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Purpose: Posterior urethral valves (PUVs) are the most common cause of congenital bladder obstruction in boys. Our aim was to assess the impact of early diagnosis and fulguration of PUVs on bladder function and compare their functional and urodynamic outcome with children who underwent delayed intervention. Materials and Methods: We retrospectively evaluated 153 patients who underwent primary valve ablation from two tertiary hospitals between 2001 and 2018. Patients have been divided into 2 groups, group 1 included 69 patients who were detected antenatally and underwent early fulguration of PUVs while group 2 included 84 children presented postnatally and underwent delayed valve ablation. The recorded data throughout follow-up in renal function tests, urodynamics and changes in the upper urinary tracts were evaluated and compared. Results: Median age at time of valve ablation was 10 days in group 1 and 7 months in group 2. The median follow-up period was 6.5 and 7 years in group 1 and 2, respectively. Chronic kidney disease (CKD) developed in 15 (22%) boys in group 1 while in group 2 it was observed in 31 (37%), p=0.04. While Q-max, mean bladder capacity and post-void residual (PVR) volumes were comparable in both groups, percent PVR was significantly higher in group 2 (3.27 vs. 1.44, p=0.002). Detrusor overactivity was slightly different in both groups (p = 0.07). Conclusions: Compared to delayed intervention, primary ablation of PUVs during the early neonatal life possibly provides the optimum chance to have optimum renal function without impact on bladder function.

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.

Persistent anhydramnios after placement of a vesicoamniotic shunt is a rare but potentially serious concern in a patient in utero. We present the case a male fetus who at 32 weeks gestation had bilateral hydroureteronephrosis, a distended bladder, and anhydramnios. A vesicoamniotic shunt was placed, but the anhydramnios persisted. Postnatally, the child was found to have a migrated shunt, left grade V vesicoureteral reflux (VUR) and posterior urethral valves (PUV). He underwent a PUV ablation and removal of the migrated shunt. The patient is now two years old and is doing well with no urinary tract infections or renal deterioration.

Posterior urethral valves (PUV) are the commonest cause of lower urinary tract obstruction detected during the antenatal and immediate postnatal period in male fetuses. Treatment options for PUV during the antenatal period include percutaneous vesico-amniotic shunt, fetoscopic fulguration of the valve and open fetal cystostomy. We report two cases of fetal cystoscopic laser valvotomy with fairly similar pre-operative profiles but contrasting postnatal outcomes, which re-iterates the fact that case selection in such cases continues to be a challenge. These are the first case reports on fetoscopic laser valvotomy in India to the best of our knowledge.

Posterior urethral valves are a common cause of congenital bladder outlet obstruction. Known associations include cardiac malformations and gastrointestinal abnormalities. In this case series, we report on two cases of PUV associated with anorectal malformations along with a case of PUV in monochorionic diamniotic twins. We explore the difficulty in achieving a diagnosis and the final management. The association of posterior urethral valves in a patient with anorectal malformation should be suspected in case of associated oligohydramnios or oliguria postnatally. There should be a high index of suspicion in twin pregnancy even if only one of the twins is suspected of bladder outlet obstruction.

A four-year-old boy presented painful episodes of priapism always before emptying the bladder since he was two years old, which led to investigation for a possible arteriovenous fistula of the perineal vascular system or a cavernous artery hyper flow. Following different exams in an attempt to establish the diagnosis, the child, actually, was found to have posterior urethral valves. The diagnosis was totally unforeseeable and the urethral valves endoscopic treatment reduced the priapism events with a positive effect on the disease.

Aim: To improve management of the posterior urethral valve (PUV) in children in a hospital environment. Patients and Methods: This was a retrospective descriptive study that covered a sixteen (16) years period, from January 1, 2002 to December 31, 2017, In pediatric surgery and urology departments of our teaching hospital, 26 cases of posterior urethral valve (PUV) were involved. The diagnosis was made by retrograde urethrocystography and micturition, supplemented by ultrasound. Results: During this period, 26 patients (1.7%) had a PUV. The mean age of the patients was 73, 2 months with extremes ranging from 6 to 180 months. Fifteen patients consulted for dysuria, 8 for complete retention of urine. Only one patient had a history of rolling the valves to the benign ones. The main complications found were urinary tract infection in 15 children, urolithiasis in 3 children and renal failure in 2 children. Two children had serum creatinine values of 50 and 58 mg/L. The lamination of the posterior urethral valves was performed in all patients. In our series, we had one death from chronic renal failure in a 6-year-old child with a bilateral mute kidney at IVU. Conclusion: The valve diagnosis of the posterior urethra is made late because the diagnosis is still postnatal.

Background: Posterior Urethral Valves (PUV) are the most common cause of congenital LUT obstruction in males. Biomarkers of glomerular or tubular injury may be of particular value in predicting the need for surgical intervention or in tracing progression of chronic kidney disease. Measurement of biomarker in urine is relatively easy. Aim: To evaluate the changes in values of urinary Transforming Growth Factor Beta 1(TGF-B1) and Monocyte Chemotactic Protein (MCP-1) before and after valve ablation and its prognostic value in Posterior urethral valve. Material and Method: This prospective study was conducted from September 2016 to August 2018. The study group included 20 consecutive male babies with the diagnosis of PUV treated and followed up versus equal numbers of age matched control without any renal or urinary tract disease. Pre-operative urine samples were collected in Operative room. Cystoscopy and valve ablation was done. Follow up was done clinically by urinary stream and radiologically with VCUG. Follow-up was planned at 1 month, 3 months and 6 months following cystoscopic valve ablation. All collected urine samples were centrifuged at 10,000 rpm for 20 minutes. Supernatant was collected and two divided aliquots were stored at -200c to be thawed on the day of assay. Optical density of each well was recorded at 450 nm and 540 nm A p-value of

This review focuses on the diagnosis, evaluation, and management of valve bladder syndrome (VBS) following initial treatment of posterior urethral valves (PUV). Based on current literature, we discuss the effect of PUV management in VBS and investigate the role of medications, catherization, surgery, and novel interventions. The current initial management of PUV involves valve ablation. Cutaneous urinary diversions are secondary options based on patient characteristics, PUV severity, and response to catheter drainage. VBS can independently occur as a consequence of PUV due to reduced nephron endowment leading to renal tubular dysfunction and polyuria, high bladder filling pressures secondary to a non-compliant bladder, and poor bladder emptying due to bladder neck hypertrophy. Options to mitigate the risk of upper tract deterioration include behavioral modifications and medical therapy (anticholinergics or alpha-blockers), bladder neck incision, clean intermittent catheterization and overnight catheter drainage, or more invasive approaches such as botulinum toxin injections and creation of a Mitrofanoff channel. Several experimental therapies to attenuate bladder fibrosis are currently under investigation. The management of VBS starts with initial management of PUV and is subsequently dependent on the evolving bladder filling dynamics and bladder neck hypertrophy affecting bladder filling and emptying. This requires a pre-emptive management protocol to identify VBS in boys with PUV to prevent renal damage using various therapeutic interventions. The lack of prospective studies to investigate effectiveness of available therapeutic options to treat VBS limits the development of an evidence-based approach for severity-based management of PUV-associated VBS.

Objectives: Posterior urethral vale is one of the major common congenital obstructive urethral lesions in male children. Endoscopic fulguration with observation is the treatment of choice for posterior urethral valves (PUV). This prospective study was carried out to assess the outcome of endoscopic fulguration of posterior urethral valves based upon the clinical, radiological and laboratory findings. Methods : The study started in Bangabandhu Sheikh Mujib Medical University Hospital in October 2004 on 50 male children with posterior urethral valves who were treated with endoscopic fulguration and came for routine follow up. Diagnosis was established by voiding cystourethrogram (VCUG) and confirmed by urethrocystoscopic findings.. The patients came for follow up after valve ablation at three months interval up to 1 year, and at six months interval for seven years. Results: Mean age of the patients was 2 years ranging from 1 to 12 years old. At diagnosis mean serum creatinine level ± SD was 90.4±44.8 μmol /litre and it decreased to 58.3±11.4μmol/litre at the end of 7 years follow up. Hydronephrotic changes decreased from decreased from 84% to 2%. Proteinuria decreased from 38% to 4%.Urinary tract infections decreased from 58% to 8% at the end of last follow up. Conclusion: In this study patients improved dramatically following fulguration of posterior urethral valves. But these patients need regular follow up because some of these patients may develop ESRD in the long run. Bangladesh Journal of Urology, Vol. 15, No. 2, July 2012 p.40-43

Background: Posterior urethral valve (PUV) is a significant cause of morbidity and mortality among male children resulting in renal failure in 25%–30% before adolescence irrespective of initial treatment. This study aimed at evaluating the early outcomes of children managed for PUV. Materials and Methods: This was a prospective study of all children who were treated for PUV between 2012 and 2016 at a single referral institution. Information reviewed included demographic and clinical data, imaging findings, pre- and post-operative serum electrolytes, and postoperative renal outcomes. Results: Twenty-nine male children were managed for PUV at a median age of 6 months including 7 (24.1%) neonates. Two (6.9%) patients had antenatal diagnosis. Micturating cystourethrogram confirmed PUV in all patients. Fourteen (48.3%) patients had impaired renal function (IRF) at presentation and 8 (57%) had improved renal function (RF) after initial catheter drainage. The mean creatinine at presentation was 1.86 ± 1.69 mg/dl and the mean serum creatinine following initial catheter drainage was 0. 93 ± 0.49 mg/dl (P = 0.003). For those patients with normal RF, the mean creatinine at presentation was 0.81 ± 0.22 mg/dl versus 0.74 ± 0.21 mg/dl (P = 0.012), following initial catheter drainage. Children with IRF on admission had mean creatinine at presentation of 2.61 ± 2.00 mg/dl compared to 1.17 ± 0.53 mg/dl (P = 0.002) after initial catheter drainage. Valve ablation was achieved with Mohan's valvotome in 26 (96.3%) patients. All patients had good urine stream at a median follow-up of 5 months. Four (13.8%) patients developed IRF at follow-up. Renal outcomes of patients presenting before 1 year and those presenting after 1 year were similar. Two children died preoperative of urosepsis and one out of hospital death given an overall mortality of 10.3% (n = 3). Conclusion: There was significant improvement in RF after initial catheter drainage. The incidence of IRF at follow-up was 13.8%. Long-term follow-up is necessary to identify patients at risk of end-stage renal disease.

Objective: To determine the diagnostic and therapeutic aspects of urinary tract anomalies in children in our environment. Patients and methods: This was a retrospective descriptive study conducted in the pediatric surgery and urology-andrology departments of the University Hospital of Brazzaville, from January 2012 to December 2017. Children aged 0 to 17 years old hospitalized for a malformation of the urinary tract were included. Results: The frequency of urinary tract anomalies was 1% of all patients admitted during the study period. The average age of the patients was 6.1 years (range, 0 - 17 years). The most common motive of consultation was ectopic urethral meatus (66.2%), vesical urine retention (18.7%) and lumbar spine pain (6.3%). Hypospadias was more common with 58.7%, followed by posterior urethral valves (20%), uretero-pelvic junction obstruction (7.5%). The most frequent preoperative complications were urinary tract infection (11.3%), urolithiasis (3.7%) and renal failure (2.5%). The treatment was surgical including urethroplasty (70.1%), or instrumental based on destruction of valves (16.9%). Conclusion: Urinary tract anomalies suffer late diagnosis and treatment in our practice. An antenatal screening strategy should be put in place to improve care to prevent the occurrence of complications.

Introduction: Posterior urethral valve (PUV) is the most common cause of bladder outlet obstruction and renal insufciency in male children. The aim of this study was to evaluate the presentation, management, challenges, and outcome of the disease in a Western Rajasthan tertiary health institution. Methods: In a retro-prospective study, 26 male children were included from November 2017 to March 2020 at Dr. S.N.Medical college, Jodhpur. Diagnosis was conrmed by voiding cysto-urethrography (VCUG). Results: A total of 26 patients were managed for PUV.The mean age of presentation was 3.45 years with 65.8% of the patients presenting after 1 year. Voiding dysfunction noted in 23(88.4%) patients was the most common presentation. Most common nding on physical examination was palpable bladder 15(57.6%) while UTI noted in 15 (57.6%) patients was most common complication. Abdominal sonography revealed dilated posterior urethra in 10(38.4%) cases, while VCUG revealed a dilated posterior urethra in 19 (75%) cases.The creatinine value at presentation ranged from 0.4-4.5 mg/dl with a mean of 1.10 ± 0.95 mg/dl. Valve ablation with a diathermy bugbee electrode & holmium laser provided relief of obstructions in the 23 (88.4%) patients who underwent the procedure without immediate complication. The period of follow-up ranged between 1 weeks to 2 years with a mean of 8.2 months. There was sustained improvement in urine stream, reduction in the mean creatinine concentration and incidence of UTI during follow-up. Conclusion: Many patients with PUVpresented late within the reviewed period. Valve ablation provided relief of obstruction in most of the cases. Efforts at improving awareness among general population and early diagnosis and referral among the health team should be encouraged. There is a need to counsel parents on the need for long-term follow-up

Posterior urethral valves (PUVs) are the most common cause of congenital urethral obstruction. However, the diagnosis of a PUV is sometimes difficult. A 13-year-old Japanese boy and his mother visited our hospital, and his mother complained that he frequently strained to void although he had no complaint. Uroflowmetry revealed a plateau-shaped curve and a voiding cystourethrography (VCUG) revealed a PUV. Thus, we performed a transurethral incision of the PUV, and his voiding status improved. Because some patients with mild PUV may not notice their dysuria, we believe that VCUG should be performed without hesitation when a urethral lesion is suspected.

Posterior urethral valves have myriad presentations based on the severity of obstruction with the milder end of spectrum often termed as mini-valves. The simultaneous occurrence of ureteropelvic junction obstruction and urethral valves has not been described before and is most likely coincidental. Herein, we discuss the management of three boys who had febrile urinary tract infection following pyeloplasty and on evaluation were found to have valves. This article highlights the need for considering these mini-valves as a possibility in boys presenting with symptoms following pyeloplasty so as to avoid delay in diagnosis and unnecessary morbidity in these children.

Purpose: Posterior urethral valves (PUVs) are the most common cause of congenital bladder obstruction in boys. Our aim was to assess the impact of early diagnosis and fulguration of PUVs on bladder function and compare their functional and urodynamic outcome with children who underwent delayed intervention. Materials and Methods: We retrospectively evaluated 153 patients who underwent primary valve ablation from two tertiary hospitals between 2001 and 2018. Patients have been divided into 2 groups, group 1 included 69 patients who were detected antenatally and underwent early fulguration of PUVs while group 2 included 84 children presented postnatally and underwent delayed valve ablation. The recorded data throughout follow-up in renal function tests, urodynamics and changes in the upper urinary tracts were evaluated and compared. Results: Median age at time of valve ablation was 10 days in group 1 and 7 months in group 2. The median follow-up period was 6.5 and 7 years in group 1 and 2, respectively. Chronic kidney disease (CKD) developed in 15 (22%) boys in group 1 while in group 2 it was observed in 31 (37%), p=0.04. While Q-max, mean bladder capacity and post-void residual (PVR) volumes were comparable in both groups, percent PVR was significantly higher in group 2 (3.27 vs. 1.44, p=0.002). Detrusor overactivity was slightly different in both groups (p = 0.07). Conclusions: Compared to delayed intervention, primary ablation of PUVs during the early neonatal life possibly provides the optimum chance to have optimum renal function without impact on bladder function.

Posterior urethral valves (PUV) constitute the most common infra-vesical urinary obstruction in boys. PUV are often accompanied by severe consequences to the lower and upper urinary tract (LUT, UUT). They also represent a major urological cause for pediatric renal transplantations. Surgical options for primary management invariably aim at abolition of valves. However, temporary urinary diversion may sometimes be a viable alternative, especially in critically ill patients or preterm infants. It was a retrospective, descriptive study which was conducted at the Department of Pediatric Surgery, Rajshahi Medical College from January 2018 to December 2018. All stable patients with the diagnosis of posterior urethral valves were included in the study. Endoscopic valve fulguration was performed in all diagnosed patients using bugbee electrode and an adequate sized cystoscope. The procedure was performed under general anesthesia and the urinary bladder was drained with a suitable size Foley's or silicon catheter for 14days. Patients were discharged from the hospital 48-72 hours after the procedure on oral antibiotics and were advised to come to the outpatient department for follow up visits for a period of 6 months. A total of 84 patients were included in the study. All were males with a mean age of 6.5years ranging from 6 months to 12 years. Sixty five patients were without any diversion while 19 had vesicostomy or ureterostomy already done in our department or somewhere else. Stricture urethra was seen in 5 patients, incontinence of urine was seen in 7 patients, nocturnal enuresis in 15 patients and recurrent urinary tract infection in 19 patients. Chronic renal failure was seen in 4 patients while 16 patients lost the follow up.18 patients had an uneventful recovery. Urethral valve ablation is the definitive treatment of posterior urethral valves. Endoscopic urethral valve fulguration is safe, effective and definitive way of management for posterior urethral valves. Early treatment improves the quality of life and prevents the ongoing renal damage. Early presentation in fetal and neonatal life has worst prognosis due to associated renal dysplasia. TAJ 2018; 31(2): 68-72

Introduction. Urethral valves (UVs) are congenital malformations of the urethra, leading to infravesical obstruction. The most common UV is the posterior urethral valve (PUV). The anterior urethral valve (AUV) is a somewhat rarer, but still well-known congenital anomaly. UVs can provoke significant obstruction of the proximal urinary system, which can later lead to disability of children and more frequent deaths.Materials and methods. The article presents a clinical example of a 32-year-old man with a congenital double urethral valve occuring in the anterior and posterior urethra, which led to the development of chronic urinary retention, urinary tract infection and chronic renal failure. The patient underwent a successful urethroplasty operation to remove the valve mechanism.Results. Six months following surgery, the patient noted the complete disappearance of symptoms, with renal function having returned to normal. No signs of recurrence of the disease or the formation of stricture of the urethra were detected by to the survey.Conclusion. A congenital variant of the double UV is an unusual extremely rare cause of infravesical obstruction. Early diagnosis and treatment of this anomaly is very important for preventing further irreversible damage to the urinary system. The presented clinical observation demonstrates the need to organise and conduct periodic preventive examinations of children of different age groups.

A posterior urethral valve (PUV) is a membranous fold within the lumen of the posterior urethra that causes functional obstruction of the urine outflow. It is a common cause of obstructive uropathy in boys during the neonatal period. The disorder varies in its severity; in extreme cases, PUVs can be incompatible with postnatal life while other patients can suffer from renal and respiratory failure associated with oligohydramnios. Milder cases can present later in life with urinary incontinence and other urinary symptoms. The management of patients with PUVs remains a clinical challenge and includes endoscopic surgical resection of the urethral valve and follow-up during early infancy and adulthood to avoid progressive bladder dysfunction and monitor for chronic renal disease. This review will address the management of PUVs during the prenatal period, the neonatal period, and throughout the entire life of the patient.

Premature newborns with posterior urethral valves (PUV) may present with medical conditions taking priority over definitive surgical care. We encountered three of such cases who underwent initial bladder decompression via transurethral catheterization and waited 2–3 weeks until they were fit enough for voiding cysto-urethrography to confirm PUV. An unexpected good urinary flow and negligible residual urine volume were documented during micturition, suggestive of valve disruption induced by insertion and prolonged duration of indwelling urethral catheter drainage. Cystoscopy documented non-obstructing remnant leaflets. Non-operative treatment may be considered as a viable alternative therapeutic option for PUV in tiny babies facing prolonged intensive care unit stay.

Neuropathic bladder may be a co-associated morbidity in newborn babies following resection of a sacrococcygeal teratoma. We report a case of a male newborn showing features of incomplete urinary voiding requiring intermittent catheterisation after operation for bladder emptying. Videourodynamic assessment excluded neuropathic bladder and posterior urethral valves were demonstrated on micturating cystography. Urology outcomes have been excellent following curative valve ablation. This report highlights the crucial importance of being aware of the rare coexistence of lower urinary tract pathology in male babies with sacrococcygeal teratoma. Routine urodynamic assessment should be considered in all children following sacrococcygeal teratoma resection.

BACKGROUND The aim of this study was to describe the complication profile of augmentation cystoplasty in contemporary paediatric urology as well as its effect on bladder metrics. METHODS Consecutive operative cases were retrospectively reviewed at a single institution over 20 years (1999-2019). Short- and long-term outcomes and complications following augmentation cystoplasty were defined. RESULTS Of the 71 operative cases; the most common underlying diagnoses were neurogenic bladder (34%), exstrophy-epispadias complex (30%) and posterior urethral valves (23%). The most common tissue-type utilized was ileal (58%) and ureteric (30%). Peri-operative urine leak affected nine (13%) children but reservoir perforations were less common (4%). Mean end-of-study detrusor pressure improved significantly following bladder augmentation (38-17 cmH2 O, P

Background The diagnosis of posterior urethral valves (PUV) encompasses a vast spectrum of disease with variable severity and clinical features. It is vital to understand the extent of developmental insult and to define the different distinct entities grouped together under the diagnostic umbrella of PUV. This would help to determine the severity of the disease, enable better prognostication, and optimize therapy. The objective of this study is to analyze the variable features of PUV and correlate the different manifestations with the embryological development of the urinary system. The possible developmental basis of anomalies in PUV is analyzed, as recognition of the underlying defect would help to determine the severity of the disease. A clinical classification and a risk stratification approach encompassing the spectrum of PUV is proposed, to help define diagnosis and guide prognosis. A combined retrospective and prospective analysis of cases diagnosed as PUV at the tertiary teaching institute over a 5-year period from July 2014 to July 2019 was done. The outcome of selected cases was analyzed, based on the risk group stratification. Results The incidence of major complications during follow-up in each risk group was assessed individually and found to be highest in the high-risk group (92%), which was significantly higher than the other groups. The intermediate risk group was found to have a complication rate of 38%, while the low-risk group had only 12.5% complication rate. The three key concepts addressed in this study pertain to the embryological basis of PUV, the necessity of a clinical subdivision of patients, and the possibility of risk stratification based on clinical criteria. The limited review of institutional series is added to highlight the method of risk stratification and its probable utility. Conclusions The most critical factors to be accounted for in the diagnosis and management of PUV are the definition of disease severity, recognition of systemic complications, and identification of children at risk of progression to ESRD. The proposed developmental defect analysis, description of PUV sequence, clinical classification, and risk stratification approach are only the means to this end of segregating patient groups within the diagnostic spectrum.

Summary Introduction Anterior urethral valves (AUV) are a rare cause of lower urinary tract obstruction which could progress to renal damage, Clinical presentation varies according with patient's age and severity of obstruction, but, in most cases, diagnosis is based on voiding cysto-urethrogram (VCUG). To date, the treatment of choice is endoscopic ablation even if approved guidelines about the overall management of AUVs, including the recognition and treatment of residual valves, are not available. Objective We describe our protocol for AUV treatment based on primary endoscopic valve ablation followed by check cystoscopy 15 days later. Study design Medical records of 5 patients with AUVs admitted from 2008 to 2018 to our Pediatric Urology Unit were retrospectively reviewed. Blood tests, urinalysis, renal US and VCUG were performed in all children, while urodynamic evaluation was performed in the 3/5 patients who could void spontaneously. All patients underwent endoscopic valves ablation and after 15 days after a second look cystoscopy was performed. Follow up was based on clinical and radiological evaluation with US, urinalysis and blood tests. Postoperative non-invasive urodynamic studies were performed in the 3/5 patient toilet-trained patients and VCUG was performed in 1/5 patient. Results and Discussion: At primary endoscopic ablation cystoscopy revealed AUVs in the penile urethra in three patients, in the penoscrotal urethra in one case, in the bulbar urethra in another case. In 3/5 patients check cystoscopy found residual valves and a second endoscopic ablation was performed. All patients achieved symptoms release and improved urodynamic parameters. No intra or post-operative complication were reported. The assessment of residual valves is variable in literature and it is usually described for posterior urethral valves (PUVs). Few series report the use of VCUG within the first week after valve ablation, our experience instead suggests that performing a second look cystoscopy, is very effective to evaluate the presence of residual AUVs and eventually proceed with further ablation. Conclusion Endoscopic ablation is the gold standard treatment for AUV, but residual valves management is not clearly defined. According to our experience, a check cystoscopy 15 days after primary ablation allows to identify and treat possible residual valves showing good results in terms of safety and efficacy.

BACKGROUND Urinary diversion using catheterizable channel among posterior urethral valve (PUV) patients may help to alleviate the functional concerns of the bladder on the allograft. Herein, we review our series of PUV patients undergoing renal transplants at a single institution to determine outcomes between those with and without pretransplant Mitrofanoff. METHODS PUV cases in our transplant database from 2000 to 2017 were identified. Data collected included clinical demographics, presentation of PUV, bladder function, implementation of dialysis and donor type. Post-transplant variables included graft function, and bladder function including incontinence and UTI development. Comparison between with vs without pretransplant Mitrofanoff patient groups was performed using Fisher's exact and t test analysis. RESULTS Overall, 22 cases were analyzed. The majority (77%) had a prenatal diagnosis of PUV, and 23% had a symptomatic presentation. Pretransplant Mitrofanoffs were performed in 40.9% of cases. The overall age at transplant was 10 ± 5 years. Five percent experienced post-transplant incontinence and 23% UTIs. No difference between groups was noted in variables including age at transplant, the timing of PUV diagnosis. Mitrofanoff group patients had more symptomatic febrile UTIs than those without Mitrofanoff (67% vs 15%; P = .03); however, there was no difference in post-transplant UTI development. Furthermore, the Mitrofanoff group seems to have later onset of dialysis or need for transplant (age at the start of dialysis 36 ± 56 vs 156 ± 25 months, P

Background: Lower urinary tract symptoms (LUTs) are common in young boys with posterior urethral valves (PUVs). It is crucial to investigate the characteristics of PUV patients with and without LUTs after valve ablation.Methods: Between January 2017 and December 2019, PUV patients visited Children's Hospital, Fudan University for following up were enrolled. Medical records and information from the patients' urodynamic studies (UDS) were reviewed.Results: A total of 54 enrolled PUV patients were divided into symptomatic PUV group (28 cases) and non-symptomatic PUV group (26 cases) according to daytime incontinence or not, and 21 OAB cases without structural abnormalities were set as UDS control group. The non-symptomatic PUV patients had lager filling volume (135 ± 46% of EBC) than the symptomatic PUV patients and OAB patients (106.1 ± 44.4% of EBC, p = 0.0255 and 88.1 ± 39.6% of EBC, p = 0.0007, respectively). The detrusor pressure at 1/4 and 3/4 of full filling was higher in PUV groups than the control group, but no significant difference was found between the PUV groups. PUV patients with LUTs had a higher rate (19/28, 67.9%) of impaired bladder compliance than non-symptomatic PUV patients (11/26, 42.3%, p = 0.0489). The PUV patients with LUTs had a trend of worse kidney functions in lower GFR, higher serum creatinine and lower estimated GFR.Conclusion: PUV patients have higher detrusor pressure regardless of the presence or absence of LUT symptoms. Bladder function assessments are needed in boys with PUV, even without incontinence symptoms after valve ablation.

Posterior urethral valves are rare, congenital obstructive membranous folds in the urethra that can lead to a range of bladder and renal complications for children. The mainstay of treatment is valve ablation. In those who cannot undergo immediate valve ablation, urinary drainage with catheterization or vesicostomy may be used as a temporizing measure until valve ablation can be performed. Diagnosis and treatment should be undertaken as early as possible to minimize the downstream risk of associated bladder and renal dysfunction in these children.

Introduction: delayed presentation of posterior urethral valves (PUVs) is a rare condition. Presentation and diagnosis of the patients with late PUVs are challenging. Voiding cystourethrogram (VCUG) is mainly practiced. In this study, we aimed to evaluate the children with late-presented PUVs, and the reliability of VCUG in this group. Materials and methods: between January 2003 and December 2017 records of patients who were diagnosed with late-presented PUVs were analyzed. Delayed presentation of PUV was defined as patients who were diagnosed and treated after infancy. Cases were examined in terms of age at diagnosis, presenting symptoms, urinalysis, urinary ultrasound, urodynamic studies, VCUG, and dimercaptosuccinic acid scintigraphy findings. Postoperative follow-up conditions were also assessed. Results: seventeen boys were diagnosed with late-presented PUVs (mean age was 7.35 years). The most common symptoms at presentation were frequency (58.8%), day and nighttime incontinence (47%), and febrile urinary infection (41%). PUV was noted by VCUG in 10 patients alone. The classical sign of dilated posterior urethra was detected in 9 patients. The 10th patient had posterior urethral irregularity. Urethra could not be evaluated due to unsuccessful voiding in one patient. Six patients had normally appearing urethra on VCUG. Reflux was detected in nine (52.9%) patients. Conclusion: late-presented PUVs may be missed on VCUG. Whether a PUV might be present is crucial in boys with a history of recurrent urinary infection, persistent reflux, and repetitive daytime incontinence. Based on our results, we conclude that cystoscopic examination should be preferred for those cases to diagnose PUVs regardless of VCUG results., NA

Anterior urethral valves are a rare cause of obstructed voiding in adolescent children and are often unheard by adult urologists. In this case report, we discuss the management of two adolescent patients who were referred to us with obstructive voiding symptoms with a diagnosis of neurogenic bladder and posterior urethral valves respectively but on evaluation were found to have anterior urethral valves. This article highlights the need for considering anterior urethral valve as an important differential diagnosis in children and adolescents presenting with obstructive voiding symptoms so as to avoid delay in diagnosis and management in young boys.

Background. Posterior urethral valves (PUV) are a common cause of congenital obstructive nephropathy. The outcome of patients with PUV at Chris Hani Baragwanath Academic Hospital in Johannesburg, South Africa, has not been documented previously. Objectives. To describe the outcome of patients diagnosed with PUV over a 29-year period from January 1985 to December 2013, and to analyse risk factors for chronic kidney disease. Methods. This was a retrospective record review of boys aged

Durch intrauterine oder postnatale sonographische Screeninguntersuchungen werden zunehmend haufig Dilatationen der Harnwege oder Nierenfehlbildungen entdeckt. Die wenigsten Harntraktanomalien bedurfen der unmittelbaren Therapie. Vor allem ureteropelvine Stenose und primarer Megaureter erfordern jedoch eine prognostische Einordnung zwischen einer korrekturbedurftigen Obstruktion und einer Dilatation ohne Auswirkung auf die Nierenfunktion. Trotz zunehmender Zahl von Biomarkern bleiben derzeit Sonographie, Diureseszintigraphie und/oder Magnetresonanztomographie die tragenden Saulen der weiterfuhrenden Diagnostik. Die Verhinderung progredienter Nierenparenchymschaden ist Hauptziel diagnostischer und therapeutischer Strategien unter Vermeidung unnotiger oder unnotig belastender Interventionen.

Posterior urethral valves are the most common cause of bladder outlet obstruction in male newborns. Initial catheter drainage alleviates the urethral obstruction before definitive management by valve ablation. Newborns with posterior urethral valves often present with hypercontractile bladders that may inhibit upper tract drainage despite bladder catheterization. Anticholinergic agents are commonly used to treat detrusor hyperactivity, with oxybutynin being the most commonly used. We report the first case of a newborn with posterior urethral valves and ureterovesical junction obstruction caused by detrusor hypertrophy who underwent urgent intravesical instillation of oxybutynin at high doses in an attempt to avoid a diversion procedure.

BackgroundAs trabeculated bladder wall is often referred to as a sign of chronically increased intravesical pressure, we investigated whether voiding cystourethrography (VCUG) or sonography reliably predicts bladder trabeculation on later urethrocystoscopy.MethodsA total of 76 consecutive patients (2012–2017) with cystoscopically confirmed posterior urethral valves (PUV) and pre-endoscopy VCUG were included. Sonography data were available for 68 of these patients. Radiological findings were reassessed and compared with endoscopic findings using Fisher’s exact test and Spearman’s rank assessment.ResultsVCUG showed a sensitivity of 83.3% and a specificity of 30% in predicting trabeculation on a later urethrocystoscopy, with no significant difference in determining mild or severe forms (p=0.51). Sonography proved a sensitivity of 27.6% and a specificity of 70%, with no correlation between sonographic signs and trabeculation on cystoscopy (r=0.1311). In addition, vesicoureteral reflux had no significant influence as a possible pressure pop-off mechanism on the development of trabeculation in our group.ConclusionsWhile VCUG predicts bladder trabeculation in children with PUV with limited reliability, sonography mostly fails to detect trabeculation. Therefore, such findings should only be used with utmost caution in relation to clinical decision-making.

Posterior urethral valves are the most common cause of partial bladder outlet obstruction (PBOO) in the pediatric population. Pathological changes in the bladder developed during PBOO are responsible for long-lasting voiding dysfunction in this population despite early surgical interventions. Increasing evidence showed PBOO induces an upregulation of hypoxia-inducible factors (HIFs) and their transcriptional target genes, and they play a role in pathophysiological changes in the obstructed bladders. We hypothesized that blocking HIF pathways can prevent PBOO-induced bladder dysfunction. PBOO was surgically created by ligation of the bladder neck in male C57BL/6J mice for 2 wk. PBOO mice received intraperitoneal injection of either saline or 17-DMAG (alvespimycin, 3 mg/kg) every 48 h starting from day 1 postsurgery. Sham-operated animals received injection of saline on the same schedule as PBOO mice and served as controls. The bladders were harvested after 2 wk, and basal activity and evoked contractility of the detrusor smooth muscle (DSM) were evaluated in vitro. Bladder function was assessed in vivo by void spot assay and cystometry in conscious, unrestrained mice. Results indicated the 17-DMAG treatment preserved DSM contractility and partially prevented the development of detrusor over activity in obstructed bladders. In addition, PBOO caused a significant increase in the frequency of micturition, which was significantly reduced by 17-DMAG treatment. The 17-DMAG treatment improved urodynamic parameters, including increases in the bladder pressure at micturition and nonvoid contractions observed in PBOO mice. These results demonstrate that treatment with 17-DMAG, a HIF inhibitor, significantly alleviated PBOO-induced bladder pathology in vivo.

Summary Paediatric urological surgery is often required for managing congenital and acquired disorders of the genitourinary system. In this Series paper, we highlight advances in the surgical management of six paediatric urological disorders. The management of vesicoureteral reflux is evolving, with advocacy ranging from a less interventional assessment and antimicrobial prophylaxis to surgery including endoscopic injection of a bulking agent and minimally invasive ureteric reimplantation. Evidence supports early orchidopexy to improve fertility and reduce malignancy in boys with undescended testes. A variety of surgical techniques have been developed for hypospadias, with excellent outcomes for distal but not proximal hypospadias. Pelvi-ureteric junction obstruction is mostly detected prenatally; indications for surgery have been refined with evidence, and minimally invasive pyeloplasty is now standard. The outlook for patients with neurogenic bladder has been transformed by a combination of clean intermittent catheterisation, algorithms of diagnostic investigations, and innovative medical and surgical therapies. Posterior urethral valves are associated with considerable mortality; fetal diagnosis allows stratification of candidates for intervention, but ongoing bladder dysfunction in patients after valve ablation remains a cause of long-term morbidity.

Congenital anterior urethral valve is a rare condition causing significant obstructive uropathy in pediatric age group. It is much rarer than posterior urethral valve. However, the clinical course is similar. We present two cases of anterior urethral valves in children.

Posterior urethral valves (PUVs) present clinically across a varied spectrum, ranging from severe obstruction with massive distension of the bladder and upper urinary tracts in the fetus, to a much more indolent course with minor secondary changes in the young or older child. Type III (diaphragm) PUVs are relatively uncommon, and are associated with difficulty in passing a transurethral catheter into the bladder as well as smooth dilation of the bladder. The following case examines the unusual experiences of both diagnosing PUVs in a teenager, and capturing visual evidence of type III valves during cystourethroscopy.

Introduction: Posterior urethral valves (PUVs) are the most common cause of obstructive uropathy in boys. While most patients in developed countries are diagnosed in the antenatal period, our patients often present late which adversely affects their outcome. This retrospective study was aimed to study the clinical profile of boys with PUV at follow-up. Methods: Records of 45 boys with PUV who were in follow-up for at least 1-year were retrieved between February and December 2015. Age of presentation, signs and symptoms, anthropometry, radiological findings, surgical interventions, and biochemical investigations were recordedin a pre-structured pro forma. The data were later analyzed on an Excel spreadsheet. Results: The mean age at the diagnosis of PUV was 16±2.8 months, and the mean age at enrollment was 70.6±53.5 months. The condition was detected antenatally in 5 (10.9%) subjects only. At the time of data collection, the mean height and weight standard deviation scores were ?1.7 and ?1.5, respectively. Hypertension was present in 29% of patients. 44.5% of subjects had moderate-to-severe malnutrition and 42.2% had short stature. Mean GFR of the subjects was 66.3 ml/min/1.73m². Bilateral hydronephrosis was present on ultrasound in 26 (57.8%) subjects. Mean anteroposterior diameter on ultrasound was 22.1 mm for the right kidney and 20.8 mm for the left kidney. Significant post-void residue was detected in 54.6% of patients. Bilateral reflux was seen in 21.7% of subjects on micturating cystourethrogram. Urodynamic studies were available for 16 patients, and 31.3% had significant post-void residual urine. Detrusor instability was present in 18.8% of subjects. 35.6% of subjects underwent valve fulguration alone as the surgical procedure while 19 (42.2%) had a diversion procedure (vesicostomy/ureterostomy) along with valve fulguration. Conclusions: Most of the patients with PUV arestill diagnosed postnatally and have significant renal damage at presentation. Urinary diversion procedures are required in almost half of these patients. All these contribute to a poor long-term outcome.