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Background • Copy number variants (CNVs) contribute significantly to normal and pathogenic genomic variations. Chromosome 17q12 microdeletion is implicated in structural or functional kidney and urethral abnormalities, MODY5 (type 5 diabetes), and neurodevelopmental or neuropsychiatric disorders. Conversely, microduplication of 17q12, though rare, elevates the risk of epilepsy and mental retardation. Case Presentation • This study focuses on a 33-year-old woman (gravida 1, para 0) who underwent amniocentesis at 22 weeks gestation due to bilateral hyperechogenic kidneys observed on prenatal ultrasound. Results • Chromosomal microarray analysis (CMA) unveiled a 1.46-Mb microdeletion on chromosome 17q12 in the fetus, spanning positions 35,802,057 to 37,261,945 (hg19). Trio whole-exome sequencing (WES) revealed 17q12 microdeletion in the fetus and 17q12 microduplication in the father. Notably, at the 3-year follow-up, the baby exhibited a normal phenotype. Conclusions • This research provides a comprehensive description of the phenotype in a rare family featuring 17q12 microdeletion and microduplication. Employing a combination of karyotype analysis, CMA, WES, prenatal ultrasound, and genetic counseling proves helpful in the prenatal diagnosis of chromosomal microdeletions/ microduplications. [ABSTRACT FROM AUTHOR]

Diphallia is an exceptionally rare genitourinary anomaly often associated with caudal duplication syndrome, which poses challenges in understanding its diverse nature. This case report presents a unique manifestation in a male infant born at 36 weeks to a mother with uncontrolled diabetes. The infant exhibited complete duplication of the penis, scrotum, testicles, urethra, bladder, sacrum, and right kidney, alongside major anomalies including Tetralogy of Fallot and lumbosacral spinal dysraphism with lipomyelomeningocele. Minor anomalies included abnormal ear creases, hypospadias, hydronephrosis, rectal-urethral fistulas, and a left club foot. This case represents the first documentation of such a complex constellation of supraumbilical presentation that includes abnormal ear creases and cardiac associations of Tetralogy of Fallot, right-sided aortic arch, and mirror aortic arch vessels. [ABSTRACT FROM AUTHOR]

Megalourethra is a rare clinical entity of absence or hypoplasia of corpus spongiosum and/or corpora carvenosa or anterior urethral valve along the pendulous urethra causing enlarged penis as a result of cystic dilatation of this part mostly during passage of urine. It may be associated with other congenital anomalies such prune belly syndrome and imperforated anus among others. We present a 10-day old neonate with scaphoid megalourethra associated with imperforated anus and pyloric stenosis. To the best of our knowledge, wehave limitedinformation on megalourethra in most parts of Africa but also, we were unable to find out any published report in East Africa regarding this rare condition especially in association with pyloric stenosis and imperforated anus. [ABSTRACT FROM AUTHOR]

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 longterm 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. [ABSTRACT FROM AUTHOR]

Boys with valve bladder syndrome (PUV) require adequate treatment of the lower urinary tract to preserve renal function and improve long-term outcomes. In some patients, further surgery may be necessary to improve bladder capacity and function. Ureterocytoplasty (UCP) is usually carried out with a small segment of intestine or, alternatively, with a dilated ureter. Our aim was to evaluate the long-term outcomes after UCP in boys with PUV. UCP had been performed in 10 boys with PUV at our hospital (2004–2019). Pre- and postoperative data were evaluated in relation to kidney and bladder function, the SWRD score, additional surgery, complications, and long-term follow-up. The mean time between primary valve ablation and UCP was 3.5 years (SD ± 2.0). The median follow-up time was 64.5 months (IQR 36.0–97.25). The mean increase in age-adjusted bladder capacity was 25% (from 77% (SD ± 0.28) to 102% (SD ± 0.46)). Eight boys micturated spontaneously. Ultrasounds showed no severe hydronephrosis (grade 3–4). The SWRD score showed a median decrease from 4.5 (range 2–7) to 3.0 (range 1–5). No conversion of augmentation was required. UCP is a safe and effective approach to improve bladder capacity in boys with PUV. In addition, the possibility of micturating naturally is still maintained. [ABSTRACT FROM AUTHOR]

Background: We sought to use deep learning to extract anatomic features from postnatal kidney ultrasounds and evaluate their performance in predicting the risk and timing of chronic kidney disease (CKD) progression for boys with posterior urethral valves (PUV). We hypothesized that these features would predict CKD progression better than clinical characteristics such as nadir creatinine alone. Methods: We performed a retrospective cohort study of boys with PUV treated at two pediatric health systems from 1990 to 2021. Features of kidneys were extracted from initial postnatal kidney ultrasound images using a deep learning model. Three time-to-event prediction models were built using random survival forests. The Imaging Model included deep learning imaging features, the Clinical Model included clinical data, and the Ensemble Model combined imaging features and clinical data. Separate models were built to include time-dependent clinical data that were available at 6 months, 1 year, 3 years, and 5 years. Results: Two-hundred and twenty-five patients were included in the analysis. All models performed well with C-indices of 0.7 or greater. The Clinical Model outperformed the Imaging Model at all time points with nadir creatinine driving the performance of the Clinical Model. Combining the 6-month Imaging Model (C-index 0.7; 95% confidence interval [CI] 0.6, 0.79) with the 6-month Clinical Model (C-index 0.79; 95% CI 0.71, 0.86) resulted in a 6-month Ensemble Model that performed better (C-index 0.82; 95% CI 0.77, 0.88) than either model alone. Conclusions: Deep learning imaging features extracted from initial postnatal kidney ultrasounds may improve early prediction of CKD progression among children with PUV. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

Urethrocystography remains the gold-standard technique for urethral pathology diagnosis. Nowadays, of the various indications for performing urethrocystography, the most common is due to a clinical suspicion of urethral stricture. Due to the high prevalence of strictures and their substantial impact on a patient's quality of life, the examination must allow the location, exclusion of multifocality, and assessment of the extent of the stricture to influence surgical planning. This article intends to demonstrate that the radiologist's role, by performing and interpreting the modality of urethrocystography, influences and is crucial for the urologic therapeutic decision and that the patients who were submitted to reconstruction by urethroplasty had a better success rate. The authors aim to review the radiological anatomy of the male urethra, discuss the modalities of choice for imaging the urethra (retrograde urethrography and voiding cystourethrography), provide an overview of the different indications for performing the study, examine the different etiologies for urethral strictures, understand the relevance of the different appearances of urethral pathology, and identify the surgical options, especially in the treatment of urethral strictures. Simultaneously, the study exposes cases of urethral trauma, fistulas, diverticulum, and congenital abnormalities. [ABSTRACT FROM AUTHOR]

Introduction: Triphallia, a rare congenital anomaly describing the presence of three distinct penile shafts, has been reported only once in the literature. This case report, based on an extensive literature review, describes the serendipitous discovery during cadaveric dissection of the second reported human case of triphallia, distinctly morphologically different from the previous case., Case Presentation: Despite the normal appearance of external genitalia on examination, the dissection of a 78-year-old white male revealed a remarkable anatomical variation: two small supernumerary penises stacked in a sagittal orientation posteroinferiorly to the primary penis. Each penile shaft displayed its own corpora cavernosa and glans penis. The primary penis and largest and most superficial of the supernumerary penises shared a single urethra, which coursed through the secondary penis prior to its passage through the primary penis. A urethra-like structure was absent from the smallest supernumerary penis., Conclusion: This case report provides a comprehensive description of the anatomical features of triphallia in a cadaver, shedding light on the morphology, embryology, and clinical implications of this anomaly. Without dissection, this anatomical variation would have remained undiscovered, suggesting the prevalence of polyphallia may be greater than expected. The single tortuous urethra present in this case, as well as the supernumerary and blind ending urethras present in many cases of penile duplication, may pose significant risk of infection, sexual dysfunction, subfertility, and traumatic catheterization., Significance: These findings underscore the importance of meticulous anatomical dissections and may act as a resource for anatomists and those studying genitourinary anomalies. Although we can only speculate as to which functional implications this patient may have experienced, understanding such anatomical variations contributes to both knowledge of human anatomy and clinical management should the condition be encountered in living individuals., (© 2024. The Author(s).)

Objective: To determine risk factors (RF) of progression to end-stage chronic renal disease (ESCRD) and the need for renal replacement therapy (RRT) in patients with posterior urethral valve (PUV)., Materials and Methods: A retrospective case and control study of patients diagnosed with PUV in the 1995-2023 period was carried out. Two study groups were created -RRT vs. no-RRT. Clinical, laboratory, and radiological variables were collected. A bivariate analysis and a binary logistic regression were conducted to detect RFs of the need for RRT., Results: 127 patients were included, 12.% of whom had undergone RRT (n= 20). Mean follow-up was 9.87 years. Mean age at clinical onset was younger in the RRT group (3 months vs. 1.23 years; p= 0.010). Pathological prenatal ultrasonography (p< 0.001), increased Nadir creatinine levels (p< 0.001) and maximum creatinine levels in the first year of life (p<  0.001), and onset with acute renal insufficiency (p= 0.03) were more frequent in the RRT group. Increased creatinine levels in the first week of life (OR: 4.74) and younger age at clinical onset (OR: 1.2) were the only independent RFs to predict the need for RRT. Diagnostic-therapeutic delay and the presence of UTIs during follow-up are not predictive of the risk of final RRT., Conclusions: In PUV children, renal functional reserve at birth is the only ESCRD risk predictor. Early clinical onset implies a higher risk of RRT.

Background: Posterior sagittal is a very well accepted approach in the treatment of anorectal malformations. This approach provides good access and exposure through the perineum to the deep pelvic structures. It reduces risk of injury to important structures as dissection remains in midline. Aims and Objectives: To access feasibility of posterior sagittal approach for non-anorectal malformation indications and to widen the spectrum. Material and Methods: We present a series of 10 cases of non-anorectal malformations operated by this approach for 4 years. Results: Six patients included in the study were of Disorders of Sexual Differentiation with pseudovagina, three of Y duplication of the urethra, and one of cervical atresia. All patients had good results. Conclusion: Posterior sagittal approach is feasible, safe with minimal bleeding, and no postoperative incontinence. It can safely be used for non-anorectal indications. [ABSTRACT FROM AUTHOR]

We present a case of antenatally detected fetal megacystis caused by an obstructing posterior urethral polyp. Antenatal and postnatal ultrasounds showed bladder wall thickening and bilateral hydroureteronephrosis, most marked antenatally. A working diagnosis of posterior urethral valves was therefore made. However, further postnatal assessment with a micturating cystourethrogram (MCUG) combined with a retrograde urethrogram identified a pedunculated urethral polyp as the cause. The addition of a retrograde urethrogram as an adjunct to the MCUG in the diagnosis of posterior urethral polyp has not previously been reported, and in this case provided diagnostic confidence of this rare condition, allowing for definitive surgical planning. [ABSTRACT FROM AUTHOR]

Introduction. Penile fracture is a rare urological emergency, the real incidence is unknown given the underreporting caused by the reduced consultation of patients, resulting from the embarrassing context. In the United States it reaches 38%, where sexual etiology predominates. In Colombia there are few published reports. The diagnosis of this entity is purely clinical. The aim of this work is to report a satisfactory evolution in terms of voiding and erectile function in a patient who underwent early cavernous and urethral reconstruction in bilateral rupture of traumatic origin by means of an unconventional surgical technique, in accordance with what has been found in the literature. Case Presentation. A 30-year-old male patient, previously healthy, consulted the emergency department for urethrorrhagia, edema and penile pain secondary to blunt trauma to the penis during sexual intercourse. The ultrasound report showed disruption in the tunica albuginea of the corpus spongiosum, hematoma and apparent urethral transection; surgical exploration was performed 6 hours later, with evidence of fracture of the corpora cavernosa, laceration of 40% of the circumference, complete transection of the urethra and corpus spongiosum. Sutures to the corpora cavernosa and end-to-end urethroplasty of the bulbar urethra were performed, with satisfactory clinical evolution. Discussion. Surgical reconstruction in less than 24 hours in penile fractures significantly reduces hospital stay and early complications; it also reduces the risk of erectile dysfunction, painful erections and voiding problems. There are no publications comparing long-term results between the two surgical approaches. Conclusions. Penile fracture is a rare pathology with clinical diagnosis, which should be managed early by any urologist and, if possible, with reconstructive experience and excellent results in sexual and voiding function. [ABSTRACT FROM AUTHOR]

Introduction: Posterior urethral valve (PUV) is life threatening congenital anomaly of urinary tract. Aim of the study was to correlate urethral ratio (UR) and bladder wall thickness (BWT) with cystoscopic findings in PUV patients to assess residual valves and to validate UR as a diagnostic tool for residual valves. It also aimed to assess the utility of bladder wall thickness in diagnosis of residual valves. Materials and Methods: A total of 31 patients were included in the prospective study done from 2017 to 2019. Calculation of UR was done in oblique VCUG films by dividing maximum posterior and anterior urethral diameter without the catheter insitu. Measurement of BWT was done at dome and bladder neck at full distension with feeding tube insitu and was done at same volume in follow up. The procedure was repeated at 3 months follow up. The findings were compared with cystoscopic findings for the status of residual valves as gold standard. Each patient served as control for self in the study. Results: Median age of presentation was 1 years with range of 1day to 10 years. The most common complains at the time of presentation in our study was straining (35.48%) followed by antenatally diagnosed patients (25.81%) and recurrent UTI (19.36%). Pre fulguration median UR was 2.45. Post Fulguration median UR was 1.20. It showed a statistically significant reduction (p < 0.001) after fulguration. Pre fulguration median BWT was 4 mm. Post fulguration median BWT was 2.5 mm. BWT showed a statistically significant reduction (p < 0.001) after fulguration as well. ROC curve was plotted for UR and BWT. BWT more than 1.95 mm (sensitivity-80%) and UR more than 1.2 (sensitivity-70%) indicates residual valves. Conclusion: A step ladder approach including BWT, UR and check cystoscopy can serve as a new diagnostic algorithm for the assessment of residual valves thereby avoiding extra radiation and general anesthesia exposure. [ABSTRACT FROM AUTHOR]

Introduction: Penile fracture accompanied by complete urethral rupture is a rare condition. This study aims to evaluate the long-term lower urinary tract symptoms and sexual functions of patients with penile fracture and concomitant complete urethral rupture who underwent urgent surgical reconstruction. Methods: Preoperative, perioperative, and long-term results of five patients who were operated on for complete urethral rupture and concomitant penile fracture were evaluated. Patients were diagnosed by physical examination and retrograde urethrography (RUG). Operation data were recorded and physical examination, International Prostate Symptom Score, International Index of Erectile Function-5 forms, and uroflowmetry results were evaluated to determine long-term results. Results: The mean age of the patients was 35.6±6.3 (25-42) years and the mean follow-up period was 18.4±9.9 months. Penile fracture occurred in three patients during sexual intercourse, one patient during masturbation, and one patient during sleep. Erectile dysfunction was not observed in any patient after the surgery. One of the patients developed anterior urethral stricture and one patient had a 15° ventral curvature during erection. Discussion and Conclusion: Urethrorrhagia and urinary retention are the major signs indicating urethral rupture. In these patients, the urethra should be evaluated with RUG preoperatively. The long-term results of early surgical repair of the corpus cavernosum and urethra in this group of patients are very good. [ABSTRACT FROM AUTHOR]

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Background: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes., Methods: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet., Results: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes., Level of Evidence: IV., Competing Interests: Conflict of interest None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Urinary drainage for posterior urethral valves can be achieved with valve ablation (VA) or diversion by vesicostomy (VES) or cutaneous ureterostomy (CU). The effect of these interventions on long-term bladder function remains debated, and voiding symptomatology after VES or CU reversal has been poorly characterized., Objective: The objective of this study was to examine the prevalence and scope of physician treatment patterns as a surrogate for retention or incontinence symptomatology among PUV patients undergoing primary VA or diversion by VES/CU and determine rates of progression to augmentation., Study Design: This is a single-institution retrospective cohort study. Retention Scores (R) were calculated 1 point for: retention behavior (double/timed void), alpha-blocker, intermittent catheterization, or overnight indwelling catheter. Incontinence Scores (I) were calculated 1 point for: incontinence behavior (double/timed void), oral medication, or botulinum toxin. Patients with R score above 3 or I score above 2 were deemed to have severe retention or incontinence symptomatology respectively. End stage bladder (ESB) was defined as need for bladder augmentation., Results: We identified 76 patients between 5 and 40 years old with median follow-up of 14.6 [5.0-40.4) years. There was no difference in the rates of severe retention or incontinence treatment pattern scoring between VA versus VES/CU (Figure). Rates of achieving R(1) status are similar between VA and VES/CU groups, though age of reaching R(1) was younger for those with VES/CU (4.8 years) compared to VA (6.6 years). There was no significant difference in rate of ESB by intervention category VA (9.4%) versus VES/CU (17.4%; p = 0.323)., Discussion: Treatment of retention symptomatology was more common than treatment of incontinence symptomatology regardless of primary management, VA or VES/CU. This study also indicates that VES/CU patients were just as responsive as VA patients to conservative treatments (behavioral changes, pharmacotherapy) for any type of bladder symptomatology as the progression to treatment of severe symptomatology and ESB were similar between cohorts. In this cohort, bladder outcomes were not associated with type of urinary diversion (VA or VES/CU)., Conclusion: Long term bladder outcomes for valve patients demonstrated similar treatment patterns and progression to end-stage bladder regardless of diversion status. Patients went on to ESB approximately 4.4 years after diagnosis at similar rates between groups., Competing Interests: Conflict of interest None., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

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., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objectives: To investigate the effects of bladder neck incision (BNI) and primary valves ablation on long-term kidney and bladder function in children with posterior urethral valves (PUV) and bladder neck hypertrophy (BNH)., Patients and Methods: From 1997 to 2016, a total of 1381 children with PUV were referred to our tertiary hospital. Of these patients, 301 PUV patients with bladder neck hypertrophy need concurrent BNI and valve ablation. All patients were followed up every 3-6 months on regular basis in first 2 post-surgical years and annually then after. The paired t-test and chi-square test were used to perform statistical analysis with p value < 0.05 defined as the level of significance., Results: Mean age at diagnosis was 7.22 ± 2.45 months (ranging from 7 days to 15 months) with a mean follow-up of 5.12 ± 2.80 years. The incidence of hydronephrosis was decreased from 266 (88.3%) at the baseline to 73 (24.3%) patients in long-term follow-up. At baseline, 188 (62.5%) patients were diagnosed with VUR, which decreased to 20 (6.6%) individuals at the end of follow-up. Bladder and renal function were improved in follow-ups following concomitant PUV ablation and BNI. No Myogenic failure was depicted in all patients with BNH. No ureteric reimplantation was needed during the two decades follow-up., Conclusion: Simultaneous valve ablation with BNI may present further profits in children with PUV and BNH particularly cases of BNH with poor bladder function at the time of presentation. This method can improve the results of urodynamic and imaging studies after the surgery. We hypothesize every child with PUV presentation who has concurrent vesicoureteral reflux, CKD or persistent hydrourethronephrosis may suffer from secondary bladder neck obstruction. This secondary bladder outlet obstruction must be managed through BNI as the surgical relief., (© 2024. The Author(s).)

Purpose: We investigated the postoperative renal function in persistent cloaca (PC) patients who underwent posterior sagittal anorecto-urethro-vaginopalsty (PSARUVP) and factors influencing the renal functional outcomes., Methods: A questionnaire survey was distributed to 244 university and children's hospitals across Japan. Of the 169 patients underwent PSARUVP, 103 patients were enrolled in the present study. Exclusion criteria was patients without data of renal prognosis., Results: The present study showed that renal anomalies (p = 0.09), vesicoureteral reflux (VUR) (p = 0.01), and hydrocolpos (p = 0.07) were potential factors influencing a decline in the renal function. Approximately half of the patients had a normal kidney function, but 45.6% had a reduced renal function (Stage ≥ 2 chronic kidney disease: CKD). The incidence of VUR was significantly higher in the renal function decline (RFD) group than those in the preservation (RFP) group (p = 0.01). Vesicostomy was significantly more frequent in the RFD group than in the RFP group (p = 0.04). Urinary tract infections (p < 0.01) and bladder dysfunction (p = 0.04) were significantly more common in patients with VUR than in patients without VUR. There was no association between the VUR status and the bowel function., Conclusions: Prompt assessment and treatment of VUR along with bladder management may minimize the decline in the renal function., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Today, prenatal diagnosis of congenital urogenital malformations is mostly dependent on anatomical variations found on imaging. However, these findings can mislead us in telling us when to intervene, and about post-natal prognosis. Since many findings are dependent on multiple assessments, delayed diagnosis can occur, leading to less optimal outcomes compared to early intervention. Analyses of fetal urinary biomarkers have been proposed as a method of finding biological changes that are predictive for diagnosis and prognosis in fetuses at risk of kidney disease. We interviewed a group of researchers that have demonstrated that by combining multiple omics traits extracted from fetal urine, the biological variability found in single omics data can be circumvented. By analyzing multiple fetal urine peptides and metabolites at single time point, the prognostic power of postnatal renal outcome in fetuses with lower urinary tract obstruction is significantly increased. In this interview, we inquired about the technical aspects of the tests, challenges, and limitations the research group have come across, and how they envision the future for multi-omics fetal analysis in the clinic., Competing Interests: Conflict of interest Two last authors are Basic Science editors of the Journal., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

We present the case of a 6-year-old girl who presented with alterations in the voiding stream. On physical examination, a very small urethral meatus was identified at the expense of a membrane. The renovesical ultrasound showed no alterations. An uroflowmetric study was performed, showing a bladder outlet obstruction pattern. The urethral meatus was calibrated and a ventral meatotomy was performed. The histological study of the resected membrane showed a transitional urethral mucosa with chronic focal inflammation and discrete hyperplasia. The patient evolved favorably, with resolution of the symptoms and no notable complications. This is, to the best of our knowledge, the first reported case with a histological study of a congenital meatal urethral stenosis. In the presence of lower urinary tract obstruction, this entity should be considered in the differential diagnosis. Surgical treatment is curative., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective:  Wound dehiscence after posterior sagittal anorectoplasty (PSARP) or anorectal vaginal urethroplasty (PSARVUP) for anorectal malformation (ARM) is a morbid complication. We present a novel anoplasty technique employing para-U-stitches along the anterior and posterior portions of the anoplasty, which helps buttress the midline U-stitch and evert the rectal mucosa. We hypothesized that, in addition to standardized pre- and postoperative protocols, this technique would lower rates of wound dehiscence., Materials and Methods:  A retrospective review of patievnts who underwent primary PSARP or PSARVUP with the para-U-stitch technique from 2015 to 2021 was performed. Wound dehiscence was defined as wound disruption requiring operative intervention within 30 days of the index operation. Superficial wound separations were excluded. Descriptive statistics were calculated. The final cohort included 232 patients., Results:  Rectoperineal fistula (28.9%) was the most common ARM subtype. PSARP was performed in 75% and PSARVUP in 25%. The majority were reconstructed with a stoma in place (63.4%). Wound dehiscence requiring operative intervention occurred in four patients, for an overall dehiscence rate of 1.7%. The dehiscence rate was lower in PSARPs compared with PSARVUPs (0.6 vs. 5.2%) and lower for reconstruction without a stoma compared with a stoma (1.2 vs. 2.0%). There were additional six patients (2.6%) with superficial wound infections managed conservatively., Conclusion:  We present the para-U-stitch anoplasty technique, which is an adjunct to the standard anoplasty during PSARP and PSARVUP. In conjunction with standardized pre- and postoperative protocols, this technique can help decrease rates of wound dehiscence in this patient population., Competing Interests: None declared., (Thieme. All rights reserved.)

Introduction: After 5 years experience with the GUD (glandular urethral disassembly) technique for distal hypospadias, we present the GUDplay technique, incorporating Thiersch-Duplay tubularization of the plate till the coronal area, disassembling the glans aggressively and refurbishing the glans., Methods: We defined the urethral plate and designed an inverted Y incision to open the glans in two wings. The glans was entirely detached from the corpora to gain a great mobility that allowed minor cranial mobilization of the urethra and caudal rotation of the wings. In sequence, there are well-known steps: Duplay urethroplasty, spongioblasts and a Dartos flap to cover the neourethra. The glans was connected to the urethra by 6.0 PDS sutures except in the ventral meatus and the glans wings are joined in the midline., Results: The 5-year-old patient had midshaft hypospadias without previous surgery. The catheter was removed after a week and the healing appears to be good., Discussion: We combined principles of total glans deconstruction in association to Duplay tubularization and then lifted it up to the tip of the glans divided in two wide and mobile wings. We have treated a small series of 6 cases without complications and mean follow-up of 6.2 months., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Lower urinary tract obstruction (LUTO) is a rare but critical fetal diagnosis. Different ultrasound markers have been reported with varying sensitivity and specificity.Aims: The objective of this systematic review and meta-analysis was to identify the diagnostic accuracy of ultrasound markers for LUTO.Materials and Methods: We performed a systematic literature review of studies reporting on fetuses with hydronephrosis or a prenatally suspected and/or postnatally confirmed diagnosis of LUTO. Bayesian bivariate random effects meta-analytic models were fitted, and we calculated posterior means and 95% credible intervals for the pooled diagnostic odds ratio (DOR).Results: A total of 36,189 studies were identified; 636 studies were available for full text review and a total of 42 studies were included in the Bayesian meta-analysis. Among the ultrasound signs assessed, megacystis (DOR 49.15, [15.28, 177.44]), bilateral hydroureteronephrosis (DOR 41.33, [13.36,164.83]), bladder thickening (DOR 13.73, [1.23, 115.20]), bilateral hydronephrosis (DOR 8.36 [3.17, 21.91]), male sex (DOR 8.08 [3.05, 22.82]), oligo- or anhydramnios (DOR 7.75 [4.23, 14.46]), and urinoma (DOR 7.47 [1.14, 33.18]) were found to be predictive of LUTO (Table 1). The predictive sensitivities and specificities however are low and wide study heterogeneity existed.Discussion: Classically, LUTO is suspected in the presence of prenatally detected megacystis with a dilated posterior urethra (i.e., the keyhole sign), and bilateral hydroureteronephrosis. However, keyhole sign has been found to have modest diagnostic performance in predicting the presence of LUTO in the literature which we confirmed in our analysis. The surprisingly low specificity may be influenced by several factors, including the degree of obstruction, and the diligence of the sonographer at searching for and documenting it during the scan. As a result, providers should consider this when establishing the differential for a fetus with hydronephrosis as the presence or absence of keyhole sign does not reliably rule in or rule out LUTO.Conclusions: Megacystis, bilateral hydroureteronephrosis and bladder wall thickening are the most accurate predictors of LUTO. Given the significant consequences of a missed LUTO diagnosis, clinicians providing counselling for prenatal hydronephrosis should maintain a low threshold for considering LUTO as part of the differential diagnosis. [ABSTRACT FROM AUTHOR]

Objective: To analyze renal glomerular and tubular function and their association in patients operated for posterior urethral valves and to prognosticate the risk for end-stage kidney disease (ESKD) METHODS: Sixty-three previously treated patients were evaluated for renal function during 1987-1991. The patients' age at evaluation was 11 years (range 2-24). Glomerular function was assessed by measuring glomerular filtration rate (GFR) and urine albumin excretion. Tubular function was determined by measuring urine concentration capacity and excretion of electrolytes (Na, K, Cl, Ca, P, Mg) and β-2-microglobulin. Additionally, the prevalence of hypertension and serum parathyroid hormone and aldosterone values were registered. Tubular function was compared with GFR and the risk of developing ESKD before November 2018.Results: Twenty of the study patients (32%) had decreased GFR. In addition, 19% had proteinuria and 56% were hypertensive. Those without proteinuria or hypertension had better GFR values (P < .01 for both). There was a significant correlation between GFR and all the tubular function (P < .05) variables (except excretion of chloride) measured. Compared to the patients with favorable renal outcome, the patients (n = 10) who later developed ESKD had significantly (P < .01) lower GFR and reduced urinary excretion of all measured electrolytes except calcium. Consistently, urine β-2 microglobulin and serum parathyroid hormone and aldosterone values were significantly higher in the patients who developed ESKD (P ≤ .01).Conclusion: Both glomerular and tubular function decline was common and several parameters were likely to predict ESKD in posterior urethral valves patients. [ABSTRACT FROM AUTHOR]

Background: Up to 50% of children with posterior urethral valves (PUV) progress to kidney failure. This study aimed to evaluate polyuria and polydipsia and other established variables with later development of kidney failure in children with PUV. Methods: Retrospective analysis of 297 children with PUV who underwent ablation of valves between January 1992 and January 2015 at our tertiary care center. Patients were divided into two groups: those who developed kidney failure (group 1) and those who did not (group 2). Specific prognostic factors for progression to kidney failure were analyzed including age at presentation < 1 year, nadir serum creatinine > 1.0 mg/dl, bilateral grade 3 or higher VUR at diagnosis, recurrent febrile UTIs, severe bladder dysfunction, polyuria, and polydipsia. Results: Thirty-eight (12.8%) patients progressed to kidney failure. Twenty-four and 64 patients were polyuric in group 1 and group 2 respectively (p < 0.001, Z-4.4666). Twenty-two and 61 patients were polydipsic in both groups respectively (p < 0.001). On univariate analysis, predicting variables were as follows: age at presentation < 1 year (p < 0.001), nadir serum creatinine > 1 mg/dl (p < 0.001), B/L high-grade VUR (p < 0.001), severe bladder dysfunction (p < 0.001), recurrent febrile UTIs (p = 0.002), polyuria (p < 0.001), and polydipsia (p < 0.001). On multivariate Cox regression analysis, severe bladder dysfunction, recurrent febrile UTIs, polyuria, and polydipsia were identified as significant prognostic factors predictive of ultimate progression to kidney failure. Conclusion: Polyuria and polydipsia along with recurrent febrile UTI and bladder dysfunction are major prognostic factors affecting long-term kidney outcome in cases of PUV. [ABSTRACT FROM AUTHOR]

Congenital anomalies of the kidney and urinary tract (CAKUT) are diagnosed in approximately 3-6 per 1000 live births and represent a spectrum of urologic conditions impacting the kidneys, ureter, bladder, and urethra.1 Although both are considered under the classification of CAKUT, there is no known unifying pathophysiologic mechanism for ureteroceles and posterior urethral valves with only 1 case report noted in the literature. Herein we report the only documented case of a patient with CAKUT related to posterior urethral valves, ureterocele, and multicystic dysplastic kidney. [ABSTRACT FROM AUTHOR]

Background: To describe the perinatal outcomes of fetoscopic urethral meatotomy (FUM) in fetuses with lower urinary tract obstruction (LUTO) by congenital megalourethra.Study Design: Between 2012 and 2020, 226 cases with LUTO were referred to our fetal surgery center in Queretaro, Mexico. We report the perinatal outcome of cases with LUTO by congenital megalourethra that were selected for FUM in an attempt to release the penile urethral obstruction.Results: Congenital megalourethra was diagnosed in 10 cases (4.4%) but only 3 cases (30%) with obstructive megalourethra and megacystis were selected for fetal surgery. Fetoscopic urethral metatotomy was successfully performed in all three cases at a median gestational age (GA) of 21.4 (18.0-26.7) weeks and with a median surgical time of 27 (12-43) min. A resolution of urethral dilatation and subsequent reduction of the penile length and normalization of both the bladder size and amniotic fluid were observed in all cases. The median GA at delivery was 35.2 (range: 30.6-38.0) weeks. There were no fetal deaths but one neonatal death (33%) secondary to renal failure and preterm delivery.Conclusion: In fetuses with LUTO by congenital obstructive megalourethra, FUM is feasible and is associated with good perinatal outcomes. [ABSTRACT FROM AUTHOR]

Individuals with spinal cord injury or neurological disorders have problems in voiding function due to the dyssynergic contraction of the urethral sphincter. Here, we introduce a closed-loop control of intraspinal microstimulation (ISMS) for efficient bladder voiding. The strategy is based on asynchronous two-electrode ISMS with combined pulse-amplitude and pulse-frequency modulation without requiring rhizotomy, neurotomy, or high-frequency blocking. Intermittent stimulation is alternately applied to the two electrodes that are implanted in the S2 lateral ventral horn and S1 dorsal gray commissure, to excite the bladder motoneurons and to inhibit the urethral sphincter motoneurons. Asynchronous stimulation would lead to reduce the net electric field and to maximize the selective stimulation. The proposed closed-loop system attains a highly voiding efficiency of 77.2–100%, with an average of 91.28 ± 8.4%. This work represents a promising approach to the development of a natural and robust motor neuroprosthesis device for restoring bladder functions. [ABSTRACT FROM AUTHOR]

Gastric cancer is a rare long-term complication in gastrocystoplasty. We report 2 cases of gastric adenocarcinoma and review the literature for similar cases. A total of 14 cases are identified. The majority of patients are males, presented with hematuria, and developed cancer at a younger age, more than 10 years after gastrocystoplasty. Long-term follow up information was limited, but 5 patients (36%) died within 5 years of diagnosis. Annual surveillance for malignancy may not be effective due to its rarity. However, symptomatic patients, particularly those 10 years after the surgery, warrant detailed evaluation to rule out neoplastic transformation. [ABSTRACT FROM AUTHOR]

Introduction The aim of this article was to assess the influence of sexual disorders after urethroplasty on patient's quality of life (QoL) and satisfaction of treatment. Material and methods We studied 106 sexually active patients who underwent urethroplasty due to urethral stricture. Patients completed the Urethral Stricture Surgery - Patient Reported Outcome Measure (USS - PROM) and International Index of Erectile Function (IIEF-5) questionnaires before and after the treatment. Spearman rank correlations were used for correlation analyses. Multiple linear regression and ordinal logistic regression analyses were used for evaluating the influence of lower urinary tract symptoms (LUTS) and IIEF-5 scores on EuroQol-5D (EQ-5D) index, EuroQol-Visual Analogue Scale (EQ-VAS), and satisfaction with treatment. Both LUTS and IIEF-5 scores were independent, significant predictors of EQ-VAS in the multiple linear regression model. Results Mean follow-up was 9 months (3-24). Reduction of LUTS and micturition improvement in the USS-PROM questionnaire after the surgery was found in 90 patients (85%). The average IIEF-5 score in the whole group did not change significantly, but in 39 cases (37%) worsened, and in 42 (39%) improved. Spearman's rank-order correlation indicated a significant positive correlation between improvement in IIEF-5 and general QoL in EQ-5D and also a positive correlation between improvement in IIEF-5 and improvement in EQ-VAS, which was also statistically significant (rho = 0.377, p <0.001). Conclusions Urethral surgery can influence sexual performance. The appearance of sexual dysfunction negatively affects the patient's quality of life, regardless of the effective restoration of the urethral patency and reduction of LUTS. [ABSTRACT FROM AUTHOR]

True ureteral diverticula are rare, may be found incidentally, or form after urinary sepsis, stones, or obstruction. Stone formation is an uncommon complication because of urine stasis or recurrent infections. Radiological opacities suggestive of a stone in an extra-anatomical site of the ureter should raise the suspicion of a ureteric diverticulum. Although a congenital ureteric diverticulum is believed to be formed because of an abortive ureteric bud, its anatomy could be completely different from that of a duplex ureter. The presence of complications requires diverticulectomy with or without reconstruction of the ureteric defect. [ABSTRACT FROM AUTHOR]

Aim: The appropriate anesthetic technique to be used in cases of proximal hypospadias in children is a subject of debate. Local or regional anesthesia as well as intubation can be used. In this study, it was aimed to evaluate retrospectively the different anesthesia and surgery techniques used and their perioperative outcomes in the cases of proximal hypospadias consulting our hospital. Materials and Methods: The hospital files of 30 cases operated on for proximal hypospadias were scanned retrospectively to evaluate the different methods of anesthesia and repair surgery implemented. The patients operated under general anesthesia were grouped on the basis of the airway equipment used for anesthesia as Laryngeal mask airway (Group 1) and endotracheal intubation (Group 2). Routine penile block implementation, effectiveness of anesthesia, side effects, hemodynamic changes and complications in all patients were recorded. Results: A total of 56 sessions of operations on 28 patients were carried out. In 26 of the session’s laryngeal mask and in 30 sessions endotracheal intubation were used. When the data of Group 1 and Group 2 were compared, the groups did not differ significantly with respect to age, anesthesia methods and duration, hypospadias repair methods, duration and sessions, intraoperative parameters, hospital stay and complications (all p>0.05). The two groups differed on the ASA physical status classification values with Group 2 values being significantly higher (p<0.05). Conclusion: Effectiveness of anesthesia by using LMA or endotracheal intubation were perioperatively equivalent during repair of proximal hypospadias with different methods of surgery. [ABSTRACT FROM AUTHOR]

Objective: To investigate the histomorphological changes of the divergent corpus spongiosum surrounding the urethral plate in hypospadias.Methods: Seventy boys with hypospadias were divided into the distal (n = 44) and proximal (n = 26) groups by the location of the ectopic meatus. The morphology of the divergent corpus spongiosum was evaluated and the histopathological studies were carried out by HE staining, Masson staining, and immunohistochemical analyses of α-SMA, CD34, TGF-β1 and androgen receptor. Western blotting was performed to measure the expression of TGF-β1 and androgen receptor.Results: The divergent corpus spongiosum was significantly thicker in the distal group than the proximal group (distal 3.14 ± 1.12 mm, proximal 1.74 ± 0.87 mm, P <.0001). Histological evaluation suggested the cavernous sinus in the hypospadias group had an abnormal structure, which was characterized by an increase in the width of the vascular lumen (control 7.20 ± 1.12 μm, hypospadias 13.75 ± 8.08 μm, P = .0068), an increased vascular wall thickness (control 5.40 ± 1.28 μm, hypospadias 14.11 ± 7.59 μm, P = .0006), a decreased vascular density (control 15.66 ± 1.17, hypospadias 7.24 ± 4.19, P <.0001) and a decreased trabecular density (control 9.80 ± 1.92, hypospadias 3.68 ± 2.87, P <.0001). The severity of the structural abnormalities was greater in the proximal group than the distal group. Immunohistochemical analysis and Western blotting showed decreased TGF-β1 and androgen receptor expression in the hypospadias group (P <.0001).Conclusions: Histomorphological analysis showed that the divergent corpus spongiosum in hypospadias is abnormal. The structural variation is positively correlated with the severity of hypospadias. [ABSTRACT FROM AUTHOR]

A 3-year-old boy presented to our pediatric urology with a history of urine flow under the scrotum when voiding in a squatting position but not when standing. And the ventral side of the front penis became enlarged during urination and dribbled afterward. Physical examination revealed the boy had 2 urethras opening at the tip of glans, and another accessory urethra opening at perineum. Rigid cystoscopy and voiding cystourethrography confirmed it to be a urethral triplication malformation. This condition, the combination of urethral diverticulum and urethral triplication, consisting of urethro-perineum fistula, has not been previously reported. [ABSTRACT FROM AUTHOR]

We present a rare case of a 7-year-old male with a perineal mass with urethral duplication. Perineal mass histopathology showed colonic mucosa. The association of heterotopic colonic mucosa with urethral duplication, hypospadias, bifid scrotum, and normal anus has not been described previously in the literature. [ABSTRACT FROM AUTHOR]

Objective: To analyze treatment results of staged surgical repair of proximal forms of hypospadias according to Bracka's technique using preputial vs buccal grafts.Material and Methods: We retrospectively reviewed 220 patients with proximal forms of hypospadias treated with Bracka's urethroplasty technique. They were divided into 2 groups: Group I-108 patients treated with preputial skin grafts in 2001-2013; Group II-112 patients who underwent urethroplasty with buccal mucosa grafts in 2013-2016.Results and Discussion: Of the Group I patients with preputial skin grafts, complications were obtained in 33 (31%) cases; in Group II-23 (20%) cases. Complications include fistulas, defects of urethra, and scar contraction of grafts. The cosmetic results according to Hypospadias Objective Penile Evaluation scale were more satisfactory when buccal mucosa grafts were used. Further studies are needed to analyze the long-term changes posturethroplasty with both preputial skin and buccal mucosa grafts.Conclusion: This is one of the only studies to compare complications and histology of the 2 free grafts: preputial skin and buccal mucosa. This study affirms that a staged surgical method with the use of free grafts according to Bracka's technique is a successful method of treatment of proximal forms of hypospadias in children achieving good functional and cosmetic results with a relatively low rate of complications. [ABSTRACT FROM AUTHOR]

The article presents a case study of a WH presented at birth with ambiguous genitalia and no clear urethral opening, he born to a 20-year-old G1P0 mother with unremarkable prenatal workup and who denied substance use during pregnancy, and physical exam demonstrated palpable bilateral inguinal testes and an anteriorly displaced anus located at the base of the scrotal raphe without obvious urethral meatus, genetics recommended an MRI of the abdomen and pelvis, which confirmed aphallia.

Cloacal dysgenesis sequence occurs as a result of complete developmental failure of the urorectal septum. Typically, the sequence is featured by a smooth perineum, without any urethral, genital or anal openings. Its clinical manifestation differs throughout gestation. We report an interesting case of first trimester megacystis with associated umbilical cord abnormalities in a female fetus having cloacal dysgenesis sequence. This rare association reflecting high urinary pressure should first suggest urethral atresia. Our case highlights the importance of routine inspection of umbilical cord in the workup of early megacystis in terms of both etiology and fetal diagnosis. [ABSTRACT FROM AUTHOR]

Background: Posterior urethral valves (PUVs) account for 17% of pediatric renal failure. The management of pregnancies involving fetuses with PUV is hampered by the fact that current clinical parameters obtained from fetal ultrasound and/or fetal urine biochemistry are insufficient to predict postnatal renal function. We previously have developed a fetal urine peptide signature (12PUV) that predicted with high precision postnatal renal failure at 2 years of age in fetuses with PUV. Here, we evaluated the accuracy of this signature to predict postnatal renal outcome in fetuses with PUV in an independent single-center study. Methods: Thirty-three women carrying fetuses with suspected PUV were included. Twenty-five fetuses received vesicoamniotic shunts during pregnancy. PUV was confirmed postnatally in 23 patients. Of those 23 fetuses, 2 were lost in follow-up. Four and 3 patients died in the pre- and perinatal periods, respectively. Follow-up renal function at 6 months of age was obtained for the remaining 14 patients. The primary outcome was early renal failure, defined by an eGFR < 60 mL/min/1.73 m2 before 6 months of age or pre- or perinatal death. Results: The peptide signature predicted postnatal renal outcome in postnatally confirmed PUV fetuses with an AUC of 0.94 (95%CI 0.74–1.0) and an accuracy of 90% (95%CI 78–100). The signature predicted postnatal renal outcome for the suspected PUV cases with an AUC of 0.89 (95%CI 0.72–0.97) and an accuracy of 84% (95%CI 71–97). Conclusions: This single-center study confirms the predictive power of the previously identified 12PUV fetal urinary peptide signature. [ABSTRACT FROM AUTHOR]

Background: The role of the voiding cystourethrogram (VCUG) in the follow-up of children with posterior urethral valves (PUVs) post-ablation has been considered a standard practice. The urethral ratio and gradient of change have proven to be useful. Objectives: We aimed to review the role of the 'ideal' ratio on predicting residual PUV post-ablation. Methods: A systematic review of the PubMed, SCOPUS and Web of Science databases was performed (April 2019). The search terms included 'Urethral Ratio and Posterior urethral valve ablation'. All cited reference lists were further evaluated for additional inclusive studies. Results: Eleven studies were identified, of which nine were relevant to the topic. Case reports, comments and adult and animal studies were excluded, leaving four studies for critical review. In total, 338 patients were assessed. The control group consisted of 167 age-matched, male children. Study regions included India and Australia. The ages ranged from 15 days to 3.4 years. Ablation methods included the use of a resectoscope with cutting diathermy, cold knife or Bugbee electrode. The mean urethral ratios in the control group ranged from 1.04 to 1.73. The suggested predictive urethral cut-off ratios recommended include 2.2 (p = 0.001), 2.5–3 and 3.5. Conclusion: Although the precise cut-off ratio could not be clearly defined in this review, a urethral ratio less than a range of 2.2–3.5 has proven to be a beneficial predictor of ablation success and should thus be incorporated into standard VCUG reporting templates in the follow-up of PUVs in male children in resource-limited settings. [ABSTRACT FROM AUTHOR]

Background: Pop-off mechanisms are potential pressure-relieving mediators in patients diagnosed with posterior urethral valves (PUV). This systematic review aimed to synthesize the existing evidence regarding the protective effect of pop-off mechanisms on renal function in children with PUV., Methods: We conducted a systematic review of the literature that involved an extensive search in the main databases of the medical bibliography. Three independent reviewers selected the relevant articles. Methodological quality was rated using the Newcastle Ottawa Scale index. We used random meta-analyses to compare different outcomes (serum creatine, Nadir serum creatinine, and Chronic Kidney Disease) between children with PUV and pop-off mechanisms and those with PUV without pop-off mechanisms., Results: 10 studies with data from 896 participants were included in this review. Seven articles reported serum creatinine values for each group and 3 of them found significant differences between groups. The random-effects meta-analysis for serum creatinine showed significant lower mean (diff = -52.88 μmol/L [95 % CI -73.65 to -32.11]) in the group of children with pop-off mechanisms, and the random-effects meta-analysis for Nadir serum creatinine showed a marginally significantly lower mean in the group of children with pop-off mechanisms (diff = -12.00 μmol/L [95 % CI -24.04 to 0.04]). The random-effect meta-analysis for Chronic Kidney Disease resulted in a significant risk reduction for the group of children with pop-off mechanisms (odds ratio = 0.48 [95 % CI 0.23 to 0.98])., Conclusions: Children with PUV and pop-off mechanisms show better renal function and lower risk of Chronic Kidney Disease than those with PUV without pop-off mechanisms suggesting these mechanisms may act as renoprotective mediators. The high heterogeneity between studies in the assessment of renal function and long-term outcomes calls for a cautious interpretation of these findings. Future studies that stratify by different types of pop-off mechanisms and use standardized metrics, such as Nadir creatinine, are needed., Competing Interests: Conflict of interest The authors declare that they have no conflict of interest., (Copyright © 2023 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Background: Concomitant anterior urethral valves (AUVs) and posterior urethral valves (PUVs) is an extremely rare congenital urologic anomaly, which may be easily overlooked in the clinic., Objective: This study assessed the prognosis of children with concomitant PUVs and AUVs., Methods: The clinical data of inpatients with concomitant AUVs and PUVs in our hospital were collected from January 1983 to June 2022. The clinical manifestations, auxiliary inspection, and treatment were described in detail., Results: In total, 6 cases of concomitant AUVs and PUVs in boys were found in our hospital, with ages ranging from 3 months to 9 years; the main clinical manifestation was abnormal urination. Four patients exhibited concomitant AUVs and PUVs preoperatively and underwent simultaneous anterior and posterior urethral valvotomy. Follow-up studies showed that 3 patients' clinical symptoms substantially improved with well-maintained renal function. One patient died of renal failure. In the other 2 patients, PUVs were initially identified and excised, but their clinical symptoms did not show substantial improvement. Following voiding cystourethrography (VCUG), the AUVs were found and obstructions were then completely relieved. However, 2 patients died of renal failure., Conclusions: If urinary symptoms cannot be substantially relieved after posterior urethral valvotomy, VCUG and cystoscopy should be repeated to shorten the interval between anterior and posterior urethral valvotomies to improve patient prognosis., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Introduction: Posterior urethral valves (PUV) is a chronic condition that can adversely impact child and family health related quality of life (HRQOL). Surprisingly, to date, the impact of this condition on the QOL of children with PUV has not been studied. Thus, our aim is to evaluate this important aspect of care using validated questionnaires in our institutional PUV clinic., Methods: From December 2022 to January 2024, the PedsQL Inventory and associated family impact module was distributed in our PUV clinic. Higher scores reflect better HRQoL and less impact on family. Children >8 years old completed the tool themselves, otherwise parents completed the tools on behalf of the child. We compared PUV scores against reported psychometric data from healthy children and other chronic illnesses., Results: A total of 112 families completed the questionnaires. The total HRQoL score was 80 ± 13. Overall, we noted that the domains reflecting psychosocial impact were significantly lower than those of physical well-being (76 ± 16 vs. 89 ± 15 for psychosocial and physical impact, respectively; p < 0.01). There was a significant difference in the psychosocial emotional impact for children managed with clean intermittent catheterization (CIC) compared to those who were not (69 ± 21 vs. 81 ± 16; p = 0.005) (Table 2). In addition, we noted that children with PUV scored similar to healthy controls, but higher than children with other chronic illnesses. With respect to the impact on family, overall, parent, and family impacts were 76 ± 19, 78 ± 21, and 81 ± 21, respectively., Discussion: Chronic illness has been associated with a negative impact on HRQoL in both adults and children. We noted similar findings the present study, particularly for young children and those who are maintained on CIC. To our knowledge, this is the first study to evaluate the impact of PUV on a child's HRQoL as well as the impact on the family, and to document a negative psychosocial impact of CIC in this population. However, there are some important limitations to be addressed including relying on families to complete questionnaires accurately during busy clinic visits and the lack of controls from the same environment., Conclusion: When compared to healthy controls, PUV patients score similarly in HRQoL assessment. In addition, children <3years of age report a negative impact on physical well being, and CIC negatively impacts the emotional well-being. These data can be used to advocate for better mental health supports and social work for these children., Competing Interests: Conflict of interest The authors have no conflicts of interest to disclose., (Copyright © 2024. Published by Elsevier Ltd.)

Background: Posterior urethral valves (PUV) represents a heterogenous spectrum in which guidelines for management are lacking particularly for those patients facing end-stage kidney disease and transplant. In this study we aim to 1) evaluate our long term PUV pediatric transplant outcomes compared to those without lower urinary tract dysfunction and 2) assess our PUV cohort for trends in bladder management and evaluate outcomes to inform development of institutional guidelines., Materials and Methods: A retrospective cohort analysis of all patients with a diagnosis of PUV who underwent kidney transplant from 2000 to 2023 was completed. A matched cohort of patients without lower urinary tract dysfunction was identified for comparison of graft function. Charts of PUV patients were reviewed for both sociodemographic and clinical variables. Patients were classified by bladder management at the time of transplantation into three separate groups for analysis: voiding, clean intermittent catheterization, and incontinent diversion. Primary outcomes of interest were eGFR, graft failure, and UTIs post-transplant., Results: 45 patients met inclusion criteria. 69% were on dialysis prior to transplant. 51% of grafts were from a deceased donor. Bladder management consisted of voiding (62%), CIC (4 via urethra, 10 via channel) (31%), and incontinent diversion (7%). 20% underwent augmentation cystoplasty (5 = ureter, 2 = gastric, 1 = colon, and 1 = ileum) prior to or at the time of transplant. Median follow up duration was 5.4 years (3.0, 10.8). Patients on CIC had higher rates of UTI; however, we found no significant difference in graft function outcomes (eGFR, graft failure) between bladder management groups or year of transplant. VUR in the transplant kidney was associated with vesicostomy (p = 0.028). 2 of 2 gastric augments developed malignancy, one of which was cause of death. Graft failure rate was 22% in both the PUV group and matched cohort, with median interval times to failure of 6.7 years and 3.7 years, respectively (p = 0.71). There were no differences in eGFR at follow-up time points between the PUV and matched cohort., Conclusions: Patients with PUV represent a spectrum of disease with heterogeneous management before and after kidney transplant. Overall, graft function outcomes were similar when compared to matched cohort without lower urinary tract dysfunction. Patients on CIC had higher rates of UTI but without impact on graft function. Gastric augmentation cystoplasty should be avoided given risk for malignancy. Guidelines to standardize evaluation and management would be helpful for patient care and outcomes., Competing Interests: Conflicts of interest These authors have no conflicts of interest pertaining to this subject matter., (Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)