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

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

Abnormal penile foreskin development in hypospadias is the most frequent genital malformation in male children, which has increased dramatically in recent decades. A number of environmental factors have been shown to be associated with hypospadias development. The current study investigated the role of epigenetics in the etiology of hypospadias and compared mild (distal), moderate (mid shaft), and severe (proximal) hypospadias. Penile foreskin samples were collected from hypospadias and non-hypospadias individuals to identify alterations in DNA methylation associated with hypospadias. Dramatic numbers of differential DNA methylation regions (DMRs) were observed in the mild hypospadias, with reduced numbers in moderate and low numbers in severe hypospadias. Atresia (cell loss) of the principal foreskin fibroblast is suspected to be a component of the disease etiology. A genome-wide (> 95%) epigenetic analysis was used and the genomic features of the DMRs identified. The DMR associated genes identified a number of novel hypospadias associated genes and pathways, as well as genes and networks known to be involved in hypospadias etiology. Observations demonstrate altered DNA methylation sites in penile foreskin is a component of hypospadias etiology. In addition, a potential role of environmental epigenetics and epigenetic inheritance in hypospadias disease etiology is suggested.

PurposeWith advances in treatment modalities and medical knowledge, girls with congenital urologic disorders are living well into adulthood. Although, sexual and reproductive function in this population is still poorly understood. The aim is to review existing literature about fertility and sexuality in women with congenital genitourinary disorders, including spina bifida (SB), bladder exstrophy–epispadias complex (BEEC) and congenital adrenal hyperplasia (CAH).MethodsThis review represents the joint SIU-ICUD (Société Internationale d’Urologie—International Consultation on Urological Disease) consultation on congenital lifelong urology. The results of this analysis were first presented at a joint consultation of the SIU and ICUD at the 2018 SIU annual conference in Seoul, South Korea. Appropriate experts were asked to write specific sections regarding sexuality and reproductive function in female patients with these complex congenital urogenital disorders. Each expert performed their own literature review which was reviewed by GDW, AFS, Hadley M. Wood and Dan Wood. Expert opinion was obtained where data are non-existent.ResultsOnly about half of the individuals with SB express a satisfactory sex life. In women with BEEC, cosmetic concerns surrounding genital appearance and function may increase psychological distress, including severe depression, suicide and sexual dysfunction. Professional health care is key for improving self-esteem and to interact in the biopsychosocial model of the quality of life. Patients with SB and BEEC should be informed about all the potential risks and difficulties before, during and after pregnancy. Screening for pelvic organ prolapse is important as it can exacerbate their already existing sexual dysfunction, difficulties achieving pregnancy and challenges with clean intermittent catheterization.ConclusionsLifelong multidisciplinary follow-up and management are complex but necessary. As these patients grow into their adolescence, they may have the desire to become involved in personal relationships and have sexual interactions. Their healthcare team needs to be increasingly sensitive to these aspects.

IntroductionThe strength of the evidence base for the comparative effectiveness of three common surgical modalities for paediatric nephrolithiasis (ureteroscopy, shockwave lithotripsy and percutaneous nephrolithotomy) and its relevance to patients and caregivers are insufficient. We describe the methods and rationale for the Pediatric KIDney Stone (PKIDS) Care Improvement Network Trial with the aim to compare effectiveness of surgical modalities in paediatric nephrolithiasis based on stone clearance and lived patient experiences. This protocol serves as a patient-centred alternative to randomised controlled trials for interventions where clinical equipoise is lacking.Methods and analysisThe PKIDS is a collaborative learning organisation composed of 26 hospitals that is conducting a prospective pragmatic clinical trial comparing the effectiveness of ureteroscopy, shockwave lithotripsy and percutaneous nephrolithotomy for youth aged 8–21 years with kidney and/or ureteral stones. Embedded within clinical care, the PKIDS trial will collect granular patient-level, surgeon-level and institution-level data, with a goal enrolment of 1290 participants over a 21-month period. The primary study outcome is stone clearance, defined as absence of a residual calculus of >4 mm on postoperative ultrasound. Secondary outcomes include patient-reported physical, emotional and social health outcomes (primarily using the Patient-Reported Outcome Measurement Information System), analgesic use and healthcare resource use. Timing and content of secondary outcomes assessments were set based on feedback from patient partners. Heterogeneity of treatment effect for stone clearance and patient-reported outcomes by participant and stone characteristics will be assessed.Ethics and disseminationThis study is approved by the central institutional review board with reliance across participating sites. Participating stakeholders will review results and contribute to development dissemination at regional, national and international meetings.Trial registration numberNCT04285658; Pre-results.

Purpose of Review As patients with spina bifida are living longer, more long-term outcomes are being reported. Today, the conversation surrounding neurogenic bladder care has evolved from reducing mortality to enhancing quality of life, namely, reducing urinary incontinence while still preserving upper tract function. In this paper, we seek to review risks of bladder malignancy in neurogenic bladder patients with history of augmentation or chronic catheters. Recent Findings Recent data has demonstrated that cancers in bladders with prior augmentation or chronic catheters tend to be advanced, aggressive, and occur at a younger age. However, bladder cancer in this population is difficult to predict and difficult to diagnose before it is too late. Summary Screening protocols and early diagnostic tools remain elusive as these cancers may present with symptoms that can be easily overlooked or attributed to other disease processes. Current work with biomarkers may yield better diagnostic tools than cystoscopy or cytology.

Background Kidney echogenicity is typically determined subjectively but may have a quantifiable relationship to kidney function. Similarly, kidney length has been shown to correlate with kidney function. This study sought to quantify echogenicity using readily available software. Secondarily, we aimed to evaluate the correlation between quantified echogenicity and kidney length to the estimated glomerular filtration rate (eGFR) in children with acute kidney injury (AKI) and chronic kidney disease (CKD). Methods In a single-center retrospective observational study, echogenicity index (EI) was determined using a ratio of right kidney to liver mean pixel density. The kidney length ratio (KLR) was determined by the actual to predicted lengths of both kidneys. Both variables were correlated to eGFR using correlation analyses and predictive capacity was determined with receiver operating characteristic curve (ROC) analysis. Results Of 94 subjects, 46% (43/94) had AKI, 28% (26/95) had CKD and 26% (25/95) were controls. The higher the EI the lower the eGFR ( r  = − 0.46, p  < 0.0001). EI between 1.0 and 1.1 predicted an eGFR < 90 ml/min/1.73m 2 with an AUC of 0.71–0.78 while an EI between 1.1 and 1.2 predicted an eGFR < 60 ml/min/1.73m 2 with AUC of 0.75–0.80. Overall, the larger the KLR the lower the eGFR ( r  = − 0.25, p 0.018). Conclusion We have developed an accessible methodology to quantify kidney echogenicity. Overall, there was an inverse correlation between EI and eGFR in pediatric CKD and AKI. However, these correlations did not persist within subgroups which could be due to small sample size and heterogeneity of etiologies. Overall, KLR had a weaker correlation to eGFR, compared to EI. Despite these correlations, both EI and KLR had “fair” to “good” performance as a biomarker for an eGFR < 60 ml/min/1.73m 2 .

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

Purpose Health-related quality of life (HRQOL) is important in spina bifida (SB) management. No clinically useful, comprehensive instrument incorporating bladder/ bowel domains exists. We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A). Methods We drafted the 53-question pilot instrument using a comprehensive item generation/refinement process. It was administered to an international convenience sample of adults with SB and controls recruited online via social media and in person at outpatient SB clinics (January 2013-September 2014). Final questions were determined by: clinical relevance, high factor loadings and domain psychometrics in an Internal Validation Sample randomly selected from United States participants (n = 250). External validity was evaluated in United States and International External Validation Samples (n = 165 and n = 111, respectively). Adults with SB completed the validated general WHOQOL-BREF and International Consultation on Incontinence Questionnaire (ICIQ). Results Mean age of 532 participants was 32 years (32.7 % males, 85.0 % Caucasian), similar to 116 controls (p ≥ 0.08). There were 474 online and 58 clinical participants (61.1 % eligible). Face validity and content validity of the 3-domain, 15-question QUALAS-A were established by patients, families and experts. Internal consistency and test-retest reliability were high for all domains (Cronbach's alpha ≥ 0.70, ICC ≥ 0.77). Correlations between QUALAS-A and WHOQOL-BREF were low (r ≤ 0.60), except for high correlations with Health and Relationships domain (0.63 ≤ r ≤ 0.71). Bladder and Bowel domain had a high correlation with ICIQ (r = -0.70). QUALAS-A scores were lower among adults with SB than without (p < 0.0001). QUALAS-A had good statistical properties in both External Validation Samples (Cronbach's alpha 0.68-0.77). Conclusions QUALAS-A is a short, valid HRQOL tool for adults with SB.

Robot-assisted laparoscopic pyeloplasty (RALP) is a minimally invasive treatment for ureteropelvic junction obstruction (UPJO). The learning curves for RALP have been well-documented, primarily in post-fellowship surgeons, but the impact of trainee involvement during fellowship training remains unclear. This study aims to (1) establish a large series post-fellowship single surgeon learning curve and (2) analyze the impact of annual trainee involvement on surgical proficiency using cumulative sum (CUSUM) analysis. A retrospective review of pediatric RALP cases from July 2012 to June 2023 at a high-volume institution was conducted. Cases performed by a single post-fellowship surgeon were included. Concomitant procedures and redo surgeries were excluded. Operative time (OT) and complication rates (CR) were tracked using CUSUM analysis. The learning curves were categorized into phases: learning, competency, case-mix, and proficiency. The effect of annual fellowship trainees was analyzed by constructing normalized learning curves per academic year. A total of 220 cases were analyzed. The post-fellowship surgeon reached competency after 29 cases, with proficiency achieved after 99 cases. Trainee involvement demonstrated a characteristic “down-up-down” pattern in OT and was not associated with increased CR during each of 6 years analyzed, reflecting an initial observation phase, increased operative time during hands-on learning, and eventual improvement. In contrast, the single year without a trainee showed a continuous downward OT trend. The complication rates remained below unacceptable thresholds and case complexity influenced OT trends. CUSUM-based analysis effectively maps the RALP learning curve, revealing distinct phases of surgical proficiency and the impact of trainee involvement. The reproducible trainee learning pattern provides insights into optimizing robotic surgery education while maintaining patient safety.