Four hour voiding observation study in infants with posterior urethral valves.

INTRODUCTION AND AIM: Posterior urethral valves (PUV) are often associated with bladder dysfunction. Our primary aim was to investigate bladder status following primary valves resection to gather evidence of function and to guide early clinical management. PATIENTS AND METHODS: Between July 2015-2020, we prospectively evaluated bladder function of PUV infants. Primary outcomes measured were number of voids, voided volume (VV), post-void residual (PVR) and bladder capacity (BC). Statistical comparisons and descriptive analysis were carried out between groups using 2-tails T test and Chi square test using an IBM SPSS Statistics program version 25. RESULTS: Sixty-one infants were included. Median age at resection was 28 days (5 days-11 months). In thirty-eight patients (62%) diagnosis was suspected antenatally. Vesico-ureteric reflux (VUR) was present in 16 (26%). The 4-h observational study was performed at median 4 days following primary cold-knife valve resection and 1 day (range from 1 to 12 days) after catheter removal. Infants voided an average 6 times (2-13 voids). Bladder capacity was large in 20 (33%) and small in 4 (6.5%) patients with a median ratio BC/eBC = 1.2 (0.49-22.6). Median residual urine was 9 mls but with great variability among the cohort (0-121 mls). A big variability in bladder emptying was noted between patients and between voids performed in one patient. In 13 infants (21%) average PVR was larger than the average voided volume. In 26 (42.6%) PVR was larger than VV at least once. Thirty-eight infants (62.3%) emptied their bladders almost completely at least once (with PVR <5 mls). No difference was found in PVR, Median BC/EBC and PVR/BC between infants with and without VUR (p = 0.654, P: 0.594 and p = 00.481, respectively). DISCUSSION: We presented our experience of non-invasive bladder function assessment in infants affected by PUV following primary valve resection. An interesting data is the great variability identified both between patients and between voids performed in a single patient. Average number of voids was similar to the voiding pattern in healthy newborns what reported by Gladh but variability was greater. We observed up to 13 voids in some infants and we can speculate this could reflect the presence of an overactive bladder. We observed at least 1 complete emptying in only 62% of infants and 21% of the cohort had PVR bigger than VV. In almost half of the infants (42.6%) PVR was larger than VV at least once. CONCLUSIONS: Around 40% of infants affected by PUV have abnormal bladder capacity and almost half of them have significant post void residuals following primary resection. Although controversies and limitations are present, we believe that this non-invasive study can provide valuable information to understand the dynamic of the bladder, particularly in children affected by PUV and allow early intervention in children considered "at risk". Having a non invasive way of assessing can help tailor intervention and be useful for future research into early bladder intervention and improving outcomes.

Elevating pediatric urology care: The crucial role of nursing research in quality improvement.

This educational article highlights the critical role of pediatric urology nursing research in improving the care of children with urological conditions. It discusses the multifaceted nature of pediatric urology nursing, addresses challenges such as limited nurse scientists and resource constraints, and highlights the need to overcome barriers to increase research involvement. The authors emphasize the importance of prioritizing research areas, the promotion of collaboration, and the provision of adequate funding and academic time for pediatric nurses to contribute to evidence-based practice, to improve patient outcomes. Furthermore, it highlights the importance of research in advancing nursing practice, shaping protocols, and advocating for the rights and needs of children with urological conditions and their families.

The importance of animal specificity in animal experimentation, part I: Anatomy in relation to pediatric urology.

Why and when is animal experimentation relevant? The answer to this question depends on the research question. In this short educational article we aim to raise awareness of the importance of formulating a very specific research question before choosing an animal species. An awareness of anatomical and physiological differences vis-a-vis similarities between species, will increase the potential for obtaining data that is relevant for translation to human conditions.

The importance of animal specificity in animal experimentation, part II: Physiological challenges and opportunities in relation to pediatric urology.

When performing animal experimentation in Pediatric Urology studies, it is important to be aware of physiological differences between species and to understand when relevant disease models are available. Diseased animal models may be more relevant in many cases, rather than performing studies in healthy and normally developed animals. For example, they may be more appropriate for the study of congenital malformations, to investigate the secondary effects of prenatal urinary obstruction, to study the effect of prenatal exposure to endogenous or exogenous factors which may lead to disease, or in testing bioengineered structures. In this short educational article, we aim to describe some disease models that have been used to simulate human pathologies and how, if properly designed, these studies can lead to important new knowledge for human translation. In addition, we also highlight the importance of formulating a research question(s) before deciding on the animal experimental model and species to choose.

A consensus process to identify research priorities in paediatric urology in the United Kingdom.

INTRODUCTION: Paediatric urologists manage a spectrum of conditions, much of the evidence for relevant treatment pathways is of low quality. For many conditions treatment varies according to location and surgeon; children with the same condition might have surgery in one unit but watchful waiting in another. Underlying this variation are differences in opinion, and insufficient high-level evidence with few prospective randomized studies. Such studies may be challenging to design, fund and recruit into, and are more likely to succeed if there is a collaborative approach. Research prioritization is a tool to identify the research of most value. Delphi methodology is an interpretive technique aiming to gain the consensus view of interested parties. The British Association of Paediatric Urologists (BAPU) set out to ascertain consensus on what paediatric urologists, working in the UK, consider to be areas of priority for research. This paper describes the process used, and the resulting list of research questions. METHODS: A scoping survey of paediatric urologists in the UK was undertaken to identify an initial set of research questions. These were refined by the BAPU research committee (BAPU RC), then prioritized using a modified Delphi process. During Stage 1a multiple new research questions were submitted leading to Stage 1b, an interim process. All UK paediatric urologists were invited to take part in Stage 2 of the prioritization process. RESULTS: Sixty-five questions were submitted to the scoping survey by 24 paediatric urologists. The BAPU RC refined these to 60 questions, which were submitted to Stage 1a of the modified Delphi process. Sixty-seven people completed Stage 1a, at the same time submitting 224 additional research questions. The BAPU RC revised the entire question set, ensuring the key subject of the original question was not altered and novel questions were retained. The BAPU RC undertook interim scoring of the resultant 79 questions, the top scoring 25 questions plus 5 lower scoring 'wild card' questions (to ensure the breadth of the specialty was represented) were put forward to Stage 2. A total of 65 people completed Stage 2, including a lay representative. A list of 30 priority research questions was generated; the top 10 includes management of neuropathic bladder, posterior urethral valves, antibiotic prophylaxis, DSD & CAH, continence, male external genitalia, VUR and transition care (Table). CONCLUSION: This process has provided BAPU, paediatric urologists in the UK, and funders with areas of research considered a priority in the specialty.

Are there anatomical limiting factors to foreskin reconstruction at the time of single-stage hypospadias repair?

INTRODUCTION: Foreskin reconstruction (FR) at the time of primary hypospadias repair is a truly anatomically complete reconstruction of the hypospadic penis. We prospectively collected penile and preputial measurement of children undergoing single-stage hypospadias repair and FR with the aim to identify possible relations between penile and preputial anatomy and the likelihood to develop complications. MATERIALS AND METHODS: We prospectively studied children who underwent single stage hypospadias repair associated with FR from 2016 to 2019. We recorded intra-operative foreskin and penile measurements and post-operative outcomes. Logistic Regression analysis was performed to explore independent factors affecting urethroplasty and skin complications. Chi square test was used to compare outcomes in different groups based on ventral foreskin defect (VFD) width, Glans size, age at surgery and meatal location. RESULTS: From a total of 181 consecutive patients, 86 boys who underwent a single stage hypospadias repair combined with FR were included in the study. Patients were excluded because they were either lost at follow up (n = 10), required a 2-stage repair (n = 2), were circumcised at birth (n = 3) or parents requested a circumcision (n = 78); in 2 patients, a decision to perform circumcision was made intraoperatively due to aesthetic reasons (monk-hood deformity of the prepuce). Median age at surgery was 17 months. Mean glans width was 14.4 mm. Mean unstretched and stretched foreskin circumference were 29.5 mm and 40.9 mm, respectively. Mean VFD (the distance between the proximal insertion of the foreskin hood on either side of the midline at the level of the coronal sulcus) was 7.2 mm (Fig. 1). At median follow-up of 8 months (6-23), 9 complications were recorded (10.4%): foreskin dehiscence occurred in 1% (1/86), a foreskin fistula was noted in 4.6% (4/86), tight, non-retractile, foreskin in 1% (1/86); urethrocutaneous fistula in 2.3% (2/86) and complete dehiscence of the glans and foreskin in 1 (1.2%). Multiple logistic regression analysis demonstrated that none of the measurements obtained was an independent risk factor for developing urethroplasty or skin complications. There was no significant difference in complications between wide VFD (>7 mm) vs. narrow VFD (≤7 mm), large glans (>14 mm) vs. small glans (≤14 mm), age at surgery ≤24 months vs. > 24 month and meatal location distal (glanular, coronal, subcoronal and distal penile) vs. proximal (midpenile, proximal penile and penoscrotal). CONCLUSION: To the best of our knowledge, this is the first study reporting a prospective and objective assessment of the foreskin in the context of single stage hypospadias repair. Individual anatomical differences in preputial and penile anatomy do not seem to affect the likelihood of skin and urethroplasty complications. FR can, therefore, be offered to all boys undergoing primary single stage hypospadias repair . Further studies on larger numbers and external validation of these measurements is necessary.

Posterior Urethral Valves, Unilateral Vesicoureteral Reflux, and Renal Dysplasia (VURD) Syndrome: Long-Term Longitudinal Evaluation of the Kidney Function.

The presence of unilateral vesicoureteral reflux (VUR), and renal dysplasia associated with posterior urethral valves (PUV) (VURD syndrome) was believed to represent a pressure-released pop-off mechanism protecting kidney function. We aimed to investigate its role with respect to long-term kidney function in a cross-sectional and longitudinal analysis. We compared the iohexol glomerular filtration rate (GFR) measured at 5 (GFR5) and 10 (GFR10) years of age in children with (Group A) and without (Group B) VURD syndrome, who underwent PUV resection under 2 years of age. VURD syndrome was diagnosed in cases of unilateral loss of kidney function (<15% on nuclear medicine test) associated with ipsilateral grade IV-V VUR. VURD syndrome was diagnosed in 16 (12.8%) out of 125 patients who met the inclusion criteria. While the median GFR5 was similar in the 2 groups [Group A: 87.3 (74.7-101.2) mL/min/1.73 m2 vs. Group B: 99.6 (77-113) mL/min/1.73 m2, p-value: 0.181], the median GFR10 values were significantly lower in children with VURD syndrome [Group A: 75.7 (71.2-85.9) mL/min/1.73 m2 vs. Group B: 95.1 (81.2-114.2) mL/min/1.73 m2, p-value: 0.009]. Similar results were obtained in a longitudinal analysis of the children with GFR measurement available both at 5 and 10 years of age [GFR5 in Group A: 93.1 (76.9-103.5) mL/min/1.73 m2 vs. Group B: 97.5 (80-113) mL/min/1.73 m2, p-value: 0.460; GFR10: Group A: 71.9 (71.9-85.9) mL/min/1.73 m2 vs. Group B: 94.8 (81.5-110.6) mL/min/1.73 m2, p-value: 0.024]. In conclusion, VURD syndrome does not show a protective role in kidney function preservation. On the contrary, it seems to be associated with a deterioration of the kidney function on a long-term follow-up.

Morbidity following suprapubic line insertion for videourodynamics in children.

INTRODUCTION: Previously in our unit, urodynamics were delayed after insertion of suprapubic (SP) lines. We postulated that performing urodynamics on the same day as SP line insertion would not result in increased morbidity. We retrospectively compared complications in those having urodynamics on the same day against those who had delayed urodynamics. PATIENTS AND METHODS: Notes were reviewed for patients undergoing urodynamics via SP lines from May 2009 until December 2018. In 2014 we modified our practice to allow urodynamics on the same day as SP line insertion in some patients. Patients undergoing videourodynamics would have two 5 Fr (mini Paed) SP lines inserted under general anaesthesia. Patients were divided into two groups: those that had urodynamics on the same day as SP line insertion and those that had urodynamics after an interval of more than one day. The outcome measure was the number of problems affecting those in each group. The two groups were compared using Mann-Whitney U tests and Fisher's Exact tests. RESULTS: There were a total of 211 patients with a median age of 6.5 years (range three months to 15.9 years). Urodynamics were performed on the same day in 86. Delayed Urodynamics were performed at an interval of more than one day in 125. Adverse events included pain or difficulty with voiding, increased urinary frequency, urinary incontinence, leak from catheter site, extravasation, extension of in-patient stay, visible haematuria, urethral catheterisation, and urinary tract infection. Problems affected 43 (20.4%) children. In the same day group, 11 (13.3%) patients had problems, in the delayed group 32 (25.6%) had problems; this was statistically significant (p = 0.03). The difference in combined incidence of important problems (requiring urethral catheterisation, extended admission or abandonment of urodynamics) was not statistically significant between the two groups. CONCLUSION: When using suprapubic catheters for urodynamics there is no additional morbidity when catheters are inserted on the same day as the urodynamics study compared to when urodynamic are delayed.

Surgical management of complicated duplex kidney: A tertiary referral centre 10-year experience.

Aim: The management of a complicated duplex kidney remains a challenge for paediatric urologists. The aim of this study is to report a 10-year experience of the surgical management of complicated duplex kidney in a single tertiary care paediatric referring hospital. Materials and Methods: Clinical records of all children who undergone a surgical procedure for complicated duplex systems between January 2009 and March 2019 at our institution were retrospectively reviewed. Clinical manifestations, surgical procedures, complications and follow-up were collected and analysed. Logistic regression was performed to explore if any patient's characteristic or underlying associated comorbidity (ureterocoele, ectopic ureter, obstruction, etc.,) could be positively linked to the chance to develop recurrent urinary tract infections (UTIs). Results: We have identified 95 children who received a surgical treatment for 102 complicated duplex kidneys. The presence of an ureterocoele was recorded in 41 (43.2%) patients, an ectopic ureter in 25 (26.3%), a vesicoureteral reflux (VUR) in 40 (42.1%), a vesicoureteric junction obstruction in 24 (25.3%) and an ureteropelvic junction obstruction in 3 (3.2%). An invasive approach such as an heminephrectomy (71.6%) was required in the majority of cases. Higher risk of developing a UTI has been demonstrated in children diagnosed postnatally (P < 0.001) and in those with an associated obstruction (P < 0.05). Conclusions: No standardised management could be recommended for the surgical treatment of complicated duplex kidney. Children without antenatal diagnosis and with either an upper tract obstruction or VUR are at greater risk to develop UTI and need to be looked after more closely.

How accurate is eyeball measurement of curvature? A tool for hypospadias surgery.

INTRODUCTION: Correction of penile curvature or "chordee" is a major component in the management of hypospadias. Accurate assessment and management of penile curvature influence both short- and long-term outcomes of surgery. AIM OF THE STUDY: The objective of this study is to investigate the accuracy of eyeball measurement and how does it compare to objective measurement by standard goniometry (SG) and smartphone app goniometry (AG). MATERIALS AND METHODS: A Dropbox file request link was shared with paediatric urologists on various social media platforms requesting participants to upload a picture of their index finger showing what they thought 30 degrees of curvature look like using their proximal inter phalangeal joint as the point of maximal curvature., The images were assessed using SG to measure the angle of curvature. The images were also assessed using AG by the principal investigator, a physician, a scrub nurse and a paediatric urology consultant., Statistical analysis was performed using SPSS statistics software version 26 (Armonk, NY: IBM Corp). A one sample t-test and a one-way chi squared test were used to evaluate significant frequency differences. Pearson correlation was used to compare AG measurements to test intra- and inter-observer reliability and to compare AG measurements vs SG measurements. Assuming 5-degree variability in goniometer measurements and 2-degree difference between the sample and population, the number of participants needed was calculated to be 49. RESULTS: Fifty-two responses were received.32.7% of respondents simulated 30° accurately (17/52). A significant proportion (23/52, 44.2%) overrepresented the degree of curvature and 23.1% (12/52) underrepresented it (p = 0.01). Compared with objective measures, eyeball estimates differed by an average of 10° ± 1.5 SE. Measurements obtained by AG were comparable to measures obtained by SG and showed excellent intra-observer and inter-observer correlation (R = 0.983, P < 0.001). DISCUSSION: We demonstrated a significant discrepancy between eyeball assessment of curvature and objective measurements in a cohort of hypospadiologists. This can be very relevant to intraoperative decision making. The limitation of the study is the use of a simulated model rather than assessment of curvature in patients with hypospadias. Another limitation is the lack of standardization of the way the pictures were taken. CONCLUSION: We demonstrated a tendency among hypospadiologists to overestimate or underestimate curvature by an average of 10° on eyeball assessment. The use of App Goniometry shows excellent interobserver reliability and is comparable to standard goniometry in curvature assessment.

Reporting ChAracteristics of cadaver training and sUrgical studies: The CACTUS guidelines.

INTRODUCTION: Recent systematic reviews highlighted increasing use of cadaveric models in the surgical training, but reports on the characteristics of the models and their impact on training are lacking, as well as standardized recommendations on how to ensure the quality of surgical studies. The aim of our survey was to provide an easy guideline that would improve the quality of the studies involving cadavers for surgical training and research. METHODS: After accurate literature review regarding surgical training on cadaveric models, a draft of the CACTUS guidelines involving 10 different items was drawn. Afterwards, the items were improved by questionnaire uploaded and spread to the experts in the field via Google form. The guideline was then reviewed following participants feedback, ergo, items that scored between 7 and 9 on nine-score Likert scale by 70% of respondents, and between 1 and 3 by fewer than 15% of respondents, were included in the proposed guideline, while items that scored between 1 and 3 by 70% of respondents, and between 7 and 9 by 15% or more of respondents were not. The process proceeded with Delphi rounds until the agreement for all items was unanimous. RESULTS: In total, 42 participants agreed to participate and 30 (71.4%) of them completed the Delphi survey. Unanimous agreement was almost always immediate concerning approval and ethical use of cadaver and providing brief outcome statement in terms of satisfaction in the use of the cadaver model through a short questionnaire. Other items were subjected to the minor adjustments. CONCLUSION: 'CACTUS' is a consensus-based guideline in the area of surgical training, simulation and anatomical studies and we believe that it will provide a useful guide to those writing manuscripts involving human cadavers.

Posterior urethral valves and urachal cyst: an unusual combination.

Combination of posterior urethral valves and urachus remnants has been described as rare occurrence. We report a case of a baby boy with normal antenatal scans, in whom the presence of large urachal cyst and posterior urethral valves causing high pressure bladder and chronic kidney disease was found. The patient underwent ablation of posterior urethral valves at 23 days of life and urachal cyst removal at age of 4 and a half months.

Development of a porcine acellular bladder matrix for tissue-engineered bladder reconstruction.

PURPOSE: Enterocystoplasty is adopted for patients requiring bladder augmentation, but significant long-term complications highlight need for alternatives. We established a protocol for creating a natural-derived bladder extracellular matrix (BEM) for developing tissue-engineered bladder, and investigated its structural and functional characteristics. METHODS: Porcine bladders were de-cellularised with a dynamic detergent-enzymatic treatment using peristaltic infusion. Samples and fresh controls were evaluated using histological staining, ultrastructure (electron microscopy), collagen, glycosaminoglycans and DNA quantification and biomechanical testing. Compliance and angiogenic properties (Chicken chorioallantoic membrane [CAM] assay) were evaluated. T test compared stiffness and glycosaminoglycans, collagen and DNA quantity. p value of < 0.05 was regarded as significant. RESULTS: Histological evaluation demonstrated absence of cells with preservation of tissue matrix architecture (collagen and elastin). DNA was 0.01 µg/mg, significantly reduced compared to fresh tissue 0.13 µg/mg (p < 0.01). BEM had increased tensile strength (0.259 ± 0.022 vs 0.116 ± 0.006, respectively, p < 0.0001) and stiffness (0.00075 ± 0.00016 vs 0.00726 ± 0.00216, p = 0.011). CAM assay showed significantly increased number of convergent allantoic vessels after 6 days compared to day 1 (p < 0.01). Urodynamic studies showed that BEM maintains or increases capacity and compliance. CONCLUSION: Dynamic detergent-enzymatic treatment produces a BEM which retains structural characteristics, increases strength and stiffness and is more compliant than native tissue. Furthermore, BEM shows angiogenic potential. These data suggest the use of BEM for development of tissue-engineered bladder for patients requiring bladder augmentation.

Long-term urology outcomes of anorectal malformation.

INTRODUCTION: Urological problems are a recognised feature of anorectal malformation (ARM). Previous assumptions of favourable long-term urinary outcomes are being challenged. OBJECTIVE: We hypothesised that urinary tract problems are common in ARM and frequently persist into adulthood. We retrospectively reviewed long-term renal and bladder outcomes in ARM patients. STUDY DESIGN: Patients with ARM born between 1984-2005 were identified from electronic hospital databases. Their case notes were reviewed. Renal outcomes included serum creatinine and the need for renal replacement therapy. Bladder outcomes included symptom review, bladder medication, need for intermittent catheterisation, videourodynamics and whether the patient had undergone augmentation cystoplasty. RESULT (TABLE 1): The case notes of 50 patients were reviewed. The median age at last follow up was 18 years (range 12-34 years). The level of fistula was noted to be high in 17 patients, intermediate in eight, and low in 10. Four had cloaca. Congenital urological abnormalities were present in 25 (50%). An abnormal spinal cord was present in 22 (44%) patients. VACTERL association occurred in 27 (54%). Chronic kidney disease stage II or above was found in 14 (28%) patients, of whom four required a renal transplant. Abnormal bladder outcomes were found in 39 (78%) patients. Augmentation cystoplasty with Mitrofanoff had been performed in 12. Of those who had not undergone cystoplasty, 17 had urinary symptoms, including urinary incontinence in 12. Of the 39 patients with abnormal bladder outcome, 19 (49%) did not have a spinal cord abnormality. There was no significant statistical association between level of ARM and abnormal renal outcome or presence of bladder abnormality. DISCUSSION: Adverse renal and bladder outcomes are common in our cohort of young people with ARM with a significantly higher incidence compared with current literature. We did not demonstrate an association between level of ARM or presence of spinal cord anomaly with persistent bladder problems. Congenital urological anomalies are more common in those who later have an abnormal renal outcome. Although this difference is statistically significant, one fifth of patients born with anatomically normal upper tracts develop reduced renal function, implying an important acquired component. CONCLUSION: Bladder problems and reduced renal function affect a significant proportion of young adults with ARM. Neither adverse outcome is reliably predicted from ARM level, congenital urological anomaly or spinal cord anomaly. We advise continued long-term bladder and kidney follow-up for all patients with ARM.

Barrier-Forming Potential of Epithelial Cells from the Exstrophic Bladder.

Bladder exstrophy (BEX) is a rare developmental abnormality resulting in an open, exposed bladder plate. Although normal bladder urothelium is a mitotically quiescent barrier epithelium, histologic studies of BEX epithelia report squamous and proliferative changes that can persist beyond surgical closure. The current study examined whether patient-derived BEX epithelial cells in vitro were capable of generating a barrier-forming epithelium under permissive conditions. Epithelial cells isolated from 11 BEX samples, classified histologically as transitional (n = 6) or squamous (n = 5), were propagated in vitro. In conditions conducive to differentiated tight barrier formation by normal human urothelial cell cultures, 8 of 11 BEX lines developed transepithelial electrical resistances of more than 1000 Ω.cm2, with 3 squamous lines failing to generate tight barriers. An inverse relationship was found between expression of squamous KRT14 transcript and barrier development. Transcriptional drivers of urothelial differentiation PPARG, GATA3, and FOXA1 showed reduced expression in squamous BEX cultures. These findings implicate developmental interruption of urothelial transcriptional programming in the spectrum of transitional to squamous epithelial phenotypes found in BEX. Assessment of BEX epithelial phenotype may inform management and treatment strategies, for which distinction between reversible versus intractably squamous epithelium could identify patients at risk of medical complications or those who are most appropriate for reconstructive tissue engineering strategies.