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.

Prenatal Presentation of a Covered Cloacal Exstrophy Variant; Early Diagnostic Challenges Within the Broad Spectrum of the Exstrophy-Epispadias Complex.

There is a broad range of variant phenotypes that can occur within the bladder exstrophy and epispadias complex spectrum. Accurate prenatal detection helps prepare families and to coordinate subspecialty resources. Here, we present the case of a patient with prenatally diagnosed patient with covered cloacal exstrophy variant along with four additional cases illustrating the nonlinear spectrum from isolated epispadias to cloacal exstrophy. Given the rarity of these variants overall and of each subtype within the spectrum, there is a need for long-term multi-institutional outcomes data to improve detection, characterization, and prognostication for these patients.

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.

Automated Society of Fetal Urology (SFU) grading of hydronephrosis on ultrasound imaging using a convolutional neural network.

INTRODUCTION: Grading of hydronephrosis severity on postnatal renal ultrasound guides management decisions in antenatal hydronephrosis (ANH). Multiple systems exist to help standardize hydronephrosis grading, yet poor inter-observer reliability persists. Machine learning methods may provide tools to improve the efficiency and accuracy of hydronephrosis grading. OBJECTIVE: To develop an automated convolutional neural network (CNN) model to classify hydronephrosis on renal ultrasound imaging according to the Society of Fetal Urology (SFU) system as potential clinical adjunct. STUDY DESIGN: A cross-sectional, single-institution cohort of postnatal renal ultrasounds with radiologist SFU grading from pediatric patients with and without hydronephrosis of stable severity was obtained. Imaging labels were used to automatedly select sagittal and transverse grey-scale renal images from all available studies from each patient. A VGG16 pre-trained ImageNet CNN model analyzed these preprocessed images. Three-fold stratified cross-validation was used to build and evaluate the model that was used to classify renal ultrasounds on a per patient basis into five classes based on the SFU system (normal, SFU I, SFU II, SFU III, or SFU IV). These predictions were compared to radiologist grading. Confusion matrices evaluated model performance. Gradient class activation mapping demonstrated imaging features driving model predictions. RESULTS: We identified 710 patients with 4659 postnatal renal ultrasound series. Per radiologist grading, 183 were normal, 157 were SFU I, 132 were SFU II, 100 were SFU III, and 138 were SFU IV. The machine learning model predicted hydronephrosis grade with 82.0% (95% CI: 75-83%) overall accuracy and classified 97.6% (95% CI: 95-98%) of the patients correctly or within one grade of the radiologist grade. The model classified 92.3% (95% CI: 86-95%) normal, 73.2% (95% CI: 69-76%) SFU I, 73.5% (95% CI: 67-75%) SFU II, 79.0% (95% CI: 73-82%) SFU III, and 88.4% (95% CI: 85-92%) SFU IV patients accurately. Gradient class activation mapping demonstrated that the ultrasound appearance of the renal collecting system drove the model's predictions. DISCUSSION: The CNN-based model classified hydronephrosis on renal ultrasounds automatically and accurately based on the expected imaging features in the SFU system. Compared to prior studies, the model functioned more automatically with greater accuracy. Limitations include the retrospective, relatively small cohort, and averaging across multiple imaging studies per patient. CONCLUSIONS: An automated CNN-based system classified hydronephrosis on renal ultrasounds according to the SFU system with promising accuracy based on appropriate imaging features. These findings suggest a possible adjunctive role for machine learning systems in the grading of ANH.

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.

Deep Learning of Videourodynamics to Classify Bladder Dysfunction Severity in Patients With Spina Bifida.

PURPOSE: Urologists rely heavily on videourodynamics to identify patients with neurogenic bladders who are at risk of upper tract injury, but their interpretation has high interobserver variability. Our objective was to develop deep learning models of videourodynamics studies to categorize severity of bladder dysfunction. MATERIALS AND METHODS: We performed a cross-sectional study of patients aged 2 months to 28 years with spina bifida who underwent videourodynamics at a single institution between 2019 and 2021. The outcome was degree of bladder dysfunction, defined as none/mild, moderate, and severe, defined by a panel of 5 expert reviewers. Reviewers considered factors that increase the risk of upper tract injury, such as poor compliance, elevated detrusor leak point pressure, and detrusor sphincter dyssynergia, in determining bladder dysfunction severity. We built 4 models to predict severity of bladder dysfunction: (1) a random forest clinical model using prospectively collected clinical data from videourodynamics studies, (2) a deep learning convolutional neural network of raw data from the volume-pressure recordings, (3) a deep learning imaging model of fluoroscopic images, (4) an ensemble model averaging the risk probabilities of the volume-pressure and fluoroscopic models. RESULTS: Among 306 videourodynamics studies, the accuracy and weighted kappa of the ensemble model classification of bladder dysfunction when at least 75% expected bladder capacity was reached were 70% (95% CI 66%,76%) and 0.54 (moderate agreement), respectively. The performance of the clinical model built from data extracted by pediatric urologists was the poorest with an accuracy of 61% (55%, 66%) and a weighted kappa of 0.37. CONCLUSIONS: Our models built from urodynamic pressure-volume tracings and fluoroscopic images were able to automatically classify bladder dysfunction with moderately high accuracy.

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.

Deep learning imaging features derived from kidney ultrasounds predict chronic kidney disease progression in children with posterior urethral valves.

BACKGROUND: We sought to use deep learning to extract anatomic features from postnatal kidney ultrasounds and evaluate their performance in predicting the risk and timing of chronic kidney disease (CKD) progression for boys with posterior urethral valves (PUV). We hypothesized that these features would predict CKD progression better than clinical characteristics such as nadir creatinine alone. METHODS: We performed a retrospective cohort study of boys with PUV treated at two pediatric health systems from 1990 to 2021. Features of kidneys were extracted from initial postnatal kidney ultrasound images using a deep learning model. Three time-to-event prediction models were built using random survival forests. The Imaging Model included deep learning imaging features, the Clinical Model included clinical data, and the Ensemble Model combined imaging features and clinical data. Separate models were built to include time-dependent clinical data that were available at 6 months, 1 year, 3 years, and 5 years. RESULTS: Two-hundred and twenty-five patients were included in the analysis. All models performed well with C-indices of 0.7 or greater. The Clinical Model outperformed the Imaging Model at all time points with nadir creatinine driving the performance of the Clinical Model. Combining the 6-month Imaging Model (C-index 0.7; 95% confidence interval [CI] 0.6, 0.79) with the 6-month Clinical Model (C-index 0.79; 95% CI 0.71, 0.86) resulted in a 6-month Ensemble Model that performed better (C-index 0.82; 95% CI 0.77, 0.88) than either model alone. CONCLUSIONS: Deep learning imaging features extracted from initial postnatal kidney ultrasounds may improve early prediction of CKD progression among children with PUV. A higher resolution version of the Graphical abstract is available as Supplementary information.

Impact of Race, Gender, and Socioeconomic Status on Symptom Severity at Time of Urologic Referral.

BACKGROUND AND OBJECTIVES: The Dysfunctional Voiding and Incontinence Scoring System (DVISS) is a validated tool to evaluate lower urinary tract dysfunction (LUTD) severity in children. DVISS provides a quantitative score (0-35) including a quality-of-life measure, with higher values indicating more/worse symptoms. Clinically, variability exists in symptom severity when patients present to pediatric urology with LUTD. We hypothesized that symptom severity at consultation varied based on race, gender, and/or socioeconomic status. METHODS: All urology encounters at a single institution with completed modified DVISS scores 6/2015-3/2018 were reviewed. Initial visits for patients 5-21 years old with non-neurogenic LUTD were included. Patients with neurologic disorders or genitourinary tract anomalies were excluded. Wilcoxon rank sum tests compared scores between White and Black patients and between male and female patients. Multiple regression models examined relationships among race, gender, estimated median household income, and insurance payor type. All statistics were performed using Stata 15. RESULTS: In total, 4086 initial patient visits for non-neurogenic LUTD were identified. Median DVISS scores were higher in Black (10) versus White (8) patients (p < 0.001). Symptom severity was higher in females (9) versus males (8) (p < 0.001). When estimated median income and insurance payer types were introduced into a multiple regression model, race, gender, and insurance payer type were significantly associated with symptom severity at presentation. CONCLUSIONS: Race, gender, and socioeconomic status significantly impact LUTS severity at the time of urologic consultation. Future studies are needed to clarify the etiologies of these disparities and to determine their clinical significance.

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.

Repurposing tools: A peña stimulator as a tool to map the limits of dissection and continence zone during bladder exstrophy repair.

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.

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.