Preputial pedicle flap ICG blood flow assessment during proximal hypospadias repair: Development of a standardized protocol.

INTRODUCTION: Blood supply to preputial flap drives outcomes of hypospadias repair. Unfortunately, we only have surgeon's subjective assessment to evaluate flap perfusion which may not be accurate. Indocyanine green (ICG) has been used in a multitude of surgeries for perfusion assessment, however, no standardized protocol has been described for use of ICG in hypospadias repairs. The aim of this study is to develop a standardized protocol of ICG use in proximal hypospadias and establish perfusion patterns of preputial flaps. STUDY DESIGN: A pilot study was conducted using ICG in patients with proximal hypospadias undergoing first stage repair with a preputial flap. The Stryker SPY PHI system and novel quantification software, SPY-QP, were used for ICG imaging. An adaptive approach was taken to develop and implement a standardized protocol (Summary Figure). Per the protocol, ICG was administered at 3 time points which were felt to be critical for assessment of flap perfusion. Of the study patients who have undergone second stage repair, ICG was also used to reassess the flap prior to tubularization of the urethra. RESULTS: A total of 14 patients underwent first stage hypospadias repair with preputial flaps and intraoperative use of ICG. Median ICG uptake of the prepuce after degloving (dose 1) was 58.5% (IQR 43-76). ICG uptake decreased after flap harvest and mobilization (dose 2) with a median ICG uptake of 34% (IQR 26-46). ICG uptake remained stable after securing the flap in place and closing the skin (dose 3) with a median ICG uptake of 34% (IQR 25-48). ICG was able to delineate subtle findings in the preputial flaps not visible to the naked eye and in one case impacted intraoperative decision making. To date, 5 patients have undergone second stage repair. Flap assessment prior to tubularization of the urethra showed hypervascularity with a median ICG uptake of 159%. CONCLUSIONS: A standardized protocol for ICG use in proximal hypospadias was successfully developed and implemented. ICG uptake in the preputial flap decreased with increasing manipulation and mobilization of the flap. ICG was able to detect changes to flap perfusion which were not able to be seen with the naked eye. Reliance on surgeon's subjective assessment of flap perfusion may be inadequate and ICG could provide a useful tool for surgeons to improve preputial flap outcomes. ICG may also enhance the learning experience for trainees and early career urologists in these complex surgeries.

Comparison of hypospadias phenotype pixel segmentation to GMS score.

BACKGROUND: Hypospadias phenotype assessment determines if the anatomy is favorable for reconstruction. Glans-Urethral Meatus-Shaft (GMS) has been adopted in an effort to standardize hypospadias classification. While extremely subjective, GMS has been widely used to classify the severity of the phenotype to predict surgical outcomes. The use of digital image analysis has proven to be feasible and prior efforts by our team have demonstrated that machine learning algorithms can emulate an expert's assessment of the phenotype. Nonetheless, the creation of these image recognition algorithms is highly subjective. In order to reduce a subjective input in the evaluation of the phenotype, we propose a novel approach to analyze the anatomy using digital image pixel analysis and to compare the results using the GMS score. Our hypothesis is that pixel cluster segmentation can discriminate between favorable and unfavorable anatomy. OBJECTIVE: To evaluate whether image segmentation and digital pixel analysis are able to analyze favorable vs unfavorable hypospadias anatomy in a less subjective manner than GMS score. METHODS: A total of 148 patients with different types of hypospadias were classified by 1 of 5 independent experts following the GMS score into "favorable" (GG), "moderately favorable" (GM) and "unfavorable" (GP) glans. From there, 592 images were generated using digital image segmentation. 584 were included for final analysis due to certain images being excluded for poor image quality or inadequate capture of target anatomy. For each image, the region of interest was segmented separately by two evaluators into "glans," "urethral plate," "foreskin" and "periurethral plate". The values obtained for each segmented region using machine-learning statistical pixel k-means cluster analysis were analyzed and compared to the GMS score given to that image using an ANOVA analysis. RESULTS: Analysis of image segmentation demonstrated that k-means pixel cluster analysis discriminated "favorable" vs "unfavorable" urethral plates. There was a significant difference between scores when comparing the GG and GM groups (p = 0.03) and GG and GP groups (p = 0.05). Pixel cluster analysis could not discriminate between "moderately favorable" and "unfavorable" urethral plates. CONCLUSIONS: Through our analysis, we found significant pairwise difference for different tissue qualities. Digital image segmentation and statistical k-means cluster analysis can discriminate anatomical features in a similar way to the GMS score. Future research can target discerning between different tissue qualities in an effort to predict surgical outcomes for hypospadias repair.

Putting the coach in the game: Current and future state of surgical coaching in pediatric urology.

BACKGROUND: Surgical coaching has been proposed as a mechanism to fill gaps in proficiency and encourage continued growth following formal surgical training. Coaching benefits have been demonstrated in other surgical fields; however, have not been evaluated within pediatric urology. The aims of this study were to survey members of The Societies for Pediatric Urology (SPU) to assess the current understanding and utilization of surgical coaching while gauging interest, potential barriers and personal goals for participation in a coaching program. METHODS: Following IRB approval, members of the SPU were invited to electronically complete an anonymous survey which assessed 4 domains: 1) understanding of surgical coaching principles, 2) current utilization, 3) interest and potential barriers to participation, and 4) personal surgical goals. To evaluate understanding, questions with predefined correct answers on the key principles of coaching were posed either in multiple choice or True/False format to the SPU membership. RESULTS: Of the 674 pediatric urologists invited, 146 completed the survey (22%). Of those, 46% correctly responded the definition of surgical coaching. Coaching utilization was reported in 27% of respondents currently or having previously participated in a surgical coaching program. Despite current participation rates, only 6 surgeons (4%) have completed training in surgical coaching, despite 79% expressing interest to participate in a surgical coaching program. The most influential barrier to participating in a coaching program was time commitment. Respondents largely prioritized technical and cognitive skill improvement as their primary goals for coaching (see figure below). CONCLUSIONS: While interest in surgical coaching is high among pediatric urologists, the principles of surgical coaching were not universally understood. Furthermore, formal coach training is markedly deficient, representing a gap in our profession and an opportunity for significant avenues for improvement, especially for technical and cognitive skills. Development of a coaching model based on these results would best suit the needs of pediatric urologists providing that the time commitment barrier for these endeavors can be mitigated and/or reconciled.

Standardization and Implementation of a Surgical Coaching Model for Pediatric Urology.

To bridge gaps in proficiency and encourage life-long learning following training, coaching models have been utilized in multiple surgical fields; however, not within pediatric urology. In this review of our methodology, we describe the development of a coaching model at a single institution. In our initial experience, the perceived most beneficial aspect of the program was the goal setting process with logistics around debriefs being the most challenging. With our proposed coaching study, we aim to develop a model based upon prior coaching frameworks,1,2 that is feasible and universally adaptable to allow for further advancement of surgical coaching, particularly within the field of pediatric urology.

Association between YouTube® videos and health literacy with postoperative healthcare utilization following pediatric urologic surgery: A randomized trial.

INTRODUCTION: Limited caregiver health literacy has been associated with poorer health outcomes in pediatric patients and may limit caregiver understanding of printed education resources. Postoperative healthcare utilization may be related to confusion about instructions or complications. OBJECTIVE: To correlate caregiver health literacy and educational video intervention with postoperative healthcare utilization following ambulatory pediatric urologic surgery. STUDY DESIGN: From July through December 2021, a randomized double-blinded trial assessed postoperative healthcare utilization following pediatric urologic surgery. Caregivers were randomized to receive standardized postoperative counseling and printed instructions (control) or access to English-language educational YouTube® videos with standardized postoperative counseling and printed instructions (intervention). Medical record abstraction was completed 30 days following surgery to identify postoperative healthcare utilization with calls, messages, add-on clinic visits, or presentation for urgent or emergent care, and postoperative complications. RESULTS: Target enrollment was achieved with 400 caregivers with 204 in the intervention and 196 in the control groups. There was a 32.5 % overall rate of postoperative healthcare utilization. Health literacy was inversely associated with total postoperative healthcare utilization (p < 0.001). There was no difference in the incidence of postoperative healthcare utilization between the control and intervention groups (p = 0.623). However, on sub-analysis of caregivers with postoperative healthcare utilization (Summary Figure), there were fewer total occurrences in the intervention group (intervention median 1, IQR 1,2.3; control median 2, IQR 1,3; p < 0.001). For caregivers with limited health literacy, there was a greater associated reduction in median calls from 2 (IQR 0,2) to 0 (IQR 0,0.5) with video intervention (p = 0.016). On multivariate analysis, total postoperative healthcare utilization was significantly associated with limited caregiver health literacy (OR 1.08; p = 0.004), English as preferred language (OR 0.68; p = 0.018), and older patient age (OR 0.95; p = 0.001). DISCUSSION: Current resources for postoperative education are limited as resources can be written above recommended reading levels and families can have difficulty recalling information discussed during postoperative counseling. Video intervention is an underutilized resource that can provide an additional resource to families with visual and auditory aids and be accessed as needed. CONCLUSION: Caregiver health literacy was inversely associated with postoperative healthcare utilization. There was no difference in the incidence of postoperative healthcare utilization with video intervention. However, on subgroup analysis, supplemental videos were associated with fewer occurrences of postoperative healthcare utilization, especially in caregivers with limited health literacy. On multivariate regression, health literacy, preferred language, and patient age were significantly associated with total postoperative healthcare utilization.

Reducing post-operative caregiver after-hours phone calls to pediatric urology providers: A quality improvement study.

INTRODUCTION: Caregiver phone calls are an important part of caring for pediatric patients. At our institution, residents respond to after-hours caregiver calls. While it is critical for families to be able to reach us for urgent concerns, the ease of access has led to overutilization with many phone calls not meeting the urgent nature that is intended for these calls. The primary aim of this quality improvement project was to decrease the number of non-urgent caregiver calls after-hours. Our secondary aim was to improve compliance with telephone encounter documentation and to standardize the documentation content. STUDY DESIGN: We conducted a single institution, multiphase quality improvement project. This started with a preintervention phase which included evaluation of our current state, identifying that most calls were for post-operative patients and that our discharge instructions inadequately detailed when caregivers should call. Notes were also inconsistently documented with no standard format. In the first PDSA cycle, launched on November 1, 2021, a standardized note template was created for documentation of caregiver telephone encounters. The PDSA cycle began on January 1, 2022 and included updates to our post-operative instructions with explicit guidance detailing when to call after-hours. Call data from September 2021 to February 2022 was reviewed including variables such as caller demographics, reason for call, and operative details. Primary outcomes were proportion of post-operative calls within 30 days and non-urgent calls. Secondary outcome was proportion of calls documented appropriately. Phases were categorized as current state (Sep/Oct 2021), PDSA cycle 1 (Nov/Dec 2021), PDSA cycle 2 (Jan/Feb 2022). RESULTS: In our current state, the majority of the calls (66%) were for post-operative patients and 59% of all calls during this period were non-urgent. The proportion of post-operative phone calls stayed stable at 67% during PDSA cycle 1, but decreased to 38% with PDSA cycle 2 with implementation of updated post-operative instructions (Summary figure) (p < 0.001). The proportion of non-urgent calls was similar (current state - 68%, PDSA cycle 1 - 72%, PDSA cycle 2-73%, p = 0.39) (Summary figure). Call documentation was also similar with a documentation rate of 79% pre-intervention and 87% post-intervention (p = 0.21) (Summary figure). CONCLUSIONS: With interventions focused on post-operative caregiver instructions, the number of post-operative phone calls decreased. Standardization of documentation was achieved. However, the overall call volume did not change, nor the proportion of non-urgent calls.

Kidney Disease in Childhood Cancer Survivors Treated With Radiation Therapy: A Comprehensive PENTEC Genitourinary Review.

PURPOSE: Kidney injury is a known late and potentially devastating complication of abdominal radiation therapy (RT) in pediatric patients. A comprehensive Pediatric Normal Tissue Effects in the Clinic review by the Genitourinary (GU) Task Force aimed to describe RT dose-volume relationships for GU dysfunction, including kidney, bladder, and hypertension, for pediatric malignancies. The effect of chemotherapy was also considered. METHODS AND MATERIALS: We conducted a comprehensive PubMed search of peer-reviewed manuscripts published from 1990 to 2017 for investigations on RT-associated GU toxicities in children treated for cancer. We retrieved 3271 articles with 100 fulfilling criteria for full review, 24 with RT dose data and 13 adequate for modeling. Endpoints were heterogenous and grouped according to National Kidney Foundation: grade ≥1, grade ≥2, and grade ≥3. We modeled whole kidney exposure from total body irradiation (TBI) for hematopoietic stem cell transplant and whole abdominal irradiation (WAI) for patients with Wilms tumor. Partial kidney tolerance was modeled from a single publication from 2021 after the comprehensive review revealed no usable partial kidney data. Inadequate data existed for analysis of bladder RT-associated toxicities. RESULTS: The 13 reports with long-term GU outcomes suitable for modeling included 4 on WAI for Wilms tumor, 8 on TBI, and 1 for partial renal RT exposure. These reports evaluated a total of 1191 pediatric patients, including: WAI 86, TBI 666, and 439 partial kidney. The age range at the time of RT was 1 month to 18 years with medians of 2 to 11 years in the various reports. In our whole kidney analysis we were unable to include chemotherapy because of the heterogeneity of regimens and paucity of data. Age-specific toxicity data were also unavailable. Wilms studies occurred from 1968 to 2011 with mean follow-ups 8 to 15 years. TBI studies occurred from 1969 to 2004 with mean follow-ups of 4 months to 16 years. We modeled risk of dysfunction by RT dose and grade of toxicity. Normal tissue complication rates ≥5%, expressed as equivalent doses, 2 Gy/fx for whole kidney exposures occurred at 8.5, 10.2, and 14.5 Gy for National Kidney Foundation grades ≥1, ≥2, and ≥3, respectively. Conventional Wilms WAI of 10.5 Gy in 6 fx had risks of ≥grade 2 toxicity 4% and ≥grade 3 toxicity 1%. For fractionated 12 Gy TBI, those risks were 8% and <3%, respectively. Data did not support whole kidney modeling with chemotherapy. Partial kidney modeling from 439 survivors who received RT (median age, 7.3 years) demonstrated 5 or 10 Gy to 100% kidney gave a <5% risk of grades 3 to 5 toxicity with 1500 mg/m2 carboplatin or no chemo. With 480 mg/m2 cisplatin, a 3% risk of ≥grade 3 toxicity occurred without RT and a 5% risk when 26% kidney received ≥10 Gy. With 63 g/m2 of ifosfamide, a 5% risk of ≥grade 3 toxicity occurred with no RT, and a 10% toxicity risk occurred when 42% kidney received ≥10 Gy. CONCLUSIONS: In patients with Wilms tumor, the risk of toxicity from 10.5 Gy of WAI is low. For 12 Gy fractionated TBI with various mixtures of chemotherapy, the risk of severe toxicity is low, but low-grade toxicity is not uncommon. Partial kidney data are limited and toxicity is associated heavily with the use of nephrotoxic chemotherapeutic agents. Our efforts demonstrate the need for improved data gathering, systematic follow-up, and reporting in future clinical studies. Current radiation dose used for Wilms tumor and TBI appear to be safe; however, efforts in effective kidney-sparing TBI and WAI regimens may reduce the risks of renal injury without compromising cure.

Comprehensive multidisciplinary phenotyping of patients with hypospadias. A pilot study.

INTRODUCTION: Hypospadias is an abnormal formation of the urethra, ventral skin, and corporal bodies. Location of the urethral meatus has historically been the phenotypic landmark that defines hypospadias. Nonetheless, classifications following location of the urethral meatus fail to consistently predict outcomes and have no correlation with the genotype. Description of the urethral plate is very subjective and difficult to reproduce. We hypothesize that the use of digital pixel cluster analysis and correlation to histological analysis can provide a novel method to describe the phenotype of patients with hypospadias. METHODS: A standardized hypospadias phenotyping protocol was developed. 1. Digital images of the anomaly, 2. Anthropometric assessment of penile dimensions (penile length, urethral plate length and width, glans width, ventral curvature), 3. Classification using the GMS score, 4. Tissue sampling (foreskin, glans, urethral plate, periurethral ventral skin) and H&E analysis by a blinded pathologist. A k-means colorimetric pixel cluster analysis was performed following the same anatomical landmark distribution as the histology samples. Analysis was performed using MATLAB v R2021b 9.11.0.1769968. RESULTS: A total of 24 patients prospectively enrolled with a standard protocol. Mean age at surgery was 16.25 months Urethral meatus was distal shaft in 7 patients, 8 coronal, 4 glanular, 3 midshaft, 2 penoscrotal. Average GMS score was 7.14 (±1.58). Average glans size was 15.71 mm (±2.33) and urethral plate width 5.57 mm (±2.06). Eleven patients underwent Thiersch-Duplay repair, 7 TIP, 5 MAGPI, and 1 a first stage preputial flap. Mean follow-up was 14.25 months ( ± 3.7 months). Two (8.3%) postoperative complications (1 urethrocutaneous fistula and 1 ventral skin wound dehiscence) were reported in the study period. Eleven (52.3%) patients with histological analysis had an abnormal pathology report. Of those, 6 (54%) had reported abnormal lymphocyte infiltration interpreted as chronic inflammation at the urethral plate. The second most common finding was hyperkeratosis visualized in the urethral plate in 4 (36.3%) and one with reported fibrosis in the urethral plate. K-means pixel analysis demonstrated a k1 mean of 64.2 for reported urethral plate inflammation vs 53.1 for non-reported urethral plate inflammation (p = 0.002) CONCLUSIONS: Current phenotyping of hypospadias using only anthropometric variables can be expanded including histological and pixel analysis correlation. Pixel clustering has a potential for a priori prediction of urethral plate quality beyond the current subjective assessment. A larger cohort will allow identification of possible predictive associations that might impact intraoperative decision-making and surgical outcomes.

Long term renal outcome and risk of elevated blood pressure in children undergoing complete primary repair of bladder exstrophy (CPRE).

INTRODUCTION: Since the initiation of staged reconstruction for bladder exstrophy (BE), hypertension has been a known complication of the procedure. Hypertension is a well-established risk factor for chronic kidney disease (CKD) progression and associated with cardiovascular/cerebrovascular morbidity and mortality. Few studies exist evaluating the risk of developing hypertension among patients with bladder exstrophy who underwent CPRE. We hypothesized that long-term blood pressure levels may be elevated in males vs females, and may be correlated with presence of hydronephrosis, bladder neck reconstruction, or continence status. OBJECTIVE: We sought to revisit our long-term experience with CPRE and determine factors associated with incidence of elevated blood pressures. METHODS: We reviewed all BE patients undergoing CPRE at our institution from 1999 to 2019. Patients were considered eligible for inclusion if last renal ultrasound was obtained at least 5 years after repair. Upper tract outcomes based on imaging, history of pyelonephritis and renal function tests measured by serum creatinine and estimated glomerular filtration rate (eGFR, Schwartz formula) were reviewed. Systolic/diastolic blood pressures (SBP/DBP) from all encounters were captured. All blood pressure values were age adjusted by percentile. RESULTS: A total of 36 patients were considered eligible for review. Median follow-up of this cohort was 10.01 (5.16-21.47) years. The mean creatinine for the patients available was 0.58 mg/dL (SD = 0.20), at mean age of 8.90 years Neither SBP or DBP were significantly elevated in males vs females, but had lower odds of elevation >90th percentile for those with higher eGFR, lower renal length, and reimplantation. Pyelonephritis incidence was 38% (n = 14) with first episode at mean age of 8.8 years, and mean of 3.7 episodes per patient. DISCUSSION: At long term follow up, blood pressures following CPRE were not significantly elevated, despite the relatively frequent occurrence of CKD, and hydronephrosis. Male gender does appear to suggest higher risk for long-term deterioration in this regard. Higher eGFR, higher renal length, and presence of ureteral reimplantation were associated with lower likelihood of systolic/diastolic blood pressure elevation. Continence status and bladder neck reconstruction were not associated with likelihood of blood pressure elevation. CONCLUSIONS: Blood pressure and upper-tract outcomes for patients undergoing CPRE at birth are positive for the majority of patients. To avoid complications from hypertension, patients should be closely evaluated as the risks associated with elevated blood pressure are significant. Ultimately, larger-scale prospective and multi-institutional studies are further needed to characterize risks of hypertension in this complex patient population.

Neural network non-linear modeling to predict hypospadias genotype-phenotype correlation.

INTRODUCTION: Hypospadias is an abnormal development of the urethral, ventral skin and corporeal bodies. Urethral meatus and ventral curvature have been historically the landmarks to define clinical severity. Genotyping has never been explored as a clinical predictor. Available reports have demonstrated a correlation between genetic mutations and syndromic hypospadias with poor surgical outcomes. We hypothesize that inclusion of genotyping can serve at classifying all types of hypospadias. We present the use of neural network algorithm to evaluate phenotype/genotype correlations and propose its potential clinical applicability. METHODS: A systematic review was performed from January 1974 to June 2022. Literature was retrieved from Medline, Embase, Web of Science and Google Scholar. Included manuscripts were those that had an explicit anatomical description of hypospadias phenotype (urethral meatus location following an anatomical description) and a defined genotype (genetic mutation) description. Cases with more than one variant/mutation were excluded. A comprehensive phenotype-genotype statistical analysis using neural network non-linear data modeling SPSS™ was performed. RESULTS: Genotype-Phenotype analysis was performed on 1731 subjects. Of those, 959 (55%) were distal and 772 (45%) proximal. 49 genes with mutations were identified. Neural network clustering predicted better for coronal (90%) and glanular (80%), and lowest for midshaft (22%) and perineal (45%). Using genes as predictor factor only, the model was able to highly and more accurately predict the phenotype for coronal and glanular hypospadias. The following genotypes showed association to a specific phenotype: AR gene n.2058G > A for glanular (p<0.0001), n.480C  >  T for coronal (p = 0.034), R840C for perineal (p = 0.002), MAMLD1 gene c.2960C > T for coronal (p< 0.0001), p. G289S for glanular (p<0.0001), gene SRD5A2 607G > A for scrotal (p<0.0001), c16C > T for penoscrotal (p<0.0001), c59 T  >  c for perineal (p = 0.042), V89L for midshaft and scrotal (p<0.0001, p = 0.041; respectively). DISCUSSION: Hypospadias phenotype has always been described from a purely anatomical perspective. Our results demonstrate that current phenotyping has poor correlation to the genotype. Higher genotype/phenotype correlation for distal hypospadias proves the clinical applicability of genotyping these cases. The concept and classification of differences in sexual development needs to be reconsidered given high positive yield reported for distal hypospadias. Given the better predictive value of genotyping in correlation to the phenotype, future efforts should be directed towards using the genotype. CONCLUSION: Hypospadias has poor phenotype/genotype correlation. Sequencing all hypospadias phenotypes may add clinical value if used in association to other predictive variables. Neural network analysis may have the ability to combine all these variables for clinical prediction.

Examination of generational impacts of adolescent chemotherapy: Ifosfamide and potential for epigenetic transgenerational inheritance.

The current study was designed to use a rodent model to determine if exposure to the chemotherapy drug ifosfamide during puberty can induce altered phenotypes and disease in the grand-offspring of exposed individuals through epigenetic transgenerational inheritance. Pathologies such as delayed pubertal onset, kidney disease, and multiple pathologies were observed to be significantly more frequent in the F1 generation offspring of ifosfamide lineage females. The F2 generation grand-offspring ifosfamide lineage males had transgenerational pathology phenotypes of early pubertal onset and reduced testis pathology. Reduced levels of anxiety were observed in both males and females in the transgenerational F2 generation grand-offspring. Differential DNA methylated regions (DMRs) in chemotherapy lineage sperm in the F1 and F2 generations were identified. Therefore, chemotherapy exposure impacts pathology susceptibility in subsequent generations. Observations highlight the importance that prior to chemotherapy, individuals need to consider cryopreservation of germ cells as a precautionary measure if they have children.

Short term reoperation rates after artificial urinary sphincter placement in pediatric patients.

INTRODUCTION: Artificial urinary sphincters (AUS) have demonstrated good functional outcomes in pediatric populations. We sought to examine the nationwide short term reoperation rates in pediatric patients after AUS placement. MATERIALS AND METHODS: An observational cohort study was designed utilizing claims from the Truven MarketScan Commercial Claims and Encounters database from 2007 to 2018. Patients under 18 years of age undergoing an AUS procedure were identified using CPT and ICD9/10 codes. Reoperations included any removal, replacement, or AUS placement codes which occurred after the initially identified placement code. Follow up time was the amount of time between AUS placement and the end of MarketScan enrollment. RESULTS: From 2007-2018, we identified 57 patients under the age of 18 who underwent AUS placement and after excluding 8 for concurrent AUS complication procedure codes and 4 for follow up < 60 days, the final cohort included 45 patients. The median age was 13 years (IQR 9-16 years) at the time of AUS placement, and the median follow up time after AUS placement was 787 days (IQR 442-1562 days), approximately 2.2 years. Total reoperation rate was 22%. Reoperations included 40% device removals (4/10) and 60% replacements (6/10). Neither gender (p = 0.70) nor age (p = 0.23) was associated with need for reoperation. Patients who had a concurrent bladder surgery had a higher rate of undergoing reoperation (50% vs. 12%, p = 0.007). CONCLUSIONS: The rate of reoperation after AUS placement approached 1 in 4 in pediatric patients. These data may be instrumental for providers and parents in counseling and decision-making regarding risks of prosthetic implantation.

Racial and ethnic disparities in the timing of orchiopexy for cryptorchidism.

INTRODUCTION: Many children do not undergo surgery for cryptorchidism in a timely fashion, increasing risk of infertility and malignancy. Racial and ethnic disparities in surgery timing has been suggested in other specialties, but has not been well-explored in Pediatric Urology. OBJECTIVES: Our aim was to investigate the association of race and ethnicity with age at orchiopexy. MATERIALS AND METHODS: We performed a retrospective cohort study of individuals <18 years of age as captured in the NSQIPP PUF from 2012 to 2016. Those with cancer were excluded. The primary outcome of interest was age at time of surgery. Secondary outcome was the proportion of individuals undergoing surgery by recommended age. Generalized linear models and logistic regression models were created for the outcomes of interest. RESULTS: The median age at orchiopexy was 17.4 months (10.7, 43.0) and overall, 51% of subjects underwent orchiopexy by 18 months of age. Non-Hispanic white individuals were most likely to have undergone orchiopexy by 18 months of age, at 56%, compared with only 44% of non-Hispanic black individuals (p < 0.001). When adjusting for co-morbidities and developmental delay, Hispanic patients underwent orchiopexy 5 months later than white patients, on average, and black patients had a delay of 7 months compared to white patients. DISCUSSION: These data suggest that orchiopexy is happening at younger ages compared to prior large-scale studies. However, minority patients are on average older at time of orchiopexy, potentially increasing future risk of infertility or malignancy. While an estimated average delay of 5-7 months may not seem high, studies suggest there is an appreciable change in risk with a 6-month delay. Patient, provider, and system-level factors likely all contribute, and these need to be further elucidated. CONCLUSIONS: Many racial and ethnic minorities with cryptorchidism have later orchiopexies, and are more likely to have surgery outside the recommended timeframe. Further investigation is warranted to determine the factors contributing to these disparities.

Integration of child life services in the delivery of multi-disciplinary differences in Sexual Development (DSD) and Congenital Adrenal Hyperplasia (CAH) care.

INTRODUCTION: Multiple studies have demonstrated the benefit of incorporating certified child life specialist (CCLS) services in various aspects of pediatric care. Although the significance of psychosocial support of patients with Disorders of Sexual Development (DSD) and Congenital Adrenal Hyperplasia (CAH) is increasingly recognized, the involvement of CCLS services into the DSD and CAH multidisciplinary care model has yet to be described. OBJECTIVE: To evaluate the feasibility, acceptability, and patient and family experience of routinely incorporating CCLS services into the multidisciplinary DSD and CAH care model. STUDY DESIGN: As part of a quality improvement initiative, CCLS services were routinely incorporated in the multidisciplinary DSD and CAH clinics at our institution. Encounters for patients seen in clinic between July 2018 through October 2019 were reviewed for demographic information, DSD diagnosis classification, CCLS documentation, and whether an exam under anesthesia (EUA) was required due to an incomplete clinical exam. CCLS documentation was reviewed for assessments, interventions, whether patients tolerated their physical exams, time of CCLS services, and additional CCLS support beyond the physical exam. All patients were limited to one physical exam per clinic visit. RESULTS: Out of the 45 encounters with CCLS involvement, 42 (93.3%) exams were well-tolerated. CCLS assessments considered patient development, communication considerations, temperament, medical stressors, coping preferences, and patient preferences for activities and distractions. Interventions included preparing patients for their physical exams, encouragement before and during exams, addressing patient stressors, distractions and coping mechanisms, and advocating for the patient. No patients required an EUA. DISCUSSION: The CCLS aimed to provide families with a sense of control during clinic visits and teach them to advocate for themselves. The CCLS helped prepare and distract patients for their clinic visit and addressed the sensitive nature of the physical exam by focusing on the emotional and development needs of patients. CCLS contributions to a positive patient experience are consistent with multiple studies demonstrating the benefit of CCLS services for pediatric care. This quality improvement initiative ultimately helped to create a positive experience for patients and families. CONCLUSION: This study demonstrates the feasibility, acceptability, and positive impact of CCLS services in the delivery of patient and family-centered care for patients with DSD and CAH as part of the multidisciplinary team model.

Digital photography in the evaluation and management of female patients with congenital adrenal hyperplasia: A standardized protocol for quality improvement.

INTRODUCTION: Digital photography can be securely stored in the medical record and enhance documentation of physical exam findings and monitor wound healing. A standardized protocol that respects the dignity of the patient and maintains the fidelity of objective documentation is needed for patients with differences in sexual development (DSD) and congenital adrenal hyperplasia (CAH). OBJECTIVE: The purpose of this study was to evaluate the feasibility, acceptability, and applications of a HIPAA-compliant digital photography protocol in the care of female patients with CAH. STUDY DESIGN: A protocol for standardized digital imaging including consent, permission, data capture, and storage in the electronic medical record (EMR) was implemented. Patients undergoing physical examination during multidisciplinary CAH clinic visits, preoperative evaluation, and postoperative follow-up from October 2020 through May 2021 were included. Male patients with CAH, patients with clitoromegaly or urogenital sinus not from CAH, and patients seen through telehealth were excluded. Consent was obtained from caregivers and permission from patients. Images of the exam were taken during clinic visits or at the time of surgery with no identifying features included. Images were directly uploaded into the patient's chart in the HIPAA-protected EMR separate from other clinical documentation and not stored on personal devices. RESULTS: There were 17 patients with CAH seen with median age 6 years (range 2 weeks-18 years). There was a median of 3 photos per patient during the study period with cooperation from both the patient and their caregiver. Amongst the patients seen, 6 patients underwent reconstruction with a median of 10 photos per patient. Images were available and used for preoperative planning and counseling. Patients with previous images did not require repeat examinations and were subjected to fewer genital examinations. Fewer providers were present during exams. Images taken by providers and caregivers during the postoperative period were used to monitor wound healing and surgical outcomes. DISCUSSION: Protocol implementation improved patient care by reducing the number of exams and number of providers present, enhancing clinical documentation, and providing a means of tracking the physical exam over time. This was in concordance with guidelines for limiting exams for patients with DSD and CAH. Implementation of best practices for medical photography was important in respecting patient dignity and confidentiality. CONCLUSION: Implementation of standardized digital photography was feasible and acceptable to patients and caregivers. Digital images reduced the need for repeat physical examination and provided a visual means of enhancing clinical documentation.

Fertility in Individuals with Differences in Sex Development: Provider Knowledge Assessment.

OBJECTIVE: Infertility is common among individuals with differences in sex development (DSD), and affected individuals and families desire fertility counseling. This survey sought to assess fertility knowledge and experiences with fertility counseling among DSD specialists for DSD conditions excluding congenital adrenal hyperplasia. DESIGN, SETTING, PARTICIPANTS, AND MEASURES: A survey was iteratively developed by members of the DSD-Translational Research Network (DSD-TRN) Fertility Preservation Workgroup and disseminated to 5 clinician groups: the DSD-TRN, the Society for Pediatric Psychology DSD Special Interest Group (SIG), the Pediatric Endocrine Society DSD-SIG, the Societies for Pediatric Urology, and the North American Society for Pediatric and Adolescent Gynecology. RESULTS: Completed surveys (n = 110) were mostly from pediatric urology (40.3%), gynecology (25.4%), and endocrinology (20.9%) specialists. Most (73/108, 67.6%) respondents reported discussing fertility potential. Sixty-seven responded to questions regarding fertility potential. Many participants answered questions about the presence of a uterus in individuals with 46,XY complete gonadal dysgenesis and about the potential for viable oocytes in individuals with 46,XY partial gonadal dysgenesis incorrectly. Comments acknowledged the need for further education on fertility in individuals with DSD. CONCLUSIONS: Many DSD providers have some knowledge of fertility potential, but knowledge gaps remain. Experts expressed a desire for education and accessible resources to counsel effectively about fertility potential for individuals with DSD.

Engagement of common pediatric urologic conditions on social media.

INTRODUCTION: As social media use continues to increase, parents and caregivers report using social media platforms as a source of health information. However, there are minimal regulations for social media content and health misinformation has been shared for various medical issues and urologic conditions. While internet content related to pediatric urology has been previously described, social media engagement for various pediatric urologic conditions have yet to be described. OBJECTIVE: To evaluate the evidence supporting articles engaged on social media that are related to common pediatric urologic conditions. STUDY DESIGN: A social media analysis tool was used to identify articles engaged through Facebook, Reddit, Twitter, and Pinterest between July 2020-2021. The top 5 articles related to toilet training, circumcision, cryptorchidism, testicular torsion, and hypospadias were identified. Article citations were reviewed and classified by Oxford levels of evidence. The content of each article was then reviewed and compared against supporting evidence on an independent literature search. Statistical analysis was completed with descriptive statistics, Mann-Whitney U, Wilcoxon signed rank, and bivariate correlation. RESULTS: Of the 25 articles reviewed, 8 (32%) were affiliated with medical journals, hospitals, or academic institutions and 17 (68%) were on non-affiliated websites with advertisements. There was greater social media engagement for articles related to toilet training and circumcision than testicular torsion, hypospadias, and cryptorchidism. No articles cited level 1 evidence and 32% of articles cited no evidence. Literature search for article content demonstrated a discrepancy between the level of evidence cited by articles compared to the evidence available in the literature to support article content. There was greater social media engagement for articles with no cited or supporting evidence and those not affiliated with medical journals, hospitals, or academic institutions. DISCUSSION: The findings in this study are consistent with trends reported for other urologic conditions, including genitourinary malignancy, female pelvic medicine and reconstructive surgery, nephrolithiasis, and sexual function. Parents without a medical background may have difficulty identifying whether articles shared on social media can be a reliable resource for health information. It is important to understand how information related to pediatric urologic conditions is engaged on social media so that misinformation can be addressed in clinical, online, and regulatory settings. CONCLUSION: There was greater social media engagement for articles with no cited or supporting evidence and those not affiliated with medical journals, hospitals, or academic institutions.

Long-term urological and gynecological outcomes following complete primary repair in females with bladder exstrophy.

INTRODUCTION: Long-term continence outcomes in patients undergoing complete primary repair of exstrophy (CPRE) have shown that a subset of patients do not achieve continence until after puberty. We aim to update the continence outcomes as well as describe gynecological outcomes for females having undergone CPRE. MATERIALS AND METHODS: This was a retrospective review between 1989 and 2019 at a single institution. Inclusion criteria were females with classic bladder exstrophy who underwent CPRE. Exclusion criteria were variant diagnoses and age <4 years. Continence was defined as dry for ≥3 h (volitional voiding or clean intermittent catheterization (CIC)). Partial continence was defined as minor stress incontinence or enuresis. Vaginal stenosis was evaluated in post-pubertal patients. RESULTS: Eighteen patients met inclusion criteria. Median age at last follow-up was 15.9 years (IQR 13.1, 18.4). All patients underwent primary closure <30 days of life (n = 18). Subsequent procedures included bladder augmentation (n = 4), continent catheterizable channel (n = 7), bladder neck injections (n = 12) and bladder neck reconstruction (n = 12). Continence and partial continence were achieved in 6/18 (33.3%) and 9/18 (50.0%), respectively, with mean 3.2 ± 2.5 continence procedures at 9.6 years (IQR 7.3, 15.2). Volitional voiding was seen in 11/18 (61.1%) and 7/18 (38.9%) performed CIC, with no significant difference in continence. Mean bladder capacity was 199 ml ± 96 versus 90 ml ± 29 in the volitional voiding versus CIC group (P = 0.0047). Eleven women with median age of 18.0 years (IQR 15.2, 21.4) had recorded menarche: 6/11 (54.5%) patients reported painful/irregular menses, controlled with hormonal therapy. Six of 11 (54.5%) women had vaginal stenosis managed with vaginal dilation (n = 2) or vaginoplasty (n = 4). Three (27.3%) reported tampon use and penetrative intercourse. CONCLUSION: Overall, the majority of women who have undergone CPRE achieved complete or partial continence, though most required additional procedures and time to attain it. Additionally, volitional voiding was achievable. Bladder capacity was significantly lower in patients dependent on CIC. Most required medical or surgical interventions for gynecologic concerns post menarche. This study underscores the unique needs of girls and young women with bladder exstrophy and further supports the importance of close long-term urologic and gynecologic management throughout development.

Experiences and attitudes of young adults with congenital bowel and bladder conditions.

INTRODUCTION: Young adults with complex congenital bowel and bladder anomalies are a vulnerable population at risk for poor health outcomes. Their experiences with the healthcare system and attitudes towards their health are understudied. OBJECTIVE: Our objective was to describe how young adults with congenital bladder and bowel conditions perceive their current healthcare in the domains of bladder and bowel management, reproductive health, and transition from pediatric to adult care. STUDY DESIGN: At a camp for children with chronic bowel and bladder conditions, we offered a 50-question survey to the 62 adult chaperones who themselves had chronic bowel and bladder conditions. Of the 51 chaperones who completed the survey (a response rate of 82%), 30 reported a congenital condition and were included. RESULTS: The cohort of 30 respondents had a median age of 23 years and almost half of the subjects (46%) reported not having transitioned into adult care. Most reported bowel (81%) and bladder (73%) management satisfaction despite high rates of stool accidents (85%), urinary accidents (46%), and recurrent urinary tract infections (70%). The majority of respondents (90%) expressed interest in having a reproductive health provider as part of their healthcare team. The median ages of the first conversation regarding transition to adult care and feeling confident in managing self-healthcare were 18 and 14 years, respectively. Most (85%) reported feeling confident in navigating the medical system. DISCUSSION: In this cohort of young adults who reported confidence with self-care and navigating the medical system, the proportion who had successfully transitioned into to adult care was low. These data highlight the need for improved transitional care and the importance of patient-provider and provider-provider communication throughout the transition process. CONCLUSION: These data highlight the need to understand the experience of each individual patient in order to provide care that aligns with their goals.

The impact of the dependent care provision on individuals with spina bifida transitioning to adulthood.

INTRODUCTION: Under the Affordable Care Act, the Dependent Care Provision (DCP) was enacted in 2010 and expanded healthcare coverage for millions of young adults ages 19-25 by allowing them to stay on their parents' insurance until age 26. It is unknown whether the DCP has impacted young adults with SB who are at risk for lapses in insurance coverage as they transition into adult care. OBJECTIVE: Our aim was to determine the impact of the DCP on access to care (insurance status) and healthcare-quality (hospital admissions for potentially preventable conditions). METHODS: Using the National Inpatient Sample (an all-payor national dataset of hospital admissions), we analyzed pre/post DCP changes for admissions of SB patients ages 19-25. Our outcomes of interest were rates of insurance coverage and proportion of admissions due to potentially preventable conditions (UTI, pyelonephritis, skin conditions, osteomyelitis, sepsis, and pneumonia). Analysis included a difference-in-differences logistic regression model which compared the pre/post DCP difference (2006-s quarter of 2010 vs. 2011-2013) in patients ages 19-25 to the difference in patients ages 26-32 who were ineligible for the DCP policy. RESULTS: For admissions of SB patients ages 19-25, the DCP was not associated with improved insurance status compared to admissions ages 26-32 (0% vs. -0.4%, p = 0.10) and rates of private insurance decreased in both age groups, but more so in ages 26-32 (-2.0% vs. -3.9%, p < 0.001). Private insurance rates increased for admissions of white patients ages 19-25 but not for black and Hispanic groups. An increase in overall insurance status was also seen in young adults from high-income zip codes. Admissions for potentially preventable conditions increased in both age groups by a similar degree (+2.6% vs. +2.5%, p = 0.82). DISCUSSION: Under the Affordable Care Act, the DCP failed to improve rates of private insurance or decrease rates of noninsurance for admissions of young adults with SB. Certain race and socioeconomic groups benefited more from this national healthcare policy. Meanwhile, admissions for potentially preventable conditions are common in spina bifida patients, and increased over the study period, suggesting a need for further investigation into optimizing the delivery of healthcare to this complex patient population. CONCLUSION: The DCP did not result in improved overall insurance rates or in improved rates of private insurance for admissions of SB patients 18-25 years old.