Current opinions regarding care of the mature pediatric urology patient

Introduction With continued improvements in pediatric urology care of patients with complex congenital genitourinary conditions, many survive into adulthood. This fact has created a challenging situation of transitioning from pediatric to adult care. Establishing long-term follow-up with appropriate specialists is a critical part of a successful transition to adulthood for this population. Objective This study sought to elucidate current practices and opinions regarding the management of adult complex genitourinary patients by pediatric urologists, in order to determine if a consensus for adult care exists. Study design An anonymous, 15-question online survey was created to address practice patterns and opinions regarding the transition of care of complex genitourinary patients. An invitation to participate was distributed via email to 200 pediatric urologists who were members of the American Urological Association. Complex genitourinary patients were defined broadly as those with a history of: spina bifida, bladder exstrophy, cloacal exstrophy, cloacal anomalies, posterior urethral valves or disorders of sex development. Fisher's exact test was used for analysis. Results The response rate was 31.0% (62/200). Two-thirds (67.7%) cared for adults with complex genitourinary conditions. Overall, 51.6% of pediatric urologists felt that general urologists best follow adult patients, but only 6.5% recommended this for patients with prior complex genitourinary reconstruction (P < 0.001). Instead, the majority (80.6%) felt that a pediatric or adult urologist with an interest and training in adolescent/transitional urology who routinely performs such procedures would provide optimal care. Follow-up by a primary care physician alone was not recommended. Recommendations did not change if patients had developmental delay or lived independently (P = 0.47 and P = 0.72, respectively). Overall, 69.4% would refer mature complex genitourinary patients to a urologist with interest and training in adolescent/transitional urology, if one was available. However, only 45.2% had such an individual available in their practice (P < 0.001). Discussion In the present study, the opinions of pediatric urologists regarding optimal providers of long-term follow-up for mature complex genitourinary patients were presented. While the results may not represent the views of the entire pediatric urology community, responses from motivated individuals with a particular interest in transition care may be especially valuable. Although the present study did not outline a mechanism for improving transitional care, it offered valuable information on prevailing opinions in this area. Finally, the opinions of mostly North American Pediatric Urologists were presented, which may not apply to other healthcare settings. Conclusions Pediatric urologists appeared to be virtually unanimous in recommending that urologists provide the most appropriate long-term follow-up of patients with congenital genitourinary conditions. Specifically, 80% recommended that patients with prior complex surgical reconstruction be followed by a urologist with specific interest, training and experience in the area of transitional urology. The data suggest that this may be an unmet need of these specialists and may signify the need for specific training in the care of such patients

Transitioning Young Adults with Neurogenic Bladder – Are We Asking Too Much?

Introduction Significant numbers of young adults with chronic health conditions fail to transition. Objective We aimed to evaluate how ready urologic patients who have transitioned were actually prepared for that process. Due to the cognitive impairments frequently seen with spina bifida (SB), we hypothesize that these individuals will be less prepared to transition medical care to adult providers compared to their healthy counterparts. Methods Participants included consecutive patients in the transitional SB clinic at our institution and controls (college student without obvious physical disability or interest in healthcare related fields aged 18-25). Both groups were administered the Transition Readiness Assessment Questionnaire (TRAQ) over a nine-month period. Five TRAQ domains assess 20 skills necessary to transition. Likert scale responses range from 1 “No, I do not know how” to 5 “Yes, I always do this when I need to” (which we considered appropriate for transitioned patients). Demographics and the number of daily medications taken were collected. Patients and healthy controls were compared using 1) total and domain TRAQ scores, 2) the proportion of non-transitioned skills (“1”) and 3) fully transitioned skills (“5”). Non-parametric statistics were used. Results 43 unique SB patients (30.8% shunted, 46.5% female) and 100 controls were enrolled. SB patients were older than controls (21 vs 20 years, p<0.001). There was no gender difference between groups (p=0.33). Transitioned patients and college students were fully transitioned only in the “Talking with Providers” domain ( Figure ). College students performed significantly better than patients in the domains of “Appointment Keeping” (p=0.04) and “Tracking Health Issues” (p=0.02). Transitioned patients were less likely to be interested in learning how to perform skills in the domains of “Appointment Keeping” and “Tracking Health Issues” (p<0.001 for both domains). Discussion We describe the transition readiness of young adults with SB compared to healthy controls and other youths with chronic health conditions. Limitations include the small sample size, potentially limiting generalizability, as well as cross-sectional nature. Conclusion “Transitioned” patients with SB had lower TRAQ scores in some domains compared to healthy college students, who themselves had scores indicating that they were not fully ready for transition. Increased attention to transition readiness in people with SB is necessary, as even healthy young adults struggle with these tasks and are poorly prepared for transition

Pediatric Sacral Nerve Stimulator Explanation due to Complications or Cure: A Survival Analysis

Introduction Historically, there have been few treatment options for children with severe, refractory bladder and bowel dysfunction (BBD). Sacral neuromodulation (SNM) continues to show promising results in this challenging pediatric population with recalcitrant lower urinary tract symptoms. At our institution, we have begun offering explantation to those with persistent improvement after >6 months of having device turned off. We hypothesized that 1.) SNM explantation for cure increases with extended follow-up, and 2.) those explanted for cure would have improved symptoms and quality of life when compared to those explanted for complication. Materials & Methods We retrospectively reviewed all consecutive patients <18 years old who underwent SNM placements at our institution (2012-2017). We excluded those without the second stage procedure. Reasons for device explantation were categorized as: cure (resolution of symptoms with the device turned off for at least 6 months), or a complication (e.g. infection, need for MRI, or pain). Non-parametric tests and survival analysis were used for analysis to account for differential follow-up time. Of those explanted, surveys were electronically sent to assess BBD severity, and overall quality of life. Results Of 67 children who underwent a first stage procedure, 62 (92.5%) underwent a second stage procedure. 61 met inclusion criteria (68.9% female, 29.5 % with previous filum section, median age at implantation 10.3 years old). During follow-up (median 2.3 years), 12 patients (19.7 %) had the SNM exchanged/revised due to lead fracture/breakage and return of urinary symptoms. To date, 50 patients remain with their SNM implanted, and 11 have been explanted. Adjusting for follow-up time, the risk of explantation was 6.5% at 2 years (2.2% for cure, 4.3% for complications) (Figure 1). Explantation increased to 24.5% at 3 years (16.5% for cure, 8.0% for complications) and 40.4% at 4 years (32.4% for cure, 8.0% for complications). Questionnaires were collected on patients post explant (median 2.2 years), with improvement in those explanted for cure compared to complication (Figure 2). Discussion SNM explantation for cure is a novel concept previously not described in the literature. Limitations of this study include the relatively small numbers, and lack of objective data in the cohort that remains with SNM device implanted. Conclusion SNM is a safe, viable option for the pediatric patient with refractory bladder dysfunction. Furthermore, SNM explantation for cure is an option with increasing likelihood after two years

Validation and Preliminary Results of the Parental Assessment of Children's External Genitalia Scale for Females (PACE-F) for Girls With Congenital Adrenal Hyperplasia

Objective To validate a parental assessment of children's external genitalia scale for females (PACE-F) for girls with congenital adrenal hyperplasia (CAH) by adapting the validated adult female genital self-image scale. Methods PACE-F was administered to parents of girls (Tanner 1, 2 months-12 years) with and without CAH. Final questions were determined by clinical relevance and psychometric properties (scores: 0-100). A reference range was established using 95% confidence interval among controls. Age-matched controls were compared to girls with CAH (1) <4 years old before and after female genital reconstruction surgery (FGRS), and (2) 4-12-year olds after FGRS. Nonparametric statistics were used. Results Participants included 56 parents of 41 girls with CAH (median 3.9 years old, 97.6% FGRS) and 139 parents of 130 girls without CAH. Face and content validity was established by families, experts, and factor analysis. Internal consistency was high (Cronbach's alpha: 0.83). Population reference score range was 66.7-100. Ten consecutive girls had pre- and post-FGRS PACE-F scores. All scores improved at 4 months after surgery and all preoperative scores were below reference range and lower than controls (P = .0001). All postoperative scores were within reference range, no different from controls (P = .18). Scores for girls with CAH after FGRS aged 4-12 years were no different from controls (100.0 vs 88.9, P = .77) and 90.0% were in reference range, as expected (P = .99). Conclusion We present a validated instrument for parental assessment of genital appearance in girls with CAH. We demonstrate improved parent-reported appearance after FGRS, with scores similar to age-matched controls

Characteristics of Female Genital Restoration Surgery for Congenital Adrenal Hyperplasia Using a Large-scale Administrative Database

Objective To analyze nationwide information on the timing of surgical procedures, cost of surgery, hospital length of stay following surgery, and surgical complications of female genital restoration surgery (FGRS) in females with congenital adrenal hyperplasia (CAH). Materials and Methods We used the Pediatric Health Information System database to identify patients with CAH who underwent their initial FGRS in 2004-2014. These patients were identified by an International Classification of Diseases, Ninth Revision (ICD-9) diagnosis code for adrenogenital disorders (255.2) in addition to a vaginal ICD-9 procedure code (70.x, excluding vaginoscopy only) or perineal ICD-9 procedure code (71.x), which includes clitoral operations (71.4). Results A total of 544 (11.8%) females underwent FGRS between 2004 and 2014. Median age at initial surgery was 9.9 months (interquartile range 6.8-19.1 months). Ninety-two percent underwent a vaginal procedure, 48% underwent a clitoral procedure, and 85% underwent a perineal procedure (non-clitoral). The mean length of stay was 2.5 days (standard deviation 2.5 days). The mean cost of care was $12,258 (median$9,558). Thirty-day readmission rate was 13.8%. Two percent underwent reoperation before discharge, and 1 (0.2%) was readmitted for a reoperation within 30 days. Four percent had a perioperative surgical complication. Conclusion Overall, 12% of girls with CAH underwent FGRS at one of a national collaborative of freestanding children's hospitals. The majority underwent a vaginoplasty as a part of their initial FGRS for CAH. Clitoroplasty was performed on less than half the patients. Overall, FGRS for CAH is performed at a median age of 10 months and has low 30-day complication and immediate reoperation rates

Long-term outcomes of catheterizable continent urinary channels: what do you use, where you put it and does it matter?

Introduction Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are commonly used catheterizable channels with similar outcomes on short-term follow-up. Their relative long-term results have not been previously published. Objective Our goal was to assess long-term durability of APV and Monti channels in a large patient cohort. Study design In this retrospective cohort study, we retrospectively reviewed consecutive patients ≤21 years old undergoing APV and Monti surgery at our institution (1990–2013). We collected data on demographics, channel type, location, continence and stomal and subfascial revisions. Kaplan–Meier survival and Cox proportional hazards analysis were used. Results Of 510 patients meeting inclusion criteria, 214 patients had an APV and 296 had a Monti (50.5% spiral Monti). Median age at surgery was 7.4 years for APV (median follow-up: 5.7 years) and 8.7 years for Monti (follow-up: 7.7 years). Stomal stenosis, overall stomal revisions and channel continence were similar for APV and Monti (p ≥ 0.26). Fourteen APVs (6.5%) had subfascial revisions compared to 49 Montis (16.6%, p = 0.001). On survival analysis, subfascial revision risk at 10 years for APV was 8.6%, Monti channels excluding spiral umbilical Monti: 15.5% and spiral umbilical Monti: 32.3% (p < 0.0001, Figure). On multivariate regression, Monti was 2.09 times more likely than APV to undergo revision (p = 0.03). The spiral Monti to the umbilicus, in particular, was 4.23 times more likely than APV to undergo revision (p < 0.001). Concomitant surgery, gender, age and surgery date were not significant predictors of subfascial revision (p ≥ 0.17). Stomal location was significant only for spiral Montis. Discussion Our study has several limitations. Although controlling for surgery date was a limited way of adjusting for changing surgical techniques, residual confounding by surgical technique is unlikely, as channel implantation technique was typically unrelated to channel type. We did not include complications managed conservatively or endoscopically. In addition, while we did not capture patients who were lost to follow-up, we attempted to control for this through survival analysis. Conclusions We demonstrate, durable long-term results with the APV and Monti techniques. The risk of channel complications continues over the channel's lifetime, with no difference in stomal complications between channels. At 10 years after initial surgery, Monti channels were twice as likely to undergo a subfascial revision (1 in 6) than APV (1 in 12). The risk is even higher in for the spiral umbilical Monti (1 in 3)

Comparison of Intraoperative and Early Postoperative Outcomes of Caudal Versus Dorsal Penile Nerve Blocks for Outpatient Penile Surgeries

Objective To compare intraoperative and 1-hour postoperative outcomes in caudal versus dorsal penile nerve block (DPNB) patients undergoing penile surgeries. Material and Methods We performed a retrospective cohort study of males 3. Secondary outcomes were intraoperative/post-anesthesia care unit (PACU) narcotics, pre-incision anesthesia time, adjusted operating room charges and complications. We performed bivariate and multivariable analyses controlling for demographic/procedure characteristics and clustering by surgeon. Results Of 738 patients, (mean age 2.1 years) 74.1% had a caudal. DPNB patients were more likely to have a maximum pain score >3 (19.5% vs. 8.1%, p 3 (95% CI 1.7- 4.4, p<0.0001) and 5.2 times the odds of intraoperative/PACU narcotic administration (95% CI 3.3-8.1, p<0.0001). In multivariable analyses, caudal patients had longer pre-incision anesthesia time (27.9 ± 7.4 vs. 19.5 ± 6.6 minutes, p<0.0001) and higher adjusted operating room charges ($12,760 ± 4077 vs.$9,402 ± 3741, p=0.01). Conclusion Caudal blocks may offer a small advantage in the immediate postoperative period although cost-effectiveness is unproven

Quality of Life Assessment in Spina Bifida for Children (QUALAS-C): Development and Validation of a Novel Health-related Quality of Life Instrument

Objective To develop and validate a self-reported health-related QUAlity of Life Assessment in Spina bifida for Children (QUALAS-C). Methods We drafted a 27-question pilot instrument using a patient-centered comprehensive item generation and refinement process. It was administered to a sample of children 8-12 years old with spina bifida (SB) recruited online via social media and in person at an outpatient SB clinic (January 2013-September 2014). Healthy controls were recruited at routine pediatrician visits. Validation and final questions were determined based on clinical relevance, high loadings on factor analysis, and domain psychometrics. Children with SB also completed the validated generic Kidscreen-27 instrument. Results Median age of 150 participants was 9.6 years (60.7% male, 72.7% Caucasian), similar to 46 controls (P ≥ .10). There were 97 online and 53 clinic participants (89.0% and 84.2% of eligible, respectively). Face and content validities of the 2-domain, 10-question QUALAS-C were established by patients, parents, and experts. Internal consistency and test-retest reliability was high for the Esteem & Independence and Bladder & Bowel domains (Cronbach's alpha: 0.72-0.76, ICC: 0.74-0.77). Correlations between QUALAS-C domains were low (r = 0.51), indicating that QUALAS-C can differentiate between two distinct health-related quality of life components. Correlations between QUALAS-C and Kidscreen-27 were also low (r ≤ 0.44). QUALAS-C scores were significantly lower in children with SB than without (P < .0001). Conclusion QUALAS-C is a short, valid health-related quality of life tool for children with SB. It will be useful in clinical and research settings