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Research ObjectiveTelemedicine is now a critical healthcare delivery modality. Prior studies of telemedicine in pediatric urology are lacking parent/patient perspectives. This study aims to assess interest in and factors associated with willingness to participate in telemedicine, and to compare perceptions of telemedicine before and after pandemic-related implementation.Study DesignThis was a cross-sectional study of parents of pediatric urology patients that attended in-person clinic visits (Pre-Telemedicine cohort; January-March 2020) and telemedicine visits (Post-Telemedicine cohort; April-July 2020).Principal FindingsThe response rates were 83.4% (Pre-Telemedicine, N =205) and 32.5% (Post-Telemedicine, N =89). Overall, most parents had a college or graduate level degree (71.9%), private insurance (71.6%), and a plurality were 36-40 years old (30.7%). Their children were mostly white (68.9%), 23.7% were Hispanic/Latino, and the median patient age was 3.3 years (range: 0.1–32.1). There were no demographic differences between the Pre-and-Post-Telemedicine sub-groups.Pre-TelemedicineMost (84.3%) were willing to participate in a new, follow-up, or post-operative telemedicine visit. There were no associations between demographics and willingness. Comfort in video visits without a physical exam (3.23% Unwilling vs 38.2% Willing, = Post-TelemedicineMost indicated that telemedicine met their needs (88.6%), and that they were satisfied with the both the quality of technology used (83%) and the interaction with the urologist (89.9%) during the telemedicine visit.Comparison of Pre-and-Post-TelemedicineIn reference to the Pre-Telemedicine respondents, Post-Telemedicine respondents more frequently strongly agreed that telemedicine is as private (51.5% vs 77.5%, p = ConclusionsMost parents had high willingness to participate and positive perceptions of telemedicine prior to the COVID-19 era. Factors associated with willingness were elucidated. Families that participated in telemedicine reduced direct costs and saved time. Lastly, these results suggest that confidence in telemedicine had increased after experience with telemedicine. These data support continued telemedicine access and coverage/reimbursement beyond the pandemic.

Objective To examine the variability of estimated glomerular filtration rate (eGFR) in emerging adults with spina bifida (SB) by comparing multiple equations across the transitional age period, hypothesizing that creatinine (Cr)-based equations show greater variability than cystatin-C (CysC)- or combination-based equations. Methods A retrospective cohort study was performed from 2012 to 2017 at a multidisciplinary SB clinic. Emerging adults were defined as patients ages 18-28 years old. Four pediatric, 3 adult, and 3 averaged eGFR equations were considered. Cross-sectional variability in eGFR data was assessed using coefficients of variation, chronic kidney disease (CKD) stage classification, and pairwise percent relative difference in eGFR between analogous pediatric and adult equations based on included lab values. Longitudinal changes in eGFR over time were compared across equations using a covariance pattern model accounting for repeated measures. Results Seventy-five emerging adults with SB (median age 21.8 years; 55% female; 83% with myelomeningocele) were included in cross-sectional analyses. Adult equations gave higher median eGFRs by 22%-27% and generally milder CKD stage classification than analogous pediatric equations. In longitudinal analyses (median follow-up of 22 months), all equations conferred negative eGFR changes over time (range −1.9 to −4.3 mL/min/1.73m2 per year) that were not significantly different. Conclusion In emerging adults with SB, adult equations demonstrated higher median eGFRs by 22%-27% compared to analogous pediatric equations, even with Cystatin-C, and generally downstaged CKD stage classification. The same eGFR equation should be used for serial kidney function monitoring in emerging adults with SB who transition care from pediatric to adult services.

Prenatal diagnoses of differences of sex development (DSD) are increasing due to availability of cell-free DNA screening (cell-free DNA screening (cfDNA)). This study explores first-hand experiences of parents whose children had prenatal findings of DSD.Eligible parents were identified through chart review at a pediatric center and interviewed about their prenatal evaluation, decision making, informational sources, and support systems. Interviews were coded using a combined inductive and deductive thematic analysis. Parents also completed quantitative measures of decisional regret.Seventeen parents (13 mothers; 4 fathers) of 13 children (with 7 DSD diagnoses) were recruited. Four children had discordance between sex predicted by cfDNA versus prenatal ultrasound, and 2 had non-binary appearing (atypical) genitalia on prenatal ultrasound. Of these 6, 3 were not offered additional prenatal testing or counseling. Most parents described tension between obtaining support through disclosure of their child's diagnosis and preserving their child's autonomy/privacy, highlighting the need for mental health support.This is the first study to gather qualitative data from parents whose children had prenatal findings of DSD. We identified multiple targets for intervention to improve care for patients with DSD across the lifespan, including improvements in clinician education, pre- and post-test counseling, and patient education materials.

Extended Summary Introduction Differences/disorders of sex development (DSD) can be detected at different ages, including prenatally. The recent implementation of prenatal genetic testing (including cell-free DNA) may affect the frequency and impact of prenatal diagnosis of DSD. Our aims were to (1) describe prenatal detection and evaluation of differences of sex development presenting to a multidisciplinary DSD clinic and (2) explore possible parental distress accompanying this evaluation. Materials and methods A retrospective chart review of mothers presenting prenatally, and patients presenting during infancy, to a multidisciplinary DSD clinic from 2013 to 2017 was conducted. Data extracted included demographics, final diagnoses, prenatal screening, prenatal evaluation, postnatal endocrine, genetic and radiologic testing, and clinician’s notes on parent/patient distress. Results Sixty-seven patients were identified; ten (15%) had prenatal detection of a suspected DSD. Of those, 4/10 were detected prenatally in the last study year alone. Within the prenatal group, 6/10 had cell-free DNA results discordant with ultrasound, 2/10 were detected by atypical genitalia on ultrasound, and 2/10 were detected through karyotyping performed for other indications. After birth, 3/10 patients were found to not have a DSD. Final diagnoses for the full study cohort are shown in the Summary Table, comparing prenatal versus postnatal presentation to our DSD clinic. Clinicians noted distress for most parents during the prenatal evaluation of a possible DSD, including one mother who reported suicidal thoughts. Discussion and conclusions Prenatal suspicion of DSD can occur through discordant prenatal testing and has been observed at our clinic in recent years, in line with other recent studies. Contributing factors to these prenatal presentations could be increased referrals to the clinic, and increased use of non-invasive prenatal testing, which can lead to inaccurate or discordant sex identification. The prenatal suspicion of a potential DSD can be associated with parental distress, underscoring the need for adequate counseling for tests that determine fetal sex, including cell-free DNA. Summary Table . Final Diagnoses, Prenatal Versus Postnatal Cohorts Total (n = 67) Prenatal cohort (n = 10) Postnatal cohort (n = 57) Final diagnosis 46, XX DSD 3 (4%) 0 3 (5%) Disorders of gonadal development 1 (1%) 1 (2%) Androgen excess 2 (3%) 2 (4%) 46, XY DSD 49 (73%) 4 (40%) 45 (79%) Disorders of gonadal development 6 (9%) 1 (10%) 5 (9%) Disorders of androgen synthesis/action 3 (4%) 1 (10%) 2 (4%) Other 40 (60%) 2 (20%) 38 (67%) Sex Chromosome DSD 12 (18%) 3 (30%) 9 (16%) 45,X 1 (1%) 0 1 (2%) 47,XXY 1 (1%) 1 (10%) 0 45,X/46,XY 8 (12%) 1 (10%) 7 (12%) 46,XX/46,XY 1 (1%) 1 (10% 0 48,XXYY 1 (1%) 0 1 (2%) No DSD diagnosis 3 (4%) 3 (30%) 0

Over half of boys in the United States undergo circumcision, which has its greatest health benefits and lowest risks when performed during the newborn period under local anesthesia. The COVID-19 pandemic has affected delivery of patient care in many ways and likely also influenced the provision of newborn circumcisions. Prior to the pandemic, we planned to conduct a qualitative study to ascertain physician perspectives on providing newborn circumcision care. The interviews incidentally coincided with the onset of the pandemic and thus, pandemic-related changes emerged as a theme. We elected to analyze this theme in greater detail. Semi-structured interviews were conducted with perinatal physicians in a large urban city from 4/2020 to 7/2020. Physicians that perform or counsel regarding newborn circumcision and physicians with knowledge of or responsibility for hospital policies were eligible. Interviews were transcribed verbatim and qualitative coding was performed. Twenty-three physicians from 11 local hospitals participated. Despite no specific COVID-19 related questions in the interview guide, nearly half of physicians identified that the pandemic affected delivery of newborn circumcision care with 8 pandemic-related sub-themes. The commonest sub-themes included COVID-19 related changes in: (1) workflow processes, (2) staffing and availability of circumcision proceduralists, and (3) procedural settings. In summary, this qualitative study revealed unanticipated COVID-19 pandemic-related changes with primarily adverse effects on the provision of desired newborn circumcisions. Some of these changes may become permanent resulting in broad implications for policy makers that will likely need to adapt and redesign the processes and systems for the delivery of newborn circumcision care.

Background A significant proportion of boys present to surgical specialists later in infancy/early childhood for elective operative circumcision despite the higher procedural risks. This study aims to assess physician perspectives on access to neonatal circumcision across the United States and identify potential reasons contributing to disparities in access. Methods A cross-sectional survey was electronically distributed to physician members of the Societies for Pediatric Urology and the American Academy of Pediatrics Section on Hospital Medicine. Hospital characteristics and circumcision practices were assessed. Associations between NC availability and institutional characteristics were evaluated using chi-squared testing and multivariable logistic regression. Qualitative analyses of free-text comments were performed. Results A total of 367 physicians responded (129 urologists [41%], 188 pediatric hospitalists [59%]). Neonatal circumcision was available at 86% of hospitals represented. On univariate and multivariate analysis, the 50 hospitals that did not offer neonatal circumcision were more likely to be located in the Western region (odds ratio [OR] = 8.33; 95% confidence interval [CI] 3.1–25 vs. Midwest) and in an urban area (OR = 4.2; 95% CI 1.6–10 vs. suburban/rural) compared with hospitals that offered neonatal circumcision. Most common reasons for lack of availability included not a birth hospital (N = 22, 47%), lack of insurance coverage (N = 8, 17%), and low insurance reimbursement (N = 7, 15%). Institutional, regional, or provider availability (68%), insurance/payment (12.4%), and ethics (12.4%) were common themes in the qualitative comments. Conclusions Overall availability of neonatal circumcision varied based on hospital characteristics, including geography. Information from this survey will inform development of interventions designed to offer neonatal circumcision equitably and comprehensively.

INTRODUCTION: Although enhanced recovery pathways (ERP) provide a safe and effective way to improve the recovery of children undergoing bladder reconstruction, ERPs have not been widely adopted in pediatric urology. We describe a quality improvement initiative and outcomes after implementing a 24-element ERP at a single, freestanding children’s hospital. STUDY DESIGN: Multiple stakeholder meetings were planned and executed, initially with pediatric practitioners with ERP experience to understand potential implementation barriers then with anesthesiologists, nurses, case managers, and other ancillary staff to draft our institution-specific ERP. A standardized order set was generated to improve ERP adherence. ERP adherence audits and cyclic performance evaluations held every 6–9 months facilitated continuous pathway refinement. Patient outcomes were compared with a pre-ERP historic cohort. RESULTS: Time from initial ERP planning to first implementation was 7 months. ERP was implemented in twenty consecutive patients undergoing bladder reconstruction (median age 11.3 years, range 4.1–21.1) who were compared to twenty consecutive pre-ERP patients (median age 11.4 years, range 7.7–25.1). Median post-operative length of stay (LOS) significantly decreased from 9 days (range 2–31) pre-ERP to 4 days (range 3–29) post-ERP (p

Key Points Question Is health literacy associated with patient-reported care transition readiness among adolescents and young adults with spina bifida? Findings In this cross-sectional, single-center study, 200 individuals completed patient-reported questionnaires between June 2019 and March 2020 during visits to a multidisciplinary pediatric spina bifida center. Health literacy was associated with care transition readiness even after adjusting for patient demographic and clinical characteristics. Meaning These findings suggest that efforts should be directed to providing relevant health education to adolescents and young adults with spina bifida., This cross-sectional study examines whether health literacy is associated with readiness to transition to adult care among adolescents and young adults with spina bifida., Importance Health literacy has been shown to play an important role in transitions of care in adult populations, with low health literacy associated with adverse health outcomes. The role of health literacy in the transition from pediatric to adult care has been less well studied. Among adolescents and young adults with spina bifida, high rates of unsuccessful transition have been shown, but how patient health literacy affects transition readiness remains unknown. Objective To determine whether health literacy is associated with transition readiness in adolescents and young adults with spina bifida. Design, Setting, and Participants This cross-sectional study involved collection of patient-reported questionnaires between June 2019 and March 2020 at a multidisciplinary spina bifida center at a single, free-standing children’s hospital. Patient demographic and clinical characteristics were obtained from medical record review. Patients were aged 12 years or older with a diagnosis of spina bifida (myelomeningocele and nonmyelomeningocele) whose primary language was English or Spanish. Data analysis was performed from October 2020 to March 2021. Exposures Health literacy as assessed by the Brief Health Literacy Screening Tool. Main Outcomes and Measures The primary outcome was total Transition Readiness Assessment Questionnaire (TRAQ) score, normalized into units of SD. Nested, multivariable linear regression models assessed the association between health literacy and TRAQ scores. Results The TRAQ and Brief Health Literacy Screening Tool were completed by 200 individuals (median [range] age, 17.0 [12.0-31.0] years; 104 female participants [52.0%]). Most of the patients were younger than 18 years (110 participants [55.0%]) and White (136 participants [68.0%]) and had myelomeningocele (125 participants [62.5%]). The mean (SD) TRAQ score was 3.3 (1.1). Sixty-six participants (33.0%) reported inadequate health literacy, 60 participants (30.0%) reported marginal health literacy, and 74 participants (37.0%) reported adequate health literacy. In univariable analysis, health literacy, age, type of spina bifida, level of education, self-administration vs completion of the questionnaires with assistance, ambulatory status, and urinary incontinence were associated with total TRAQ score. In all nested, sequentially adjusted, multivariable models, higher health literacy remained a significant, stepwise, independent variable associated with higher TRAQ score. In the fully adjusted model, having adequate compared with inadequate health literacy was associated with an increase in normalized TRAQ score of 0.49 SD (95% CI, 0.19-0.79). Conclusions and Relevance Patient-reported transition readiness is associated with health literacy, even after adjustment for education level and other demographic and clinical factors. Developing and implementing health literacy–sensitive care programs during the transition process may improve patient transition readiness.

AIMS Current estimated glomerular filtration rate (eGFR) equations may be inaccurate in patients with spina bifida (SB) because of reduced muscle mass and stature. Cross-sectional and longitudinal variability of eGFR were analyzed in these patients across multiple equations, hypothesizing greater variability in creatinine-based than cystatin-C (Cys-C)-based equations. METHODS This retrospective cohort study included children (age, 1-17.9 years) and adults (≥18 years) with SB from 2002-2017 at a large SB clinic. Those without all data needed to calculate eGFR were excluded. Four pediatric and three adult eGFR equations were compared for cross-sectional outcomes of eGFR and elevated office blood pressures using chronic kidney disease (CKD) stage classification, and for longitudinal outcome of eGFR slope over time using covariance pattern models accounting for repeated measures. RESULTS One hundred and eighty two children and 75 adults had greater than or equal to 1 set of data measurements; 118 and 52, respectively, had greater than or equal to 2 sets. The pediatric bedside Schwartz equation had the highest median eGFR and coefficient of variation. CKD stage classification by eGFR showed large differences across equations in children, with rates of eGFR

Summary Introduction Enhanced Recovery Pathways (ERPs), also known as ERAS® pathways, are standardized pathways composed of 21–24 perioperative elements designed to improve post-surgical recovery. ERP has been shown to be safe and effective in children undergoing bladder reconstruction but has not been widely utilized. Objective The aim of this study was to assess utilization of ERPs in pediatric urology and identify barriers to establishing these standardized pathways. Study design Pediatric urologists who were members of the Societies for Pediatric Urology (SPU) were surveyed regarding their familiarity with standardized ERPs, current use of ERP elements, and encountered or perceived barriers to standardized ERP implementation. Willingness to implement ERP elements in a child undergoing bladder reconstruction was assessed with a 5-point Likert scale. Descriptive analysis was performed; Fisher's exact test was performed to assess associations between respondent demographics and ERP familiarity. Results Of 714 distributed surveys, 113 (16%) valid responses were collected. 69% of respondents were male, 58% practiced at academic institutions, and 57% performed 1–5 bladder reconstructions a year. 61% were somewhat familiar or not familiar with standardized ERP. While 54% currently utilize individual ERP elements, only 20% have standardized pathways. Out of 24 possible ERP elements, a median of 15 elements (range 0–24) were implemented by the respondents whether they reported they were implementing ERP elements or had standardized pathways in place. 15 of 24 ERP elements were found to be nearly universally acceptable, with greater than 90% of respondents being somewhat or very willing to implement them in the presented case scenario (Summary Figure). 62% and 56% of those who currently implement ERP elements and experienced barriers noted lack of administrative/leadership support and inability to achieve consensus among pediatric colleagues, respectively, as common barriers in standardization. For those who have not attempted standardization, the most common perceived barrier was pathway unfamiliarity (48%). Discussion Over half of respondents were not familiar with enhanced recovery pathways but were willing to implement a majority of the pathway elements, suggesting potential for ERP standardization in pediatric urology. Buy-in from colleagues and leadership would be necessary to overcome perceived barriers of standardized pathway development. Conclusion Administrative support and more widespread knowledge of ERP amongst pediatric urologists are necessary to facilitate further implementation in children undergoing bladder reconstruction. Download : Download high-res image (691KB) Download : Download full-size image Summary Figure .

OBJECTIVES: Seventeen states do not provide Medicaid coverage for neonatal male circumcision, despite American Academy of Pediatrics recommendations supporting access for families that choose it. Our study objectives were to (1) compare state-specific trends in neonatal circumcision to previously established estimates and (2) assess the impact of changes in Medicaid coverage of the procedure. METHODS: The State Inpatient Databases were used to determine rates of neonatal male circumcision in 4 states (CO, FL, MI, and NY) at 4 time points (2001, 2006, 2011, 2016). Neonatal circumcision was defunded by Medicaid in Florida (2003) and Colorado (2011). A multivariable logistic regression model was created to assess associations between patient and state characteristics and odds of neonatal circumcision. RESULTS: Overall, 54.5% of neonates underwent circumcision. States where Medicaid defunded neonatal circumcision revealed a decrease in circumcision rates in subsequent years (47.4% to 37.5% in FL; 61.9% to 52.0% in CO). Neonates with private insurance had higher odds of circumcision compared with those with public insurance (adjusted odds ratio [aOR] 2.23; 95% confidence interval [CI] 2.21–2.25). When Medicaid coverage was available, Black neonates had higher odds of circumcision compared with white neonates (aOR 1.44; 95% CI 1.42–1.46). When Medicaid coverage was not available, Black neonates had lower odds compared with white neonates (aOR 0.40; 95% CI 0.39–0.41). CONCLUSIONS: State-specific data reveal trends in neonatal circumcision similar to previous national estimates. Colorado and Florida revealed 20.9% and 16.0% reductions in neonatal circumcision rates, respectively, after defunding. Black neonates appeared to be disproportionately affected by changes in Medicaid coverage.

This survey study assesses possible barriers to neonatal male circumcision by evaluating circumcision-related requirements and procedures among 44 birthing hospitals in the Chicago area in Illinois.

Purpose Little is known about the phenotypes, diagnoses, and sex of rearing of infants with atypical genital development in the United States. As part of a multicenter study of these infants, we have provided a baseline report from US difference/disorder of sex development clinics describing the diagnoses, anatomic features, and sex of rearing. We also determined whether consensus guidelines are followed for sex designation in the United States. Methods Eligible participants had moderate-to-severe genital atypia, were aged

Purpose Little is known about the reproductive desires of transgender and gender-nonconforming (TGNC) adolescents who may seek gender-affirming medical care that leads to infertility. The current study addressed this gap by examining attitudes toward fertility and family formation in a diverse sample of TGNC youth. Method An online survey about sexual/reproductive health in sexual and gender minority (SGM) adolescents ages 14–17 years was conducted from September to October 2016. Results A total of 156 TGNC adolescents (M age = 16.1 years; 83.3% assigned female at birth; 58.3% youth of color) responded. Overall, 70.5% of TGNC adolescents were interested in adoption and 35.9% in biological parenthood; more gender-nonconforming youth (43.8%) than transgender youth (25.8%) expressed interest in biological fertility. Discussions with health-care providers about fertility and reproductive health were uncommon—only 20.5% of youth had discussed fertility in general and only 13.5% had discussed effects of hormones on fertility. However, 60.9% of respondents were interested in learning more about their fertility and family building options. Key themes emerging from qualitative comments included concerns related to fertility/reproductive health (e.g., stigma of SGM parenthood, effect of gender-affirming treatments on fertility), and the need for additional reproductive health information both tailored to their individual experience and for SGM individuals more generally. Discussion TGNC adolescents expressed interest in multiple family building options, including adoption and biological parenthood, and identified a need for more information about these options. Thus, clinicians working with adolescents should be aware of the unique fertility and reproductive health needs of TGNC youth.

We sought to determine the presence of germ cells in the gonads of patients with disorders of sex development to establish whether preservation of germ cells for future fertility potential is possible. We hypothesized that germ cells are present but vary by age and diagnosis.We reviewed histology from patients with disorders of sex development who underwent gonadectomy/biopsy from 2002 to 2014 at a single institution for pathological classification of the gonad, composition of gonadal stroma and germ cell presence.A total of 44 patients were identified and germ cells were present in 68%. The presence and average number of germ cells per mmGerm cells were present in the majority of our cohort and the presence decreased with age. This novel, fertility driven evaluation of germ cell quantity in a variety of disorders of sex development suggests that fertility potential may be greater than previously thought. Further studies must be done to evaluate a larger population and examine germ cell quality to determine the viability of these germ cells.

This retrospective chart review aims to address gaps in the literature regarding the efficacy and interaction of gonadotropin-releasing hormone agonists (GnRHa) and gender-affirming hormone therapies in medical transition regimens in transgender adolescents. We abstracted and reviewed data from 83 patients at our pediatric gender clinic, and found that patients who initiated treatment with GnRHa before gender-affirming hormones (estrogen, testosterone) required lower doses of those hormones than those who did not use GnRHa. The results of this preliminary research provide a foundation for future long-term prospective studies aimed to better understand these relationships.

INTRODUCTION AND OBJECTIVES:Among some populations, access to neonatal circumcision (NC) has become increasingly limited, despite evidence that benefits justify NC access and reimbursement. This st...

INTRODUCTION AND OBJECTIVES:Emerging adults with spina bifida (SB) may face inaccurate kidney function assessment as they bridge the age gap between pediatric and adult equations for estimated glom...

Twelve providers (eight institutions) participating in a Gender and Sex Diversity Fertility Working Group completed a survey assessing their hospital's transgender, disorders/differences of sex development (DSD), and fertility healthcare teams. Participants also prioritized the development of a: (1) Provider Assessment Tool (questionnaire assessing knowledge/feelings about future fertility), or (2) decision aid (DA). Healthcare team presence by institution: transgender (6/8; 75%), DSD (75%), and fertility preservation (75%). Two-thirds of providers reported that the DA was most needed. Respondents recommended the DA to: help manage complex information, have a take-home reference, and provide neutral information. Other identified resource needs included: fertility specialists in clinic and financial resources.

Children and adolescents with gender and sex diversity include (1) gender-nonconforming and transgender individuals for whom gender identity or expression are incongruent with birth-assigned sex (heretofore, transgender) and (2) individuals who have differences in sex development (DSD). Although these are largely disparate groups, there is overlap in the medical expertise necessary to care for individuals with both gender and sex diversity. In addition, both groups face potential infertility or sterility as a result of desired medical and surgical therapies. The Ann & Robert H. Lurie Children's Hospital of Chicago (Lurie Children's) gender and sex development program (GSDP) provides specialized multidisciplinary care for both transgender and DSD patients. In response to patient concerns that recommended medical treatments have the potential to affect fertility, the Lurie Children's GSDP team partnered with experts from the Oncofertility Consortium at Northwestern University to expand fertility preservation options to gender and sex diverse youth. This article summarizes the results of a meeting of experts across this field at the annual Oncofertility Consortium conference with thoughts on next steps toward a unified protocol for this patient group.

Background Current organizational guidelines regarding use of antibiotics during urinary tract catheterization are based on limited evidence and are not directly applicable to the pediatric urology population. We seek to improve understanding of this population by first evaluating current practices. This study aims to investigate practice patterns and attitudes of pediatric urologists regarding the use of antibiotics in the setting of urinary tract catheterization. Methods An online survey was sent to members of the Society for Pediatric Urology. Questionnaire sections included demographics, general questions about antibiotic use with catheterization, and specific clinical scenarios. Descriptive statistics were used, and chi-square analysis was performed to examine associations between demographics and specific responses. Results Of 448 pediatric urologists surveyed, 154 (34%) responded to the survey. A majority of surveyed urologists (78%) prescribe daily prophylactic antibiotics with a hypospadias stent in place, but extensive variation in use of antibiotics was reported with other catheters and tubes. Extensive variation in practice patterns was also reported for three case scenarios regarding antibiotic prophylaxis with catheterization. Urologists > 50 years of age and fellowship-trained urologists were more likely to prescribe antibiotics for hypospadias stents (p = 0.02, p = 0.03), but no other significant associations between demographic characteristics and antibiotic use were found. Conclusions There is substantial variation in practice patterns among surveyed pediatric urologists regarding prophylactic antibiotic use with urinary catheterization. This variation, combined with a lack of objective data and increasing pressure to decrease infectious complications and combat antibiotic resistance, highlights the need for development of management guidelines for this unique population. Electronic supplementary material The online version of this article (10.1186/s12894-017-0268-5) contains supplementary material, which is available to authorized users.

One potential strategy for improving voiding diary completion rates and data quality is use of a mobile electronic format. We evaluated the acceptability and feasibility of mobile voiding diaries for patients with nonneurogenic lower urinary tract dysfunction, and compared mobile and paper voiding diaries.We prospectively enrolled children presenting with daytime symptoms of lower urinary tract dysfunction between July 2012 and April 2013. We enrolled an initial cohort of patients who were provided a paper voiding diary and a subsequent cohort who were provided a mobile voiding diary. We conducted in person interviews and assessed completion rates and quality, comparing paper and mobile voiding diary groups.We enrolled 45 patients who received a paper voiding diary and 38 who received a mobile voiding diary. Completion rates were 78% for paper voiding diaries and 61% for mobile voiding diaries (p = 0.10). Data quality measures for patients completing paper vs mobile voiding diaries revealed a larger proportion (63% vs 52%) providing a full 5 days of data and a smaller proportion (20% vs 65%) with data gaps. However, the paper voiding diary also demonstrated a lower proportion (80% vs 100%) that was completely legible and a lower proportion (40% vs 65%) with completely prospective data entry.The use of a mobile voiding diary was acceptable and feasible for our patients with lower urinary tract dysfunction, although completion rates were somewhat lower compared to paper voiding diaries. Data quality was not clearly better for either version. The mobile voiding diary format may offer data quality advantages for select groups but it did not display significant superiority when provided universally.

Summary Background Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management. Objective This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function. Study design A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Children's Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion. Results Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis ( Summary Table ). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering. Discussion Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis. Conclusions This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD. Summary Table . Group 1 Group 2 Group 3 Group 4 Adjusted P-value Age at diagnosis 2–18 months >18 months unknown n 19 11 7 6 Most recent method of bladder emptying Voiding 7 (36.8%) 3 (27.3%) 2 (28.6%) 1.00 CIC 13 (68.4%)* 8 (72.7%) 5 (71.4%) 1.00 No hydronephrosis on recent ultrasound 13 (68.4%) 5 (45.5%) 3 (42.8%) 0.90 Recent serum creatinine (ng/dl) Median 0.4 0.4 0.55 0.91 IQR 0.3–0.5 0.4–0.7 0.4–0.8 0.91 *One patient utilized both CIC and voiding to empty.

We describe our experience with 2-stage proximal hypospadias repair. We report outcomes, and patient and procedure characteristics associated with surgical complications.We retrospectively studied patients with proximal hypospadias who underwent staged repair between January 1993 and December 2012. Demographics, preoperative management and operative technique were reviewed. Complications included glans dehiscence, fistula, meatal stenosis, nonmeatal stricture, urethrocele/diverticula and residual chordee. Cox proportional hazards model was used to evaluate the associations between time to surgery for complications and patient and procedure level factors.A total of 134 patients were included. Median patient age was 8.8 months at first stage surgery and 17.1 months at second stage surgery, and median time between surgeries was 8 months. Median followup was 3.8 years. Complications were seen in 71 patients (53%), with the most common being fistula (39 patients, 29.1%). Reoperation was performed in 66 patients (49%). Median time from urethroplasty to surgery for complication was 14.9 months. Use of preoperative testosterone decreased risk of undergoing surgery for complication by 27% (HR 0.73, 95% CI 0.55-0.98, p = 0.04). In addition, patients identified as Hispanic were at increased risk for undergoing surgery for complications (HR 2.40, 95% CI 1.28-4.53, p = 0.01).We review the largest cohort of patients undergoing 2-stage hypospadias repair at a single institution. Complications and reoperation rates were approximately 50% in the setting of complex genital reconstruction.

Summary Background Management of vesicoureteral reflux (VUR) continues to be controversial. In conditions of uncertainty, decision analytic techniques such as cost-utility analysis (CUA) can help to structure the decision-making process. However, CUA analyses require a “utility,” a value between 0 (death) and 1 (perfect health) corresponding to the quality of life associated with a health state. Ideally, utility values are elicited directly from representative community samples, but utilities have not been rigorously measured for pediatric urology conditions. Objectives To elicit utility scores for VUR and open anti-reflux surgery (ARS) from a representative, well-characterized community sample of adults who have been parents. Methods Cross-sectional survey of nationally representative adults who had ever been parents. Each respondent saw one of four descriptions of VUR, with or without continuous antibiotic prophylaxis (CAP) and occurrence of febrile urinary tract infection (UTI). A 6-week postoperative health state following ARS was also assessed. We used the time trade-off (TTO) method to elicit utility scores. Factors associated with utility score were assessed with a multivariate linear regression model. Results The survey was completed by 1200 individuals. Data were weighted to adjust for demographic differences between responders and non-responders. Mean age was 52 ± 15 years, 44% were male, and 68% were White. In terms of education, 29% had a college degree or higher. The mean utility score for VUR overall was 0.82 ± 0.28. VUR utility scores did not differ significantly based on inclusion of CAP or UTI in the health state description (p = 0.21). The 6-week postoperative period garnered a utility of 0.71 ± 0.43. Discussion Our results showed that VUR has a mean utility score of 0.82, which indicates that the community perceives this condition to be a substantial burden. For comparison, conditions with similar utility scores include compensated hepatitis B-related cirrhosis (0.80) and glaucoma (0.82); conditions with higher utilities include neonatal jaundice (0.99) and transient neonatal neurological symptoms (0.95); and conditions with lower utility scores include severe depression (0.43) and major stroke (0.30). Our results suggest that parents consider the burden associated with VUR to be significant, and that the impact of the condition on families and children is substantial. Conclusions VUR is perceived as having a substantial impact on health-related quality of life, with a utility value of 0.82. However, use of CAP and occurrence of UTI do not seem to affect significantly the community perspective on HRQOL associated with living with VUR. Table . Utility scores for VUR, stratified by inclusion of continuous antimicrobial prophylaxis and/or UTI in the VUR health state description (weighted linear regression) Weighted N (%) VUR utility (±SD) Univariate p Overall sample 1200 0.82 ± 0.28 – VUR health state group 0.209 +antibiotic prophylaxis, +UTI 303 (25) 0.84 ± 0.26 +antibiotic prophylaxis, -UTI 292 (24) 0.79 ± 0.30 -antibiotic prophylaxis, +UTI 313 (26) 0.82 ± 0.28 -antibiotic prophylaxis, -UTI 293 (24) 0.81 ± 0.27

Few standards exist for reporting results of voiding cystourethrogram (VCUG). To assess the variation in reporting of VCUG findings from different facilities using a standardized assessment tool. VCUG reports were evaluated for demographic, technical, anatomical and functional information. Reports were categorized by age, gender, indication and vesicouretal reflux (VUR) status. Institutions were classified as a free-standing pediatric hospital (n = 3), pediatric hospital within a hospital (n = 11), or non-pediatric facility (n = 24) and reports were classified as having been read by a pediatric radiologist or not. Each category of outside reports (n = 152) was randomly matched with a twice-larger group of Hospital A reports from the same category (n = 304). Multivariate linear regression was used to analyze the association between the primary outcome (percentage of items described in dictated VCUG report) and the type of radiologist and institution. Of the 456 studies, 66% were in girls, 56% were in those

Although unilateral ureteral reimplantation for vesicoureteral reflux is highly successful, new contralateral reflux will develop postoperatively in some patients. We examined predictors and clinical outcomes of postoperative contralateral vesicoureteral reflux.We reviewed patients who underwent nontapered unilateral reimplantation for primary vesicoureteral reflux graded on a 3-point scale at our institution from January 1990 to December 2002, and identified those with subsequent contralateral vesicoureteral reflux. We analyzed the association of patient/procedure characteristics with incidence, and time to resolution of contralateral reflux and postoperative urinary tract infection. Multivariable models controlled for variables associated with incidence and time to resolution of contralateral reflux.A total of 395 patients (77.2% female, median age 5.3 years) underwent ureteral reimplantation for vesicoureteral reflux. Preoperative reflux was grade 1 in 2.8% of patients, grade 2 in 56.6% and grade 3 in 40.6%. Technical success was 95.4%. After reimplantation 39 patients (9.9%) had new contralateral reflux (grade 1 in 7, grade 2 in 27 and grade 3 in 5). Median followup was 51.8 months. On multivariate analysis younger age (less than 6 years, OR 3.7, p = 0.006) and low observed bladder capacity as percent of predicted bladder capacity (less than 50% of predicted capacity, OR 6.3, p = 0.02) were significant predictors of contralateral reflux. Contralateral reflux resolved in 21 of 27 patients (77.8%) on subsequent cystography at a median of 21.5 months. Two patients underwent reimplantation for persistent contralateral reflux. Four of 39 patients (10.3%) with contralateral reflux had postoperative febrile urinary tract infections at a median of 26 months, with spontaneous resolution of contralateral reflux in all.Younger patients and those with low observed vs predicted bladder capacity may be at increased risk for postoperative contralateral vesicoureteral reflux. A majority of contralateral reflux will resolve spontaneously, and the clinical course is typically benign.

Success rates of ureteral reimplantation for primary vesicoureteral reflux are high. Few studies document the natural history of children with persistent vesicoureteral reflux. We reviewed their clinical outcomes and long-term resolution.We performed a retrospective review of all children with persistent vesicoureteral reflux (grade 1 or greater) into the reimplanted ureter(s) on initial cystogram after reimplantation for primary vesicoureteral reflux at our institution from January 1990 to December 2002. We evaluated subsequent cystograms (graded on the 3-point radionuclide cystogram scale), surgery and urinary tract infection. We performed survival analyses of time to resolution of persistent (grade 1 or greater) and clinically significant (grade 2 or greater) vesicoureteral reflux in patients with more than 1 postoperative cystogram.Of 965 patients 59 (94 ureters) had persistent vesicoureteral reflux (6.1%), including 19 grade 1/3, 29 grade 2/3 and 11 grade 3/3. Median patient age at reimplantation was 1.9 years (range 0.8 to 5.1) and 62.7% were female. Preoperative vesicoureteral reflux grade was 2/3 in 42.4% and 3/3 in 57.6%, and 30.5% of patients had ureteral tapering. Median followup was 47.1 months (IQR 19.3-650.3). Reflux was resolved in 26 of 36 (72.2%) patients and median time to resolution was 20.4 months. Grade 2 or greater reflux on postoperative cystogram resolved in 21 of 32 (65.6%) patients and median time to resolution was 20.4 months. There were 10 patients with persistent vesicoureteral reflux at last cystogram, grade 1 or 2 in 9 and 3/3 in 1 patient. One patient underwent repeat reimplantation for persistent vesicoureteral reflux and 7 (13%) had postoperative febrile urinary tract infection at a median of 37 months postoperatively (IQR 1.7-64.4).Persistent vesicoureteral reflux after reimplantation resolves spontaneously in most children and can be managed nonoperatively with good long-term outcomes.

The impact of diagnostic genitourinary imaging on patients and families is poorly understood. We measured patient and family reaction to commonly performed genitourinary imaging studies using a standardized measurement tool.We surveyed families undergoing genitourinary imaging (renal ultrasound, voiding cystourethrography, radionuclide cystogram, static renal scintigraphy and diuretic renal scintigraphy) using a Likert scaled 11-item survey to assess impact across 4 domains (pain, anxiety, time, satisfaction). Survey scores were analyzed using ANOVA and linear regression.A total of 263 families were surveyed (61 renal ultrasound, 52 voiding cystourethrogram, 55 radionuclide cystogram, 47 mercaptoacetyltriglycine dynamic renal scintigraphy, 48 dimercaptosuccinic acid static renal scintigraphy). Mean patient age was 2.1 years old. Of the patients 45% were male and 77% were white. Patient age, gender and prior genitourinary imaging experience varied by study type. Study type was significantly associated with total and weighted scores on the genitourinary imaging survey (both p0.0001). Renal ultrasound was scored as better and mercaptoacetyltriglycine dynamic renal scintigraphy was worse than voiding cystourethrogram, radionuclide cystogram and dimercaptosuccinic acid static renal scintigraphy, which did not differ from each other. Other factors associated with worse total scores included patient age 1 to 3 years (p0.001) and nonwhite race (p = 0.04). Gender, prior testing history, wait time and parent education were not associated with total scores. In the multivariate model renal ultrasound remained the best and mercaptoacetyltriglycine dynamic renal scintigraphy the worst (p0.0001). In a direct comparison dimercaptosuccinic acid static renal scintigraphy and voiding cystourethrogram total scores did not differ (p = 0.59).There were significant differences among genitourinary imaging studies regarding the patient/family experience, but there was no overall difference between dimercaptosuccinic acid static renal scintigraphy and voiding cystourethrogram. These findings may be useful to aid decision making when considering genitourinary imaging for children.

Objective To evaluate the long-term durability of successful ureteral reimplantation (UR) for vesicoureteral reflux (VUR) through a review of late cystography (LC) findings. Materials and Methods We performed a retrospective chart review of all children with primary VUR who underwent successful open UR (grade 0 VUR into the reimplanted ureter[s] on initial cystogram) at our institution from January 1990 to December 2002. We identified successful UR patients who underwent LC ≥1 year after UR and reviewed the results for the presence of recurrent VUR into the reimplanted ureter(s). Results Seven-hundred ninety-four patients underwent successful open UR for primary VUR, of whom 60 (7.6%) had a subsequent LC. Preoperative VUR grade was ≤II in 20 (34.5%) and ≥III in 38 (65.5%). Median age at UR was 3.5 years (IQR 1.3–6.2 years); 51 (85%) were female. UR was intravesical in 45 (75%) and bilateral in 19 (32%). LC was performed at a median of 38.7 months after UR (IQR 19.6–66.1 months). Indication for LC was febrile urinary tract infection (UTI) in 16 (27%), nonfebrile UTIs in 15 (25%), follow-up of contralateral VUR in 16 (27%), and other clinical indications in 13 (21%). The recurrence rate was 0%; of the 79 reimplanted ureters, 100% (95% CI 95.4–100) had no VUR (grade 0). Conclusion Among children who underwent successful open UR for primary VUR, there was no VUR recurrence on extended follow-up. This suggests that the late durability of open antireflux surgery is excellent.

We have previously shown that nonwhite patients with ureteropelvic junction obstruction undergo pyeloplasty at a younger age than white patients. The mechanisms behind this finding are unclear, since there is no known racial variation in the natural history of ureteropelvic junction obstruction. We used a detailed clinical database to explain this phenomenon.We retrospectively reviewed all patients undergoing primary pyeloplasty at our institution between 1992 and 2008. More than 360 data points were abstracted for each patient, including self-reported race, socioeconomic status, symptom duration and presentation.Of 847 patients undergoing pyeloplasty during the study period 741 met inclusion criteria. Nonwhite patients underwent surgery at a younger age (0.6 years) than whites (2.6 years, p0.0001). When stratified by timing of clinical presentation (prenatal vs postnatal), there was no significant difference in race among patients presenting prenatally (0.37 vs 0.36 years, p = 0.22). Nonwhite patients presenting postnatally were significantly younger than white patients (6.3 vs 8.2 years, p = 0.03). This finding appeared to be due to differences in age at initial clinical presentation (5.4 vs 7.0 years, p = 0.03) and in time from initial clinical presentation to urological evaluation (0.6 vs 3.2 months, p = 0.03). These differences persisted after correcting for other factors, including markers of socioeconomic status.Consistent with previous studies, we found that nonwhite patients underwent primary pyeloplasty at a younger age than whites. This difference is limited to patients presenting after birth. Prenatally diagnosed patients underwent surgery at similar ages regardless of race.

The basis for vesicoureteral reflux (VUR) is considered to be primarily genetic, with a 30–50% incidence of VUR in first-degree relatives of patients. The search for the causative gene or genes has been elusive, likely because of VUR being genetically heterogeneous with complex inheritance patterns. In this study, a genome-wide analysis of VUR with high-density single nucleotide polymorphisms was conducted with the aim of identifying susceptibility loci for VUR in 98 families with two or more affected children. Using the affected sib-pair method of analysis in 150 sib-pairs, we identified a genome-wide statistically significant linkage peak with an LOD score greater than 4 on chromosome 5 and two linkage peaks with LOD scores greater than 3.6 on chromosomes 13 and 18 were identified in these 98 families. These results suggested that multiple genes are likely to contribute to the formation of VUR phenotype. Further mapping of these linkage peaks may help identify the causative genes.