Quality of Life and Bladder Symptoms in Adolescents and Young Adults With Spina Bifida Who Catheterize via Urethra Versus Catheterizable Channel.

PURPOSE: To assess associations between health-related quality of life (HRQOL), bladder-related QOL, bladder symptoms, and bladder catheterization route among adolescents and young adults (AYA) with spina bifida (SB). MATERIALS AND METHODS: Clinical questionnaires administered to individuals ≥ 12-years-old requiring catheterization between June 2019-March 2020 in a SB center were retrospectively analyzed. Questionnaires were completed in English or Spanish independently or with caregiver assistance. Medical records were reviewed for demographic and clinical characteristics. Primary exposure was catheterization route (urethra or channel). Primary outcome was HRQOL, measured by Patient Reported Outcome Measure Information System Pediatric Global Health 7 (PGH-7). Secondary outcomes were bladder-related QOL and bladder symptoms, measured by Neurogenic Bladder Symptom Score (NBSS). Nested, multivariable linear regression models assessed associations between catheterization route and questionnaire scores. RESULTS: Of 162 patients requiring catheterization, 146 completed both the PGH-7 and NBSS and were included. Seventy-three percent catheterized via urethra and 27% via channel. Median age was 17.5 years (range 12-31), 58% of patients were female, and 80% had myelomeningocele. Urinary incontinence was more common among those who catheterized via urethra (60%) compared to channel (33%). On adjusted analyses, catheterization route was not significantly associated with PGH-7 or NBSS bladder-related QOL scores. More bladder symptoms were associated with worse bladder-related QOL. Patients who catheterized via channel had fewer bladder symptoms than those who catheterized via urethra. CONCLUSIONS: Catheterization route was not significantly associated with QOL. Though catheterization via channel was associated with fewer bladder symptoms, only degree of current bladder symptoms was significantly associated with bladder-related QOL.

Assessing Readiness for Transition From Pediatric to Adult Gender Affirming Care.

PURPOSE: Transitioning from pediatric to adult care is a critical juncture in the health of adolescents. Little is known about how best to optimize transition to adult care among transgender and nonbinary (TGNB) youth. While the Transition Readiness and Assessment Questionnaire (TRAQ) has been validated in other pediatric populations, it has not been studied in TGNB youth. Our aims were to pilot the use of the TRAQ for TGNB patients, describe transition readiness patterns, and identify factors associated with transition readiness. METHODS: The TRAQ was introduced into routine clinical care for patients and their caregivers in a large, urban pediatric gender program in the spring of 2021. We performed a retrospective chart review comparing TRAQ responses based on demographic and clinical data. RESULTS: We collected TRAQs from 153 adolescents (mean age: 19 years [standard deviation 2.36], range: 11-24). The TRAQ demonstrated good internal reliability with a Cronbach alpha of 0.926. Patients scored highest in the TRAQ subdomains of talking with providers and tracking health issues and lowest in the subdomains of managing medications and appointment keeping. Age and presenting to the appointment alone were associated with higher TRAQ scores. DISCUSSION: We found that the TRAQ is internally reliable in a sample of TGNB youth. Factors associated with higher TRAQ scores and patterns identified in TRAQ score subdomains provide an insight into the needs of TGNB youth preparing to transition to adult gender-affirming care. Future research should focus on tracking transition readiness longitudinally, developing and evaluating interventions to improve transition readiness, and assessing post-transition outcomes.

Male perspectives on clinical communication about sexual health in spina bifida.

AIM: To assess perspectives on clinical communication about sexual health in young adult males with spina bifida. METHOD: Semi-structured interviews were conducted between February and May 2021 with males at least 18 years of age with spina bifida to assess their perspectives and experiences of sexual health communication with clinicians. Demographic and clinical characteristics were obtained from chart review and a survey of patients. Interviews were transcribed verbatim, and conventional content analysis was used for transcript coding. RESULTS: Twenty individuals participated with a median age of 22 years 6 months (range 18-29 years). Sixteen had myelomeningocele. Most identified as heterosexual (n = 17) and not sexually active (n = 13). Barriers and facilitators of successful interactions were identified. Barriers for participants included general discomfort with talking about sex and variability in individual preferences for how conversations occur. Facilitators included participants' comfort with their urologist and discussing sex in relation to disability. Suggestions for improving discussions included (1) notifying individuals that discussion about sex will occur before clinic visits; (2) creating space for discussions; (3) respecting individuals' readiness to discuss; and (4) making discussions disability specific. INTERPRETATION: Young adult males with spina bifida are interested in discussing sexual health with their clinicians. Great variability exists about conversation preferences, emphasizing the need to individualize clinical communication about sex. Current health guidelines for males may not be in line with individuals' wishes. WHAT THIS PAPER ADDS: Great variability exists in individual preference around sexual health communication. Patient-level barriers hinder successful conversations about sex. Individuals have great insight into how conversations about sex can be improved.

Physician Perspectives on Performing Newborn Circumcisions: Barriers and Opportunities.

OBJECTIVE: Over half of infant boys born in the United States undergo newborn circumcision. However, available data indicate that boys who are publicly insured, or Black/African American, have less access to desired newborn circumcision, thus concentrating riskier, more costly operative circumcision among these populations. This study ascertains perinatal physician perspectives about barriers and facilitators to providing newborn circumcisions, with a goal of informing future strategies to ensure more equitable access. METHODS: Qualitative interviews about newborn circumcision care were conducted from April-June 2020 at eleven Chicago-Area hospitals. Physicians that provide perinatal care (pediatricians, family medicine physicians, and obstetricians) participated in qualitative interviews about newborn circumcision. Inductive and deductive qualitative coding was performed to identify themes related to barriers and facilitators of newborn circumcision care. RESULTS: The 23 participating physicians (78% female, 74% white, median 16 years since medical school graduation [range 5-38 years], 52% hospital leadership role, 78% currently perform circumcisions) reported multiple barriers including difficulty with procedural logistics and inconsistent clinician availability and training; corresponding suggestions for operational improvements were also provided. Regarding newborn circumcision insurance coverage and reimbursement, physicians reported limited knowledge, but noted that some insurance reimbursement policies financially disincentivize clinicians and hospitals from offering inpatient newborn circumcision. CONCLUSIONS: Physicians identified logistical/operational, and reimbursement-related barriers to providing newborn circumcision for desirous families. Future studies and advocacy work should focus on developing clinical strategies and healthcare policies to ensure equitable access, and incentivize clinicians/hospitals to perform newborn circumcisions.

Association Between Quality of Life and Neurogenic Bowel Symptoms by Bowel Management Program in Spina Bifida.

OBJECTIVE: To compare differences in bowel-specific quality of life (QOL), overall qQOL, and neurogenic bowel dysfunction (NBD) severity by bowel management program in patients with spina bifida (SB). METHODS: We performed a retrospective cross-sectional study of patients ≥12 years old at our multidisciplinary SB center who completed both a modified Peristeen NBD questionnaire (assessing bowel symptom severity and bowel-specific QOL) and the Patient-Reported Outcomes Measurement Information System Pediatric Global Health questionnaire (assessing overall QOL). Nested, multivariable models were fit for associations between outcomes and bowel management program (enemas, conservative management, and none). RESULTS: A total of 173 patients, 56.1% female and 64.6% with myelomeningocele, were included in our analysis. Median age was 18.2 years old. Patients reported using enemas (n = 42), conservative management (n = 63), and no bowel program (n = 68). When adjusting for covariates, there was no significant association between bowel-specific QOL nor overall QOL across bowel management programs. However, the use of conservative management compared to enemas was associated with worse bowel symptoms severity (adjusted beta=2.58, 95%CI=[0.09,5.06]). Additionally, greater bowel symptom severity was significantly associated with lower overall QOL (adjusted beta=-0.33, 95%CI=[-0.57,-0.10]). CONCLUSION: NBD symptom severity in SB is more strongly associated with QOL than the individual bowel program being utilized. Our findings suggest that different degrees of NBD require different invasiveness of bowel programs, but it is the outcome of the bowel management program and not the specific program itself that is most associated with QOL.

Retrograde incision from orifice (RIO) technique for endoscopic incision of ureterocele: 15 years of outcomes.

INTRODUCTION: Management of obstructing ureterocele often includes endoscopic transurethral incision (TUI) that can be challenging secondary to uncertainty in anatomic landmarks with risk of serious complications. To this end, we innovated a technique using predictable landmarks that begins endoscopic incision at the ureterocele orifice and extends retrograde proximal to the bladder neck (Figure). OBJECTIVE: With over 15 years of experience in performing this retrograde incision from orifice (RIO) technique, we aimed to examine post-operative outcomes and risk of surgical failure after RIO compared to traditional TUI techniques for ureteroceles. We hypothesized that clinical outcomes after RIO would be superior to traditional endoscopic approaches to decompression of obstructing ureterocele in infants. STUDY DESIGN: A retrospective study of patients ≤12 months old who underwent TUI ureterocele at our institution between 2007 and -2021 was conducted. Pre-, intra- and post-operative characteristics were compared between patients who underwent RIO vs non-RIO TUI. Primary outcome was post-incision febrile urinary tract infection (fUTI). Secondary outcome was a composite failure measure of fUTI, secondary surgery, de novo bladder outlet obstruction, or vesicoureteral reflux. Multivariable Cox proportional hazard models were fitted to compare the time-to-event risk of primary and secondary outcomes between groups. RESULTS: Ninety patients with 92 ureteroceles were included (49 RIO, 43 non-RIO). Median follow-up from TUI was 33 months. RIO had a shorter median operative duration (27 vs 35 min, p = 0.021). Primary and secondary outcomes were similar between groups (fUTI: 29% RIO vs 19% non-RIO, p = 0.27; composite failure 54% RIO vs 69% non-RIO, p = 0.15). In multivariable Cox proportional hazard models, there was no significant difference in risk of fUTI (RIO aHR 0.98, 95% CI 0.38-2.54, p = 0.97) or composite failure (RIO aHR 0.80, 95% CI 0.45-1.44, p = 0.46) between TUI techniques. DISCUSSION: RIO technique for TUI ureterocele is attractive in that it uses predictable anatomic landmarks making it simple to perform. In analyzing this 15-year institutional experience of TUI ureterocele, RIO showed similar success to non-RIO endoscopic incisions. This study is a retrospective, non-randomized, single-institutional study over 15 years and is therefore subject to change in surgeon practice over time and variable practices between providers. CONCLUSIONS: Given comparable success and durability over time to other TUI ureterocele techniques, and with the advantage of operator ease using consistent anatomic landmarks, RIO is a worthy option for endoscopic ureterocele decompression.

Differential Insurance Plan Coverage and Surgeon Reimbursement of Pediatric Circumcision at an Urban, Midwestern Hospital.

OBJECTIVE: To explore private vs public pediatric circumcision insurance coverage and surgeon reimbursement. METHODS: A telephone survey about circumcision coverage (Current Procedural Terminology codes: 54150, 54161) was conducted in October 2021 with insurance plan representatives from the 12 plans that comprised ≥1% of institutional pediatric urology visits to compare plan characteristics and coverage details. Circumcision billing data were collected at one pediatric hospital to assess surgeon reimbursement (insurance+patient payment) by plan type using bivariate statistics. RESULTS: Ten plans (5 private and 5 public) responded (83.3% response rate). All except one public plan covered newborn circumcision. For non-newborn circumcisions, most public plans (80%) had unrestricted coverage, whereas all private plans required medical necessity. Median reimbursement for newborn circumcision (CPT: 54150) was $484 for private and $78 for public plans, P < .001 while median reimbursement for non-newborn circumcision (CPT: 54161) was $314 for private and $147 for public plans, P < .001. CONCLUSION: Private insurance plans reimburse significantly more than public plans for newborn circumcision. For non-newborn circumcision, private plans reimburse more than public but the coverage is more restricted, with a smaller differential between newborn and non-newborn circumcision. This coverage and reimbursement structure may indirectly encourage newborn circumcision for privately insured boys and non-newborn circumcision for publicly insured boys.

"I Just Haven't Done Any of That": Applicability of the International Index of Erectile Function in Young Men With Spina Bifida.

PURPOSE: We evaluate the applicability of the International Index of Erectile Function in young men with spina bifida and identify spina bifida-specific sexual experiences not captured by this measure. MATERIALS AND METHODS: Semistructured interviews were conducted between February 2021 and May 2021 with men ≥18 years of age with spina bifida. The International Index of Erectile Function was completed by participants, and perspectives on its applicability were discussed. Participant experiences and perspectives around sexual health were discussed to identify aspects of the sexual experience not well captured by the International Index of Erectile Function. Demographic and clinical characteristics were obtained from a patient survey and chart review. Conventional content analysis framework was used for transcript coding. RESULTS: Of 30 eligible patients approached, 20 participated. Median age was 22.5 years (range 18-29), and 80% had myelomeningocele. Most identified as heterosexual (17/20, 85%), were not in a relationship (14/20, 70%), and were not currently sexually active (13/20, 65%). Some perceived the International Index of Erectile Function as applicable, while others reported it was not, as they do not define themselves as sexually active. Aspects of the sexual experience not captured by the International Index of Erectile Function included (1) lack of control over sexual function, (2) poor lower body sensation, (3) urinary incontinence, (4) spina bifida-specific physical limitations, and (5) psychosocial barriers. Participant suggestions for improving the International Index of Erectile Function to increase its applicability were identified. CONCLUSIONS: While many perceived the International Index of Erectile Function as applicable, the measure inadequately captures the diverse sexual experiences of young men with spina bifida. Disease-specific instruments to evaluate sexual health are needed in this population.

Robot-assisted Laparoscopic Pyeloplasty: Experience of a Single Pediatric Institution, Including Long-term and Safety Outcomes.

OBJECTIVE: To evaluate the efficacy and safety of robot-assisted laparoscopic pyeloplasty (RALP) and describe the short and long-term outcomes of pediatric RALP. METHODS: We retrospectively reviewed all patients ..±21 years old who underwent primary RALP from 7/2007 through 12/2019. Patients were excluded from postoperative analysis if follow-up data after stent removal was not available. The primary outcome was surgical success, defined as radiographic improvement of hydronephrosis without need for reoperation. Secondary outcomes were time to reoperation and 90-day complication rate. RESULTS: A total of 356 patients underwent primary repair of ureteropelvic junction obstruction during the study period; 29.ßpatients were limited to intraoperative data due to lack of follow-up imaging. Radiographic improvement at latest follow-up was seen in 308/327 (94.2%). Ten of 327 patients (3.1%) underwent reoperation: 7 were identified within 1 year of RALP and 3 were identified over 1 year after RALP. The median time to reoperation was 13.0 months (IQR 9.3-21.7). We defined long-term as>3 years after pyeloplasty. Over one-third (122/327, 37.3%) of the cohort had>3 years of follow-up, none of whom developed evidence of recurrent obstruction requiring reoperation beyond 3 years. Complications occurred within 90 days of surgery in 20/327 (6.1%). CONCLUSION: This largest single-institution series confirms short- and long-term surgical effectiveness and safety of RALP. Our data also indicate that most patients who needed reoperation were identified within 1 year, and reoperation more than 3 years after RALP is rare.

Prospective evaluation of a pediatric urodynamics protocol before and after limiting urine cultures.

PURPOSE: While our institution has historically obtained a urine culture (UCx) from every child at the time of urodynamics (UDS), no consensus exists on UDS UCx utility, and practice varies widely. This study aims to prospectively study our symptomatic post-UDS UTI rate before and after implementing a targeted UCx protocol. MATERIALS AND METHODS: A 2-part prospective study of patients undergoing UDS at one pediatric hospital was undertaken, divided into Phase 1 (7/2016-6/2017) with routine UCx at the time of UDS and Phase 2 (7/2019-6/2020) after implementation of a protocol limiting UCx at the time of UDS to only a targeted subset of patients. The primary outcome was symptomatic post-UDS UTI, defined as positive UCx ≥10ˆ4 CFU/mL and fever ≥38.5 °C or new urinary symptoms within seven days of UDS. RESULTS: A total of 1,154 UDS were included: 553 in 483 unique patients during Phase 1 and 601 in 533 unique patients during Phase 2. Age, sex, race, ethnicity, and bladder management did not differ significantly between phases. All 553 UDS in Phase 1 had UCx at the time of UDS, compared to 34% (204/601) in Phase 2. The rate of positive UCx decreased from 39% in Phase 1-35% in Phase 2. Three patients developed symptomatic post-UDS UTI in each study period, resulting in a stable post-UDS UTI rate of 0.5% (3/553) in Phase 1 and 0.5% (3/601) in Phase 2. These patients varied in age, sex, UDS indication, and bladder management. Four of the six (67%) patients had positive UCx at the time of UDS, one had a negative UCx, and one had no UCx under the targeted UCx protocol. Predictors of symptomatic post-UDS UTI could not be evaluated. DISCUSSION: In the largest prospective study to date, we found that symptomatic post-UDS UTI was <1% and that UCx at the time of UDS can safely be limited at our hospital. This reduction has important implications for cost containment and antibiotic stewardship. We will continue iterative modifications to our protocol, which may eventually include the elimination of UCx at the time of UDS in all groups. CONCLUSIONS: This 2-part prospective evaluation at one pediatric hospital determined that the symptomatic post-UDS UTI rate remained <1% with no identifiable predictors after limiting previously universal UCx at the time of UDS to only a targeted subset of patients.

Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study.

BACKGROUND: We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study. METHODS: We performed a cross-sectional study of people with CE and covered variants managed at five participating institutions. Those with <1 year follow-up or born with variants without hindgut involvement were excluded. Primary outcomes were methods of urinary and bowel management. Urinary management included: voiding via urethra, clean intermittent catheterizations (CIC), incontinent diversion and incontinent in diaper. Bowel management included: intestinal diversion (colostomy/ileostomy) and pull-through (with/without MACE). We evaluated three age groups: children (<10 years), older children (10 to <18) and adults (≥18). We assessed if management varied by age, institution or time (born≤2000 vs. >2000). RESULTS: A total of 160 patients were included (40% male). Median follow-up was 15.2 years (36% children, 22% older children, 43% adults). While 42% of children were incontinent in diapers, 73% of older children and adults managed their bladder with CIC, followed by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%). Among older children and adults, 86% did not evacuate urine per urethra and 28% of adults had an incontinent urinary diversion. No child or adult voided per urethra. Age-adjusted odds of undergoing incontinent diversion was no different between institutions (p = 0.31) or based on birthyear (p = 0.08). Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining patients had a pull-through, half with a MACE. The probability of undergoing bowel diversion varied significantly between institutions (range: 55-91%, p = 0.001), but not birth year (p = 0.85). SUMMARY: We believe this large long-term data presents a sobering but realistic view of outcomes in CE. A limitation is our data does not assess comorbidities or patient-reported outcomes. Rarity of volitional urethral voiding in CE forces the question of whether is a potentially unachievable goal. We advocate thoughtful surgical decision making and thorough counseling about appropriate expectations, distinguishing between volitional voiding and urinary and fecal dryness. CONCLUSIONS: In this long-term, multi-institutional study of patients with CE, 94% of older children and adults manage their bladder with incontinent diversion or CIC. Nearly 80% of patients, regardless of age, have an intestinal diversion. Given that no patients were dry and voided via urethra and 86% of older patients do not evacuate urine per urethra, these data bring into question what functional goals are achievable when performing reconstructive surgery for these patients.

A fresh "slant" on modified Mitchell bladder neck reconstruction: A contemporary single-institution experience.

Introduction: Patients with neurogenic urinary incontinence due to an incompetent outlet may be offered bladder neck reconstruction, but the quest for the perfect surgical-outlet procedure continues. Our aim was to characterize continence and complications after modified Mitchell urethral lengthening/bladder neck reconstruction (MMBNR) with sling and to introduce a modification of exposure that facilitates subsequent steps of MMBNR. Methods: A single-institution, retrospective cohort study of patients who underwent primary MMBNR between May 2011 and July 2019 was performed. Data on demographics, urodynamic testing, operative details, unanticipated events, continence, bladder changes, and additional procedures were collected. A 2013 modification that permits identification of the incompetent bladder neck prior to urethral unroofing was applied to the last 17 patients. The trigone and bladder neck are exposed via an oblique low anterolateral incision on the bladder. Ureteral reimplantation is not routinely performed. Focal incision of the endopelvic fascia after posterior plate creation limits breadth of blunt dissection for sling placement. Descriptive statistics were utilized. Results: A total of 25 patients (13 females) had MMBNR with sling at a median age of 10 years [interquartile range (IQR) 8-11]. Bladder augmentation was performed concurrently in 14/25 (56%) patients. At a median of 5.0 (IQR 3.9-7.5) years follow-up after MMBNR, 9/11 (82%) without bladder augmentation and 13/14 (93%) with bladder augmentation had no leakage per urethra during the day without further continence procedures. Of the three patients with persistent incontinence, two achieved continence with bladder wall Botox injection (overall continence 24/25, 96%). New and recurrent vesicoureteral reflux was noted in five patients and one patient, respectively. Two patients required subsequent bladder augmentation for pressures and one other will likely require it. None have required bladder neck closure or revision. Conclusion: MMBNR with sling provides promising continence per urethra in neurogenic bladder with low need for secondary continence procedures. Ongoing modifications may achieve elusive total continence.

Variation in care between pediatric and adult patients presenting with nephrolithiasis to tertiary care pediatric emergency departments in the United States (2009-2020).

BACKGROUND: Individuals with nephrolithiasis frequently present to the Emergency Department (ED). Safety and quality principles are often applied in pediatric EDs to children presenting with nephrolithiasis, such as limiting ionizing radiation exposure and opioid analgesics. However, it is unknown whether pediatric EDs apply these same principles to adult patients who present with nephrolithiasis. We hypothesized that adult patients would be associated with higher use of radiation-based imaging and opioid analgesics. OBJECTIVE: To assess variations in diagnostic and treatment interventions and hospital utilization between pediatric and adult patients presenting to the pediatric ED with nephrolithiasis. STUDY DESIGN: A retrospective cohort study was conducted, examining outcomes for pediatric (<18-years-old) versus adult (≥18-years-old) patients in 42 pediatric EDs from 2009 to 2020 using the Pediatric Health Information System (PHIS) database. Patients with an ICD-9/10 principal diagnosis code of nephrolithiasis with no nephrolithiasis-related visits within the prior 6 months were included. Primary outcomes were imaging, medications, and surgical interventions. Secondary outcomes were hospital admissions, 90-day ED revisits, and 90-day readmissions. Generalized linear mixed models with random effects were used to adjust for confounding and clustering. RESULTS: In total, 16,117 patients with 17,837 encounters were included. Most hospitals were academic (95.2%), and a plurality were located in the South (38.1%). Most patients were <18-years-old (84.4%, median (interquartile range): 15 (12-17)-years-old), female (57.9%), and White (76.3%), and 17.1% were Hispanic/Latino. Most had no complex chronic conditions (89.2%) and no chronic disease per pediatric medical complexity algorithm (51.5%). For the primary outcome, adults, relative to pediatric patients, who presented to the pediatric ED with nephrolithiasis had higher adjusted odds of receiving computerized tomography (CT) scans (Odds Ratio [OR] 1.43 [95% Confidence Interval [CI] 1.29-1.59]) and opioid analgesics (OR 1.45 [95%CI 1.33-1.58]) (Summary Figure). Secondary outcomes showed that adults, relative to pediatric patients, had lower adjusted odds of hospital admissions, 90-day ED revisits, and 90-day readmissions. DISCUSSION: Our results suggest that certain pediatric safety and quality principles, such as limiting ionizing radiation exposure and opioid analgesic prescriptions, are not being equally applied to pediatric and adult patients who present to pediatric EDs with nephrolithiasis. The mechanism of these findings remains to be elucidated. CONCLUSIONS: Variations in care for individuals with nephrolithiasis reflect an opportunity for quality improvement in pediatric EDs and inform work exploring optimal care pathways for all patients presenting to the pediatric ED with nephrolithiasis.

The Association of Health Literacy with Health-Related Quality of Life in Youth and Young Adults with Spina Bifida: A Cross-Sectional Study.

OBJECTIVE: The objective of the study was to determine if health literacy is associated with health-related quality of life (HRQOL) in adolescents and young adults (AYAs) with spina bifida. STUDY DESIGN: Between June 2019 and March 2020, the Patient-Reported Outcome Measurement Information System Pediatric Global Health-7 (PGH-7), a measure of HRQOL, and the Brief Health Literacy Screening Tool (BRIEF) were administered to patients ≥12 years old with a diagnosis of spina bifida seen in our multidisciplinary spina bifida center. Questionnaires were completed at scheduled clinic visits. The primary outcome was the PGH-7 normalized T-score. The primary exposure was the BRIEF score. Demographic and clinical characteristics were obtained from the medical record. Nested, multivariable linear regression models assessed the association between health literacy and the PGH-7 score. RESULTS: Of 232 eligible patients who presented to clinic, 226 (97.4%) met inclusion criteria for this study. The median age was 17.0 years (range: 12-31). Most individuals were female (54.0%) and had myelomeningocele (61.5%). Inadequate, marginal, and adequate health literacy levels were reported by 35.0%, 28.3%, and 36.7% of individuals. In univariable analysis, higher health literacy levels were associated with higher PGH-7 scores. In nested, sequentially adjusted multivariable linear regression models, a higher health literacy level was associated with a stepwise increase in the PGH-7 score. In the fully adjusted model, adequate health literacy and marginal health literacy, compared with inadequate health literacy, were associated with increases in a PGH-7 score of 3.3 (95% CI: 0.2-6.3) and 1.1 (95% CI: -2.0 to 4.2), respectively. CONCLUSIONS: Health literacy was associated with HRQOL after adjusting for demographic and clinical factors. Strategies incorporating health literacy are needed to improve HRQOL in AYAs with spina bifida.

Can diagnostic and imaging recommendations from the 2011 AAP UTI guidelines be applied to infants <2 months of age?

INTRODUCTION: In 2011, the American Academy of Pediatrics (AAP) published guidelines regarding diagnosis and management of children 2-to-24-months-old with initial febrile urinary tract infection (fUTI). Available data were insufficient to determine whether evidence from studies of 2-to-24-month-olds applies to those <2-months-old, so they were excluded. OBJECTIVE: This study aimed to 1) compare demographic, clinical, imaging and outcomes between patients <2-months-old and those 2-to-24-months-old hospitalized with fUTI, and 2) assess whether diagnostic and imaging recommendations of the AAP 2011 guidelines apply to those <2-months-old. STUDY DESIGN: A cohort study of patients ≤24-months-old hospitalized at a children's hospital with fUTI from 2016 to 2018 was conducted. Data were collected via a prospectively generated electronic medical record note template, supplemented with retrospective chart review. Primary outcomes included differences in demographics, clinical presentation, urine culture results, and imaging utilization/results by age group. Secondary outcomes included surgical procedures, UTI recurrence, and 90-day all-cause readmissions and emergency department (ED) revisits. Univariate and bivariate statistics were utilized to compare age groups. RESULTS: Overall, 137 patients were included (median age 70 days, 55.5% male [92.1% uncircumcised], 53.3% Hispanic/Latino, 89.8% 1st fUTI). There were no demographic differences between groups, except children <2-months-old were more frequently male (71.2 vs 43.6%, p = 0.002). The Summary Table compares clinical factors and imaging utilization by age. There were no differences in urinalysis or urine culture results between groups. Patients <2-months-old had shorter fever duration, lower maximum temperature, and lower white blood cell counts. Voiding cystourethrograms (VCUGs) were recommended and obtained more frequently in patients <2-months-old, but there were no differences in renal and bladder ultrasound (RBUS) or VCUG results between age groups. There were no differences in UTI recurrence (13.6% of <2-months-old vs 14.1% of 2-to-24-months-old, p = 1.00) or fUTI recurrence (13.6 vs 7.7%, p = 0.40) within 1 year, 90-day readmission (6.8 vs 6.4%, p = 1.00), or 90-day ED revisit (22.0 vs 20.5%, p = 1.00). DISCUSSION: There were minimal differences between the <2-months-old and 2-to-24-months-old age groups in demographics, laboratory (including microbial) or imaging results, or clinical outcomes. Patients <2-months-old were more frequently male and less ill. These data support applying urinalysis and urine culture diagnostic criteria, and universal RBUS, from the AAP guidelines to patients <2-months-old. Given utilization differences, applicability of VCUG guideline recommendations requires further clarification for patients <2-months-old. CONCLUSION: Laboratory testing and RBUS recommendations from the AAP guidelines may be safely applied to infants <2-months-old. Further studies are needed to clarify optimal VCUG recommendations.

Botulinum toxin in patients at-risk for bladder augmentation: Durable impact or kicking the can?

PURPOSE: Deferring bladder augmentation (BA) may be desirable in a pediatric neurogenic bladder (NGB) with worsening compliance, but prior studies have questioned whether onabotulinum toxin A (BTX) demonstrates durable improvement in compliance. We present our overall experience with BTX and its role in mitigating the "at-risk" NGB, as determined by urodynamic characteristics. METHODS: A retrospective single-institution review of all BTX procedures performed during January 2010 to October 2018 was conducted. Patients with <12 months follow-up after first BTX injection were excluded. Urodynamic studies (UDS) were reviewed to assign National Spina Bifida Patient Registry (NSBPR) as well as institutionally developed (LCH) risk classification groups. Patients were considered "at-risk" for BA if they had any of the following: NSBPR risk grade of intermediate or hostile; LCH risk grades indicating end-fill pressure >25 cmH2 O or detrusor sphincter dyssynergia (DSD); upper tract changes including new hydronephrosis; new or worsening vesicoureteral reflux; or other signs of a hostile urinary tract (i.e., febrile UTIs). UDS risk grades pre and post-first BTX injection were compared in this "at-risk" group, when available. RESULTS: Thirty-nine patients underwent 162 injection procedures over a median follow-up of 65 months interquartile range (IQR 49-81). Median age at first BTX was 10 years and the median number of BTX injections per patient was 4 (IQR 2-7; range 1-12) with a median time of 6 months between injections (IQR 4-10). Twenty-six patients were deemed "at-risk" at the time of first BTX injection, and of those, 16 (61.5%) proceeded to BA at a median of 36 months (IQR 22.5-42). A small number (four) had BA due to upper tract changes or worsening pressures on BTX, while nine patients (five with CKD) proceeded to BA given a lack of sufficient improvement to consider BTX a long-term viable option. Post-first BTX UDS demonstrated downgrading of risk group in 38% and 63% using NSBPR and LCH classifications, respectively. CONCLUSIONS: Encouraging improvements in the urodynamic risk group were noted in some patients. With careful counseling and follow-up, BTX may safely extend the time to BA in some "at-risk" patients.

Prenatal detection and evaluation of differences of sex development: A qualitative interview study of parental perspectives and unmet needs.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.