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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.

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.

Early kidney and anatomic features may be predictive of future progression and need for additional procedures in patients with posterior urethral valve (PUV). The objective of this study was to use machine learning (ML) to predict clinically relevant outcomes in these patients. Patients diagnosed with PUV with kidney function measurements at our institution between 2000 and 2020 were included. Pertinent clinical measures were abstracted, including estimated glomerular filtration rate (eGFR) at each visit, initial vesicoureteral reflux grade, and renal dysplasia at presentation. ML models were developed to predict clinically relevant outcomes: progression in CKD stage, initiation of kidney replacement therapy (KRT), and need for clean-intermittent catheterization (CIC). Model performance was assessed by concordance index (c-index) and the model was externally validated. A total of 103 patients were included with a median follow-up of 5.7 years. Of these patients, 26 (25%) had CKD progression, 18 (17%) required KRT, and 32 (31%) were prescribed CIC. Additionally, 22 patients were included for external validation. The ML model predicted CKD progression (c-index = 0.77; external C-index = 0.78), KRT (c-index = 0.95; external C-index = 0.89) and indicated CIC (c-index = 0.70; external C-index = 0.64), and all performed better than Cox proportional-hazards regression. The models have been packaged into a simple easy-to-use tool, available at https://share.streamlit.io/jcckwong/puvop/main/app.py ML-based approaches for predicting clinically relevant outcomes in PUV are feasible. Further validation is warranted, but this implementable model can act as a decision-making aid. A higher resolution version of the Graphical abstract is available as Supplementary information

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.

Objectives The aim of this retrospective study is to describe the importance of serum creatinine levels for the long-term renal outcome in cases of posterior urethral valve. Methods Records of 94 patients who underwent cystoscopic valve ablation were evaluated retrospectively. Demographic variables, radiologic findings, and serum creatinine levels were recorded. Patients were grouped according to the serum creatinine levels in the first month of life. Twenty-four patients' serum creatinine levels were found to be above 1 mg/dL in group 1 and ≤1 mg/dL in 70 patients in group 2. Results The initial findings of 74 patients were as follows: pyelonephritis in 24 (32.43%) patients, hydronephrosis in 20 (27.02%), and inability to urinate in 18 (24.32%). Prenatal hydronephrosis was present in 36 cases (38.29%). Of the 92 voiding cystograms, 53.26% had findings that included vesicoureteral reflux. The mean serum creatinine levels of the patients in group 1 increased above 1 mg/dL in the 2nd year, the significant difference between the two groups lasted up to 7 years of age, and no significant difference was observed at the age of 7. The mean serum creatinine levels of the patients in group 2 were observed to increase above 1 mg/dL by the 10th year. Conclusions In patients with posterior urethral valves, nadir creatinine, especially higher than 1 mg/dL, should be considered as high risk for end-stage renal disease. Therefore, clinicians should improve awareness about early detection and treatment of posterior urethral valves, and these patients should be monitored as early as possible for the risk of chronic kidney disease.

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.

A significant proportion of PUV becomes symptomatic after the perinatal period. Voiding cystourethrography (VCUG) often fails to identify PUVs. This study evaluates the relationship between the radiological appearance of the posterior urethra, potential secondary radiological signs and endoscopically documented PUV in boys with febrile UTIs, VUR, refractory symptoms of bladder overactivity or suggestive sonography findings. Data on VCUG findings and endoscopy from 92 boys (mean age 27 months) who underwent endoscopic PUV incision between 2012 and 2017 following a VCUG were reviewed. 24 boys with endoscopically unsuspicious urethras were included as control group (mean age 27.5 months). Statistical analysis was performed using Fisher’s exact test. In patients with PUV, the urethra was suspicious on a preoperative VCUG in 45.7%, whereas it appeared normal in 54.3%. Abortive forms of PUV were more frequently found in patients with a radiologically unsuspicious urethra (30%vs.16.7%, p = 0.15). Bladder neck hypertrophy on VCUG (16.7%vs.60.9%, OR 7.5, p

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

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.

Congenital pulmonary airway malformation is a rare developmental anomaly of the lower respiratory tract. It is classified into three major types depending on the size and number of cysts. Though the type II CPAM is known to be associated with congenital malformations, co-existence with a lower urinary tract obstruction (LUTO) has not been documented. We describe a novel combination of CPAM with megacystis due to posterior urethral valves in one of the twins of a monozygotic twin pregnancy. Further, we discuss the cardiovascular effects of megacystis. This was previously considered to be due to pressure effect of megacystis on iliac veins and inferior vena cava. Recently activation of RAS system has been shown to predict the severity of cardiac disease. Further studies will clarify the precise etiology of cardiac dysfunction in LUTO and megacystis.

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

INTRODUCTION AND OBJECTIVE:Approximately 20% of boys with posterior urethral valves (PUV) develop ESRD; however, few factors associated with the risk of ESRD have been identified. To predict renal ...

INTRODUCTION AND OBJECTIVE:Posterior urethral valves (PUV) are a leading cause of chronic renal failure in childhood. A consistently validated prognostic indicator of renal outcomes is the creatini...

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

Objective: To analyze which factors in the history of a posterior urethral valve (PUV) patient relate to lower urinary tract symptoms (LUTS) in adulthood. This study also aimed to evaluate whether the patients have developed signs of a myogenic failure of detrusor. Materials and methods: LUTS were evaluated with a DAN-PSS questionnaire in 78 adult patients treated for PUV in childhood. Symptom scores (SS) were compared with patient characteristics and types of treatment. The results of uroflowmetry and post-voiding residual (PVR) measurements were collected from their latest visit to Helsinki University Children's Hospital. Results: The median total symptom score (TSS) was 1 (IQR = 0-5, range = 0-18). The patient age during the investigation and SS were associated (R = 0.220, p = 0.032), but the primary creatinine level and SS were not (R = 0.260, p = 0.081). The median age for achieving continence was 6.3 years. The SS and the age when becoming continent were not associated (p = 0.365 and p = 0.679, respectively). In the age group of 39 years or more, 19 patients with a previous bladder neck incision (BNI) had a higher storage-SS than 15 patients without any previous bladder neck incision (1 (IQR = 0-5) vs 0 (IQR = 0-0), p = 0.030). According to symptoms and post-voiding residual measurements, myogenic failure in adults seems to be rare. Conclusions: LUTS are common, but severe symptoms referring to myogenic failure are rare in men treated for PUV in childhood. The specific reasons for LUTS are difficult to indicate and even the sickest children do not necessarily have LUTS in adulthood.

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.

OBJECTIVE To construct reference values for fetal urinary bladder distension in pregnancy and use Z-scores as a diagnostic tool to differentiate posterior urethral valves (PUV) from urethral atresia (UA). METHODS This was a prospective cross-sectional study in healthy singleton pregnancies aimed at constructing nomograms of fetal urinary bladder diameter and volume between 15 and 35 weeks' gestation. Z-scores of longitudinal bladder diameter (LBD) were calculated and validated in a cohort of fetuses with megacystis with ascertained postnatal or postmortem diagnosis, collected from a retrospective, multicenter study. Correlations between anatomopathological findings, based on medical examination of the infant or postmortem examination, and fetal megacystis were established. The accuracy of the Z-scores was evaluated by receiver-operating-characteristics (ROC)-curve analysis. RESULTS Nomograms of fetal urinary bladder diameter and volume were produced from three-dimensional ultrasound volumes in 225 pregnant women between 15 and 35 weeks of gestation. A total of 1238 urinary bladder measurements were obtained. Z-scores, derived from the fetal nomograms, were calculated in 106 cases with suspected lower urinary tract obstruction (LUTO), including 76 (72%) cases with PUV, 22 (21%) cases with UA, four (4%) cases with urethral stenosis and four (4%) cases with megacystis-microcolon-intestinal hypoperistalsis syndrome. Fetuses with PUV showed a significantly lower LBD Z-score compared to those with UA (3.95 vs 8.83, P

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

Objectives Lower urinary tract obstruction (LUTO) has different etiologies. The most common form is posterior urethral valve (PUVs). It is a condition that can lead to end stage renal disease (ESRD) in later life and severe renal and pulmonary damage in utero. Those born alive may end up with residual kidney disease leading to life long morbidity or death. This condition can be diagnosed through prenatal ultrasound but because diagnosis is done late when kidney injury has already happened it does not guarantee healthy survival after birth. There are various treatments in utero and after birth but they all have their complications. In developing countries diagnosis is possible but in most cases pregnant women with fetuses with PUVs present very late in pregnancy. In utero interventions are in most cases not possible and the only way out is expectant management with serial ultrasound assessment until the fetus has achieved level of maturity at which time it is delivered and further postnatal evaluations and treatment given. This series is aimed at highlighting the prenatal ultrasound features of PUVs and the management challenges faced by physicians in developing countries. Case presentation The three cases were managed in three different hospitals. All the cases presented at different gestational ages and different levels of fetal affectation. Because of these, outcomes of management and prognosis of each case differ. Conclusions Fetal PUVs pose specific challenges in management because of late presentation and lack of resource for intervention even where cases present early with minimal renal damage.

Fetal lower urinary tract obstruction (LUTO) is classically based on prenatal ultrasound identification of a dilated/ thick-walled bladder, bilateral hydronephrosis, dilated ureters and a dilated posterior urethra (also known as the "keyhole sign") in a male fetus. Although the most common underlying diagnosis is posterior urethral valves, the prenatal appearance may be similar with urethral atresia or stenosis, the Prune-Belly Syndrome, or even a cloacal anomaly in a female. These conditions form part of the Congenital Anomalies of Kidney and Urinary Tract (CAKUT) spectrum, which is the commonest cause of end-stage renal disease in children. Although it is difficult to predict postnatal renal function from the prenatal appearance, studies have recently identified predictive features (based on ultrasound findings and fetal biochemistry), and established staging systems to assist with counselling, and, where indicated, patient selection for in-utero intervention. Current in-utero therapy includes amnio-infusion, vesico-amniotic shunting, and fetal cystoscopy with valve ablation or urethral stenting. Postnatal survival and renal functional outcomes, complications and management uncertainties are described, highlighting areas of future development.

Posterior urethral valves (PUVs) are frequently recognized during the perinatal period. Delayed diagnosis is reported usually within the first decade of life with diverse clinical presentations. In the current case report, we describe a 45 years old man patient who presented with aspermia and primary infertility for 8 years in whom his diagnostic workup revealed radiological imaging suggestive of PUVs. This phenomenon was confirmed by cystourethroscopy that showed obstructive valve. Endoscopic ablation resulted in significant improvement of his seminal parameter with successful conception.

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.

Aim To investigate and describe factors contributing to the impact of caring for a child with posterior urethral valves and to determine the extent to which families view their care as family-centered, and whether this can affect the impact of care. Methods A cross sectional, survey-based study involving primary caregivers of children with posterior urethral valves. Caregivers completed the Impact on Family Scale (IOFS) and the Measure of Processes of Care (MPOC). Regression analyses were used to assess the relationship between child-related variables, demographic variables, MPOC, and IOFS scores. Results 31 primary caregivers completed both questionnaires. Caregivers of children with moderate/severe chronic kidney disease (CKD) scored higher on the IOFS compared to those caring for children with mild CKD. Caregivers rated care received highly but reported lower scores in subscales related to information provision. Receiving less respectful and supportive care was significantly associated with lower IOFS scores. Conclusion The impact of caring for a child with posterior urethral valves is variable and is strongly determined by the severity of the associated CKD. Further research is required to show whether improving family-centeredness of care can reduce the impact of caring for children with posterior urethral valves.

Background. Congenital urethral obstruction occurs most frequently as a result of urethral valves. The diagnosis is usually confirmed pre- or neonatally. Though not ideal, delayed diagnosis can occur in childhood, adolescence, or adulthood. Despite a normal prenatal ultrasound, there may still be a delayed diagnosis of urethral valves. Methods. We present 4 patients with delayed diagnosis of posterior urethral valves (PUV) and 1 patient with delayed diagnosis of anterior urethral valves (AUV) who were evaluated by a pediatric urologist at our Institution. We determined the age and symptoms at presentation, physical examination, micturating cystourethrogram (MCU) findings prior to the valve ablation, renal function before and after valve ablation, type of valve ablation, and urine culture and sensitivity. The urological courses following urethral valve ablation including urodynamic study findings are described. Results. The median age at presentation was 33 months. All 5 patients presented with decreased urine output and urinary retention. All 5 patients underwent a MCU that demonstrated bladder trabeculations (3 cases), vesicoureteral reflux (3 cases), and bladder diverticula (2 cases). A urethral valve ablation was performed in all cases. Four patients underwent a renal function panel prior to this procedure, and their serum BUN/creatinine levels decreased 1 day postoperatively. Conclusion. Pediatricians should consider urethral valves as causing urethral obstruction although the prenatal ultrasound may be normal. Early diagnosis and prompt treatment of urethral valves may mitigate the potentially devastating morbidities such as renal failure, congestive heart failure, and respiratory distress that may ensue.

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.

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.

Aim: Describe the clinical, radiological and management of patients with posterior urethral valves. Materials and Methods: This was a retrospective descriptive study over a period going from January 2011 to December 2016, reviewing 12 cases of patients treated for valves of the urethra posterior to the medical surgical center of urology in Cameroon at Douala. The variables studied were clinical, paraclinical, therapeutic and evolutionary. Results: In 5 years, we had diagnosed and treated 12 cases of posterior urethral valves, an average of 2.4 per year. The average age of patients was 41.6 months with extrems of 3 and 156 months. The reason for consultation was dominated by urinary tract infections. Urethrocystography and echography were performed in all patients and allowed to objectify a bilateral ureterohydronephrosis, a diverticular bladder in 9 cases and 4 cases of vesico-ureteral reflux, including a grade 4 according to the classification of Cendron, 8 patients suffered from acute urinary failure. Renal scintigraphyrealised on 3 patients had as objective in 1 patient the existence of a disfunctioning kidney. The treatment consisted of an endoscopic incision of the valves (n = 8), a vesicostomy (n = 3) and an iterative urethral dilatation with double J catheter placement (n = 1). The evolution was favorable (n = 7), and 5 cases of death were recorded. Conclusion: The endoscopic incision is the gold-standard of the treatment of the valves of the posterior urethra. The vesicostomy is an alternative in the absence of adequate technical platform. Despite adequate support, the prognosis remains burdened with significant mortality.

This case describes a 7-week-old male infant presenting with vomiting and decreased urine output. Initial point-of-care ultrasound (POCUS) demonstrated a normal pylorus; however, assessment of bladder volume revealed the problem. The bladder was distended with a thickened, trabeculated wall and there was bilateral hydroureter, consistent with bladder outlet obstruction. Renal POCUS revealed bilateral hydronephrosis and perinephric fluid collections consistent with calyceal rupture. A voiding cystourethrogram confirmed the diagnosis of posterior urethral valves which were eventually ablated by urology. To our knowledge, this is the first case report of POCUS leading to a diagnosis of posterior urethral valves in an infant. This case highlights how POCUS can expedite evaluation of decreased urine output.