Parental decision regret after pediatric urologic surgeries compared to decisions of everyday life.

INTRODUCTION: Parents are at risk of decision regret (DR) for decisions affecting their children. The Decision Regret Scale (DRS) measures medical DR but lacks context outside of healthcare. OBJECTIVE: To compare parental DR 1) between common pediatric urologic surgeries and everyday decisions and 2) with preference to make a different choice. METHODS: We conducted a cross-sectional online survey of randomly selected parents >1year (y) after their children underwent: orchiopexy (males ≤10y), open ureteral reimplant (OUR, females 2-6y), open pyeloplasty (OP, ≤2y), or robotic pyeloplasty (RP, 5-17y) (2017-2021). Higher DRS scores indicate increased DR (none: 0, mild: 1-25, moderate: 30-50, strong: 55-75, very strong: 80-100). Parents completed DRS on four decisions: their child's surgery, most recent/current romantic relationship, most recent leased/purchased car, and most recent purchased meal. Parents reported if they would make the same choice (yes/no). Nonparametric statistics were used. RESULTS: We surveyed 191 parents (orchiopexy n = 52, OUR n = 50, OP n = 51, RP n = 38). The median parent age was 36y (mothers: 86%). Some DR was reported for all decisions, but with significant differences in DR severity. The lowest median DRS score was seen with surgery (orchiopexy 0 [IQR 0-10], OUR 0 [IQR 0-5], OP 0 [IQR 0-0], RP 0 [IQR 0-0]), with no difference between surgery groups (p = 0.78). This was followed by relationship (0, IQR 0-20), car (15, IQR 0-25), and meal (20, IQR 0-30, p < 0.001). Most parents did not report any DR regarding surgery (orchiopexy 69%, OUR 74%, OP 76%, RP 76%, with no difference between surgery groups p = 0.85, Summary Figure). Comparatively, 59% of parents did not have any regret about their relationship, 37% their car, and 28% their meal (p < 0.001). All surgical DR was mild or moderate. No parent (0%) would have chosen differently for their child's surgery versus 4-12% for non-surgical decisions (p < 0.001). Overall, increasing DR corresponded to increasing desire to have made a different choice (DRS≤10: 0%, DRS 45-50: 32%, DRS 55-60: 66%, DRS≥75: 100%, p < 0.001). CONCLUSION: Parental DR varied between urological surgical and non-surgical decisions. It was lowest after surgery. Some regret was reported after every decision, but the subset of parents with regret was smallest after surgical decisions. Positive DRS scores do not necessarily correspond to parents wishing they made a different choice.

Objectified kidney ultrasound echogenicity and size metrics as potential predictors for kidney function in children.

BACKGROUND: Kidney echogenicity is typically determined subjectively but may have a quantifiable relationship to kidney function. Similarly, kidney length has been shown to correlate with kidney function. This study sought to quantify echogenicity using readily available software. Secondarily, we aimed to evaluate the correlation between quantified echogenicity and kidney length to the estimated glomerular filtration rate (eGFR) in children with acute kidney injury (AKI) and chronic kidney disease (CKD). METHODS: In a single-center retrospective observational study, echogenicity index (EI) was determined using a ratio of right kidney to liver mean pixel density. The kidney length ratio (KLR) was determined by the actual to predicted lengths of both kidneys. Both variables were correlated to eGFR using correlation analyses and predictive capacity was determined with receiver operating characteristic curve (ROC) analysis. RESULTS: Of 94 subjects, 46% (43/94) had AKI, 28% (26/95) had CKD and 26% (25/95) were controls. The higher the EI the lower the eGFR (r = - 0.46, p < 0.0001). EI between 1.0 and 1.1 predicted an eGFR < 90 ml/min/1.73m2 with an AUC of 0.71-0.78 while an EI between 1.1 and 1.2 predicted an eGFR < 60 ml/min/1.73m2 with AUC of 0.75-0.80. Overall, the larger the KLR the lower the eGFR (r = - 0.25, p 0.018). CONCLUSION: We have developed an accessible methodology to quantify kidney echogenicity. Overall, there was an inverse correlation between EI and eGFR in pediatric CKD and AKI. However, these correlations did not persist within subgroups which could be due to small sample size and heterogeneity of etiologies. Overall, KLR had a weaker correlation to eGFR, compared to EI. Despite these correlations, both EI and KLR had "fair" to "good" performance as a biomarker for an eGFR < 60 ml/min/1.73m2.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

People with spina bifida use their MACE on long-term follow-up: A single institutional retrospective cohort study.

OBJECTIVE: While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. METHODS: All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). RESULTS: Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). COMMENT: Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. CONCLUSIONS: Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care.

Age-based risk of end-stage kidney disease in patients with myelomeningocele.

OBJECTIVE: We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. METHODS: We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). RESULTS: Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. COMMENT: Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. CONCLUSIONS: While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.

Ask the parents: Testing the acceptability and usability of a hypospadias decision aid.

INTRODUCTION: In previous work, we engaged key stakeholders to create a web-based decision aid (DA) prototype to facilitate shared decision making about hypospadias. OBJECTIVE: The study's objective was to use a human-centered design approach to assess the DA's acceptability and usability and revise it prior to pilot testing. METHODS: We recruited English-speaking parents (≥18 years old) of sons with hypospadias (≤5 years) for a two-phase process of semi-structured phone/video interviews to obtain feedback about our DA prototype. DA webpages included: "Hypospadias," "Surgery Basics," "No Surgery," "Family Stories," "Help Me Decide," and "FAQs." In both phases, participants viewed the DA using the "think aloud" technique and completed several validated scales to evaluate its acceptability and usability. In phase 1, we collected feedback about the "Homepage" organization, values clarification methods (VCM), and webpage content. In phase 2, participants searched the DA for answers to hypospadias-related questions, provided feedback on testimonial videos and VCM, and shared their preferences about data visualizations. All interviews were audio recorded. After each phase, transcripts were qualitatively analyzed to identify key areas for revision. Revisions were made between phase 1 and 2 to improve the DA's acceptability and usability. RESULTS: We interviewed 20 participants (10/phase): median age 33.7 years, 60% female, 80% White. Mean score on the Preparation for Decision Making Scale: 86.8 (out of 100). We revised: 1) VCM, focusing on pros/cons of surgery and question prompts, 2) "Homepage," adding webpage descriptions (Extended Summary Figure), 3) menu organization, 4) "Surgery Day" webpage, adding general anesthesia risk information, and 5) "Hypospadias" webpage, adding an icon bar graph to help participants visualize statistics. Participants thought the testimonial videos were relatable and the VCMs would prepare them for their visit with their child's urologist. DISCUSSION: Ours is the first parent-centered DA developed and pre-tested for hypospadias. Using validated usability and acceptability scales, participants highly rated the DA in helping them arrive at a decision about surgery. Study limitations include the sample's lack of diversity (i.e., educated, health literate) and participants already decided about their son's hypospadias management before enrolling. To learn more about the DA's usability and acceptability, we plan to pilot test it in a clinical setting. CONCLUSIONS: Participants found our DA informative in understanding hypospadias. There was a high perceived level of preparation for hypospadias decision making. Participatory research methods, such as "think aloud," may be helpful when testing DAs as they privilege the patient's experience.

Split appendix catheterizable urinary channels are at no higher risk of undergoing revision compared to channels made with the intact appendix.

OBJECTIVE: To assess long-term APV and split-appendix MACE durability and to compare split and intact appendix APVs in a large patient cohort. METHODS: This retrospective cohort study included consecutive patients ≤21 years old undergoing an APV at our institution (1990-2019). Main outcomes were stomal and subfascial revisions. Kaplan Meier survival and Cox proportional hazards analysis were used. RESULTS: A total of 339 patients underwent APV creation at a median 7.4 years old (41% female vs. 59% male; 37% umbilical stoma vs. 63% other). In total, 36 patients underwent a stomal revision and 19 a subfascial revision (median channel follow-up 6.3 years). On survival analysis, the risk of stomal revision of the APV was 9.1% at 5 years, 12.6% at 10 years and 16.5% at 15 years. Risk of subfascial revision of the APV was 5.1% at 5 years, 7.0% at 10 years and 8.2% at 15 years. A split-appendix APV was performed in 118 (34.8%) of 339 patients. They had a shorter follow-up compared to those with an intact APV (5.1 vs. 7.0 years, p = 0.03). After correcting for differential follow-up time, there was no significant difference between groups for stomal revisions (HR 1.11, p = 0.76) or subfascial revisions (HR 0.80, p = 0.67, Figure). Risk of APV stomal revision was independent of stomal location and age at surgery (p ≥ 0.37). Similarly, risk of subfascial APV revision was independent of stomal location and age at surgery (p ≥ 0.18). Risk of stomal revision for split-appendix MACE channels was 16.2% at 5, 10 and 15 years (similar to split-appendix APV and all APVs, p ≥ 0.26). Risk of MACE subfascial revision was 5.5% at 5 years, 5.5% at 10 years and 14.7% at 15 years (similar to split-appendix APV and all APVs, p ≥ 0.36). COMMENT: We focused on surgical complications, as these entail the highest morbidity, however, we did not assess non-surgical, percutaneous or endoscopic management which also impact long-term outcome and patient quality of life. We did not compare the outcomes of the split-appendix MACE to an intact-appendix MACE cohort, as this patient population was not captured in this review. CONCLUSIONS: The split-appendix technique has durable long-term results for both the APV and MACE channels, which are comparable to the technique utilizing the intact appendix. Channel complications occur over the channel's lifetime, as 1 in 8 APVs in the entire cohort underwent a stomal revision and 1 in 14 APVs underwent a subfascial revision at 10 years after surgery.

Spinal column shortening for secondary tethered cord syndrome: radiographic, clinical, patient-reported, and urodynamic short-term outcomes.

OBJECTIVE: Tethered cord syndrome (TCS) is a clinical and radiographic diagnosis of pathological stretching of the spinal cord leading to progressive loss of neurological function. The gold standard treatment for TCS is a tethered cord release. However, detethering involves significant risks of spinal cord injury and high rates of retethering. To mitigate these risks, the concept of spinal column shortening (SCS) to decrease spinal cord tension has become an alternative to detethering. In this study, the authors applied SCS to a pediatric and emerging adult population affected by secondary TCS. METHODS: A retrospective review of a prospective database at the authors' tertiary pediatric institution was performed. The Pediatric Quality of Life Inventory, patient- and parent-reported outcomes, and urodynamics were used to evaluate the outcomes of TCS treated with SCS. RESULTS: A total of 41 patients with secondary TCS were treated with SCS. The average age at the time of surgery was 15.9 years (range 5-55 years). Preoperative symptoms evaluated included pain (33 patients), weakness (30 patients), and bladder/bowel dysfunction (39 patients). The most common level of spinal column osteotomy was T12, with spinal fusion between T10 and L2. The mean follow-up time was 22.6 months (range 8-45 months). For patients with at least 12 months of follow-up, subjective clinical improvements were reported in 21/23 (91.3%) of those with preoperative pain (p < 0.01); in 16/24 (66.7%) of patients with weakness (p < 0.01), and in 15/29 (51.7%) of those with bladder/bowel dysfunction (p < 0.01). The median differences in initial and most recent Pediatric Quality of Life Inventory results were +5 for patient-reported scores (n = 19, p = 0.04) and +5 for parent-reported scores (n = 19, p = 0.08). Formal urodynamics performed at a median of 3.5 months after surgery documented stable to improved bladder function in 16/17 patients, with a median improvement in one classification category (n = 17, p = 0.01). CONCLUSIONS: SCS continues to represent a safe and efficacious alternative to traditional spinal cord untethering for TCS in children and emerging adults, as documented by objective formal urodynamics and patient- and parent-reported outcomes.

Incidence of nephrolithiasis after bladder augmentation in people with spina bifida.

INTRODUCTION: Risks of nephrolithiasis after bladder augmentation in people with spina bifida (SB) remain unclear. Annual incidence of nephrolithiasis in the general population is 0.01% for 10-14 years old, 0.07% for 15-19 years old and 0.2% for 20-24 years old. Our aim was to assess the incidence and risk factors of nephrolithiasis in SB patients after augmentation. METHODS: Patients with SB and augmentation followed at our institution were retrospectively reviewed (born ≥1972, surgery 1979-2019). Patients were screened annually with renal bladder ultrasound and abdominal radiograph. Main outcome was nephrolithiasis treatment. Kaplan-Meier survival and Cox proportional hazards analysis were used. Possible predictors were assessed using stepwise forward selection (variables with p < 0.1 on univariate analysis included in multivariate analysis). RESULTS: 427 patients with SB and augmentation were included (51.8% female, 74.9% shunted). Median age at augmentation was 8.5 years (median follow-up: 12.4 years, ileum segment: 81.0%, bladder neck procedure: 60.7%, urinary channel: 74.2%) and 28.8% developed bladder stones. Overall, 47 (11.0%) patients were treated for nephrolithiasis. After correction for differential follow-up, nephrolithiasis was treated in 7.3% at 10 years, 13.2% at 15 years, and 18.0% at 20 years (Figure). Patients presented with either a urinary tract infection (46.8%), on screening (44.7%), or pain (8.5%). Stones were treated percutaneously, endoscopically or by ESWL (63.8%/34.0%/10.7%, respectively). Most were calcium stones (58.3%). On multivariate analysis, compared to younger patients, patients augmented at ≥10 years of age had 1.84 times the risk of nephrolithiasis (p = 0.01). Nephrolithiasis was more common in those who developed bladder stones (HR = 3.00, p < 0.0001). Among those with both renal and bladder stones, bladder stones typically preceded nephrolithiasis (55.2%), were treated concurrently (31.0%) and 13.8% occurred after nephrolithiasis. Gender, wheelchair use, bowel segment used, MACE and skeletal fractures were not associated with higher nephrolithiasis risk (p ≥ 0.11). DISCUSSION: This study of a large cohort of SB patients with long-term follow-up highlights that the risk of nephrolithiasis is cumulative and related to bladder stone formation, age at augmentation and time since augmentation. An association with bladder stones suggests potential shared metabolic causes. The study's retrospective design likely led to underestimating the risk of nephrolithiasis by not capturing spontaneously passed stones. CONCLUSION: Approximately 1% of patients with SB develop nephrolithiasis annually after augmentation. Close long-term surveillance after augmentation is strongly indicated, as nephrolithiasis incidence in augmented patient with SB is at least 10 times higher than general population. Patients with bladder stones are especially at risk.

Fertility and sexuality issues in congenital lifelong urology patients: male aspects.

PURPOSE: To review existing literature about fertility and sexuality of boys born with complex congenital genitourinary anomalies. METHODS: A Pubmed review was performed in December 2018 to identify the most relevant original manuscripts regarding male complex congenital conditions affecting the urogenital system in male patients including spina bifida (SB), bladder exstrophy-epispadias complex (BEEC) and hypospadias. A comprehensive review was drafted exploring sexual dysfunction from a medical, psychosexual, surgical and reproductive point of view during transition from childhood (or adolescence) to adulthood. RESULTS: About 75% of men with SB have erectile dysfunction (ED) (Gamé et al. in Urology 67(3):566-570, 2006; Diamond et al. in 58(4):434-435, 1986). Most SB patients have impaired sexual development mainly due to diminished self-esteem, dependence on caregivers and lack of privacy (Blum et al. in Pediatrics 88(2):280-285, 1991). Men with BEEC have fewer intimate relationships than women because of the greater difficulties with issues regarding their genitalia and sexual activities (Deans et al. in Am J Obstet Gynecol 206(6):496.e1-496.e6, 2012). However, a good quality of life is achievable with the effective use of coping strategies (Deng et al. in Transl Androl Urol 7:941, 2018; Rikken et al. in BMC Womens Health 18(1):163, 2018; Friedler et al. in Reprod Biomed Online 32(1):54-61, 2016). Chordee occurs in 25% of all hypospadias patients. More severe hypospadias is related to a greater risk for complications. The long-term sexual quality of life (QoL) in men who underwent hypospadias surgery is influenced by a lot of factors. Therefore, an interactive and dynamic biopsychosocial model of sexual QoL was proposed. CONCLUSIONS: The care of patients with congenital urologic conditions becomes a challenge especially in the period of 'transition'. The goal of follow-up is a holistic management viewed from a medical, psychosexual, surgical end reproductive point. All patients should be asked for specific urinary, fecal or sexual concerns.

The basics of transition in congenital lifelong urology.

PURPOSE: Transition in urology is defined by the process that allows an adolescent or a young adult with a congenital or acquired urogenital anomaly to assume increasing responsibility for their own health care and to become the primary decision maker in their care. METHODS: A review of the literature regarding transitional care for lifelong urologic congenital anomalies was performed with the aim of reporting expert opinion when data are non-existent. This review focuses on special considerations for adolescents and young adults with spina bifida, bladder exstrophy, anorectal malformations and differences of sexual development. RESULTS: Urologic goals during the transition from childhood to adulthood continue to include attention to the preservation of renal function and optimization of lower urinary tract function. Additional concerns include care to decrease long-term surgical complications (especially after augmentation cystoplasty), to monitor for malignancy, to prepare for sex activity and fertility, and to help the adult patient in decision making. Transition aims to maximize quality of life and independence by ensuring uninterrupted appropriate care through a multidisciplinary approach which varies by geographical location and healthcare setting. Barriers include patient and family factors as well as provider and system related factors. A dedicated team is an important element of successful transition.

User-centered development of a hypospadias decision aid prototype.

INTRODUCTION: Many parents who choose hypospadias repair for their sons experience decisional conflict (DC) and decisional regret (DR). We previously found that parental decision-making about hypospadias surgery is a complex process characterized by cyclic information-seeking to alleviate anxiety and confusion. OBJECTIVE: The objective of this study was to engage parents of hypospadias patients and pediatric providers in the co-design of a decision aid (DA) prototype to facilitate shared decision-making about hypospadias surgery and address DC and DR. METHODS: From August 2018 to January 2019, we conducted three co-design workshops with parents of hypospadias patients, pediatric urology and general pediatric providers to discuss their recommendations for a DA prototype. Activities were audio recorded and professionally transcribed. Transcripts and worksheets were analyzed by six coders using qualitative analysis to identify key aspects of a hypospadias DA desired by stakeholders. We conducted a collaborative design and prototyping session to establish key features and requirements, created a content map visualizing this work, and then developed a DA prototype. RESULTS: Parent participants included 6 mothers and 4 fathers: 8 Caucasian, 2 African-American; median age 31 years. Providers included pediatric urology (n = 7) and general pediatric providers (n = 10): median age 47.5 years, 83.3% Caucasian, 58.3% male, 58.3% MD's and 41.7% nurse practitioners. Participants created user-friendly, interactive DA prototypes with "24/7" availability that had three key functions: 1) provide accurate, customizable, educational content, 2) connect parents with each other, and 3) engage them in a decision-making activity. The prototype consisted of five modules (Extended Summary Figure). "Hypospadias Basics" includes epidemiology and a hypospadias severity scale. "Surgery Basics" includes goals, illustrated steps, and pros/cons of surgery. "Testimonials" includes videos of parents and adolescents discussing their experiences. "Help me Decide" includes a decisional conflict scale and a decision-making activity (i.e. values clarification method). "Frequently Asked Questions" covers general hypospadias information, perioperative expectations and a review of postoperative care. DISCUSSION: To our knowledge, this is the first DA prototype developed for a pediatric urology condition using a human-centered design approach to engage many key stakeholders in the development process. One limitation of this study is the small population sampled, which limits generalizability and means that our findings may not reflect the views of all parents or pediatric providers involved in hypospadias decision-making. CONCLUSIONS: We created a parent-centered hypospadias DA prototype that provides decision support in an online, interactive format. Future directions include further testing with usability experts, providers and parents.

User testing of a hypospadias decision aid prototype at a pediatric medical conference.

INTRODUCTION: Based on our previous qualitative work, we created a web-based decision aid (DA) prototype to facilitate shared decision-making regarding hypospadias. OBJECTIVE: The objective of this study was to obtain rapid feedback on the prototype as part of an iterative, human-centered design process. METHODS: We conducted this study at a statewide, pediatric educational conference in May 2019, recruiting attendees by verbal/written announcements. The DA consisted of: hypospadias overview and surgery "storyboard," frequently asked questions, parent testimonials, and a values clarification exercise. Participants viewed the DA on a tablet as they participated in semi-structured, qualitative interviews covering website acceptability, usability, and preference for surgical photographs versus illustrations. Three coders used qualitative content analysis to identify themes and resolved disagreements by consensus. RESULTS: Of 295 conference attendees, all 50 who approached us agreed to participate. Responses from 49 participants were available for analysis: 67% female, ages 20-69, 65% Caucasian, 55% MDs. 96% of participants thought the website design matched its purpose; 59.1% preferred surgical illustrations, 8.2% preferred photos, 30.6% preferred both and 2.0% did not like either. Participants recommended improvements in: a) usability/accessibility (e.g. site navigation, visual layout, page length), b) content coverage (e.g. epidemiology, consequences of no/delayed surgery, lifelong risks), c) parent-centeredness (e.g. reading level/writing style) and d) implementation (provider tools, printable handouts). The Extended Summary Figure shows a revised image of the first step of a hypospadias repair based on feedback about participants' preferences for illustrations rather than photographs. DISCUSSION: The main strength of our study was the valuable feedback we obtained to inform critical revisions of the DA prototype. We also demonstrated the feasibility and efficacy of a conducting a usability evaluation of a web-based DA in a medical conference setting. One limitation of this study is that the relatively small population sampled limits generalizability and our findings may not reflect the views of all providers who care for hypospadias patients. CONCLUSIONS: The vast majority of providers thought that the design of the Hypospadias Homepage matched its purpose and most preferred surgical illustrations rather than photos to demonstrate the steps of hypospadias surgery. Based on their feedback, we plan to focus our efforts in the following areas: 1) improvement of navigation/menus, 2) reduction in the amount of text per page, 3) expansion of specific content coverage and 4) inclusion of "parent-friendly" visuals such as infographics to represent quantitative data and colorful illustrations to depict hypospadias and its surgical repair.

The need for specialized training for adults with congenital urologic conditions: differences in opinion among specialties.

INTRODUCTION: The aim of this study was to survey pediatric urology fellowship directors (PFD) and adult reconstruction fellowship directors (AFD) to assess who they believe has sufficient training to care for adults with congenital urologic conditions (ACUC). MATERIAL AND METHODS: An online survey was created to assess attitudes towards specific training to care for ACUC. The survey was administered to 27 PFD and 26 AFD [16 from genitourinary reconstructive surgery (GURS) and 10 from female pelvic medicine and reconstructive surgery (FPMRS)]. Both groups were asked if specific training is warranted, and if general urologists, pediatric urologists or adult reconstructive urologists were sufficiently trained to care for ACUC. RESULTS: A total of 26 (96%) PFD and 10 (39%) AFD completed the survey. All PFD were fellowship trained in pediatrics. Of the AFD, 5 were GURS trained, 4 were FPMRS trained and 1 was not fellowship trained. The majority (65% PFD, 90% AFD) believed specific training is warranted. Few believed general urologists have sufficient training (8% PFD, 20% AFD). Most PFD believed pediatric urologists have sufficient training (85%), but a minority believed those with adult reconstructive training do (40%). Conversely, a minority of AFD believed that pediatric urologists have sufficient training (40%), while those with adult reconstructive training do (FPMRS: 67%, GURS: 60%). CONCLUSIONS: Both pediatric and adult reconstructive urologists believe specific training to care for adults with congenital urologic conditions is warranted. Neither group considers the other to be ideally suited to care for this complex patient group. This suggests both groups may have something to learn from each other.

Provider perspectives on shared decision-making regarding hypospadias surgery.

INTRODUCTION: Many parents experience decisional conflict and decisional regret around hypospadias surgery. The utilization of a shared decision-making (SDM) process may mitigate these issues, however addressing the principal components of the SDM process is a complex task that requires the investment of providers. OBJECTIVE: The purpose of this study was to facilitate a discussion about SDM anchored on hypospadias with pediatric urology and general pediatric providers to explore perspectives, clinical applications and barriers to adopting SDM in clinical practice. STUDY DESIGN: We conducted two focus groups in order to engage pediatric urology and general pediatric providers in guided discussions about SDM anchored on hypospadias. All activities were audio recorded and professionally transcribed. The transcripts were analyzed by three coders using directed qualitative content analysis techniques to identify themes and relationships between themes to inform the development of an affinity diagram (Extended Summary Figure). RESULTS: Two focus groups were held; one with seven pediatric urology providers in November 2018 and one with ten general pediatric providers in January 2019 (median age 51 years, 88.2% Caucasian, 58.8% female, 70.6% physicians and 29.4% nurse practitioners). Both groups identified some of the key components of SDM including engaging families in decision-making, informing them about treatment options and clarifying values/preferences (Extended Summary Figure). They thought that SDM was useful for discussing preference-sensitive conditions (e.g. hypospadias) and addressing parental compliance. General pediatric providers also suggested that SDM helped them avoid unnecessary referrals to specialists. Both groups identified parental, provider and systemic barriers to the adoption of SDM: a) desire for paternalism, b) misperceptions about medical evidence, c) completion of parental decision-making prior to the clinical visit, d) provider bias/lack of interest and e) time constraints/productivity pressures. DISCUSSION: Providers who care for hypospadias patients are knowledgeable about SDM and its potential clinical applications. They identified several potentially modifiable barriers to the adoption of a SDM process about hypospadias surgery in a pediatric clinical setting. CONCLUSIONS: Based on feedback from providers, we plan to implement a hypospadias decision aid early in the parental decision-making process about hypospadias such as in the postpartum unit and at well-child visits in the newborn period and provide a provider training session about SDM to address the identified knowledge gaps.

Estimating and tracking renal function in children and adults with spina bifida.

PURPOSE: Estimated glomerular filtration rate (eGFR) in the general population is stable in children after 2 years of age until adulthood. In the first three decades after age 18, eGFR decreases by 0.3-0.8 ml/min/1.73 m2/year. Little data exists regarding eGFR changes in the spina bifida (SB) population given variability in muscle mass. In the absence of a validated SB-specific eGFR formula, the performance of different eGFR formulas may vary. We performed a cross-sectional study (1) to determine trends in eGFR with increasing age in children and adults with SB and (2) to compare eGFRs calculated using different formulas. METHODS: We retrospectively reviewed records of patients 2-50 years old with SB followed at our institution (2014-2019). We determined eGFR using four pediatric formulas (2-17 years: CKiDSCr, CKiDCys, CKiDSCr-Cys, ZappitelliSCr-Cys) and four adult formulas (18 + years: MDRDSCr, CKD-EPISCr, CKD-EPICys, CKD-EPISCr-Cys). One eGFR per patient was included (most recent eGFR for those with serial measurements). Patients were categorized as chronic kidney disease (CKD) stage 2 (eGFR 60-89) and Stage 3+ (<60). Non-parametric tests, linear regression, and Spearman's correlation were used for analysis. RESULTS: Among 209 children with SB (median age 10.3 years), depending on the formula used, eGFR decreased by -0.7 to -1.8 ml/min/1.73 m2/year (CKiDCys, CKiDScr, p ≤ 0.001), remained stable (CKiDSCr-Cys, p = 0.41), or increased by +2.7/year (ZappitelliSCr-Cys, p < 0.001) (Figure). The proportion of children with CKD 2 or higher varied between formulas (11.5-58.9%, p < 0.001). Correlations between pediatric formulas were negligible to moderate. Comparing any two formulas, 12.0-65.6% of children were assigned a different CKD stage. Among 164 adults (median age 26.3), eGFR decreased for each formula (range: -1.3 to -2.2/year, p ≤ 0.01) (Figure). The proportion of adults with CKD 2 or higher varied between formulas (9.2-30.5%, p < 0.001). Correlations between adult formulas were moderate to very high. Comparing any two formulas, 8.5-26.8% of adults were assigned a different CKD stage. COMMENT: We cannot reliably determine whether eGFR changed during childhood. Among adults, eGFR decreased with age for every formula evaluated at greater than twice the general population rate. Without a validated SB-specific eGFR formula, we are left with formulas providing different results. CONCLUSIONS: Estimated GFR among adults with SB appears to deteriorate at a higher rate than in the general population. Due to lack of precision of accepted eGFR formulas in the SB population, this may be a real phenomenon, an artifact of inaccurate eGFR formulas, or both. A validated SB-specific eGFR formula is needed.

Additional Surgeries after Bladder Augmentation in Patients with Spina Bifida in the 21st Century.

PURPOSE: We determined the long-term risks of additional surgery after bladder augmentation in a modern spina bifida cohort accounting for differential followup. MATERIALS AND METHODS: We retrospectively reviewed patients with spina bifida who were born after 1972 and were followed at our institution after augmentation surgery performed between 1979 and 2018. Outcomes included diversion, bladder stones, perforation, reaugmentation, laparotomy for bowel obstruction, and benign and malignant bladder tumors. Survival analysis was used for the entire cohort and the modern cohort (detubularized and reconfigured ileocystoplasty beginning in 2000). RESULTS: A total of 413 patients were included in the study. At a median followup of 11.2 years 80.9% of the patients had undergone ileocystoplasty and 44.1% had undergone 370 additional surgeries. Ten-year risk of any reoperation was 43.9%, with 17.4% of patients undergoing 2 or more and 9.9% undergoing 3 or more additional surgeries. Outcomes included conversion to a diversion (2.7% at 10-year followup) and bladder stones (28.2% with recurrence in 52.4%) irrespective of detubularized reconfigured status (p ≥0.20). Bladder perforation risk was 9.6% for patients undergoing vs 23.7% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.01). Similarly reaugmentation rate was 5.3% for patients undergoing vs 15.2% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.001). Finally, 10-year reperforation risk was 32.1% for patients undergoing vs 73.8% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.053). Other risks included bowel obstruction (4.5% with recurrence in 15.8%), nephrogenic adenoma (2.2% with regrowth in 48.2%) and malignancy (0.0% at 20 years). For 222 patients in the modern cohort (median followup 9.1 years) 10-year risk of any reoperation was 46.0%, which consisted of diversion in 4.0%, stones in 32.9% (recurrence in 44.5%), perforation in 8.8% (recurrence in 42.2%), reaugmentation in 4.3%, obstruction in 4.9% (recurrence in 10.0%), adenoma in 4.7% (regrowth in 40.0%) and cancer in 0.0%. CONCLUSIONS: Bladder augmentation is long-lasting. While benefiting continence and renal outcomes, this operation frequently requires additional surgeries, necessitating close followup. Since survival analysis based risks of alternative management options such as incontinent diversion are unavailable, comparisons with augmentation are unfeasible.

Presentation and Management Patterns of Lower Urinary Tract Symptoms in Adults Due to Rare Inherited Neuromuscular Diseases.

OBJECTIVE: To describe the urologic sequalae of several rare congenital neuromuscular diseases. METHODS: We retrospectively reviewed medical records at Gillette Specialty Healthcare (2014-2018) of patients presenting to urology clinic with lower urinary tract symptoms and select rare congenital diseases: muscular dystrophy, spinal muscular atrophy, and Rett syndrome. RESULTS: Muscular dystrophies (n = 19) are X-linked myogenic disorders characterized by progressive muscle wasting and weakness. Men present to the urologist at variable ages, typically with complaints of functional incontinence and normal cystometrograms; we manage them with oral anticholinergic medications, condom catheter, or suprapubic catheter. Spinal muscular atrophy (n = 6) is a rare autosomal recessive disease characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem leading to progressive muscle weakness and atrophy. Patients typically present with nephrolithiasis and urinary retention in late adolescence/early adulthood, but timing varies. Filling cystometrograms have been normal. We allow passive retention with intermittent catheterization and creation of catheterizable channels, when indicated. Rett syndrome (n = 5) is a rare, noninheritable genetic condition affecting females characterized by a brief period of normal development followed by loss of speech and purposeful hand use; there are characteristic behaviors. Patients present in early adulthood with complaints of urinary retention. We manage retention with permissive retention or sphincter chemodenervation. CONCLUSION: Several congenital neuromuscular conditions can cause lower urinary tract symptoms when these individuals become adults. We have discussed the clinical characteristics and management of select neurogenic and myogenic bladder conditions seen in adults with congenital conditions.

Risk of Reaugmentation after Enterocystoplasty Using a Reconfigured Bowel Segment in Patients with Spina Bifida: A Bi-Institutional Cohort Study.

PURPOSE: Reaugmentation cystoplasty rates vary in the literature but have been reported as high as 15%. It is likely that bladders augmented with detubularized and reconfigured bowel are less likely to require reaugmentation. We assessed the incidence of reaugmentation among patients with spina bifida at 2 high volume reconstruction centers. MATERIALS AND METHODS: We retrospectively reviewed medical records of patients with spina bifida who underwent enterocystoplasty before age 21 years (1987 to 2017). Those who did not undergo augmentation with a detubularized and reconfigured bowel segment were excluded from analysis. Data on demographic and surgical variables were collected. Reaugmentation was the main outcome. One analysis was performed using the entire cohort and another analysis was restricted to patients with ileocystoplasty performed in the last 15 years (2002 to 2017). Survival analysis was used. RESULTS: A total of 289 patients were identified. Enterocystoplasty was performed in patients at a median age of 8.1 years (median followup 11.3, IQR 5.2-14.9). Most initial augmentations were performed using ileum (93.4%), followed by sigmoid (6.2%). Seven patients underwent reaugmentation, including 6 with initial augmentation using ileum and 1 with initial augmentation using sigmoid. On survival analysis risk of reaugmentation was 1.1% at 5 years and 3.3% at 10 years after the original surgery. All reaugmentations occurred within the first 9 years of followup. In the more contemporary cohort (162, median followup 7.0 years) only 1 patient underwent reaugmentation at 2.0 years. CONCLUSIONS: The risk of reaugmentation after enterocystoplasty with a detubularized and reconfigured bowel in the spina bifida population is lower than that reported in initial series.

Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida.

BACKGROUND: Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. METHODS: We retrospectively reviewed patients born with SB 2005-2009, presenting to our institution within 1 year of birth. Outcomes at 8-11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists' interpretation and initial findings compared with final outcomes. RESULTS: Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8-11 years old, one had confirmed chronic kidney disease (stage 2). CONCLUSIONS: Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8-11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.