Mental health, psychosocial functioning, and health-related quality of life of children and adolescents with bladder exstrophy, cloacal exstrophy, and epispadias: A scoping review.

BACKGROUND: The exstrophy-epispadias complex (EEC) is a spectrum of rare congenital defects affecting the genitourinary system that includes epispadias, bladder exstrophy, and cloacal exstrophy. EEC patients may require several surgeries throughout their lifetime and experience a number of difficulties that may impact their quality of life (QoL), mental health, and psychosocial functioning. OBJECTIVE: The purpose of this scoping review is to systematically map existing literature that explores the mental health, psychosocial functioning, and QoL of children and adolescents with EEC. STUDY DESIGN: A search strategy on multiple bibliographic databases was created using a combination of Medical Subject Headings (MeSH) and keywords for the concepts of bladder exstrophy, cloacal exstrophy, or epispadias. RESULTS: The initial search identified 6303 unique articles. 254 articles were selected for full-text review, and 76 articles were identified for data extraction. Articles were excluded if they solely focused on adults. 25 articles are included in this review regarding child and adolescent experiences with EEC. DISCUSSION: Many child-focused studies utilized HRQoL measures to explore changes to an individual's quality of life after undergoing specific continence surgeries, with many studies finding improved QoL after surgery. As children age into adolescence, the research also demonstrates that experiences with QoL may change, with additional differences by gender. Body- and self-image seemed to become more of a concern as patients aged into adolescence, with special consideration for cloacal exstrophy patients who often undergo routine neonatal assignment genetic male to female. CONCLUSIONS: The existing literature suggests that current measures do not demonstrate that children and adolescents with EEC function differently than the general population, but children with EEC have a greater likelihood of experiencing a wide range of emotional and behavioral problems as they reach adolescence. Future studies should further explore manifestations of mental health and QoL throughout the life course.

Prevalence of disorders of sex development in patients with hypospadias and cryptorchidism.

BACKGROUND: Hypospadias and undescended testicles (UDT) are common congenital conditions, affecting approximately 1 in 125 and 1 in 33 boys, respectively. There has been limited contemporary data regarding the prevalence of Disorders of Sex Development (DSD) in patients with a history of both hypospadias and UDT. OBJECTIVE: Our objective is to determine the prevalence of DSD among patients presenting with hypospadias and UDT. DESIGN, SETTING, AND PARTICIPANTS: Electronic medical records were reviewed, and a retrospective chart review was conducted on 177 patients that were evaluated at our institution from 2000 to 2021 with a diagnosis of hypospadias and UDT. The degree of hypospadias, presence of and palpability of UDT, and prevalence and type of DSD were recorded. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: The degree of hypospadias, presence of and palpability of UDT, and prevalence and type of DSD were recorded. Chi-squared and Fisher's exact tests were conducted for descriptive statistical analyses as appropriate. Logistic regression analyses were conducted, with adjusted models including demographic and relevant clinical data. RESULTS: 177 patients were identified with both hypospadias and UDT, with 17/111 (15.3 %) diagnosed with DSD. The most common etiology of DSD was mixed gonadal dysgenesis (35.3 %; n = 6). Proximal hypospadias made up only 59.6 % of those without DSD vs. 100 % of those with confirmed DSD (p = 0.0044). A significantly smaller proportion of DSD patients had bilaterally palpable gonads compared to those without DSD (29.4 % vs. 79.8 %, p < 0.0001). Compared to those with palpable testes, patients with one non-palpable testicle had up to 26.67 times greater odds of DSD. CONCLUSIONS: To date, we present the largest cohort of patients that have undergone DSD work up for the combined presentation of hypospadias and UDT. Our findings highlight an increase in DSD diagnosis in proximal hypospadias patients with non-palpable UDTs, which offers further evidence and support for pursuing DSD diagnostic work-up in all proximal hypospadias patients with UDTs.

Human umbilical cord allograft associated with higher pediatric urethrocutaneous fistula repair success rates.

BACKGROUND: Urethrocutaneous fistula (UCF) formation is a known complication following hypospadias repair, affecting between 5 and 70% of cases. Moreover, approximately 30% of patients experience refistulization after UCF repair. The use of extracellular matrices, such as AmnioCord, a dehydrated human umbilical cord allograft manufactured by MiMedx®, may mitigate high rates of refistulization. OBJECTIVE: To determine whether the use of AmnioCord during UCF repair is associated with reduced incidence of subsequent UCFs among pediatric patients. DESIGN, SETTING, AND PARTICIPANTS: Electronic medical records were retrospectively reviewed among 60 patients who underwent at least one UCF repair at a pediatric hospital in a large urban setting in the U.S. between January 2012-June 2018. Patients were followed through January 2024. OUTCOME MEASUREMENTS AND STATISTICAL ANALYSIS: Adjusted generalized estimating equation regression models were used to assess the association between AmnioCord use and rates of refistulization, while controlling for confounding variables. RESULTS: The number of fistula presentations per patient ranged from one to four; 53.3% had one fistula, 30.0% had two, and 16.6% had three or more. Overall fistula repair success rate was 56.8% but significantly differed by AmnioCord use in adjusted modelling; 69.4% of cases that received AmnioCord were successfully repaired, compared to 47.2% of cases that did not receive AmnioCord. In adjusted models, patients who received AmnioCord had 2.66 times greater odds of surgical success than patients who did not receive AmnioCord (95% CI:1.13-6.24; p = 0.02). CONCLUSIONS: This study demonstrates a positive association between AmnioCord use and successful UCF closure among pediatric patients.

Incidence and predictors of delays in starting fellowship training in urology.

INTRODUCTION: The completion of residency and start of fellowship training marks a critical transition for urologists in the pursuit of subspeciality training. Most graduating urology residents are under contract until June 30, and most fellowships are scheduled to begin on July 1. There has been no investigation into the practical implications of fellowship delays in urology from a trainee perspective. Our research study aimed to investigate the incidence and predictors of delays in fellowship starts. METHODS: Pediatric urology fellows that began their fellowship training between 2019 and 2023 and endourologic fellows that began their fellowship training between 2017 and 2022 were surveyed using SurveyMonkey®. A total of 250 endourology (EU) fellows and 90 pediatric urology (PU) fellows were contacted. RESULTS: A total of 26.0% and 14.3% of EU and PU fellows, respectively, experienced a delay in their training, despite many leaving their residency positions early (33.8% vs. 44.9%, p=0.2097); 11.7% and 8.2% of EU and PU fellows, respectively, experienced delays they reported to be "very stressful" and 9.1% and 4.1%, respectively, found them "somewhat stressful." Delays of 2-4 weeks were experienced by 5.2% and 6.1%, 4-6-week delays by 7.8% and 4.1%, and delays >6 weeks by 2.6% and 0% of EU and PU fellows, respectively (p=0.0007). CONCLUSIONS: Delays in fellowship training do occur at a notable rate, despite nearly half of urology fellows leaving their residency training positions early, with unclear impacts on patient care and resident colleague well-being. This research highlights the importance of fellowship programs considering delaying fellowship starts to mid-July or August, with support of the prior fellow cohorts.

Long-Term outcomes of cecostomy tube insertion for patients with bowel dysfunction: A retrospective review.

BACKGROUND: Constipation is common in the pediatric population and in severe forms it can lead to debilitating fecal incontinence which has a significant impact on quality of life. Cecostomy tube insertion is a procedural option for cases refractory to medical management, however there is limited data investigating the long-term success and complication rate. METHODS: A retrospective review was performed evaluating patients at our centre undergoing cecostomy tube (CT) insertion between 2002 and 2018. The primary outcomes of the study were the rate of fecal continence at 1-year, and the incidence of unplanned exchanges prior to annual scheduled exchange. Secondary outcomes include the frequency of anaesthetic requirements and length of hospital stay. Descriptive statistics, t-test, and chi-square analysis was performed where appropriate using SPSS v25. RESULTS: Of 41 patients, the average age at the time of initial insertion was 9.9 years with the average length of stay in hospital being 3.47 days. The most common etiology of bowel dysfunction was spina bifida, which was present in 48.8% (n = 20) of patients. Fecal continence was achieved in 90% (n = 37) of patients at 1 year and the average rate of cecostomy tube exchange was 1.3/year with an average of 3.6 general anaesthetics being required by patients and the average age of no longer requiring one being 14.9 years. DISCUSSION: Analysis of patients undergoing cecostomy tube insertion at our centre has further supported the use of cecostomy tubes as a safe and effective option for management of fecal incontinence refractory to medical management. However, a number of limitations exist in this study including its retrospective design and failure to investigate changes in quality of life using validated questionnaires. Additionally, while our research provides greater insight to practitioners and patients what degree of care and types of complications or issues they may encounter with an indwelling tube over the long-term, our single-cohort design limits any conclusions that could be made regarding optimal management strategies for overflow fecal incontinence through direct comparison with other management strategies. CONCLUSIONS: CT insertion is a safe and effective method for managing fecal incontinence due to constipation in the pediatric population, however, unplanned exchange of tube due to malfunction, mechanical breakage, or dislodgment occurs frequently and may impact quality of life and independence. LEVEL OF EVIDENCE: IV.

Renal outcomes of children born with posterior urethral valves at a tertiary center: A 15-year retrospective review.

INTRODUCTION: Posterior urethral valves (PUVs) is a congenital condition in which an obstruction in the urethra prevents drainage of urine from the bladder in males, with up to 60% of children diagnosed developing chronic kidney disease (CKD). The primary aim of this study was to identify novel factors that may predict development of CKD and end-stage real disease (ESRD ) in children with PUVs to potentially address modifiable factors and delay progression. The secondary aim was to compare rates of catheterization and incontinence between our patients and other case series to provide information to parents about long-term bladder outcomes. METHODS: A single-center, retrospective cohort study was performed of all children referred to our multidisciplinary clinic for PUV diagnosis between 2005 and 2019. Univariable associations of different variables with the composite outcome CKD or ESRD were evaluated. RESULTS: Thirty of 46 patients (65%) developed CKD, with the majority (40%) being stage 2 CKD (n=12). Seven of 30 patients (23%) developed ESRD requiring renal replacement therapy. Fourteen of 26 (30%) required clean intermittent catheterization (CIC) initiation, with a median CIC initiation age of 4.3 years. Creatinine nadir post-valve ablation, oligohydramnios, and initiation of CIC are significant predictors of CKD development. CONCLUSIONS: This review reiterates that children born with PUVs have a high morbidity rate, with a high proportion developing CKD. Using a multidisciplinary approach to PUV patient care allows for better family education, early intervention of bladder dysfunction, and possibly better long-term preservation of renal function.

Characterizing patients with multiple same-sided ureteric stones.

PURPOSE: Patients presenting with multiple same-sided ureteric stones (MSSUS) are a unique population with nuanced prognostic and treatment considerations, which have yet to be characterized in the literature. Therefore, our purpose was to examine outcomes of patients with MSSUS vs those with single ureteric stones (SUS). METHODS: A retrospective review of prospectively collected patients included adults (> 18yo) with [Formula: see text] 2 ipsilateral ureteral stones without prior treatment for their current stone burden. A historical comparison group was used as a control population. Univariate logistic regression analyses and descriptive statistics were performed with SPSS® 20.0 (p < 0.05). RESULTS: Seventy-nine MSSUS patients were compared to 101 SUS patients. MSSUS patients had 2.21 [Formula: see text] 0.66 ureteric stones and had significantly smaller lead stones (MSSUS 6.4 mm vs SUS 7.2 mm, p = 0.03). MSSUS patients were more likely to have had prior stones (66 vs 42%) and 5.9 times more likely to have had prior stone procedures. Conservative management was successful in 30% MSSUS vs 19% SUS (p = 0.073), and there were no differences in resolution time (p = 0.44). For patients proceeding to intervention, (MSSUS n = 52, SUS n = 78), ureteroscopy was performed on 58% MSSUS vs 51% SUS patients (p = 0.302), and shockwave lithotripsy in 10% MSSUS vs 26% SUS (p = 0.01). MSSUS were more likely to spontaneously pass a stone prior to planned intervention (OR = 41.1; 95% CI = 12.0-140.7; p = 0.0001). CONCLUSION: MSSUS patients are more likely recurrent stone formers with extensive stone histories and current stone burdens. Conservative management appears as successful between groups, with no difference in resolution time. When employed, ureteroscopy outcomes do not different between cohorts.

Understanding failures in getting it up: The prevalence and predictors of failed ureteral access in ureteroscopy.

INTRODUCTION: Failed access ureteroscopy (FA) describes the inability to gain adequate access to a stone to allow for treatment. The purpose of this study was to identify the prevalence of, and factors predicting FA in patients presenting with renal and ureteral stones. METHODS: We conducted a retrospective review of all ureteroscopy (URS) procedures performed for renal and ureteral stones by three endourologists over a six-month period at our center. All patients who underwent URS for the purpose of stone treatment were included. Patients were excluded if they underwent URS for non-stone diagnosis or treatment. FA was investigated in relation to demographics, medical history, stone-specific characteristics, procedure-specific characteristics, etc. Statistical analysis consisted of descriptive statistics, as well as Chi-squared and t-test analysis using SPSS statistical software version 24.0. RESULTS: A total of 188 cases were reviewed, with 8% of patients experiencing FA. Patient age, gender, body mass index (BMI), American Society of Anesthesiologists (ASA) score, emergency cases, previous stone treatment, use of computed tomography (CT) imaging, presence of hydronephrosis, and surgeon did not differ significantly between FA and successful access (SA) groups. Stone size (9.88±5.8 vs. 8.76±4.3 mm; p=0.361) was also not significantly different. However, a significant difference was noted in time from first diagnosis to URS (128 vs. 65 days, p=0.044) between the FA and SA groups, respectively. Similarly, for ureteral stones, the FA group had a significantly greater proportion of stones located in the proximal ureter (62.5% vs. 22.0%, p=0.043). CONCLUSIONS: Proximal ureteric stones were more likely to result in FA URS, and FA procedures were more likely to be preceded by extended time from first diagnosis to URS. Further investigation is necessary, and all endourology centers should track their own personal outcome data to allow for more meaningful analysis to be performed to improve patient outcomes.

The association between rurality and return to work for workers' compensation claimants with work-related musculoskeletal injuries: An analysis of workers who failed to return to work within typical healing time frames.

OBJECTIVES: The objectives of this study have been to: 1) describe and compare urban and rural injured worker populations in Alberta, Canada; 2) identify return-to-work outcomes in urban and rural populations; 3) examine the relationship between geographic location of residence and recovery from work-related musculoskeletal injury; and 4) investigate if this relationship is attenuated after controlling for other known risk factors. MATERIAL AND METHODS: This study was a secondary analysis utilizing data of a population of musculoskeletal injury claimants who underwent clinical/RTW (return to work) assessment between December 2009 and January 2011 collected by the Workers' Compensation Board of Alberta. Descriptive statistics were computed for 32 variables and used for comparing urban and rural workers. The logistic regression analysis was performed to test the association between geographic location of residence and likelihood of return-to-work. RESULTS: Data on 7843 claimants was included, 70.1% of them being urban and 29.9% - rural. Rural claimants tended to have spent less time in formal education, have a blue-collar job, have no modified work available, have a diagnosed comorbidity, and not been enrolled in a specialized rehabilitation program. They were 1.43 (1.12-1.84) times the odds more likely than urban claimants to be continuing to receive full disability benefits 90 days after their RTW assessment, and 1.68 (1.06-2.67) times the odds as likely to report a recurrence of receiving disability benefits. CONCLUSIONS: Rural residence was associated with prolonged work disability, even after controlling for age, job type, education level, health utilization and other potential confounders. Further research is required to explore why injured workers in rural settings experience prolonged reception of disability benefits and have greater rates of recurrence of receiving disability benefits. Int J Occup Med Environ Health 2017;30(5):715-729.