successful radiologic method of evaluating the pediatric male urethra.

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium.

INTRODUCTION: Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. OBJECTIVE: We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). STUDY DESIGN: As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. RESULTS: 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128 ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3 h, 9 (13%) patients have dry intervals of 1-3 h and 44 (61%) patients had dry intervals <1 h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p = 0.3). DISCUSSION: In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. CONCLUSIONS: Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

Caregiver distress: A mixed methods evaluation of the mental health burden of caring for children with bladder exstrophy.

Introduction: Caring for children with bladder exstrophy-epispadias complex (BEEC) exacts a long-term emotional toll on caregivers. Previous studies leave a gap in understanding the impact that caring for a child with BEEC has on caregivers in low- and middle-income countries (LMIC). We hypothesize that families and caregivers experience psychological distress that has long gone unaddressed. Materials and methods: From 2018 to 2020, researchers conducted a multi-method evaluation of caregiver distress with participants recruited as part of the annual International Bladder Exstrophy Collaboration based in Ahmedabad, Gujarat, India. In 2018, pilot data was collected through cognitive interviews. In 2019, researchers conducted structured interviews predicated on themes from the previous year, which subsequently prompted formal mental health screenings in 2020. Caregivers who reported suicidal thoughts were immediately referred for intervention. Results: In 2018, caregivers described the primary source of stigma arose from their village (n = 9, 26.5%). Caregivers also identified long-term concerns (n = 18, 52.9%), including future fertility and marital prospects, as sources of anxiety. In 2019, caregivers substantiated preliminary findings with the primary source of anticipated (n = 9, 31%) and experienced (n = 19, 65.5%) stigma again stemming from their communities. Both cohorts identified the collaboration as a positive source of support (n = 23, 36.5%). In 2020, caregivers stated decreased emotional wellbeing as number of subsequent repairs increased (n = 54, 75%, p = 0.002). Caregivers of children who underwent initial surgery within 5 years of screening reported higher anxiety (n = 46, 63.8%) and this was exacerbated as the number of subsequent repairs increased (p = 0.043). Conclusion: Complex, long-term course of care, including additional surgeries, significantly impacts caregiver distress in the LMIC setting. Screening for caregivers of children with complex congenital anomalies, like BEEC, should be an essential element of any comprehensive effort to alleviate the global burden of disease.

Incidence and resolution of de novo hydronephrosis after pediatric robot-assisted laparoscopic extravesical ureteral reimplantation for primary vesicoureteral reflux.

INTRODUCTION: With the advent of robot-assisted laparoscopic ureteral reimplantation (RALUR) for primary vesicoureteral reflux (VUR), understanding and minimizing its complications continues to be critical. Incidence of de novo hydronephrosis after RALUR could be indicative of an outcome that needs further study or could be a benign transient finding. OBJECTIVE: In the present study, we hypothesized that the incidence of de novo hydronephrosis after RALUR is low and resolves spontaneously. METHODS: An IRB-approved prospective single-institutional registry was utilized to identify all patients undergoing RALUR via an extravesical approach from 2012 to 2020. Patients with primary VUR and minimal (Grade I SFU) or no hydronephrosis prior to surgery were included. Patients who had other associated pathology or underwent concomitant procedures were excluded. Preoperative characteristics including VUR and hydronephrosis grades as well as post-operative clinical details and hydronephrosis grades were aggregated and analyzed. RESULTS: 86/172 (50%) patients (133 ureters), with median reflux grade of 3 (IQR: G2, G3) met the inclusion criteria. Patients underwent RALUR at a median age of 5.7 years (IQR: 3.4, 8.7) with median 36.2 months (IQR: 19.6, 63.6) follow-up. Renal ultrasound at 4-6 weeks post-op showed de novo hydronephrosis in 18 (13.5%) ureters; the complete resolution was seen in 13 ureters (72%) at a median of 4.5 months from surgery. Among the 5 with non-resolved hydronephrosis (SFU G2:4, G3:1), 2 patients (3 ureters) underwent subsequent interventions. DISCUSSION: The present study evaluating the natural history of de novo hydronephrosis after RALUR-EV performed for primary VUR, is to our knowledge the largest cohort of patients undergoing RALUR that this has been studied in. In our cohort, the incidence of de novo hydronephrosis after RALUR was 13.5%, similar to rates reported in two OUR cohorts, and significantly lower than reported incidence rates of 22-26% in several OUR cohorts, and 30% in a RALUR cohort. In the present cohort, hydronephrosis resolved spontaneously in more than 72% of cases. The median time from surgery until resolution of hydronephrosis was 4.5 (1.6, 10.5) months, which is shorter in comparison to the average time to resolution of 7.6 months, reported by Kim et al. in an earlier study. CONCLUSIONS: De novo hydronephrosis after RALUR can be followed with serial renal ultrasounds. The majority of de novo hydronephrosis post-RALUR is transient and resolves spontaneously within a year of surgery with a very low re-intervention rate.

Quantifying Glans Width Changes in Response to Preoperative Androgen Stimulation in Patients Undergoing Hypospadias Repair.

PURPOSE: Testosterone (T) administration prior to hypospadias surgery to increase glans size remains controversial. Understanding T's effect on glans width (GW) is essential to understanding its potential impact on surgical outcomes. We hypothesized that preoperative T in prepubertal boys significantly increases GW at the time of hypospadias surgery. MATERIALS AND METHODS: Our single institutional database was queried to identify patients who underwent hypospadias surgery from 2016 to 2020, in which data for T administration and GW were available. Descriptive, nonparametric and categorical statistics were performed as indicated. RESULTS: A total of 579 patients were eligible for analysis. Median age at surgery was 0.9 years (IQR 0.6-1.6). A total of 247/579 patients (42.7%) received T. The median GW at surgery was 15 mm (IQR 13-17). When comparing patients who had T administered to those who did not, we found a significant difference in GW at surgery (16 mm vs 14 mm, p <0.001). The median change in GW from the office to surgery was 4 mm for those receiving T vs 0 mm for those not receiving T (p <0.001). We identified a greater change in GW from preoperative to intraoperative measurements in patients who received 2 doses of T vs 1 dose (4 mm vs 2 mm, p <0.001). A histogram plot revealed the distribution of GW change at surgery. CONCLUSIONS: In our prospectively collected cohort of patients undergoing hypospadias surgery, we were able to quantitate the change in GW from preoperative T. Two doses of T resulted in a significant increase in GW vs 1 dose.

Posterior Urethral Valves Outcomes Prediction (PUVOP): a machine learning tool to predict clinically relevant outcomes in boys with posterior urethral valves.

BACKGROUND: Early kidney and anatomic features may be predictive of future progression and need for additional procedures in patients with posterior urethral valve (PUV). The objective of this study was to use machine learning (ML) to predict clinically relevant outcomes in these patients. METHODS: Patients diagnosed with PUV with kidney function measurements at our institution between 2000 and 2020 were included. Pertinent clinical measures were abstracted, including estimated glomerular filtration rate (eGFR) at each visit, initial vesicoureteral reflux grade, and renal dysplasia at presentation. ML models were developed to predict clinically relevant outcomes: progression in CKD stage, initiation of kidney replacement therapy (KRT), and need for clean-intermittent catheterization (CIC). Model performance was assessed by concordance index (c-index) and the model was externally validated. RESULTS: A total of 103 patients were included with a median follow-up of 5.7 years. Of these patients, 26 (25%) had CKD progression, 18 (17%) required KRT, and 32 (31%) were prescribed CIC. Additionally, 22 patients were included for external validation. The ML model predicted CKD progression (c-index = 0.77; external C-index = 0.78), KRT (c-index = 0.95; external C-index = 0.89) and indicated CIC (c-index = 0.70; external C-index = 0.64), and all performed better than Cox proportional-hazards regression. The models have been packaged into a simple easy-to-use tool, available at https://share.streamlit.io/jcckwong/puvop/main/app.py CONCLUSION: ML-based approaches for predicting clinically relevant outcomes in PUV are feasible. Further validation is warranted, but this implementable model can act as a decision-making aid. A higher resolution version of the Graphical abstract is available as Supplementary information.

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

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.

Primary vs redo robotic pyeloplasty: A comparison of outcomes.

INTRODUCTION: Robot-assisted laparoscopic pyeloplasty (RALP) is a safe and efficacious option for repair of UPJO. We hypothesize that redo-RALP is technically more difficult but has comparable outcomes to primary RALP. METHODS: An IRB-approved single institutional registry was utilized to identify all patients undergoing primary or redo RALP from 2012 to 2019. Redo RALP consisted of pyeloplasty and ureterocalicostomy (RALUC). Peri-operative and post-operative details and outcomes were aggregated. Successful reconstruction was defined as resolution of symptoms, improved hydronephrosis and no need for additional procedures. RESULTS: From 399 patients who underwent UPJO repair at our center, a total of 306 with a median age of 4.9 years at surgery and a median follow-up of 18.5 months were included: 276 primary and 30 redo (21 RALP and 9 RALUC). Redo group had significantly longer procedure time and length of stay compared to the primary group. However, no significant difference was noted in the post-operative complications, need for additional endoscopic procedures or redo reconstruction, and success between the two groups. Multivariate analysis showed that when controlled for age, gender, 30-days post-operative complication and anatomy of obstruction, redo as compared to primary reconstruction did not have a significant effect on success. DISCUSSION: This study is the largest controlled cohort in the pediatric population comparing redo RALP with an established control group -primary RALP. This retrospective chart review possesses the biases innate to any retrospective study. The low number of re-operative cases as well as low rate of failure in redo RALP further complicates identification of statistically significant predictors of outcomes following redo RALP. CONCLUSION: Redo RALP is an efficient and safe approach for reconstruction of recurrent UPJO, with low complication rate and high success rate, comparable to primary RALP.

Variant of Bladder Exstrophy With an Intact Penis: Surgical Options and Approach.

Variants of bladder exstrophy are a rare but diverse spectrum of bladder exstrophy-epispadias complex. This case series describes a group of 4 unique exstrophy variant cases who had an intact phallus, but a completely open bladder plate. These patients underwent exstrophy repair and concomitant umbilicoplasty at the Civil Hospital, Ahmedabad as part of the US-India Multi-institutional Bladder Exstrophy Collaboration and were followed at the same institution. We believe that a detailed assessment of bladder neck prior to reconstructive repair and bladder closure would be beneficial in these cases as the extent of bladder neck involvement would affect reconstructive approach.

Utilization of Robot-assisted Surgery for the Treatment of Primary Obstructed Megaureters in Children.

OBJECTIVE: To describe the technical aspects of robot assisted laparoscopic ureteral reimplantation (RALUR) for the management of primary obstructive megaureter (POM) and report initial outcomes, safety, and feasibility of the procedure. METHODS: Using an IRB- approved robotic surgery registry, we performed a retrospective chart review of patients undergoing RALUR for POM between April 2009 and May 2019. RESULTS: A total of 18 patients underwent RALUR using a modified Lich-Gregoir technique for management of POM and 7 (38.9%) of these underwent intracorporeal ureteral tapering at the time of surgery. At median follow up of 27.5 (IQR 11-50) months, no patient required reoperation for recurrent obstruction and all patients had improvement in hydronephrosis postoperatively. 30-day complications were low with 1 Grade I, 2 Grade II and 1 Grade III Clavien-Dindo complication. The most common issue postoperatively was febrile urinary tract infection, occurring in 6 patients (33.3%), at an average of 3.2 months after surgery. Increased operative time was the only significant difference between the tapered verses nontapered group. CONCLUSION: We present the largest series of RALUR for POM to date. Based upon our initial experience with this technique we believe it is technically feasible and reproducible with good outcomes and low complication rates. Future studies are needed to track long-term outcomes and better understand indications for and the utility of ureteral tapering as well as how to minimize febrile UTIs postoperatively. Additional follow up is needed to determine the efficacy of RALUR as compared to open ureteral reimplantation for POM.

Evaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model.

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.

Does contrast-enhanced ultrasound have a role in evaluation and management of pediatric renal trauma? A preliminary experience.

BACKGROUND: To report our initial experience using intravenous contrast-enhanced ultrasound (CEUS) in pediatric renal trauma with the potential for substantial radiation reduction. METHODS: A retrospective review of all patients who underwent intravenous CEUS at our institution between May 2015 and January 2018 for the suspicion of blunt renal trauma. CEUS was obtained either as an immediate or short-term comparison to contrast-enhanced computed tomography (CECT), or in outpatient follow-up. RESULTS: CEUS was performed on 7 patients (9 kidneys) with age range 2 months to 16 years old. CEUS was utilized as a comparison to CECT in 4 of 7 patients for initial evaluation, clinical change, or short-term follow-up. CEUS alone was used in one patient with low suspicion for renal injury. In the remaining two patients, CEUS was obtained as a follow-up study weeks after the initial CECT, following conservative management. All patients with confirmed renal injury by CECT (n = 5) underwent a follow-up CEUS at 1-2 months. CONCLUSIONS: In an era of conservative management for renal trauma in which operative intervention is dictated more often by the clinical course than radiographic findings, it is reasonable to consider alternative imaging methods such as CEUS in stable patients to decrease radiation exposure. LEVEL OF EVIDENCE RATING: IV.

Follow-up and Outcomes of Patients With Long-term Cutaneous Vesicostomies at a Single Institution.

OBJECTIVE: To identify and describe the characteristics of a group of patients at our institution who are satisfied with cutaneous vesicostomy for long-term bladder management and never undergo subsequent continence surgery. METHODS: Using an IRB-approved registry of our patients undergoing reconstructive urinary tract surgery since 1985, we identified patients who underwent vesicostomy creation and were 15 years or older as of October 2017 and performed a retrospective chart review. RESULTS: Sixty-seven patients were included with the most common diagnosis being spina bifida and the average duration of vesicostomy 14.3 years (range 8 months to 35 years). In follow-up, 38 patients (56.7%) had conversations regarding further surgery and 31 (46.3%) had their vesicostomies taken down. Of the 36 patients who still had their vesicostomy at last follow-up, 29 (80.6%) had spina bifida and 14 (38.9%) were satisfied, 1 (2.8%) was unhappy, 4 (11.1%) were deceased and satisfaction was unknown for 17 (47.2 %). CONCLUSION: Although often intended to be temporary, in our experience many patients keep their vesicostomy for a long time and into adulthood, with generally high satisfaction and low complications. In the properly selected patient, vesicostomy may be a good long-term operation to prevent infection and renal deterioration, though more rigorous studies of these patients are needed.

A Teenager With Recurrent Epididymitis.

A 17-year-old man with a history of imperforate anus presented to clinic with recurrent epididymitis. A fluoroscopic voiding cystourethrogram demonstrated urethra-ejaculatory duct reflux. A narrowing was also noted in the distal prostatic urethra with dilation of the proximal urethra. Subsequent cystoscopy revealed a patent urethra with a hypertrophic external sphincter as the culprit. Pelvic floor physical therapy was undertaken with resolution of urinary symptoms and testicular pain.