PURPOSE: Cognitive developmental disorders (CDD) including intellectual disability (ID), cerebral palsy (CP), Down's syndrome (DS), and autism spectrum disorder (ASD) are often associated with lower urinary tract dysfunc...PURPOSE: Cognitive developmental disorders (CDD) including intellectual disability (ID), cerebral palsy (CP), Down's syndrome (DS), and autism spectrum disorder (ASD) are often associated with lower urinary tract dysfunction. This study aimed to characterize urodynamic (UD) patterns in pediatric patients with CDD and analyze their relationship with clinical variables and treatment outcomes. MATERIALS AND METHODS: A retrospective observational study was conducted including 79 pediatric patients (ages 4-17) with confirmed CDD who underwent comprehensive video urodynamic evaluations between 2009 and 2024 at a tertiary pediatric center. Data analyzed included age, sex, diagnosis, urodynamic parameters (detrusor overactivity, bladder compliance, detrusor-sphincter dyssynergia, bladder sensation), vesicoureteral reflux (VUR), and treatment modalities. Statistical associations between CDD type and UD parameters were examined using chi-square tests (p < 0.05). RESULTS: The mean age was 9.2 years, with male predominance (70.9%). Diagnoses included CP (38.0%), ID (32.9%), ASD (24.1%), and DS (5.1%). Dysfunctional voiding was the most frequent diagnosis (32.9%), particularly in CP (43.3%) and ASD (36.8%). Detrusor overactivity was common in ID (57.7%) and CP (40.0%) but rare in ASD (5.3%). Detrusor-sphincter dyssynergia was seen in 56.7% of CP and 26.9% of ID patients and absent in ASD. Decreased bladder compliance (<20 ml/cmHO) occurred in 90.0% of ID and 57.7% of CP patients but was preserved in ASD. Absent urgency sensation was found in 50.0% of CP and 19.2% of ID patients. VUR was present in 10.5% of ASD and 6.7% of CP patients. Clinical management was most frequent in ID (96.2%) and ASD (94.7%) patients. Clean intermittent catheterization was required in 43.3% of CP and 12.5% of ID cases. Surgical intervention was necessary in 12.7% of patients, mainly in CP (20.0%). CONCLUSIONS: Distinct urodynamic patterns were observed across cognitive developmental disorders. CP patients showed predominantly neurogenic patterns with high rates of detrusor-sphincter dyssynergia and need for invasive management. ID patients exhibited storage-phase dysfunction responsive to medical therapy, while ASD patients showed preserved motor coordination and compliance, suggesting primarily behavioral or sensory dysfunction. Urodynamic studies are crucial for individualized diagnosis and management in children with cognitive developmental disorders.
BACKGROUND: Cystine stones are a rare yet challenging cause of pediatric nephrolithiasis, marked by high recurrence and repeated surgical needs. Although endourological advances have improved the safety of percutaneous n...BACKGROUND: Cystine stones are a rare yet challenging cause of pediatric nephrolithiasis, marked by high recurrence and repeated surgical needs. Although endourological advances have improved the safety of percutaneous nephrolithotomy (PCNL), long-term renal outcomes remain strongly influenced by adherence to medical therapy. OBJECTIVE: To evaluate the efficacy, safety, recurrence patterns, and long-term renal outcomes of PCNL for cystine stones in children across a 25-year institutional experience. STUDY DESIGN: This retrospective review included pediatric patients with cystine stones who underwent PCNL between 2000 and 2024 at a tertiary referral center. Surgical success was defined as stone-free status. Complications were graded using the modified SATAVA and Clavien-Dindo systems. All patients received urine alkalization and thiol therapy, with adherence assessed by the Medication Possession Ratio. Long-term renal function and blood pressure were evaluated during follow-up. RESULTS: A total of 127 PCNLs were performed in 94 renal units of 78 patients (median age at first PCNL 6 years [IQR 2-14]; 56% male, 44% female), with a median follow-up of 216 months (IQR 141-287). The overall stone-free rate was 64%, increasing to 81% when combined with simultaneous endoscopic procedures. Repeat PCNL for recurrence occurred in 42.5% of renal units (n = 40/94), and regrowth in 11.7% of the renal units (n = 11/94). Only nine (11.5%) of patients were fully adherent to medical therapy. Kaplan-Meier analysis showed a significantly shorter time to repeat PCNL in non-adherent renal units (log-rank p = 0.016); among those that experienced an event, the median time to first repeat PCNL was 15 months, whereas no repeat PCNL events were observed in adherent renal units. During follow-up, 18 patients (23%) developed hypertension and eight (10.3%) developed renal function decline, all confined to the non-adherent group. DISCUSSION: Although PCNL provided high clearance with good procedural safety, long-term success was limited by poor adherence. Recurrence and renal deterioration rates were comparable to those reported in previous series. Major limitations include the retrospective, single-center design and incomplete metabolic data. CONCLUSIONS: PCNL is a safe and effective option for pediatric cystine stones, but sustained medical adherence is essential to prevent recurrence and preserve renal function, ensuring durable long-term outcomes.
OBJECTIVE: Pediatric kidney stone disease is a health problem that may require invasive surgical interventions during childhood and can impose a substantial psychological burden on both the child and the family. Uncertai...OBJECTIVE: Pediatric kidney stone disease is a health problem that may require invasive surgical interventions during childhood and can impose a substantial psychological burden on both the child and the family. Uncertainty regarding anesthesia, perioperative safety, postoperative pain, and early recovery/stone clearance may increase both parental and child anxiety. This study assessed perioperative anxiety in children undergoing kidney stone surgery and their parents, and identified demographic and clinical factors associated with anxiety. MATERIALS AND METHODS: This prospective observational study was conducted at a tertiary academic urology center between January and July 2025. The study population consisted of 100 children aged 3-18 years scheduled to undergo ureterorenoscopy (URS) for kidney stones, along with their parents. Anxiety levels of the children were assessed using the Facial Image Scale (FIS), whereas parental anxiety was assessed using the State-Trait Anxiety Inventory, including state (STAI-S) and trait (STAI-T) components. The scales were administered before surgery and at postoperative 1., 2., and 6. h. Data were analyzed using descriptive statistics, analysis of variance, and linear regression methods. RESULTS: The median age of the children included in the study was 7.5 years, and the mean duration of surgery was 44.39 ± 14.60 min. There was no difference between the STAI-S and STAI-T scores of mothers and fathers during the preoperative period. However, in the postoperative 1. h, anxiety levels increased significantly in both parents (p = 0.001) and then gradually decreased. All mothers, and the parents of the children aged 4-<10 years had significantly higher anxiety levels at all preoperative and postoperative time points (p < 0.001). Anxiety scores were higher in mothers in the American Society of Anesthesiologists (ASA) III group, and anxiety levels were also significantly increased in parents with a history of previous major surgery (p < 0.05). In contrast, increasing experience with stone surgery had a reducing effect on parental anxiety (p < 0.01). FIS scores increased significantly during the transfer of the patients from the waiting room to the operating room (p < 0.001). Although FIS scores decreased somewhat in the inpatient unit, they still remained above their preoperative levels. CONCLUSION: Parental anxiety during pediatric stone surgery increases significantly, especially in the early postoperative period, and is influenced by factors such as the child's age, ASA score, and prior surgical history. The results indicate that the psychological state of the parents is closely related to the emotional and clinical recovery processes of their children. Children's anxiety rose during transfer to the operating room and remained above baseline postoperatively.
INTRODUCTION: Bladder exstrophy-epispadias complex (BEEC) involves osseous pelvic defects and a wide pubic diastasis (PD). PD widens over time after the initial closure, but the degree and impact of this increase is not...INTRODUCTION: Bladder exstrophy-epispadias complex (BEEC) involves osseous pelvic defects and a wide pubic diastasis (PD). PD widens over time after the initial closure, but the degree and impact of this increase is not well delineated. Using the transverse distance between posterior inferior iliac spines (dPIIS) as an internal comparison, we aimed to: 1) compare dPIIS, PD, and M-factor (PD/dPIIS) measurements made on radiographs to computed tomography (CT) images and between reviewers; 2) assess the measurements at birth and over time for BEEC and for age- and gender-matched controls; 3) determine how the M-factor compares with the PD over time. METHODS: This was a single institution, IRB-approved retrospective study of patients with BEEC who underwent a complete primary repair of bladder exstrophy (CPRE) or repair of epispadias or cloacal exstrophy with bilateral osteotomies between 5/1/2013 and 3/1/2020. PD and dPIIS were measured on cases and controls at 4 timepoints: preoperative, intraoperative, early post-operative (6-8 weeks) and late post-operative (greater than 6 months). For statistical analysis intra class correlations were used to assess inter-rater reliability. Mixed model two-way ANOVA was done with subjects as random effect, and group (patient/control), exam time as the two fixed effects. RESULTS: Forty-three cases (33 male) ranging in age from 1 day to 129 months (IQR 15.25 months) during the time points were evaluated. There was excellent agreement for measurements among the 6 raters. Following BEEC repair with osteotomies, PD and the M-factor decreased significantly. In the entire cohort, due to early post-operative spreading of the bones, there was no difference between pre-operative and early post-operative PD (35.2 ± 8.6 mm to 34.1 ± 9.8 mm, p = 0.08), however there was a significant difference in the M-factor between those time points (0.89 ± 0.2 to 0.73 ± 0.2, p < 0.01). With gradual increase in PD over time, there was a significant difference between early and late post-operative PD measurements (35.2 ± 8.6 mm to 39.8 ± 9.9 mm, p < 0.01), but not in the M-factor (0.73 ± 0.2 to 0.75 ± 0.2 (p = 0.45). CONCLUSION: PD in patients with BEEC changes over time due to surgery as well as growth, while a ratio of PD to an internal reference (dPIIS), does not change. This M-Factor ratio can better determine the initial extent of PD within the context of the size of the child, as well as standardize assessment of changes after surgery and during growth and development.
BACKGROUND: Persistent penile curvature remains a major challenge in proximal hypospadias repair. The TAIPEI (Taping In Between Penile Incisions) procedure is a novel postoperative rehabilitation strategy that maintains...BACKGROUND: Persistent penile curvature remains a major challenge in proximal hypospadias repair. The TAIPEI (Taping In Between Penile Incisions) procedure is a novel postoperative rehabilitation strategy that maintains penile straightening through caregiver-applied taping between staged repairs. Despite promising surgical outcomes, the real-world feasibility of long-term home taping and family adherence has not been systematically evaluated. OBJECTIVE: To assess the feasibility of the TAIPEI procedure based on caregiver-reported experience and to identify factors associated with perceived difficulty in performing taping. STUDY DESIGN: A cross-sectional, anonymous online questionnaire was distributed to families of patients who underwent staged hypospadias repair at a single tertiary institution between 2020 and 2024. Eligible participants were caregivers who had performed penile taping for at least three months. The survey evaluated ease of taping, time requirement, retaping frequency, financial burden, child adaptation, and potential side effects. RESULTS: A total of 95 caregivers completed the questionnaire (response rate: 95/129, 73.6%). Most respondents (90%) rated taping as easy or moderately easy to perform, and 55% reported adaptation within three months. On average, 59% maintained taping for over 14 h per day, and 80% re-applied tape fewer than five times daily, with each session lasting under 5 min. Perceived taping challenges were significantly associated with shorter daily taping duration, more frequent retaping, longer taping sessions, and difficulty with child cooperation. No significant associations were found between difficulty and tape consumption, cost, or skin irritation. DISCUSSION: These findings indicate that with proper instruction and follow-up support, most families can successfully implement the TAIPEI procedure at home. While compliance and adaptation vary, key challenges are behavioral rather than procedural or financial. Limitations include potential response bias and the single-center design. CONCLUSIONS: The TAIPEI procedure is a feasible and manageable family-centered postoperative rehabilitation strategy to correct penile curvature between staged repairs. Early caregiver education, clear demonstration of technique, and structured follow-up support appear critical for success. CLINICAL/TRANSLATIONAL APPLICABILITY: This study provides the first quantitative assessment of caregiver experience with the TAIPEI procedure. The results support its use as a practical, low-cost, family-integrated rehabilitation method and may inform postoperative protocols and counseling in pediatric urology.
BACKGROUND: While anatomical success rates following hypospadias repair exceed 90%, the psychological and social impact remains poorly understood. This synthesis aimed to integrate and assimilate published evidence on qu...BACKGROUND: While anatomical success rates following hypospadias repair exceed 90%, the psychological and social impact remains poorly understood. This synthesis aimed to integrate and assimilate published evidence on quality-of-life outcomes in hypospadias patients, examining satisfaction, psychological adjustment, and predictive factors. METHODS: Nine studies encompassing 8409 participants (467 cases; 7942 controls) with corrected and uncorrected hypospadias were analysed. Parameters included health-related quality of life (HRQoL), patient satisfaction, genital self-perception, sexual function, and psychological adjustment. RESULTS: Overall outcomes were favourable, with high patient satisfaction (92.6%), excellent functional results (>90% acceptable HOSE scores), and HRQoL scores comparable to normative populations. Decision regret remained minimal (79.0% patients, 81.7% parents reporting no regret). However, a striking paradox emerged: significant psychosocial challenges persist in a substantial minority. Genital self-perception was consistently impaired compared to controls (13.3 vs 15.8, p < 0.001), with 53% of patients scoring below thresholds indicating need for psychological support. Predictive modelling revealed that negative genital self-perception and reduced orgasmic function depicted a significant association with impaired mental health-related quality of life (R = 0.51), while medical variables showed no predictive value. Comparison with untreated hypospadias demonstrated clear surgical benefits. CONCLUSIONS: Hypospadias repair achieves favourable quality of life outcomes with high satisfaction and minimal regret. Comparison with untreated cases demonstrates clear benefits of surgical intervention across multiple quality of life domains. However, in a significant subset of patients, persistent genital self-perception impairment despite successful surgical outcomes highlights the need for enhanced psychological support and long-term follow-up incorporating patient-centred measures.
INTRODUCTION: Prune Belly Syndrome extends beyond the classical triad of deficient abdominal wall musculature, urinary tract anomalies, and cryptorchidism. Urological abnormalities may be severe enough to require renal t...INTRODUCTION: Prune Belly Syndrome extends beyond the classical triad of deficient abdominal wall musculature, urinary tract anomalies, and cryptorchidism. Urological abnormalities may be severe enough to require renal transplantation during their lifetime. The RUBACE score is currently the most comprehensive tool for categorising the multisystem manifestations of the syndrome. We hypothesized that higher RUBACE scores would be associated with poorer post renal transplantation outcomes. PATIENTS AND METHODS: We conducted a retrospective review of 500 paediatric renal transplantations performed between April 2008 and October 2021, identifying all patients with Prune Belly Syndrome undergoing their first transplant. Demographic variables were collected, and RUBACE scores were assigned based on clinical assessment and physical examination prior to renal transplantation. Descriptive analyses were performed using the software R 4.4.3. Qualitative variables were expressed as percentages, and numeric variables as medians and interquartile (IQR 25-75). Associations between RUBACE scores graft loss or mortality were evaluated using a permutation test with 100.000 simulations, adopting a level of significance of 5%. RESULTS: In the period of the study, 22 patients with Prune Belly Syndrome underwent their first renal transplantation. During follow-up, 11 patients (50%) experienced at least one febrile urinary tract infection, 3 (14%) had graft loss, and 2 (9%) died. Those with graft loss had higher RUBACE scores than those without, although this difference was not statistically significant (p = 0.16). RUBACE scores were higher among patients who died (p = 0.03). DISCUSSION: Intestinal, osteoarticular, neurologic, pulmonary and cardiac manifestations impact the quality of life and survival of patients with Prune Belly Syndrome and should be considered in multidisciplinary care when preparing these patients for renal transplantation. Many of the patients in the present study needed procedures to allow adequate bladder emptying and to reduce complications such as recurrent urinary tract infections. Until now, no previous study has evaluated RUBACE score with renal outcomes in paediatric Prune Belly patients that received renal transplantation. CONCLUSIONS: Renal transplantation in children with Prune Belly Syndrome presents unique clinical challenges. RUBACE score may assist in risk stratification, helping to identify patients at higher risk of graft loss and mortality after transplantation.
BACKGROUND: Urological complications remain a significant cause of morbidity in pediatric kidney transplantation (KT). While ureteroneocystostomy (UNC) according to Lich-Gregoir is the standard technique for urinary reco...BACKGROUND: Urological complications remain a significant cause of morbidity in pediatric kidney transplantation (KT). While ureteroneocystostomy (UNC) according to Lich-Gregoir is the standard technique for urinary reconstruction, pyeloureterostomy (PU) with native ureter ligation has emerged as a potential alternative, though its role as a first-line approach is still debated. METHODS: We retrospectively reviewed all pediatric KTs performed in two university hospitals between January 2013 and January 2023. Patients with documented vesicoureteral reflux were excluded. Two groups were compared according to the urinary reconstruction technique: PU with native ureter ligation versus UNC. Data collected included patient demographics, perioperative characteristics, postoperative complications, renal function, pain management, and 2-year graft survival. RESULTS: A total of 74 patients were analyzed (26 PU; 48 UNC). Baseline characteristics were comparable between groups. Two-year graft survival was similar (PU: 90% vs. UNC: 89%). Renal function, assessed by glomerular filtration rate (GFR) at follow-up, showed no significant difference. The overall rate of major urological complications requiring surgical revision was comparable (PU: 11% vs. UNC: 10%). PU was associated with a higher incidence of transient graft pelvic dilatation (27% vs. 0%), which spontaneously regressed in most cases, while symptomatic vesicoureteral reflux occurred only in the UNC group (6.2%). Ligation of the native ureter did not result in specific morbidity or increased pain. CONCLUSIONS: First-line PU with ligation of the native ureter is a safe and effective alternative to UNC in pediatric KT. It avoids bladder dissection, preserves better vascularization of the donor ureter, and facilitates endourological procedures, without increasing complication rates. Prospective multicenter studies with longer follow-up are warranted to confirm long-term outcomes and better define optimal surgical strategies.
BACKGROUND: Enuresis is a common pediatric urological condition in which sleep-related mechanisms (sleep fragmentation and impaired arousal) may contribute to nocturnal bladder control problems. In parallel, problematic...BACKGROUND: Enuresis is a common pediatric urological condition in which sleep-related mechanisms (sleep fragmentation and impaired arousal) may contribute to nocturnal bladder control problems. In parallel, problematic digital media use has emerged as a potentially modifiable exposure that may disrupt sleep in children. OBJECTIVE: To investigate the relationship between problematic media use and sleep disorders in children diagnosed with enuresis and to identify sociodemographic and behavioral factors associated with increased sleep problems. MATERIALS AND METHODS: This cross-sectional study was conducted with the parents of 186 children aged 6-12 years who presented to the pediatric urology clinic with enuresis. Parents completed the Problematic Media Use Scale-Parent Form (PMUS-PF) and the Sleep Disorder Scale for Children (SDSC). One parent per child participated in the study The analyses used descriptive statistics, Mann-Whitney U, Kruskal-Wallis, t-test, ANOVA, and Spearman correlation tests. RESULTS: The average age of the children is 8.1 ± 2.0, with 62.9% being boys and 37.1% being girls. A moderate positive correlation was found between problematic media use and sleep disorders (r = .445, p < .001); this relationship was particularly evident in the subscales of sleep initiation and maintenance disorders (r = .401; p < .001), sleep-wake transition disorders (r = .342; p < .001), and excessive sleepiness disorders (r = .283; p < .001). According to the variable of children's average daily internet usage time, statistically significant differences were found between the PMUS-PF total score and the SDSC total score, and the subscale scores for "Sleep Initiation and Maintenance Disorders" and "Sleep-Wake Transition Disorders" (p < .05). It was found that children who used the internet for more than 5 h per day had higher scores on the aforementioned scale and subscale compared to other groups. CONCLUSION: Problematic media use is associated with sleep disturbances especially difficulty falling asleep, sleep-wake transition problems, and excessive daytime sleepiness in children diagnosed with enuresis. Incorporating the assessment of sleep patterns and digital media habits into the routine clinical evaluation of enuretic children may be valuable, and family-based behavioral interventions supporting healthy media use and sleep hygiene should be considered as adjunctive strategies in enuresis management.
INTRODUCTION: We reviewed our data to evaluate changes in estimated glomerular filtration rates (eGFR) and chronic kidney disease (CKD) status in pediatric patients with classic bladder exstrophy (CBE) that have previous...INTRODUCTION: We reviewed our data to evaluate changes in estimated glomerular filtration rates (eGFR) and chronic kidney disease (CKD) status in pediatric patients with classic bladder exstrophy (CBE) that have previously undergone staged repairs with primary closure followed by isolated bladder neck repair (BNR). METHODS AND MATERIALS: Medical records of patients with CBE (1986-2020) were retrospectively reviewed and included if the patient had undergone a staged repair without bladder augmentation and creatinine measurements were obtained prior to 18 years of age. Schwartz formula was used to calculate eGFRs and CKD status as determined by nephrology evaluations. Non-parametric statistical analysis was performed. RESULTS: Twenty-two children (68% male) underwent primary closure (94% immediate neonatal) followed by BNR at median age of 5.1 years. At median follow-up after BNR of 16 years, 16 (73%) were augmented or diverted primarily for urodynamic deterioration or upper tract changes (81%). Mean preoperative eGFR was 109 ml/min/1.73 m at which time no patient had a CKD classification based on eGFR criteria. Ninety-two percent had a decline in eGFR with mean change of -30 ml/min/1.73 m (p < 0.001) observed prior to 18 years of age, corresponding to a -2.2 ml/min/1.73 m (p = 0.02) annual rate of decline. A significant difference in eGFR between the two time points (before BNR and prior to age 18) was observed in male (p < 0.001) but not female (p = 0.29) patients. Prior to age 18, five patients met CKD criteria and one had CKD3+ at a median age of 14 years; all followed with a nephrologist. CONCLUSIONS: Renal outcomes data following exstrophy repairs continue to be sobering, with nearly a 2-point annual decline in eGFR in patients undergoing staged repair including isolated BNR. We did note a significantly higher risk of renal functional decline during childhood in boys. More attention to the early surveillance of renal function following exstrophy repair is needed.
BACKGROUND: There is no consensus on the ideal follow-up testing after pyeloplasty for ureteropelvic junction obstruction (UPJO) in children. Although diuretic renogram (DR) is considered the gold standard to define succ...BACKGROUND: There is no consensus on the ideal follow-up testing after pyeloplasty for ureteropelvic junction obstruction (UPJO) in children. Although diuretic renogram (DR) is considered the gold standard to define successful pyeloplasty, percent reduction in antero-posterior diameter (PR-APD) on post-operative ultrasound (USG) has been reported to be useful to identify successful pyeloplasty. We sought to further explore PR-APD ranges in the first post-operative USG that would have high specificity to identify successful and failed pyeloplasty. METHODS: From a 10-year database of pediatric pyeloplasties performed in 4 centers, cases with follow-up USG at 3 months post-pyeloplasty and follow-up DR were selected for analysis. On the basis of drainage on follow-up DR, the patients were divided into group 1 (successful) and group 2 (failures). PR-APD was compared between the two groups, and receiver operating curve (ROC) analysis was performed to assess the cutoff value of PR-APD that had the best discriminative ability to predict success. Further, within group 2, PR-APD was compared between early (2a) and late (2b) failures. RESULTS: Three hundred and fifty-nine children (323 group 1, 36 group 2) who underwent primary dismembered pyeloplasty for unilateral UPJO were included. Both groups were comparable for demographic parameters and mean pre-operative APD. On the 3-months post-operative USG, group 1 had significantly lower post-operative APD (p = 0.0001) and higher PR-APD (62.8% vs 24.6%, p < 0.001) than group 2. On ROC analysis, PR-APD of 42% had 91% sensitivity and 100% specificity for predicting success of pyeloplasty. PR-APD <15% predicted early failures with 100% specificity, while PR-APD between 16 and 42% was associated with 45% risk of late pyeloplasty failure. DISCUSSION: We identified the cut-off values of PR-APD that could predict success and early failure of pyeloplasty with high specificity. While earlier reports had limited number of failed cases, our data was unique that we had a sizable number of failed pyeloplasty cases, thus enabling meaningful comparison of PR-APD between successes and failures. In addition, we identified a subgroup at risk of late failure. These findings might be useful to avoid routine post-operative DR studies after pyeloplasty, detect early failures and identify the few patients who benefit from long-term follow-up. CONCLUSIONS: On the 3-month post-operative USG after pyeloplasty, PR-APD 42% or more predicted success with 100% specificity, while PR-APD <15% was 100% specific in predicting early failures. Those with PR-APD 16-42% may be at risk for late failures and may benefit from long-term follow-up.
INTRODUCTION: Achieving urinary continence is an important objective of surgical reconstruction for bladder exstrophy-epispadias complex (BEEC). Despite its importance to postoperative quality of life, there remains no u...INTRODUCTION: Achieving urinary continence is an important objective of surgical reconstruction for bladder exstrophy-epispadias complex (BEEC). Despite its importance to postoperative quality of life, there remains no universally accepted definition of continence in this patient population, with existing evidence suggesting highly inconsistent definitions across studies. OBJECTIVE: We aimed to determine whether a standardized definition of continence exists following intervention for BEEC and to characterize inconsistencies in reporting through a comprehensive systematic review. METHODS: Following PRISMA guidelines, a systematic search of PubMed, Embase, and Scopus databases was conducted to identify studies that defined/described post-intervention continence in pediatric and young adult populations (≤30 years old) with BEEC. RESULTS: Of 1565 studies identified, 89 were included (n = 4057 subjects; 68% males). Classic bladder exstrophy was the primary condition in 49 studies (n = 2366), epispadias in 7 studies (n = 179), and cloacal exstrophy in 5 studies (n = 184). Twenty-eight studies assessed >1 condition, forming the "BEEC" subgroup (n = 1328). The most commonly utilized definition was "≥3-h dry interval in between voiding/catheterization" (50 studies; 56%). However, reporting was inconsistent regarding pad use, nighttime leakage, catheterization, and continence-promoting drug usage. Continence data were obtained from the patient/parent in 21 studies, while 66 did not specify whether continence was clinician- or patient-assessed. Use of the 3-h definition did not differ significantly by primary diagnosis (p = 0.69), study setting (single-vs. multicenter; p = 0.46), publication date (≥2012; p = 0.28), or country (p = 0.10). CONCLUSIONS: Definitions of continence in BEEC remain highly variable. The most frequently used definition of continence included 3-h dry intervals in between voiding/catheterization. However, there were omissions in key factors such as pad use, nighttime leakage, and catheterization. Establishing a standardized definition of continence is essential for measuring success in this critical outcome and ultimately improving patient care.
INTRODUCTION: As the exstrophy-epispadias complex (EEC) has not only physical, but also complex psychological consequences, further research is required to understand these in greater detail. This study aimed to test hyp...INTRODUCTION: As the exstrophy-epispadias complex (EEC) has not only physical, but also complex psychological consequences, further research is required to understand these in greater detail. This study aimed to test hypotheses concerning the association between shame, disclosure, friendship quality and life satisfaction among individuals with EEC, in order to identify specific areas for potential patient support. SAMPLE AND METHODS: During the pre-registered study, data were collected online and anonymously via an Instagram account and four support groups in Germany and Switzerland. This cross-sectional survey incorporated the "Chronic Illness-related Shame Scale", four subscales of the "McGill Friendship Questionnaire", and the "Short Scale to Measure General Life Satisfaction", with minor modifications. The disclosure scale was self-created and tested using exploratory factor analysis. The reliability of the scales ranged from acceptable to very good. For hypothesis testing, Pearson correlation and linear regressions were performed (α = 0.05). To examine sex differences, t-tests were conducted. RESULTS: 106 adults (47 men, 58 women, 1 divers or no answer) completed the questionnaire. On average, shame was reported in the lower to mid-range of the scale, while disclosure, friendship quality, and life satisfaction were reported at moderate to high levels. In line with the hypotheses, the analyses revealed significant negative correlations between EEC-related shame and social support and intimacy within the best friendship, life satisfaction, and the propensity to disclose EEC with approach goals, thus indicating an association between lower levels of shame and more positive social factors. Additionally, in affected friendships, a positive correlation between disclosure with approach goals and positive feelings, social support, and reliable alliance was found. Contrary to expectations, men reported a marginally insignificant tendency to experience more EEC-related shame than women. Women scored higher than men in terms of friendship quality and disclosure. DISCUSSION: In EEC, the level of shame and disclosure appears to be important in friendships. Furthermore, shame seems to play an important role in life satisfaction. Men, in particular, may be vulnerable in relation to most of these constructs. The results are largely consistent with previous research. Limitations of this study are especially the predominance of support groups and the underrepresentation of men. The findings suggest the need for practical support. CONCLUSION: The study emphasized the importance of psychosocial factors in EEC. Future research should continue to focus on psychosocial aspects of EEC, with a particular emphasis on health services research.
Vesicoscopic Cohen ureteral reimplantation is technically demanding but has good success. However, when the ureteral orifice is situated distally in close proximity to the bladder neck, there is little space for adequate...Vesicoscopic Cohen ureteral reimplantation is technically demanding but has good success. However, when the ureteral orifice is situated distally in close proximity to the bladder neck, there is little space for adequate submucous tunnel creation. In this tricky anatomical situation, we describe a technique of repositioning the ureteral hiatus cranially on the posterior bladder wall so that a good cross-trigonal tunnel can be created. This technique was employed by us successfully in 5 ureters with good success and no major complications. To our knowledge, this modified technique of vesicoscopic ureteral repositioning and cross-trigonal reimplantation has not been described before.