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Purpose:: While intradetrusor botulinum neurotoxin (BoNT) injection is a well-established treatment of neurogenic detrusor overactivity, its administration in individuals with complex genitourinary (GU) reconstruction can be difficult. The objective of this report is to highlight our institution’s experience with challenges and practical solutions in delivering BoNT to this unique patient population. Materials and Methods:: We performed a retrospective review of our institution’s database of adults with congenital neurogenic lower urinary tract dysfunction (NLUTD) with history of complex GU reconstruction who received intradetrusor BoNT injections performed by a single surgeon between 2016 and 2023. In patients with multiple injections, the most recent procedure was used as the index case for analysis. Results:: A total of 17 patients were included, with a mean age of 27 years (range 21–46). The most common diagnosis was myelomeningocele (n=13), followed by sacral agenesis (n=2), Williams syndrome (n=1), and urethral atresia (n=1). The most common GU reconstruction was augmentation cystoplasty (n=11). 75% (12/16) of the patients had a continent catheterizable channel (CCC) and 31% (5/16) had undergone prior bladder neck reconstruction or closure. Conclusion:: Additional considerations and instruments are required for safe and effective BoNT injections in individuals with congenital NLUTD and reconstruction. Adequate preparation by the staff and the provider may be needed. Our recommendation generally favors the utilization of a flexible cystoscope or a rigid scope with a smaller sheath size to minimize trauma to the CCC, bladder neck, or urethra. Additionally, it is advisable to consider reconstituting BoNT in higher concentrations to enable the administration of fewer injections. Furthermore, performing these procedures in the operating room under anesthesia allows for the development of a well-considered strategy prior to any attempts in the office setting We hope that this manuscript sparks discussion between pediatric urologists and adult urologist to expand our knowledge on strategies needed for delivering BoNT to individuals with complex and restricting GU surgeries.

Background: The neurogenic bladder symptom score (NBSS) has been widely used to specifically measure symptoms and consequences of neurogenic bladder (NB). The cognitive interviewing (CI) is effective in assessing item clarity and identifying key issues related to the comprehension of the instrument. We aim to translate the NBSS into Chinese and use the CI approach to explore the thought processes of patients with NB in responding the Chinese Version of the NBSS, identify and modify the factors hinder the thought processes to enhance the face validity of the NBSS. Methods: The translation of the NBSS into Chinese was conducted with the guidance of the recommended frameworks. Patients with NB were recruited by purpose sampling. CI with the combination of thinking aloud and verbal probing techniques were used to explore thought processes. The interviews were transcribed and analyzed based on Tourangeau four-stage response model. Results: Two rounds of CI were carried out. The problems of comprehension, judgement and response mapping were identified in 8 items. Four items were revised based on the results of the interview. The revised items were verified and eventually integrated into the final version. Conclusion: The Chinese Version of the NBSS was easy to comprehend and use. The use of CI methodologies can increase the comprehensibility and cultural applicability of the NBSS, providing the evidence for the development of a clearer and more appropriate questionnaire.

This is the third manuscript from the Neuro-urology Promotion Committee of the International Continence Society (ICS) discussing uncommon neuro-urological conditions that are not well described in urological literature. Readers are referred to the previous documents for a more detailed understanding of how neurological disease might affect lower urinary tract function ( https://doi.org/10.1016/j.cont.2022.100022) ( https://doi.org/10.1016/j.cont.2023.101043). Eleven conditions are covered. This includes five genetic conditions — Angelman disease, spinocerebellar ataxias, Fabry’s disease, neurofibromatosis and spinal muscular atrophy. Two infection associated conditions are covered including Human T-lymphotropic Virus Type 1 associated myelopathy/ tropical spastic paraparesis and Creutzfeldt–Jackob disease. Four other conditions covered are cerebral palsy, Lennox–Gastaut syndrome, idiopathic normal pressure hydrocephalus and spinal vascular malformation. Key aspects and specific recommendations related to clinical practice are summarized in tables. Knowledge of unusual neurological conditions that can affect the lower urinary tract is important for ensuring a timely and precise diagnosis. This document from the ICS, along with Parts I and II published earlier, can serve as a reference for clinicians presented with unusual forms of neurogenic lower urinary tract dysfunction.

Vesicostomy is a temporary solution for patients with bladder outlet challenger who are not eligible for catheterization.A 73 year-old man who has successfully managed his bladder using a vesicostomy for 59 years. Complications of prolapse and dermatitis led to vesicostomy closure and cystostomy placement at age 73.Vesicostomy can be safely used to preserve bladder and upper urinary tract health for a lifetime.

This literature review provides a comprehensive overview of the development and application of herpes simplex virus type 1 (HSV-1) vectors in gene therapy, with a specific focus on functional urology. Gene therapy is becoming more and more a clinical reality as more and more products getting market-approval. The text highlights the advantages of viral vectors, especially HSV-1, in delivering therapeutic genes due to their large cargo capacity, efficient growth in tissue culture, and tropism for neuronal cells. The neuronal system is crucial in functional urology because the intricate network of nerves or neuronal structures control and regulate the complex functions of the urinary system. In functional urology, HSV-1, vectors show promise in addressing conditions such as bladder pain syndrome, diabetic detrusor underactivity, and neurogenic detrusor overactivity. Studies demonstrate successful localized expression of therapeutic genes in animal models, indicating potential applications in treating bladder-related disorders.This review underscores the promising frontier that HSV-1 vectors represent in functional urology, acknowledging the need for further research to optimize transduction efficiency, address potential immunogenic concerns, and ensure long-term effectiveness.

Aim:: Intravesical onabotulinum toxin A (BTX) injection is a widely recognized treatment for idiopathic detrusor overactivity (DO) and neurogenic detrusor overactivity (NDO) refractory to adjustments in oral medications.The primary end point of our study was to evaluate patients’ comfort and tolerability with intravesical BTX treatment given in an office-based setting inside a hospital. As a secondary end point, for a subgroup of patients who had previously undergone the procedure in an operating room, we aimed to ascertain if there was any difference in patients’ preferences between the office and operating room settings. Methods:: A cross-sectional descriptive study was designed to evaluate patients’ comfort and tolerability with intravesical BTX treatment given in an office-based setting. It was based on a questionnaire investigating the patients’ overall experience during the procedure and subsequent recovery. Results:: A total of 74 consecutive patients treated between January 2016 and July 2022 were enrolled: 48 males and 26 females; the average age was 57.09 years, ranging from 23–84 years. Among them, 66.2% presented with NDO, and 33.8% with DO. Conclusions:: According to our study, intravesical BTX injections in an office-based setting inside a hospital appear to be a well-tolerated procedure. Most of the patients in our study who experienced both options finally preferred the office setting rather than the operating room. This is an important result, especially if we consider that most patients are required to repeat the treatment over time.

Chronic urine retention due to functional reasons is a difficult to treat condition. Low-pressure, low-flow voiding dysfunction also called as neurogenic bladder is a common functional reason of chronic urinary retention. Conventional pharmacotherapy has not been promising in such conditions and the symptomatic management is done through regular catheterisation. Catheterisation due to its social and medical limitations has a high discontinuation rate even though if it is recommended. In this scenario, any possibility of alternative interventions leading to the clinical improvements without catheterisation comes with hope as a lead to the future medicine. We present here case of a young woman suffering with chronic urinary retention due to neurogenic bladder, previously on regular intermittent catheterisation for few months and subsequently treated through Ayurveda interventions aiming to improve bladder contractility to the extent of complete recovery raises a high hope for treating such cases if such observations are being brought to the serious scientific enquiry and are translated into regular treatment strategy for similar clinical conditions.

This is the second manuscript from the Neuro-urology Promotion Committee of the International Continence Society (ICS) discussing uncommon neuro-urological conditions that are not well described in urological literature. Readers are referred to the first document for a more detailed understanding of how neurological disease might affect lower urinary tract function ( https://doi.org/10.1016/j.cont.2022.100022).Eleven conditions are covered. This includes five genetic conditions — Duchene muscular dystrophy, mitochondrial encephalopathy, Down’s syndrome, adrenoleukodystrophy and Ochoa’s syndrome. Three infection-associated conditions are covered including central nervous system tuberculosis, schistosomal myeloradiculopathy and post-polio syndrome. Three other conditions covered are myasthenia gravis, syringomyelia and the vegetative state. Key aspects and specific recommendations related to clinical practice are summarized in tables.Knowledge of unusual neurological conditions that can affect the lower urinary tract is important for ensuring a timely and precise diagnosis. This document from the ICS, along with Part I published earlier, can serve as a reference for clinicians presented with unusual forms of neurogenic lower urinary tract dysfunction.

Objectives: This study aimed to determine patients’ perceived benefits of a WeChat applet for self-management of patients with neurogenic bladder (NGB) and identify the key factors hindering their adoption. Methods: In the qualitative study, 19 NGB patients were invited for semi-structured interviews. They were hospitalized in the rehabilitation departments of two tertiary hospitals in Shenzhen and tried out the self-management applet for two weeks. Data were analyzed using the content analysis method. Results: The results indicated that the WeChat applet of self-management was helpful and embraced by the NGB patients. Three perceived benefits were identified 1) being accessible, flexible, and intuitive to users, 2) driving bladder self-management, and 3) directing the way for care partners and family members. Challenges hindering the adoption of the applet included 1) negative attitudes of patients towards bladder self-management and patient characteristics, 2) concerns about the risks of mHealth, and 3) the necessity of applet upgrading. Conclusion: This study showed feasibility of the WeChat applet for self-management among NGB patients to meet their needs for access to information during hospitalization and after discharge. The study also identified facilitators and barriers to patient use, providing valuable information for healthcare providers to implement mHealth interventions to promote self-management among NGB patients.

Objective: This study examines the outcome of augmentation cystoplasty (AC) in children with stages III and IV chronic kidney disease (CKD) secondary to neurogenic bladder in which transplantation was not imminent. Methods: Hospital records of all children with CKD stages III and IV who underwent AC between 2008 and 2017 were retrieved to study outcome and complications including estimated glomerular filtration rate (eGFR, National Kidney Foundation Calculator), somatic growth (percentiles, compared with population data), and febrile urinary tract infections (fUTIs) requiring admission. Statistical analysis was performed using R. Results: AC was performed in 13 children with CKD stages III and IV (10 girls; median 8.0 years) with median follow-up of 51 months. Patients had incontinence (10/13), reflux (7/13), and hydronephrosis (13/13) despite antimuscarinics and intermittent catheterization. Bladder capacity was 74% of expected and median compliance was 5 mL/cm H20 (inter-quartile range 4 mL/cm H20). All underwent ileocystoplasty (25 cm bowel). One each had nephrectomy and mitrofanoff conduit. All had resolution of incontinence. One had acute kidney injury that recovered. Initial eGFR at presentation (24 mL/min/1.73 m2) improved with conservative management alone (52 mL/min/1.73 m2, p=0.004). This improved further 1 year following AC (61 mL/min/1.73 m2, p=0.036) with stable function at 7 years. There was improvement in somatic growth, hydronephrosis, and fUTI despite no ureteric re-implantation. Conclusion: AC carries acceptable morbidity in children with CKD stages III and IV secondary to neurogenic bladder. Surgery is effective with improvements in continence, eGFR, somatic growth, and propensity for fUTIs. Ureteric re-implantation might not be necessary.

Introduction: The National Spina Bifida Patient Registry (NSBPR) assesses bladder and bowel incontinence using ordinal categories, but prior NSBPR analyses employed binary classification. Our aims were to 1) perform the first NSBPR analysis of bladder and bowel incontinence as ordinal outcomes to compare to the binary definition and subject variables; 2) explore the correlation of incontinence with undergarment usage, and 3) assess incontinence status following continence surgeries., Methods: Data from NSBPR participants' most recent clinic visit from 2013 to 2020 were analyzed. Ordinal categories of incontinence were compared to previously used binary definitions. Incontinence surgical outcomes were analyzed for those with data at least three months post-operatively. Chi-square tests evaluated associations among categorical variables. Univariate and ordinal logistic regression models were used to test associations of ordinal incontinence status with patient and condition factors. Statistical tests were 2-sided; p values < 0.05 were considered significant., Results: Analysis of 7217 individuals using ordinal incontinence outcomes showed little difference from previously used binary outcomes. The final multivariable logistic regression models with ordinal multinomial outcomes showed that associations of incontinence with age, sex, race/ethnicity, health insurance, level of lesion, and continence management technique were similar to prior studies. Among those reporting never being incontinent of both bladder and bowel, 14% reported using protective undergarments. Of the 500 individuals who had bladder outlet surgery, 38% reported never being incontinent of urine. Of 1416 individuals who had appendicostomy (ACE) bowel surgery, 48% reported never being incontinent of stool., Discussion: Our current analysis showed that ordinal continence outcome classification had similar continence findings as previous studies using the binary definition of continence. Expanding the binary definition of continence to include monthly episodes of incontinence did not greatly increase the proportion of continent individuals and, therefore, would have not likely made meaningful differences in continence outcomes in prior NSBPR analyses. However, it is known that even mild incontinence can affect quality of life, therefore, capturing any level of incontiennce is of clinical importance. Confirmation of the association of continence outcomes with sociodemographic, condition-related, and interventional factors with both approaches further validates previous analyses using the binary definition of continence., Conclusion: The previously used binary definition of bladder and bowel continence appears robust. Undergarment choice was a poor surrogate for reported incontinence. After bladder and bowel continence surgeries, 38% and 48%, respectively, reported never being incontinent., Competing Interests: Conflict of interest MSK serves as advisor for Coloplast A/C, Humlebaek, Denmark., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Segmental spinal dysgenesis is a rare and complex congenital condition affecting the dorso-lumbar spine, characterized by focal spinal cord dysgenesis and kypho-scoliotic deformity. It arises due to notochord malformation during embryogenesis. The case in question involves a 2-year-old female child. She presented to the outpatient department of our hospital with a history of inability to walk and increased frequency of micturition. The patient's mother had no antenatal visits. Upon examination, the patient was found to have a scoliotic deformity. Magnetic resonance imaging (MRI) of the spine revealed an absence of the spinal cord and spinal nerves from the T5 to L2 levels. A relatively thick spinal cord was visible from the L2 to L4 level. There was a complete absence of the spinal canal at the D10 and D11 levels, along with dorsal levoscoliosis. Segmental anomalies of the vertebrae were also noted in the dorsal spine. Additionally, imaging showed features of neurogenic bladder and mild left hydroureteronephrosis. The child underwent rehabilitation and surgical correction of the scoliosis., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Introduction: Ureteroscopy (URS) for urolithiasis in pediatric patients may be particularly challenging for patients with co-morbidities that increase the risk for stone formation and recurrence. Patients with neurogenic bladders (NGB) and/or patients that are non-ambulatory are reported to have higher rates of additional comorbidities and a particularly increased risk of developing urolithiasis, and higher rates of infections and post-operative complications., Objective: To report outcomes of URS for stone disease in pediatric patients with NGB and/or non-ambulatory status and compare these outcomes to patients without these co-morbidities., Methods: An IRB-approved prospective single institutional registry was used to retrospectively identify all patients under 18 years of age who underwent URS for stone disease between July 2012 and July 2021, excluding bladder stones. Patients were categorized in two groups: patient with NGB with or without non-ambulatory status versus all other patients (control). Baseline demographics, pre-operative radiologic imaging, intra-operative details, and post-operative outcomes including 30-day complications were aggregated and compared between the two groups., Results: 275 URS in 198 patients were performed during the study period, and 49 (18 %) of these were performed on patients with NGB. Pre-operative imaging showed significantly higher number of stones (3 vs 2, p = 0.003) and larger total stone burden in patients with NGB than those without NGB (15 mm vs 9 mm, p = 0.009). Patients with NGB had a significantly longer length of procedure (86 vs 60 min, p = 0.002), increased need for staged procedures, increased length of stay (1 vs 0 days, p < 0.001), and increased use of an extended duration of antibiotics prior to the procedure (<0.001). There was no difference in need for passive dilation, stent placement, or other intra-operative parameters. There was no statistical difference in the incidence of 30-day complications between the two groups post-operatively. However, there was a higher incidence of febrile UTIs (8.2 % vs 1.3 %, p = 0.021) in patients with NGB and a lower incidence of pain related complications (0 % vs 9.3 %, p = 0.032). Patients with NGB had a higher incidence of requiring ipsilateral URS for recurrent stone disease within a year of surgery (34.6 % vs 18.9 %, p = 0.01)., Conclusion: The results show that URS for urolithiasis can be done safely and effectively in pediatric patients with neurogenic bladders. The increased risk of infectious complications within 30 days of surgery warrants careful pre- and post-operative antibiotic care plan for this patient population., Competing Interests: Conflict of interest The authors declare that they have no known competing interests that could have appeared to influence the work reported in this article., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Surgical management for neurogenic bladder requires complex decision-making by physicians, patients, and caregivers. Assessing decisional regret (DR) as a patient-reported outcome among caregivers could inform future counseling and shared decision-making., Objective: To assess DR among caregivers of children with neurogenic bladder following reconstructive bladder surgery., Study Design: A questionnaire including a validated DR survey was distributed to English-speaking caregivers of children with neurogenic bladder who had undergone reconstructive bladder surgery at Children's Hospital Colorado. DR scores range from zero to 100, with higher numbers indicating higher regret. Wilcoxon rank sum test and Spearman correlation were performed to assess differences in DR scores by patient demographic factors or disease factors., Results: Forty-five of 210 English-speaking caregivers completed the DR survey. The median DR score was 5, with 40% of subjects reporting with a DR score of zero and 24% of subjects with a DR score of 30 or higher. Patient sex and Mitrofanoff leakage were found to be associated with DR, with caregivers of male patients reporting significantly higher DR. Surgical procedure did not have a statistically significant impact on DR scores., Discussion: Sparse existing data exploring DR among patients with neurogenic bladder suggest DR following reconstructive bladder surgery is low, with few identifiable predictors of regret. While the majority of caregivers in our study report little or no DR, one quarter of caregivers report moderate to high DR. The limitations of this study include small cohort size, low response rate, exclusion of non-English speaking patients, and the potential for recall bias due to the survey design of the study., Conclusion: Caregiver DR following bladder reconstruction in children with neurogenic bladder is generally low, however a subset of caregivers reports significant DR. This study suggests that caregivers of male children may have higher DR, a finding that merits further investigation., Competing Interests: Conflict of interest The authors have no competing interests to declare., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Bladder augmentation with catheterizable channel creation is indicated for patients with neurogenic bladder (poorly complaint/high pressure) to preserve kidney function and provide long-term social continence when conservative medical management has failed. An open surgical approach is currently the gold standard. Methods: A 10-year-old-male with a history of neurogenic bladder due to sacral agenesis and caudal regression syndrome (wheelchair bound), solitary left kidney with worsening vesicoureteral reflux (grade 3), and continued daytime and nighttime incontinence underwent an open bladder augmentation ileocystoplasty, with creation of an appendicovesicostomy catheterizable channel, and bladder neck reconstruction. Results: Total operative time was 230 min. EBL was less than 50 cc. Patient was discharged on post-operative day 9. At the 6 month post-operative visit, patient is selfcatheterizing the APV channel every 2-3 h with daytime outputs of 300 mL-500 mL and nighttime output between 200 mL-300 mL. Renal ultrasound shows improved grade 3 hydronephrosis, with improved renal pelvis dilation and more robust appearing renal parenchyma. Conclusion: This video demonstrates the steps of an open bladder augmentation ileocystoplasty, with creation of an appendicovesicostomy catheterizable channel, and bladder neck reconstruction.

The management of patients with neurogenic lower urinary tract dysfunction has been well-described, however this is most frequently discussed for common conditions such as spinal cord injury or multiple sclerosis. Our objective was to review uncommon neurologic disorders and summarize both the underlying disease process, and the relevant disease-specific research on the impact of the neurologic condition on the lower urinary tract. Among the degenerative and traumatic brain disorders, we have included frontotemporal dementia, amyotrophic lateral sclerosis, Huntington’s Disease, progressive supranuclear palsy, corticobasal degeneration, multiple system atrophy, and traumatic brain injury. Among the autoimmune disorders, we reviewed transverse myelitis, neuromyelitis optica spectrum disorders, Myelin oligodendrocyte glycoprotein antibody-associated disease, glial fibrillary acidic protein astrocytopathy, and meningitis-retention syndrome (a form of aseptic meningitis that presents with urinary retention). Hereditary spastic paraplegia, VACTERL association, and several peripheral neuropathies (Guillain Barre syndrome, chronic inflammatory demyelinating polyneuropathy, autoimmune autonomic gangliopathy, Wolfram syndrome spectrum disorder (a progressive peripheral neuropathy disorder with early onset diabetes, optic atrophy and megacystis in the early stage), Charcot Marie Tooth disease, and amyloid neuropathy are included. Practice points specific to the disorders are included where appropriate.

Objective:: To use existing clinical trial data to assess the impact of prophylactic antibiotics on the 1-year UTI rate among people with different neurologic diseases, and to determine if UTIs impact renal function. Methods:: We conducted a secondary analysis of community dwelling participants with a neurologic disease and intermittent catheter use who participated in a 12-month randomized trial (AnTIC) of low dose antibiotic prophylaxis. We calculated incident rate ratios (IRR) of symptomatic UTIs that required antibiotics. Renal function was assessed using the estimated glomerular filtration rate. Results:: We identified 138 patients who had a neurologic disease (multiple sclerosis (25%), spinal cord injury (21%), spina bifida (18%), and other disorders (36%)). The incidence of symptomatic, antibiotic treated urinary infections was 1.48 per person–year in the prophylaxis group, and 2.51 per person–year in the usual care group; the IRR was 0.59 (95% CI 0.46, 0.76) in favor of continuous antibiotic prophylaxis. The IRR was lowest (most protective) among those with spinal cord injury (IRR 0.23, p

Streptococcus salivarius (S. salivarius) is an oral commensal bacterium that rarely causes disease. Here, we report a case of emphysematous cystitis due to S. salivarius infection in a patient with a neurogenic bladder. A 56-year-old woman was hospitalized and managed for left putamen hemorrhage. Afterward, she developed poor oral intake. Although the patient was afebrile, laboratory test results suggested an inflammatory response. Urinalysis revealed pyuria and hematuria. Abdominal computed tomography revealed a thickened urinary bladder wall and intraluminal gas. Additionally, she was diagnosed with a neurogenic bladder as she had approximately 200 mL of residual urine. The patient was diagnosed with emphysematous cystitis, and S. salivarius was isolated from urine culture specimens. The patient’s condition improved immediately after treatment, which included bladder drainage and administration of appropriate antibiotics. We could not find any report on S. salivarius causing urinary tract infections, such as emphysematous cystitis. Accordingly, we report this case along with a review of the literature.

Suprapubic catheter insertion is frequently indicated for patients with a neurogenic bladder. It's an effective and safe way to drain the bladder and can be managed in a community setting by a trained health professional. Here we report a rare case of a 73-year-old male-presenting with a two-day history of frank haematuria secondary to a recent suprapubic catheter change. Imaging confirmed an obstruction of the right ureteric orifice due to migration of the catheter into the ureter. Once the catheter was withdrawn into the correct location his haematuria and acute renal injury resolved and was discharged the following day.

Introduction: Children and adolescents with neurogenic bladder often need clean intermittent catheterization (CIC) over a long period. Our study aimed to identify factors that affect CIC compliance and to determine if CIC compliance affected short-term urological outcomes among patients in Malaysia., Study Design: 50 patients aged 2-18 years who perform CIC were included in this cohort study. Patient compliance with CIC was evaluated using the validated Intermittent Catheterization Adherence Scale (ICAS). CIC difficulties were assessed using the validated Intermittent Catheterization Difficulty Questionnaire (ICDQ). Data was obtained on patients' co-morbidity, caregiver factors, socio-economic factors, CIC technique, access to catheters and facilities, urinary tract infections, incontinence, urology tests and treatment. Statistical analysis was performed., Results: Mean age was 6.68 ± 4.34 years. 32 (64%) patients commenced CIC within the first month of life. Mean daily CIC frequency was 4.70 ± 1.33.30 (60%) participants showed strong adherence to CIC. 39 (78%) participants were able to catheterize with no or minor difficulties. Pain (6, 12%), transient blocking sensation (6.12%), and urinary incontinence (3, 6%) were the predominant difficulties encountered. CIC performed by caregiver was associated with improved adherence compared to patient self-catheterization (p = 0.039). The mean age of participants who self-catheterized was 10.7 ± 3.7 years. Strong adherence was also observed among patients who purchased their own CIC catheters (p = 0.007). Participants with lower ICDQ score were more likely to be compliant with CIC (p = 0.007). CIC adherence was not affected by patient's age, gender, co-morbidity, mobility, caregiver factors, socio-economic factors, and age at initiation of CIC. There was no significant association between CIC adherence and febrile urinary tract infections, upper tract deterioration, and bladder stones at 6 months follow-up., Discussion: There is lower CIC adherence when a child begins to self-catheterize and healthcare providers should be alert during this period of transition. Though most patients with spina bifida have decreased urethral sensation, some patients do experience significant pain during CIC which may impact their compliance. These patients would need a review of their catheterization techniques to improve adherence. The limitations of our study are its modest sample size from a single center and short study period. Our study provides insights into the feasibility of instituting CIC in developing countries., Conclusion: Strong CIC adherence was observed among patients who were catheterized by their caregiver, purchased their own CIC catheters, and encountered minimal difficulties during catheterization. CIC adherence had no effect on short-term urological outcomes., Competing Interests: Conflict of interest All authors declare that no competing interests exist., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: This review aims to assess the efficacy of transcutaneous electrical nerve stimulation (TENS) for neurogenic bladder after spinal cord injury (SCI)., Materials and Methods: A systematic search was conducted of seven electronic data bases from inception to Dec 31, 2022, to identify randomized controlled trials that studied TENS for neurogenic bladder after SCI. The primary outcomes were maximum cystometric capacity (MCC) and residual urine volume (RUV). Secondary outcomes included maximum detrusor pressure, flow rate, and bladder diary. Random effects models were used in all analyses., Results: Eleven trials involving 881 participants were included. Meta-analysis showed that TENS in addition to conventional treatment had larger MCC (mean difference [MD] 50.55 ml, 95% CI 27.81-73.29, p＜0.0001) and lower RUV (MD -22.96 ml, 95% CI -33.45 to -12.47, p＜0.0001) than did conventional treatment only. Compared with magnetic stimulation, no differences were observed with TENS for MCC (MD -14.49 ml, 95% CI -48.97 to 19.98, p = 0.41) and RUV (MD 25 ml, 95% CI -61.79 to 111.79, p = 0.57). There also were no differences in MCC (MD -7.2 ml, 95% CI -14.56 to 0.16, p= 0.06) and (MD -5.2 ml, 95% CI -60.00 to 49.60, p = 0.851) when compared with solifenacin succinate and pelvic floor biofeedback, respectively., Conclusions: TENS may be an effective treatment option for neurogenic bladder after SCI., Competing Interests: Conflict of Interest The authors reported no conflict of interest., (Copyright © 2023 International Neuromodulation Society. Published by Elsevier Inc. All rights reserved.)

Introduction: Intra-detrusor botulinum toxin (Botox) injection is a minimally invasive alternative to augmentation cystoplasty in patients with refractory neurogenic bladder. Botox was first used for neurogenic bladder children two decades ago. However, there are no existing guidelines on indications or use among patients with spina bifida. Furthermore, there are little data regarding its use relative to bladder augmentation and patient volume on a national scale., Objective: We sought to investigate the contemporary trends of intra-detrusor Botox injection and augment cystoplasty in free-standing children's hospitals., Study Design: We queried the Pediatric Health Information System database to identify spina bifida patients from 2016 to 2019 who underwent intra-detrusor Botox injection and augment cystoplasty based on CPT and ICD-10 codes. Total spina bifida population under care in the free-standing children's hospitals was estimated by all inpatient and ambulatory surgery encounters as denominators to calculate frequency by time for both intra-detrusor Botox injections and augmentation cystoplasty., Results: In total, we included 1924 intra-detrusor Botox injections and 842 augmentation cystoplasties. 1413 (51.1%) patients were female. Median age at surgery was 10.0 (interquartile range 6.98-13.5) years. There was a significant increase in intra-detrusor Botox injection frequency (p < 0.001). While there was an overall decreasing, but not significant, trend for augmentation cystoplasty, there was a significant increase in this procedure during the summer months compared to the rest of the year (p < 0.001, Figure 1). Sensitivity analysis using only first intra-detrusor Botox injection per patient demonstrated similarly significant increasing trend., Discussion: Use of intra-detrusor Botox injection for the management of neurogenic bladder has significantly increased among patients with spina bifida while augmentation cystoplasty has slightly decreased, but not significantly., Conclusions: Over time, practice patterns for the treatments of neurogenic bladder among spina bifida children have favored minimally invasive Botox injections while augmentation cystoplasty use has not significantly changed., Competing Interests: Conflicts of interest No authors have any conflicts of interest to disclose., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Dr. Ochoa proposed a condition known as Urofacial syndrome (UFS) which is characterized by the abnormal facial expression while smiling along with dysfunctional lower urinary tract in 1987. This study investigated 7 years old boy who presented with recurrent urine retention, dysuria, and inversion facial expression. Radiological and genetic work-up diagnosed him with UFS.

A 2-month-old full-term female presented with a large anterior sacral meningomyelocele resulting in transient obstructive uropathy with bilateral hydronephrosis and acute kidney injury. After initial bladder decompression and surgical resection of the meningomyelocele, there was spontaneous resolution of bladder function confirmed with urodynamics. Anterior spinal meningomyelocele (ASM) is a rare neural tube defect that may present with urinary dysfunction secondary to compression of the bladder and sacral nerve roots or congenital defects to the bladder nervous supply. Obstructive uropathy due to ASM may spontaneously resolve after surgical resection.

Spinal cord injury often results in neurogenic bladder condition and eventually lead to an end-stage renal disease requiring kidney transplantation. However, transplantation in abnormal bladder carries special considerations. We report a case of an adult male with end-stage chronic kidney disease and small bladder capacity after having spinal cord injury. The evaluation of videourodynamic showed reduced compliance and detrusor overactivity during filling phase. Kidney transplantation and vesicostomy was performed. Eighteen months follow-up after surgery showed that kidney function could be maintained. The prevention of increasing bladder pressure and UTI should be monitored to prevent the damage of the graft kidney.

Schistosomose mansoni is an endemic disease in Brazil. It rarely affects the central nervous system, particularly in children. We report the case of a child that presented with an acute spinal cord compression condition, resulting from a mass that proved to be a granuloma caused by a Schistosoma mansoni infection. Proper treatment had no effect on the regression of urological symptoms and the child had to undergo a Mitrofanoff principle.

Spinal cord injury (SCI) is a devastating injury to the central nervous system in which 60 to 80% of patients experience chronic pain. Unfortunately, this pain is notoriously difficult to treat, with few effective options currently available. Patients are also commonly faced with various compounding injuries and medical challenges, often requiring frequent hospitalization and antibiotic treatment. Change in the gut microbiome from the “normal” state to one of imbalance, referred to as gut dysbiosis, has been found in both patients and rodent models following SCI. Similarities exist in the bacterial changes observed after SCI and other diseases with chronic pain as an outcome. These changes cause a shift in the regulation of inflammation, causing immune cell activation and secretion of inflammatory mediators that likely contribute to the generation/maintenance of SCI pain. Therefore, correcting gut dysbiosis may be used as a tool towards providing patients with effective pain management and improved quality of life.

Introduction/background: Enhanced Recovery After Surgery (ERAS) is a fundamental shift in perioperative care that has consistently demonstrated an improved outcome for a wide variety of surgeries in adults but has only limited evidence in the pediatric population., Objective: We aimed to assess the success with and barriers to implementation of ERAS in a prospective, multi-center study on patients undergoing complex lower urinary tract reconstruction., Study Design: Centers were directed to implement an ERAS protocol using a multidisciplinary team and quality improvement methodologies. Providers completed pre- and post-pilot surveys. An audit committee met after enrolling the first 5 patients at each center. Pilot-phase outcomes included enrollment of ≥2 patients in the first 6 months of enrollment, completion of 90 days of follow-up, identification of barriers to implementation, and protocol adherence., Results: A total of 40 patients were enrolled across 8 centers. The median age at surgery was 10.3 years (IQR 6.4-12.5). Sixty five percent had a diagnosis of myelomeningocele, and 33 % had a ventriculoperitoneal shunt. A bladder augmentation was performed in 70 %, Mitrofanoff appendicovesicostomy in 52 %, Monti ileovesicostomy in 15 %, and antegrade continence enema channel in 38 %. The most commonly perceived barriers to implementation on the pre-pilot survey were "difficulty initiating and maintaining compliance with care pathway" in 51 % followed by a "lack of time, money, or clinical resources" in 36 %. The pre-pilot study experience, implementation, and pilot-phase outcomes are provided in the Table. All primary and secondary outcomes were achieved., Discussion: The findings of the present study were similar to several small comparative studies with regard to the importance of a multidisciplinary team, strong leadership, and continuous audit for successful implementation of ERAS. Similar barriers were also encountered to other studies, which primarily related to a lack of administrative support, leadership, and buy-in from other services. The limitations of the present study included a relatively small heterogeneous cohort and absence of a comparative group, which will be addressed in the larger exploratory phase of the trial. The findings may also not be generaziable due to the need for sustainable processes that were unique to each center as well as an absence of adequate volume or resources at smaller centers., Conclusions: ERAS was successfully implemented for complex lower urinary tract reconstruction across 8 centers through a multidisciplinary team, structured approach based on the local context, and focus on a continuous audit., Competing Interests: Conflicts of interest None., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Neurogenic bladder was first confirmed as a urological sequela of Congenital Zika Syndrome (CZS) in 2018. Further clinical-epidemiological evidence also confirmed neurogenic bowel dysfunction and cryptorchidism. To strengthen the care for these children, the Congenital Zika Virus Bladder and Bowel Sequelae Network (RASZ in Brazilian) was created, including six integrated centers in Brazil. This article represents the initial outcome of the efforts by RASZ., Objective: To evaluate the prevalence of bladder and bowel dysfunction, cryptorchidism and other urological sequelae related to CZS in cohorts attended in six Brazilian states., Study Design: Observational, prospective, multicenter study including children with CZS assisted in one of six RASZ collaborative centers between June 2016 and February 2023. Data were collected from patient's first assessment using the same protocols for urological and bowel evaluation. Categorical variables were analyzed by frequency of occurrence and numerical variables by mean, median, and standard deviation. The study was approved by the Research Ethics Committees of each center, all parents/caregivers provided written informed consent., Results: The study included 414 children aged 2 months to 7 years (mean 2.77 years, SD 1.73), 227 (54.8 %) were male and 140 (33,8 %) referred urological and bowel symptoms on arrival. Prevalence of both urological and bowel sequelae was 66.7 %, 51 % of children aged 4 years and older had urinary incontinence (UI). UTI was confirmed in 23.4 % (two presented toxemia) and among males, 18.1 % had cryptorchidism. Renal ultrasonography, performed in 186 children, was abnormal in 25 (13.4 %), 7 had hydronephrosis. Among the 287 children who performed urodynamics, 283 (98.6 %) were altered: 232 had a lower bladder capacity, 144 a maximum bladder pressure of ≥40 cm H2O, and 127 did not satisfactorily empty their bladder., Discussion: A higher prevalence of NLUTD, neurogenic bowel and cryptorchidism was confirmed in children with CZS. Early diagnosis and appropriate treatment, including a multidisciplinary approach, may reduce the risk of UTIs, UI and kidney damage. A limitation of the study was the inability of children to complete the protocol, specifically urodynamic evaluation, and ultrasonography. In both exams, the percentage of abnormal cases was higher than that expected in the normal population., Conclusion: A 66,7 % prevalence of combined urological sequelae and bladder-bowel dysfunction related to CZS was confirmed in patients evaluated in six Brazilian cohorts. The most frequent changes were related to NLUTD, neurogenic bowel, and cryptorchidism. Prevalence may be underestimated due to access restrictions to diagnostic tests., Competing Interests: Conflict of interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Neurological defects in children with cerebral palsy (CP) not only affect their motor skills but also lead to bladder and bowel problems. Although most children with CP have achieved urinary control, more than 50% of cases experience lower urinary tract symptoms (LUTS). Common LUTS complaints observed in CP include delayed toilet training, urinary incontinence, increased frequency of urination, urgency, urinary hesitancy, and recurrent urinary tract infections., Objective: This study aimed to prospectively evaluate and compare the effectiveness of two different physiotherapy approaches, sacral Transcutaneous Electrical Nerve Stimulation (TENS) and massage, on lower urinary tract dysfunction in children with CP., Method: A total of 54 children with CP who had the Dysfunctional Voiding Scoring System (DVISS) of 8.5 or higher were included in the study. Children were randomized to the TENS (TG; n = 27) and Manual Therapy (MG; n = 27) groups. TENS application was performed 2 sessions in a week for 20 min for a total of 12 weeks. The electrodes used during the application were adhered bilaterally to the parasacral region (S2-S4). 4 electrodes of 5 × 5 cm were used. Classical bowel massage was applied to the MG with the friction massage technique twice a week for 12 weeks. Manual therapy applications were performed in the form of abdominal, colon, and friction massage, twice a week for 20 min by the physiotherapist. Massage was applied to the abdominal region between the lower subcostal border and the anterior superior iliac spine. Questionnaires were applied before and after treatment interventions: DVISS, functional bladder capacity (FBC), frequency of voiding, and urinary incontinence episodes evaluated by bladder diary, Bristol Gaita Scale, and Pediatric Incontinence Quality of Life Scale (PIN-Q) used., Results: The decrease in the episodes of incontinence was higher in the TG (p = 0.037; p < 0.05). FBC increased after treatment in both groups, but there was no statistically significant difference between the groups (p = 0.683; p > 0.05). Manual therapy was more effective in improving constipation symptoms. In both groups, DVISS and PIN-Q values decreased after treatment, but the decrease in TG was statistically significant in the evaluation made between groups (p = 0.001; p < 0.01)., Conclusion: Both parasacral TENS and massage provided a significant improvement in LUTS, constipation, and quality of life but TENS showed a bigger improvement. We suggest adding these interventions to the treatment of bladder and bowel problems in CP children., Competing Interests: Conflict of interest None., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Incontinent pediatric neurogenic bladder (NB) patients face social ostracization and potential renal deterioration. Reconstructive surgery, after maximal medical therapy, requires a difficult decision-making process. Current literature for NB surgeries is difficult to interpret given definitions of dryness, use of augmentation cystoplasty (AC) and the lack of renal preservation. This study assesses the results of a defined surgical protocol to treat incontinent NB patients, using a new composite outcome measure, which includes upper tracts status and a definition of dryness., Materials and Methods: This is a retrospective cohort study assessing 33 consecutive incontinent NB patients (Spina bifida 31, Sacral agenesis- 2) who underwent one of 2 procedures between 2008 and 2021. AC with a Mitrofanoff procedure (MP) was performed in patients who had a high detrusor leak point pressure (DLPP) and significant bladder trabeculations (N = 21, Group 1). Children with a low DLPP and non-trabeculated bladders, underwent a modified Young-Dees-Leadbetter/Mitchell procedure with a 360° autologous rectus fascial sling (BOP) with concomitant AC and MP (N-12, Group 2). Post-operative success was defined using a composite grading of success assessing dryness, upper tract stability and medication use., Results: The mean age at surgery was 11.6 years (SD = 6 years), with 21 in Group 1 and 12 in Group 2. Mean follow-up was 3.25 years, with a minimum 24-month follow-up period. Success rate was 90% in Group 1 and 66% in Group 2. No patient had upper tract deterioration following surgery. Redo-surgical intervention, was required in 38% of Group 1 and 50% of Group 2 patients. These include 3 bladder neck injections in Group 1 and 2 bladder neck closure in Group 2, with a final success rate to 95 % in Group 1 and 83 % in Group 2., Discussion: Achieving dryness and preserving upper tracts is a challenge in incontinent NB patients. Dryness rates achieved in this study is comparable, given complications and redo-surgery. Primary bladder neck closure is a radical intervention, but Group 2 patients, may benefit from an upfront discussion of the pros and cons of a bladder neck closure primarily or as a secondary procedure., Conclusions: Isolated AC obtains acceptable results for a selected subset of incontinent NB patients with significant bladder trabeculation. For those requiring a BOP, the success rate is relatively lower with the higher rate of potential complications and need for redo-surgery., Competing Interests: Conflict of interest None., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Early B-cell factor 3 (EBF3) is a transcription factor involved in neuronal differentiation and maturation. Pathogenic variants are associated with hypotonia, ataxia, and delayed development syndrome (HADDS) (MIM#617330). Urologic manifestations are common and may have implications regarding long term renal function., Objective: To review all known patients with pathogenic variants of the EBF3 gene resulting in HADDS with urologic manifestations. We hypothesize a high rate of bladder dysfunction secondary to the EBF3 variant's impact on relaxation of the urinary sphincter leading to detrusor sphincter dyssynergia (DSD)., Methods: The PubMed database was queried for publications of the EBF3 mutation between January 2017 and January 2023. Search terms were "EBF3 mutation OR HADDS AND urology OR phenotype". Retrospective analysis of HADDS patients cared for in our institution was performed. Demographic and clinical information was collected., Results: We identified 52 patients (33F:19M) through literature (28F:18M) and retrospective review (5F:1M). There was a high prevalence of genitourinary physical exam abnormalities, history of urinary tract infection, vesicoureteral reflux (VUR), and diagnosis of neurogenic bladder. Within the literature review cohort, 67% had a urologic diagnosis. Females were disproportionately affected with urologic manifestations. In our cohort, four of six children were diagnosed with VUR and severe voiding dysfunction consistent with neurogenic bladder (67%). These children were managed with a vesicostomy. Five children had bowel dysfunction requiring therapy. Urodynamics suggested a high prevalence of external sphincter dyssynergia. Less severe forms of DSD were felt to be implicated in the abnormal voiding parameters in children who presented later in life based on non-invasive flow studies., Discussion: There is significant variability in the phenotypic presentation of patients with HADDS. While EBF3 plays a clear role in neurodevelopment, it also impacts muscle development and may impact muscle relaxation. The location of the genetic variant may impact the degree of DSD, with more severe forms leading to earlier presentations. Initial work-up should include a renal ultrasound (RUS) and post void residual (PVR). Consideration can be given to obtaining a VCUG, DMSA scan or urodynamic studies. Yearly screening should be pursued with an RUS and PVR in those with an initial unremarkable work-up given the variable timing and severity of presentation., Conclusion: Urologic manifestations of HADDS include high rates of bladder dysfunction secondary to DSD, vesicoureteral reflux, urinary tract infection, and cryptorchidism. These patients are at risk of renal deterioration if urinary abnormalities are not properly diagnosed and managed., Competing Interests: Conflict of interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: To report our experience and results in terms of complications, reoperation rate and urinary continence with the Salvage Continent Vesicostomy (SCV) technique in pediatric patients with history or need of enterocystoplasty and absent appendix., Methods: Retrospective review of all patients with a history of a continent catheterizable channel surgery performed in our institution between June 2016 and January 2022. Only patients with a SCV surgery with a minimum 6-month post operative follow up were included in this cohort and divided in group 1 (history of previous bladder augmentation) and group 2 (simultaneous bladder augmentation and SCV). Primary outcome of the study was to assess both continence and postoperative complication rates. Early complications were assessed using the Clavien-Dindo classification (I-V). Late complications were focused on the need of further subfascial revision., Results: 84 patients with a history of a continent stoma creation surgery were identified. In 20 of them (12 males) a SCV was performed. The mean age at surgery was 10.38 (range 4.87-15.6) years and the median postoperative follow-up time was 32 (range 6-64) months. Eleven patients were included in Group 1, while 9 patients in Group 2. Early complications occurred in 4 patients (20%), two of them required a re-intervention (Clavien-Dindo IIIb). Stoma subfascial revision was further required in 2 patient (10%). Continence rate at last follow up was 95%., Conclusions: In our early experience, the salvage continent vesicostomy has proven to be a simple continent stoma technique with acceptable both continence and complication rates that can be used in selected patients with a history or need of bladder augmentation and absent appendix., Competing Interests: Conflict of interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Purpose: To review the urological outcomes of proactive versus delayed management of children with a neurogenic bladder (NB)., Methods: We performed a literature search on EMBASE, MEDLINE, Scopus, Web of Science, and Cochrane Central Register of Controlled Trials between January 1, 2000 to August 21, 2023 for studies investigating the management of spina bifida-associated NB in pediatric patients (0-18 years of age). Proactive management was defined as use of clean intermittent catheterization, and/or anticholinergics at presentation, or based on initial high-risk urodynamic findings by 1 year of age. Delayed management was defined as beginning management after 1 year of age or no intervention. Outcomes included incidence or diagnosis of secondary vesicoureteral reflux (VUR), urinary tract infection (UTI), and renal deterioration, which included renal scarring, loss of differential renal function on a nuclear scan, or a decrease in renal function defined by glomerular filtration rate or serum creatinine estimation. Forest plots were synthesized using the Inverse Variance method with random-effect model. The Risk of Bias was assessed using the ROBINS-I tool., Results: We included 8 observational studies on 652 pediatric patients with spina bifida-associated NB (mean follow-up - 7 years). Proactive management following initial assessment was associated with significantly lower risks of secondary VUR (OR 0.37 [0.19, 0.74], p = 0.004), non-febrile UTI (OR 0.35 [0.19, 0.62], p = 0.0004), and renal deterioration (OR 0.31 [0.20, 0.47], p < 0.00001)., Discussion: Delayed management of NB potentially has 3 times higher risks of secondary VUR, non-febrile UTI, and renal deterioration. However, the evidence is limited by the high risk of bias due to lack of randomization and standardized reporting in observational studies., Conclusion: While further well-defined prospective studies with long-term follow-up should be conducted to confirm this finding, this study supports the EAU/ESPU recommendations for early intervention in children with NB., Competing Interests: Conflict of interest All authors declare no conflicts of interest., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Neurogenic bladder is a common complication after spinal cord injury (SCI) that carries substantial burdens on the inflicted individual. The objective of this study is to build a prediction model for neurogenic bladder recovery 1 year after traumatic SCI., Methods: We queried the National Spinal Cord Injury Model Systems database for patients with traumatic SCI who had neurogenic bladder at the time of injury. The primary outcome of interest was the complete recovery of bladder function at 1 year. Multiple imputations were performed to generate replacement values for missing data, and the final imputed data were used for our analysis. A multivariable odds logistic regression model was developed for complete bladder recovery at 1 year., Results: We identified a total of 2515 patients with abnormal bladder function at baseline who had an annual follow-up. A total of 417 patients (16.6%) recovered bladder function in 1 year. Predictors of complete bladder recovery included the following baseline parameters: sacral sensation, American Spinal Injury Association (ASIA) impairment score, bowel function at baseline, voluntary sphincter contraction, anal sensation, S1 motor scores, and the number of days in the rehabilitation facility. The model performed with a discriminative capacity of 90.5%., Conclusions: We developed a prediction model for the probability of complete bladder recovery 1 year after SCI. The model performed with a high discriminative capacity. This prediction model demonstrates potential utility in the counseling, research allocation, and management of individuals with SCI., (Published by Elsevier Inc.)

Urine samples of female patients with overactive bladder (OAB) are characterized by low levels of nerve growth factor (NGF) and elevated concentrations of nitric oxide (NO) compared to healthy controls. We therefore examined how NO might regulate NGF synthesis using rat bladder smooth muscle (SMCs) and urothelial (UROs) cells in culture. In UROs, incubation in hyperglycemic conditions to mimic insulin insensitivity present in the OAB cohort increased secretion of NO and concomitantly decreased NGF, except when the NO synthase inhibitor, l-NAME (1 mM) was present. Sodium nitroprusside (SNP) (300 μM, 24 h), a NO generator, decreased NGF levels and decreased cyclic GMP (cGMP) content, a process validated by the cGMP synthase inhibitor ODQ (100 μM). Alternatively, SNP increased mRNA of both NGF and matrix metalloproteinase-9 (MMP-9). MMP-9 knockout of UROs by Crispr-Cas9 potently decreased the effect of SNP on NGF, implying a dependent role of NO on MMP-9. On the other hand, matrix metalloproteinase-7 (MMP-7) activity was increased by SNP, which taken together with increase in NGF mRNA, suggests a compensatory mechanism. In SMCs, hyperglycemic conditions had the same effect on extracellular content of NO and NGF than in UROs. SNP also decreased NGF secretion but increased cGMP content. Stable permeable analogs of cGMP 8-(4-Chlorophenylthio)-cGMP (1 mM) and N2,2'-O-Dibutyryl-cGMP (3 mM) inhibited NGF release. NGF and MMP-9 mRNA expression was unchanged by SNP. Deletion of MMP-9 in SMCs by Crispr-Cas9 did not alter the effect of SNP. Finally, SNP decreased MMP-7 activity, diminishing the conversion of proNGF to NGF. These results demonstrate that enhanced NO secretion triggered by high glucose decreases NGF secretion through pathways unique for each cell type that involve cGMP and proteases MMP-7 and MMP-9. These results might help to explain our observations from the urine from patients with OAB associated with metabolic syndrome., Competing Interests: Declaration of competing interest The authors have declared that no conflict of interest exist., (Copyright © 2023. Published by Elsevier Inc.)

A boy with myelomeningocele who had sigmoidocolocystoplasty and ureteric reimplantation when 2-years old and normal annual cystoscopies, developed hematuria and abdominal pain with liver dysfunction 14 years postoperatively. Computed tomography showed a tumor on the left side of the augmented bladder, a large lymph node, and large multiple probable metastases in the liver. Cystoscopy 3 months earlier had been normal, but when repeated showed a tumor originating from the augmented sigmoid colon. Biopsy showed undifferentiated sarcoma. Despite chemotherapy, he died 3 months later. The diagnosis at autopsy was undifferentiated sarcoma originating from the sigmoid colon. We report the first case of undifferentiated sarcoma developing 14 years after sigmoidocolocystoplasty for meningomyelocele, and also review the 55 cases of post-bladder augmentation malignancy in the literature.

Introduction: Mirabegron is a beta-3 adrenergic receptor agonist that received FDA approval in 2021 to treat neurogenic detrusor overactivity (NDO) in children ages three years and older. Despite its safety and efficacy, access to mirabegron frequently remains restricted by payor coverage policies., Objective: This cost minimization study sought to determine the cost implications from a payor perspective of mirabegron use at different points in the treatment pathway for pediatric NDO., Study Design: A Markov decision analytic model was constructed to assess the costs for eight treatment strategies over a 10-year period, using six-month cycles (Table). Five strategies involve mirabegron use as first-, second-, third-, or fourth-line therapy. Two strategies, including the "base case," entail use of anticholinergic medications followed by onabotulinum toxin type A (Botox) injection and augmentation cystoplasty. A strategy involving first-line Botox was also modeled. The effectiveness, adverse event rates, attrition rates, and costs associated with each treatment option were obtained from the clinical literature and adjusted to a six-month cycle. Costs were adjusted to 2021-dollar value. A discount rate of 3% was used. To quantify uncertainty, costs and treatment transition probabilities were modeled as gamma and PERT distributions, respectively. One-way sensitivity analyses were performed. Probabilistic sensitivity analysis (PSA) was conducted using a Monte Carlo simulation with 100,000 iterations. Analyses were performed using Treeage Pro (Healthcare Version)., Results: The least costly strategy involved first-line mirabegron (expected cost $37,954). All strategies involving mirabegron were less costly than the base case ($56,417). On PSA, first-line mirabegron was the least costly strategy in 88.9% of cases (mean $37,604, 95% CI: $37,579-37,628); in 100% of cases, the least costly strategy involved mirabegron use. Cost savings associated with mirabegron use were attributable to decreased use of augmentation cystoplasty and Botox injections., Discussion: This is the first study to compare costs across multiple strategies involving mirabegron to treat pediatric NDO. Mirabegron use likely yields cost savings for the payor: the least costly strategy involved first-line mirabegron, and all pathways incorporating mirabegron were less costly than those without mirabegron use. These findings provide an updated cost analysis for the treatment of NDO by investigating mirabegron use alongside more established treatment options., Conclusion: Use of mirabegron for the treatment of pediatric NDO is likely associated with cost savings as compared to treatment pathways without mirabegron. Expansion of payor coverage for mirabegron, as well as clinical studies to study first-line mirabegron use, should be considered., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Detrusor contraction in bladder exstrophy (BE) patients following reconstruction is poorly understood as there are few published studies assessing urodynamic findings in this population. Understanding the ability of the detrusor to contract in BE patients early after closure may be able to inform the longer-term management and potential for the development of future continence in this population., Objective: We sought to evaluate early detrusor contraction using urodynamic studies (UDS) in children who had previously undergone complete primary repair of bladder exstrophy (CPRE). We hypothesized that a majority of children with BE would display the presence of normal detrusor contractile function after CPRE., Study Design: A retrospective review of our prospectively collected database was performed for all patients with a diagnosis of classic BE who underwent primary CPRE between 2013 and 2017. From this cohort we identified patients with at least one post-operative UDS at 3 years of age or older who had undergone an initial CPRE. Our primary outcome was the presence of a detrusor contraction demonstrated on UDS., Results: There were 50 children (31 male, 19 female) with CBE who underwent CPRE between 2013 and 2017.There were 26 (13 male, 13 female) who met inclusion criteria. Median age was 3.5 (IQR: 3.2-4.7) years at the time of UDS Sixteen of the 26 (61.5%) generated a sustained detrusor contraction generating a void, with a median peak voiding pressure of 38 cm H20 (IQR: 28-51). The median bladder capacity reached was 48 ml, which represented a median of 30% of expected bladder capacity. The median post void residual (PVR) for the entire cohort was 26 ml (IQR: 9, 47) or 51% (IQR: 20%-98%) of their actual bladder capacity, while the median PVR for those children with a sustained detrusor contraction was 18 ml (IQR: 5, 46) or 33% (IQR: 27%, 98%) of their actual bladder capacity. Intraoperative bladder width and bladder dome to bladder neck length did not correlate with the presence of voiding via a detrusor contraction (p = 0.64)., Discussion: We present the first study assessing early UDS finding of detrusor contraction in BE patients after CPRE. In our cohort, 61.5% of patients were able to generate a sustained detrusor contraction on UDS which is a higher percentage than has been reported in previous series. A difference in initial surgical management may account for these findings., Conclusion: At short term follow up, the majority of children in our cohort were able to produce sustained detrusor contractions sufficient to generate a void per urethra with a modest post void residual volume. Long-term follow-up and repeated UDS will be needed to track detrusor contractility rates, bladder capacities, compliance, post void residuals and ultimately continence rates over time., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Lower urinary tract dysfunction (LUTD) is becoming more common in aging societies worldwide. In patients with LUTD, the risk of urinary tract infection (UTI) rises due to several distinct mechanisms, including easy bacterial access to the urinary tract, impaired bacterial washout, and an impaired innate defense system. The pathophysiology of LUTD varies depending on whether it is neurogenic or non-neurogenic, as well as by gender; therefore, the etiology and characteristics of UTI differ according to the type of LUTD. Patients with neurogenic LUTD, especially those with spinal cord injury, have a high risk of febrile UTI, and strict bladder management is required to prevent UTI. Clean intermittent catheterization with or without appropriate pharmacological therapy is also strongly recommended for patients with neurogenic LUTD at risk of febrile UTI, unable to void, or with high post-void residual volume. In contrast, both male and female patients with non-neurogenic LUTD have a lower risk of symptomatic UTI. There is insufficient evidence for non-neurogenic LUTD regarding the association between symptomatic UTI but not asymptomatic bacteriuria and LUTD severity, including the presence of post-void residual volume, or whether therapeutic intervention for LUTS reduces the incidence of UTI, particularly in male patients. In this narrative review, we aimed to highlight the pathogenesis, epidemiology, and management of UTI in patients with LUTD., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Japanese Society of Chemotherapy, Japanese Association for Infectious Diseases, and Japanese Society for Infection Prevention and Control. Published by Elsevier Ltd. All rights reserved.)

Introduction: Bladder augmentation is an option to protect kidneys and treat incontinence in frequent neurogenic and congenital non-neurogenic disorders. However, patients may need also other procedures to treat incontinence or to aid catheterizations. In addition, the patients are prone to complications., Objective: To evaluate complications or additional surgeries in augmentation cystoplasty according to the etiology of the bladder dysfunction., Study Design: Data on additional surgical procedures related to bladder augmentation were collected from 128 patients who had undergone enterocystoplasty between 1990 and 2019, with a median age of 10.1 years (IQR 7.2-12.8) and a follow-up time of 11.8 years (IQR 5.7-18.6) after augmentation., Results: Eighty-three patients had a neurogenic disease, and 45 patients had a non-neurogenic disease. Bladder neck operations were performed either at the time of or after augmentation for 62 patients (48.4%), more commonly in the non-neurogenic than neurogenic group <0.01. In addition, continent stoma operations in 56 (36.8%) patients were more common in the non-neurogenic group (p < 0.01). Ten patients received kidney transplantation (four in the neurogenic, six in the non-neurogenic group); seven of these patients originally had dysplastic kidneys, two developed nephrological kidney disease, and one had hydronephrosis and renal insufficiency at the time of diagnosis of neurogenic bladder. Surgical problems resulting from bladder augmentation were treated in 28 patients (21.8%). The risk for surgical treatment because of complications was 4%, 17%, 24%, 27% and 27% at 1, 5, 10, 15 and 20 years follow up respectively. Complications were more common in patients in non-neurogenic than in patients with neurogenic group (p < 0.01). However, the greater proportion of continent stomas in non-neurogenic group explained the difference (Fig.). The most frequent complication requiring surgery during follow-up was bladder stones, which occurred in 24 (18.8%) patients. The first stone was treated at a median of 3.3 years (range 0.3-14.2) after augmentation. Stones were more common in patients with a continent stoma than without (p < 0.01). Other indications for surgical treatment were as follows: obstructive ileus in five patients, reduced bladder capacity in three patients, bladder perforation in two patients and lobulated bladder with infections in one patient., Conclusions: The risk for surgical treatment was about 27% in 20-year follow-up after bladder augmentation, because of augmentation related complications. The most prevalent complication was bladder stone, that was mainly associated with continent stomas. Continent stomas were most prevalent in patients with non-neurogenic diagnosis., Competing Interests: Conflicts of interest None., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Enterocystoplasty (EC), appendico- or ileovesicostomy (APV), and appendicocecostomy (APC) can facilitate continence and prevent renal damage for patients with congenital urologic or bowel disease. Bowel obstruction is a well-documented complication of these procedures, and the etiology of obstruction is variable. The aim of this study is to determine the incidence and describe the presentation, surgical findings, and outcomes of bowel obstruction from internal herniation due to these reconstructions., Methods: In this single institution retrospective cohort study patients who underwent EC, APV, and/or an APC between 1/2011 and 4/2022 were identified via CPT codes within the institutional billing database. Records for any subsequent exploratory laparotomy during this same timeframe were reviewed. The primary outcome was an internal hernia of bowel into the potential space between the reconstruction and the posterior or anterior abdominal wall., Results: Two hundred fifty seven index procedures were performed in 139 patients. These patients were followed for a median of 60 months (IQR 35-104 months). Nineteen patients underwent a subsequent exploratory laparotomy. The primary outcome occurred in 4 patients (including one patient who received their index procedure elsewhere) for a complication rate of 1% (3/257). The complications occurred between 19 months and 9 years after their index procedure (median 5 years). Patients presented with bowel obstruction; two patients also had sudden pain following an ACE flush. One complication was caused by small bowel and cecum passing around the APC and subsequently volvulizing. A second was caused by bowel herniating behind the EC's mesentery and the posterior abdominal wall. A third was caused by bowel herniating behind the APV mesentery and subsequently volvulizing. The exact etiology of fourth internal herniation is unknown. Of the three surviving patients, all required resection of ischemic bowel and 2 required resection of the involved reconstruction. One patient died intraoperatively from cardiac arrest. Only 1 patient required a subsequent procedure to regain lost function., Conclusion: Internal herniation caused by small or large bowel passing through a defect between the mesentery and abdominal wall or twisting around a channel occurred in 1% of 257 reconstructions performed over 11 years. This complication can arise many years after abdominal reconstruction, resulting in bowel resection and possibly takedown of the reconstruction. When anatomically possible and technically feasible, the surgeon should close any potential spaces created during the initial abdominal reconstruction., Competing Interests: Conflicts of interest The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2023 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

A 34-year-old man presented with sudden voiding dysfunction and lower limb paraplegia. As a central nervous system disorder was suspected, he was referred to the neurology department. Under the diagnosis of neurosarcoidosis, steroid pulse therapy was initiated. To ensure the effect of this therapy, the patient was referred back for urodynamic testing. Urodynamic testing indicated that the urethral sphincter was not relaxed and could not void. Due to the sudden appearance of repeated and refractory opisthotonus, tetanus was strongly suspected. After administration of antibiotics and tetanus immune globulin, those symptoms disappeared.

Objective: To observe the therapeutic effect of bladder function training combined with repetitive sacral root magnetic stimulation (rSMS) on neurogenic bladder in patients with spinal cord injury., Methods: From January 2019 to May 2021, we selected 68 patients with neurogenic bladder and spinal cord injury who were hospitalized in the rehabilitation department of our hospital. Patients were divided into the rSMS group (36 cases) and control group (32 cases) according to a random number table. The rSMS group was treated with bladder functional training combined with rSMS, while the control group was treated only with bladder functional training. The training lasted for 40 days. Finally, we compared the therapeutic effects in the 2 groups., Results: There was no significant difference in sex, age, course of disease, site, and grade of spinal cord injury between the 2 groups. There was no statistically significant difference in urodynamics between the 2 groups before treatment. After treatment, the residual urine volume of the rSMS group was 82.7 ± 34.6 mL, maximum urine flow rate was 15.7 ± 4.8 mL/second, bladder volume was 388.6 ± 51.3 mL, and bladder pressure was 36.3 ± 6.3 cm H 2 O. After treatment, the residual urine volume in the rSMS group was significantly less than that in the control group (P < 0.05). The maximum urine flow, bladder volume, and bladder pressure in the rSMS group were higher than those in the control group, and the differences were statistically significant (P < 0.05). After treatment and after 40 days of follow-up, the Barthel index scores of the patients in the rSMS group were 69.5 ± 9.3 and 73.5 ± 8.2, respectively, which were significantly higher than those of the patients in the control group (P < 0.05). The effective rates were 94.4% in the rSMS group and 81.3% in the control group, and the difference was statistically significant (P < 0.05)., Conclusions: Compared with bladder function training alone, bladder function training combined with rSMS was more effective in the treatment of neurogenic bladder, particularly for detrusor hyporeflexia/areflexia; it significantly improved patients' ability to urinate autonomously, reduced the residual urine volume of the bladder, and improved patients' quality of life., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Neurogenic bladder is a common source of morbidity in patients with spina bifida and can cause renal damage. Medical management may include imaging, urodynamic studies (UDS), laboratory testing, clean intermittent catheterization (CIC), and medication. There is ongoing debate regarding the optimal management regimen. Approaches are described by two paradigms: proactive and expectant management. In a proactive approach, invasive interventions like CIC and UDS are initiated before the onset of renal abnormalities. In expectant management, UDS, CIC, and medications are started after abnormalities are identified. In this scoping review, we aim to comprehensively review existing literature on outcomes of proactive and expectant management of neurogenic bladder in patients with spina bifida., Methods: We searched multiple databases and screened articles for inclusion using PRISMA-ScR guidelines. Included studies reported clinical outcomes of any aspect of proactive or expectant neurogenic bladder management in patients with spina bifida., Results: Ultimately, 74 articles were included for review including 67 cohort studies, 4 cross-sectional studies, 2 sequential cohort studies, and 1 randomized control trial. Eleven studies directly compared management strategies. There was substantial heterogeneity in study designs, management protocols, and reported outcomes. Most studies addressed multiple simultaneous aspects of management without specifically analyzing individual aspects. However, some commented on individual aspects of management including UDS (13), CIC (32), imaging (7), and medication (5). Although there was no consensus about optimal management, all direct comparisons of paradigms supported a proactive approach., Conclusion: Our review identified a broad body of literature about optimal management of neurogenic bladder. Existing studies vary greatly in terms of treatment protocols, measured outcomes, and management recommendations. Overall, studies that directly compare management are scarce but favor proactive management. Given the implications on clinical outcomes, it is crucial to focus future work on directly comparing management strategies and isolating the effects of different individual management elements., Competing Interests: Conflicts of interest None., (Copyright © 2022 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Bladder symptoms are common in Parkinson disease (PD), affecting quality of life. Medications commonly used such as antimuscarinics can cause frequently intolerable side effects, and therefore, new, better tolerated approaches are needed. Neuromodulation techniques have an established role in urologic disorders; these techniques include tibial nerve stimulation (TNS) and sacral neuromodulation (SNM), which are localized therapies lacking the side effects associated with medication., Objectives: This study aimed to undertake a systematic review of the literature reporting the use of neuromodulation techniques for the treatment of bladder symptoms in PD and related conditions., Materials and Methods: A systematic search of data bases was conducted including MEDLINE, CENTRAL, and Web of Science. Studies were required to present specific outcomes for individuals with PD for neuromodulation interventions., Results: Ten primary studies were identified concerning detailed outcomes of neuromodulation on bladder symptoms in PD, including seven for TNS, one for SNM, and one using transcranial magnetic stimulation (TMS). Two further mixed cohort studies documented minimal data on individuals with PD. All studies demonstrated benefit in a range of outcome measures following neuromodulation. Two randomized sham-controlled studies were carried out using TNS, with one clearly demonstrating superiority over sham, although difficulties with achieving believable yet ineffective sham treatment are highlighted. Further studies reported limited, uncontrolled outcomes of SNM in patients with PD, demonstrating benefit., Conclusions: Evidence from case series suggests benefit from TNS in PD, with limited literature on SNM or TMS. Placebo effect from neuromodulation is a concern, and only limited controlled data exist. Future well-designed and sham-controlled studies need to be completed to provide definitive data on the benefit of neuromodulation in PD. Definitively proving the utility of a neuromodulation modality will allow better treatment of bladder symptoms without the need for pharmacologic measures that cause side effects., (Copyright © 2022 International Neuromodulation Society. Published by Elsevier Inc. All rights reserved.)