The association between caudal anesthesia and increased risk of postoperative surgical complications in boys undergoing hypospadias repair.

INTRODUCTION: Recent reports have suggested that caudal anesthesia may be associated with an increased risk of postoperative surgical complications. We examined our experience with caudal anesthesia in hypospadias repair to evaluate for increased risk of urethrocutaneous fistula or glanular dehiscence. METHODS: All hypospadias repairs performed by a single surgeon in 2001-2014 were reviewed. Staged or revision surgeries were excluded. Patient age, weight, hypospadias severity, surgery duration, month and year of surgery, caudal anesthesia use, and postoperative complications were recorded. Bivariate and multivariate statistical analyses were performed. RESULTS: We identified 395 single-stage primary hypospadias repairs. Mean age was 15.6 months; 326 patients had distal (83%) and 69 had proximal (17%) hypospadias. Caudal anesthetics were used in 230 (58%) cases; 165 patients (42%) underwent local penile block at the discretion of the surgeon and/or anesthesiologist. Complications of urethrocutaneous fistula or glanular deshiscence occurred in 22 patients (5.6%) and were associated with caudal anesthetic use (OR 16.5, 95% CI 2.2-123.8, P = 0.007), proximal hypospadias (OR 8.2, 95% CI 3.3-20.0, P < 0.001), increased surgical duration (OR 1.01, 95% CI 1.01-1.02, P < 0.001), and earlier year of practice (OR 3.0, 95% CI 1.2-7.9, P = 0.03 for trend). After adjusting for confounding variables via multivariable logistic regression, both caudal anesthetic use (OR 13.4, 95% CI 1.8-101.8, P = 0.01) and proximal hypospadias (OR 6.8, 95% CI 2.7-16.9, P < 0.001) remained highly associated with postoperative complications. CONCLUSIONS: In our experience, caudal anesthesia was associated with an over 13-fold increase in the odds of developing postoperative surgical complications in boys undergoing hypospadias repair even after adjusting for urethral meatus location. Until further investigation occurs, clinicians should carefully consider the use of caudal anesthesia for children undergoing hypospadias repair.

Rare variants in tenascin genes in a cohort of children with primary vesicoureteric reflux.

BACKGROUND: Primary vesicoureteral reflux (PVUR) is the most common malformation of the kidney and urinary tract, and reflux nephropathy is a major cause of chronic kidney disease in children. Recently, we reported mutations in the tenascin XB gene (TNXB) as a cause of PVUR with joint hypermobility. METHODS: To define the role of rare variants in tenascin genes in the etiology of PVUR, we screened a cohort of patients with familial PVUR (FPVUR) and non-familial PVUR (NFPVUR) for rare missense variants inTNXB and the tenascin C gene (TNC) after excluding mutations in ROBO2 and SOX17. RESULTS: The screening procedure identified 134 individuals from 112 families with PVUR; two families with mutations in ROBO2 were excluded from further analysis. Rare missense variants in TNXB were found in the remaining 110 families, of which 5/55 (9%) families had FPVUR and 2/55 (4%) had NFPVUR. There were no differences in high-grade reflux or renal parenchymal scarring between patients with and without TNXB variants. All patients with TNXB rare variants who were tested exhibited joint hypermobility. Overall we were able to identify causes of FPVUR in 7/57 (12%) families (9% in TNXB and 3% in ROBO2). CONCLUSIONS: In conclusion, the identification of a rare missense variant in TNXB in combination with a positive family history of VUR and joint hypermobility may represent a non-invasive method to diagnose PVUR and warrants further evaluation in other cohorts.

Emergent care patterns in patients with spina bifida: a case-control study.

PURPOSE: Individuals with spina bifida are typically followed closely as outpatients by multidisciplinary teams. However, emergent care of these patients is not well defined. We describe patterns of emergent care in patients with spina bifida and healthy controls. MATERIALS AND METHODS: We reviewed Nationwide Emergency Department Sample data from 2006 to 2010. Subjects without spina bifida (controls) were selected from the sample using stratified random sampling and matched to each case by age, gender and treatment year at a 1:4 ratio. Missing emergency department charges were estimated by multiple imputation. Statistical analyses were performed to compare patterns of care among emergency department visits and charges. RESULTS: A total of 226,709 patients with spina bifida and 888,774 controls were identified. Mean age was 28.2 years, with 34.6% of patients being younger than 21. Patients with spina bifida were more likely than controls to have public insurance (63.7% vs 35.4%, p <0.001) and to be admitted to the hospital from the emergency department (37.0% vs 9.2%, p <0.001). Urinary tract infections were the single most common acute diagnosis in patients with spina bifida seen emergently (OR 8.7, p <0.001), followed by neurological issues (OR 2.0, p <0.001). Urological issues were responsible for 34% of total emergency department charges. Mean charges per encounter were significantly higher in spina bifida cases vs controls ($2,102 vs $1,650, p <0.001), as were overall charges for patients subsequently admitted from emergent care ($36,356 vs $29,498, p <0.001). CONCLUSIONS: Compared to controls, patients with spina bifida presenting emergently are more likely to have urological or neurosurgical problems, to undergo urological or neurosurgical procedures, to be admitted from the emergency department and to incur higher associated charges.

Estimating the nationwide, hospital based economic impact of pediatric urolithiasis.

PURPOSE: The incidence of urolithiasis is increasing in children and adolescents but the economic impact of this problem is unclear. We examined 2 large databases to estimate the nationwide economic impact of pediatric urolithiasis. MATERIALS AND METHODS: We analyzed the 2009 NEDS and KID, used ICD-9-CM codes to identify children 18 years or younger diagnosed with urolithiasis and abstracted demographic and charge data from each database. RESULTS: We identified 7,348 weighted inpatient discharges in KID and 33,038 emergency department weighted encounters in NEDS. Of the patients 32% and 36% were male, respectively. Inpatients were younger than those who presented to the ED (mean age 13.9 vs 15.7 years). Most patients had private insurance (52.9% to 57.2%) and the South was the most common geographic region (39.5% to 44.4%). The most common procedures were ureteral stent placement in 20.4% to 24.1% of cases, followed by ureteroscopy in 3.8% to 4.4%. Median charges per admission were $13,922 for a weighted total of $229 million per year. Median emergency department charges were $3,991 per encounter for a weighted total of $146 million per year. CONCLUSIONS: Each day in 2009 in the United States an estimated 20 children were hospitalized and 91 were treated in the emergency department for upper tract stones. A conservative estimate of 2009 annual charges related to pediatric urolithiasis in the United States is at least $375 million. This is likely a significant underestimate of the true economic burden of pediatric urolithiasis because it accounts for neither outpatient management nor indirect costs such as caregiver time away from work.

Inflammatory response to Escherichia coli urinary tract infection in the neurogenic bladder of the spinal cord injured host.

PURPOSE: Urinary tract infections cause significant morbidity in patients with spinal cord injury. An in vivo spinal cord injured rat model of experimental Escherichia coli urinary tract infection mimics human disease with enhanced susceptibility to urinary tract infection compared to controls. We hypothesized that a dysregulated inflammatory response contributes to enhanced susceptibility to urinary tract infection. MATERIALS AND METHODS: Spinal cord injured and sham injured rats were inoculated transurethrally with E. coli. Transcript levels of 84 inflammatory pathway genes were measured in bladder tissue of each group before infection, 24 hours after infection and after 5 days of antibiotic therapy. RESULTS: Before infection quantitative polymerase chain reaction array revealed greater than twofold up-regulation in the proinflammatory factor transcripts slc11a1, ccl4 and il1ß, and down-regulation of the antimicrobial peptides lcn2 and mpo in spinal cord injured vs control bladders. At 24 hours after infection spinal cord injured bladders showed an attenuated innate immune response with decreased expression of il6, slc11a1, il1ß and lcn2, and decreased il10 and slpi expression compared to controls. Despite clearance of bacteriuria with antibiotics spinal cord injured rats had delayed induction of il6 transcription and a delayed anti-inflammatory response with decreased il10 and slpi transcript levels relative to controls. CONCLUSIONS: Spinal cord injured bladders fail to mount a characteristic inflammatory response to E. coli infection and cannot suppress inflammation after infection is eliminated. This may lead to increased susceptibility to urinary tract infection and persistent chronic inflammation through neural mediated pathways, which to our knowledge remain to be defined.

Use of nephron sparing surgery and impact on survival in children with Wilms tumor: a SEER analysis.

PURPOSE: Nephron sparing surgery is the standard of care for many adults with renal tumors and has been described in some children with Wilms tumor. However, beyond case series the data concerning nephron sparing surgery application and outcomes in patients with Wilms tumor are scarce. We examined nephron sparing surgery outcomes and factors associated with its application in children with Wilms tumor. MATERIALS AND METHODS: We retrospectively reviewed the 1998 to 2010 SEER database. We identified patients 18 years old or younger with Wilms tumor. Clinical, demographic and socioeconomic data were abstracted, and statistical analysis was performed using multivariate logistic regression (predicting use of nephron sparing surgery limited to unilateral tumors smaller than 15 cm) and Cox regression (predicting overall survival) models. RESULTS: We identified 876 boys and 956 girls with Wilms tumor (mean ± SD age 3.3 ± 2.9 years). Of these patients 114 (6.2%) underwent nephron sparing surgery (unilateral Wilms tumor in 74 and bilateral in 37). Median followup was 7.1 years. Regarding procedure choice, nephron sparing surgery was associated with unknown lymph node status (Nx vs N0, p <0.001) and smaller tumor size (p <0.001). Regarding survival, only age (HR 1.09, p = 0.002), race (HR 2.48, p = 0.002), stage (HR 2.99, p <0.001) and lymph node status (HR 2.17, p = 0.001) predicted decreased overall survival. Survival was not significantly different between children undergoing nephron sparing surgery and radical nephrectomy (HR 0.79, p = 0.58). CONCLUSIONS: In children with Wilms tumor included in the SEER database nephron sparing surgery has been infrequently performed. Nephron sparing surgery application is associated with smaller, bilateral tumors and with omission of lymphadenectomy. However, there are no evident differences in application of nephron sparing surgery based on demographic or socioeconomic factors. Despite lymph node under staging, overall survival is similar between patients undergoing nephron sparing surgery and radical nephrectomy.

TNXB mutations can cause vesicoureteral reflux.

Primary vesicoureteral reflux (VUR) is the most common congenital anomaly of the kidney and the urinary tract, and it is a major risk factor for pyelonephritic scarring and CKD in children. Although twin studies support the heritability of VUR, specific genetic causes remain elusive. We performed a sequential genome-wide linkage study and whole-exome sequencing in a family with hereditary VUR. We obtained a significant multipoint parametric logarithm of odds score of 3.3 on chromosome 6p, and whole-exome sequencing identified a deleterious heterozygous mutation (T3257I) in the gene encoding tenascin XB (TNXB in 6p21.3). This mutation segregated with disease in the affected family as well as with a pathogenic G1331R change in another family. Fibroblast cell lines carrying the T3257I mutation exhibited a reduction in both cell motility and phosphorylated focal adhesion kinase expression, suggesting a defect in the focal adhesions that link the cell cytoplasm to the extracellular matrix. Immunohistochemical studies revealed that the human uroepithelial lining of the ureterovesical junction expresses TNXB, suggesting that TNXB may be important for generating tensile forces that close the ureterovesical junction during voiding. Taken together, these results suggest that mutations in TNXB can cause hereditary VUR.

A multi-site cultural and linguistic adaptation of a hypospadias decision aid for Latinx communities.

INTRODUCTION: Latinx, Spanish-speaking (LSS) patients are more likely to experience decisional conflict and regret about healthcare decisions than non-Hispanic, white, English-speaking patients. OBJECTIVE: To adapt the Hypospadias Hub (Hub), a rigorously developed and tested web-based decision aid (DA), for LSS parents. METHODS: Guided by the Ecological Validity Model (EVM), a heuristic framework was followed to culturally adapt the Hub (see Extended Summary Figure). In stage 1, recommendations were obtained from a focus group with members of the institution's Latinx Community Review Board (Latinx CRB) and semi-structured interviews with pediatricians with Latinx-focused practices. In stage 2, preliminary cultural modifications were made, the Hub was translated into Spanish, and a second focus group with the Latinx CRB was convened to review the revised Hub. In stage 3, semi-structured interviews with LSS parents of healthy boys (i.e., without hypospadias) ≤ 5 years old were conducted to identify any cultural adaptations and/or usability issues regarding the revised Hub. In stage 4, based on parents' feedback, final revisions to the Hub were made. The focus groups and parent interviews were conducted in Spanish; then, the recordings were professionally transcribed in Spanish and translated into English. Interviews with pediatricians were conducted in English; then, the recordings were professionally transcribed. Three coders conducted a qualitative content analysis to identify areas for revision. Changes were applied iteratively. RESULTS: Participants included 3 Latinx CRB members (2 women, 1 did not disclose gender; mean age = 48.3, SD = 21.2), 3 non-Latinx pediatricians (2 women, 1 man; mean age = 49.6, SD = 9.1), and 5 Latinx mothers (mean age = 34.0, SD = 1.26). Participants recommended: 1) featuring video testimonials from Latinx families or including Spanish voice-overs/subtitles; 2) diversifying racial/ethnic/geographic representation and including extended families in photographs/illustrations; 3) adding information about health insurance coverage and circumcision, 4) reassuring parents that the condition is not their fault, 5) considering cultural values (e.g., reliance on expert advice), and 6) clarifying medical terminology. Feedback related to seven EVM dimensions: concepts, content, context, goals, language, metaphors, and methods. DISCUSSION: Participants perceived the Hub to be informative in guiding parents' treatment decisions. Revisions were reasonable and acceptable for a linguistic and cultural adaptation for LSS parents. CONCLUSIONS: We identified and implemented preliminary cultural modifications to the Hub and applied user-centered design methods to test and revise the website. The product is a culturally appropriate DA for LSS parents. Next, English and Spanish-versions of the Hub will be tested in a randomized controlled trial.

Enhanced susceptibility to urinary tract infection in the spinal cord-injured host with neurogenic bladder.

Neurogenic bladder predisposes to recurrent urinary tract infections (UTI) and renal failure, and susceptibility is commonly ascribed to urinary stasis from elevated residual urine volumes. Escherichia coli UTI was modeled in the spinal cord-injured (SCI) rat with the hypothesis that SCI animals would require fewer bacteria to establish infection, have an exaggerated inflammatory response, and have delayed clearance of infection compared to normal-voiding controls. T10 SCI rats and controls had median infectious doses (ID50) of 10(2) and 10(5) CFU, respectively. Mean residual volumes in the SCI animals did not correlate with susceptibility to initiation of UTI or outcome. In the acute infection, control and SCI rats developed acute cystitis and pyelitis without acute differences in histopathological scores of inflammation. However, in vivo imaging of infected animals revealed persistently higher levels of bacteria in the SCI urine and bladders than were seen for controls over 2 weeks. Likewise, at 2 weeks, acute and chronic inflammatory infiltrates persisted in the bladders and kidneys of SCI rats, whereas inflammation largely resolved within the controls. Together these data demonstrate that SCI rats exhibit delayed clearance of infection and exaggerated inflammatory responses in bladders and kidneys; however, the severity of residual volumes does not predict increased susceptibility to UTI. These studies suggest that host-dependent mechanisms that are discrete from alterations in bladder physiology influence UTI susceptibility with the SCI-neurogenic bladder. This model will allow elucidation of SCI-neurogenic bladder-mediated changes in host response that yield UTI susceptibility and may lead to new preventative and therapeutic options.

Experience with glycerin for antegrade continence enema in patients with neurogenic bowel.

PURPOSE: Malone antegrade continence enemas are used in the management of neurogenic bowel to attain fecal continence. Several different irrigation solutions have been described but glycerin, an osmotic laxative that promotes peristalsis, has rarely been mentioned or studied. We assessed clinical outcomes in our patients with a Malone antegrade continence enema using glycerin based irrigation. MATERIALS AND METHODS: We retrospectively reviewed patients with neurogenic bowel who underwent a Malone antegrade continence enema procedure between 1997 and 2011. Glycerin diluted with tap water followed by a tap water flush is our preferred irrigation protocol. Bowel regimen outcomes examined included fecal continence, emptying time, leakage from stoma, enema volume, frequency and independence. RESULTS: Of the 23 patients with followup greater than 6 months 19 used glycerin based irrigation. Average age at surgery was 8.8 years. Patients using glycerin instilled a median of 30 ml (mean 29) glycerin and 50 ml (131) tap water. Fecal continence rate was 95% and stoma leakage rate was 16%, and only 16% of patients required daily irrigation. CONCLUSIONS: Glycerin is a viable and effective alternative irrigant for antegrade enemas of neurogenic bowel, with an excellent fecal continence rate. The volume of irrigant needed is typically less than 90 ml, which is much less than in published reports using tap water alone.

Contemporary epidemiological trends in complex congenital genitourinary anomalies.

PURPOSE: Anecdotal evidence suggests that complex congenital genitourinary anomalies are occurring less frequently. However, few epidemiological studies are available to confirm or refute this suggestion. MATERIALS AND METHODS: The Kids' Inpatient Database (KID) is a national, all payer database of several million inpatient pediatric hospitalizations per year, including complicated and uncomplicated in-hospital births. We reviewed the 1997 to 2009 KID to determine the birth prevalence of spina bifida, posterior urethral valves, bladder exstrophy, epispadias, prune belly syndrome, ambiguous genitalia and imperforate anus. For posterior urethral valves and prune belly syndrome we limited our search to newborn males only. RESULTS: During the study period, there was a diagnosis of spina bifida in 3,413 neonates, bladder exstrophy in 214, epispadias in 1,127, ambiguous genitalia in 726, prune belly syndrome in 180, posterior urethral valves in 578 and imperforate anus in 4,040. We identified no significant change in the birth prevalence of spina bifida (from 33.9 new spina bifida births of 100,000 uncomplicated births to 29.0/100,000, p = 0.08), posterior urethral valves (from 10.4/100,000 to 11.0/100,000, p = 0.51), prune belly syndrome (from 4.8/100,000 to 3.3/100,000, p = 0.44) or ambiguous genitalia (from 5.82/100,000 to 5.87/100,000, p = 0.38). There was a significant decrease in the birth prevalence of bladder exstrophy (from 2.4/100,000 to 1.6/100,000 uncomplicated births, p = 0.01) and a significant increase in epispadias (from 8.0/100,000 to 11.6/100,000) and imperforate anus (from 33.6/100,000 to 35.0/100,000, each p = 0.04) during the study period. CONCLUSIONS: The birth prevalence of spina bifida, posterior urethral valves and prune belly syndrome appears to have been stable in the last 12 years. Epispadias, ambiguous genitalia and imperforate anus diagnoses in newborns became more common in the same period, while bladder exstrophy diagnoses became less common.

The effect of dietary sodium and fructose intake on urine and serum parameters of stone formation in a pediatric mouse model: a pilot study.

PURPOSE: Dietary factors have a role in stone disease in adults but little is known about the relationship in children. We tested whether diet could alter urine and serum parameters in a pediatric mouse model. MATERIALS AND METHODS: We randomized 30 female BALB/c mice at age 3 weeks to mouse chow, a complex carbohydrate Western diet or a high fructose, high sodium Western diet. Body weight was measured twice weekly. Urine was periodically collected and mice were sacrificed on day 30. Samples were pooled to analyze serum and urine electrolyte factors. RESULTS: There was no significant difference in body weights among the groups at any time or in kidney weight at sacrifice (each p >0.15). There was no observable increase in urine stone forming analytes across the arms except uric acid, which tended to be higher in the Western diet and high fructose, high sodium Western diet groups. There was a substantial decrease in urinary stone inhibitors (magnesium and citrate), a decrease in urinary potassium and an increase in serum calcium in the 2 Western diet groups. CONCLUSIONS: This pilot study suggests that consuming a Western diet, especially one composed of high fructose and sodium, may alter parameters that predispose to stone formation in children by decreasing stone inhibitors. Results require validation in larger studies and ultimately in humans.

Outcomes of intermediate-risk hydronephrosis in pediatric patients.

PURPOSE: Hydronephrosis is a common antenatal diagnosis and is present in approximately 1-5% of pregnancies. The urinary tract dilation (UTD) classification system was introduced in 2014 and stratifies post-natal hydronephrosis risk into three groups: low-risk (P1), intermediate-risk (P2), and high-risk (P3). Recommendations for P3 hydronephrosis have been established, whereas those for P1 and P2 UTD are often left to the discretion of providers with P1 considered low-grade and less concerning significant pathology. Given the obscure nature of P2 hydronephrosis, we sought to determine the natural history and outcomes of pediatric patients with P2 hydronephrosis within a single institution. MATERIALS AND METHODS: Children <18 years old diagnosed with hydronephrosis between January 2015 and December 2018 were identified by ICD-9 and ICD-10 codes. Patients with P1 hydronephrosis, P3 hydronephrosis, known vesicoureteral reflux, complex anomalies (ex. Posterior urethral valve), neurological impairments, neurogenic bladder secondary to spinal abnormalities, and <6 months of follow-up were excluded. The development of urinary tract infection (UTI; ≥100 000 CFU/mL of bacterial growth, UA > 10 WBCs/hpf with fever >38C), need for surgical intervention (impaired renal function, worsening hydronephrosis, and/or delayed drainage on diuretic renography), and stability of hydronephrosis were collected retrospectively. RESULTS: Eighty-seven patients [105 renal units (RU)] were included. Twenty-six patients (30%) were female and 61 (70%) were male. Of the male patients, 30 (49%) of them were circumcised. The median age at initial evaluation was 1 month, and the median duration of follow-up was 13 months. Thirty-four (32%) RU had complete resolution, 24 (23%) improved to P1 hydronephrosis, 33 (31%) remained stable, and 14 (13%) progressed to P3 hydronephrosis. The median duration to resolution and improvement was 8.5 months and 5 months, respectively. Eleven (11%) RU required surgical intervention, 10 of which underwent pyeloplasty, with a median duration to intervention of 9 months. Fifty-five patients (63%) received antibiotic prophylaxis (amoxicillin or sulfamethoxazole-trimethoprim) for a median duration of 5 months. Nine patients (10%) developed a UTI, 3 of which were taking antibiotic prophylaxis at the time of infection. CONCLUSIONS: Intermediate-risk hydronephrosis diagnosed in the pediatric population will either improve, resolve, or remain stable during 1-year follow-up in 87% of RU. Only 11% of RU required surgical intervention, and 19% of patients developed a UTI in the absence of antibiotic prophylaxis. These findings will assist with counseling parents concerning the importance of follow-up imaging and monitoring for UTI. However, the low risk of surgical intervention is encouraging and should be discussed with the children's caretakers.

A multi-site pilot study of a parent-centered tool to promote shared decision-making in hypospadias care.

BACKGROUND: Using a user-centered design approach, we conducted a two-site pilot study to evaluate a decision aid (DA) website, the Hypospadias Hub, for parents of hypospadias patients. OBJECTIVES: The objectives were to assess the Hub's acceptability, remote usability, and feasibility of study procedures, and to evaluate its preliminary efficacy. METHODS: From June 2021-February 2022, we recruited English-speaking parents (≥18 years old) of hypospadias patients (≤5 years) and delivered the Hub electronically ≤2 months before their hypospadias consultation. We collected website analytic data using an ad tracker plug-in. We inquired about treatment preference, hypospadias knowledge, and decisional conflict (Decisional Conflict Scale) at baseline, after viewing the Hub (pre-consultation), and post-consultation. We administered the Decision Aid Acceptability Questionnaire (DAAQ) and the Preparation for Decision-Making Scale (PrepDM) which assessed how well the Hub prepared parents for decision-making with the urologist. Post-consultation, we assessed participants' perception of involvement in decision-making with the Shared Decision-making Questionnaire (SDM-Q-9) and the Decision Regret Scale (DRS). A bivariate analysis compared participants' baseline and pre/post-consultation hypospadias knowledge, decisional conflict, and treatment preference. Using a thematic analysis, we analyzed our semi-structured interviews to uncover how the Hub impacted the consultation and what influenced participants' decisions. RESULTS: Of 148 parents contacted, 134 were eligible and 65/134 (48.5%) enrolled: mean age 29.2, 96.9% female, 76.6% White (Extended Summary Figure). Pre/post-viewing the Hub, there was a statistically significant increase in hypospadias knowledge (54.3 vs. 75.6, p < 0.001) and decrease in decisional conflict (36.0 vs. 21.9, p < 0.001). Most participants (83.3%) thought Hub's length and amount of information (70.4%) was "about right", and 93.0% found most or everything was clear. Pre/post-consultation, there was a statistically significant decrease in decisional conflict (21.9 vs. 8.8, p < 0.001). PrepDM's mean score was 82.6/100 (SD = 14.1); SDM-Q-9's mean score was 82.5/100 (SD = 16.7). DCS's mean score was 25.0/100 (SD = 47.03). Each participant spent an average of 25.75 min reviewing the Hub. Based on thematic analysis, the Hub helped participants feel prepared for the consultation. DISCUSSION: Participants engaged extensively with the Hub and demonstrated improved hypospadias knowledge and decision quality. They felt prepared for the consultation and perceived a high degree of involvement in decision-making. CONCLUSION: As the first pilot test of a pediatric urology DA, the Hub was acceptable and study procedures were feasible. We plan to conduct a randomized controlled trial of the Hub versus usual care to test its efficacy to improve the quality of shared decision-making and reduce long-term decisional regret.

In focus: perplexing increase of urinary stone disease in children, adolescent and young adult women and its economic impact.

Background: Urinary stone disease (USD) historically has affected older men, but studies suggest recent increases in women, leading to a near identical sex incidence ratio. USD incidence has doubled every 10 years, with disproportionate increases amongst children, adolescent, and young adult (AYA) women. USD stone composition in women is frequently apatite (calcium phosphate), which forms in a higher urine pH, low urinary citrate, and an abundance of urinary uric acid, while men produce more calcium oxalate stones. The reasons for this epidemiological trend are unknown. Methods: This perspective presents the extent of USD with data from a Canadian Province and a North American institution, explanations for these findings and offers potential solutions to decrease this trend. We describe the economic impact of USD. Findings: There was a significant increase of 46% in overall surgical interventions for USD in Ontario. The incidence rose from 47.0/100,000 in 2002 to 68.7/100,000 population in 2016. In a single United States institution, the overall USD annual unique patient count rose from 10,612 to 17,706 from 2015 to 2019, and the proportion of women with USD was much higher than expected. In the 10-17-year-old patients, 50.1% were girls; with 57.5% in the 18-34 age group and 53.6% in the 35-44 age group. The roles of obesity, diet, hormones, environmental factors, infections, and antibiotics, as well as the economic impact, are discussed. Interpretation: We confirm the significant increase in USD among women. We offer potential explanations for this sex disparity, including microbiological and pathophysiological aspects. We also outline innovative solutions - that may require steps beyond typical preventive and treatment recommendations.

How dry is dry? A review of definitions of continence in the contemporary exstrophy/epispadias literature.

PURPOSE: Definitions of continence following surgery in children with exstrophy-epispadias complex vary widely. We assessed the most common definitions of continence and evaluated the clinical significance of usage patterns for those definitions. MATERIALS AND METHODS: We searched MEDLINE and EMBASE (2000 to 2011) for English language reports describing postoperative continence outcomes in children with exstrophy-epispadias complex. Articles were evaluated and data were abstracted by 2 reviewers. We assessed patient level factors such as age, preoperative diagnoses and use of additional therapies, as well as study level factors such as continence definition(s), country of origin and method of data collection. RESULTS: We identified 884 articles, of which 87 met inclusion criteria. In total these studies included continence outcomes data on 2,681 patients (57% male). Only 59 studies (68%) clearly defined the term "continence." The most common definition of continence was dry with voiding/catheterization every 3 hours (used in 23 studies, or 39%, defining continence). There was no association between publication date (p = 0.17), study location (p = 0.47) or study size (p = 0.81) and continence definition. There was a trend toward improved reporting of methods for continence ascertainment in more recent years (p = 0.02). Of the 2,681 children included 1,372 (51%) were dry by the definition used in their study. CONCLUSIONS: The most frequent definition of continence was "dryness with voiding or catheterization at 3-hour intervals." However, definitions were highly variable and many authors did not define continence at all. To better define outcomes, we recommend that a standardized definition of continence be established and used in future reports.

Upper pole pathologies in duplex kidneys: an analysis of predictive factors for surgery and urinary tract infections from the Mid-Atlantic Pediatric Academic Consortium.

BACKGROUND/OBJECTIVE: While there is significant data on the natural history and outcomes for prenatal hydronephrosis in simplex kidneys, duplex kidneys tend to be less studied. Management can be quite variable based on provider preference. We aimed to describe practice patterns from several tertiary academic institutions, identify clinical predictors for surgical intervention and urinary tract infection (UTI) for upper pole pathology, and demonstrate the natural history of lower pole vesicoureteral reflux (VUR). METHODS: We conducted a retrospective review of patients from 4 Mid-Atlantic institutions between 2015 and 2020. Inclusion criteria included patients with a duplex kidney with upper pole pathology and/or lower pole VUR. The primary outcome was predictive factors for surgical intervention and UTI. The secondary outcome was to assess the natural history of lower pole VUR including resolution rates by grade. Linear regression identified clinical predictors for UTI events. Multivariate logistic regression identified predictors of surgical intervention, UTI, and lower pole VUR resolution. Descriptive statistics and regression modeling analyses were performed using SAS. RESULTS: Two hundred forty-two patients were included with a total of 271 duplex renal units. Hydronephrosis grade (both SFU and UTD grading) and number of prior UTI events were statistically significant predictors for surgical intervention (p = 0.03/0.001 and p = 0.002 respectively). Ectopic ureter (p = 0.004), ureterocele (p = 0.02), and obstruction (p = 0.04) were the only pathologies predictive for surgery. Male gender and circumcision were significantly associated with decreased UTI risk (p = 0.03 and p = 0.01). On linear regression modeling, antibiotic prophylaxis after the first year of life was associated with decreased risk of further UTI events (p = 0.03); however, antibiotic prophylaxis within the first year of life did not decrease UTI risk (p = 0.14). For VUR outcomes, 65.0% of grades 1-3 VUR and 52.2% of grades 4-5 had resolution/improvement at mean time of 2.1 years. There were no predictive factors for resolution/improvement of VUR. CONCLUSIONS: Hydronephrosis grade and UTI events were significant predictors for surgical intervention for upper pole pathology. Pathologies that were predictive for surgery included ectopic ureter, ureterocele and obstruction. Male gender, circumcision and antibiotic prophylaxis after the first year of life were associated with a decreased UTI risk. Roughly 58% of lower pole VUR spontaneously improved/resolved. Identification of these risk factors aids in standardization of care practices to reduce long-term UTI risk and inform counseling with families about possible need for surgical intervention and expectations for long term outcomes.

Defining Urinary Tract Infections in Children With Spina Bifida: A Systematic Review.

CONTEXT: Children with spina bifida are at high risk for urinary tract infections (UTI). However, there is no standardized definition of UTI in this population, leading to variability in both clinical management and research. This was highlighted in the 2013 systematic review on the same topic. OBJECTIVE: Evaluate the frequency with which researchers are defining UTI in their studies of children with spina bifida and to determine what parameters are used. DATA SOURCES: We searched Medline and Scopus databases for articles that included pediatric patients with spina bifida and used UTI as an outcome. STUDY SELECTION: Exclusion criteria included publication before October 1, 2012, non-English language, and nonprimary research articles. DATA EXTRACTION: Two independent reviewers each extracted data. RESULTS: A total of 39 studies were included; 74% of these analyzed included an explicit definition of UTI. The most commonly used definition included a combination of symptoms and culture results (34.5%), whereas 31% used a combination of symptoms, culture results, and urinalysis data. Only 3.4% of articles used a urine culture alone to define UTI. CONCLUSIONS: More articles that focus on children with spina bifida included a definition of UTI. However, significant variability persists in the definition of UTI in this patient population.