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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.

Introduction Posterior urethral valve (PUV) is the leading etiology of lower urinary tract obstruction (LUTO) in boys and is an important cause of end-stage renal disease (ESRD) in children. The aim of this article is to review the current status on antenatal intervention for PUV as well as postnatal medical and surgical management. Main body MEDLINE, EMBASE, Pubmed and Google Scholar search was conducted throughout December 2020 using the keywords: posterior urethral valves, congenital urinary obstruction, antenatal, valve bladder syndrome, medical, anticholinergics, alpha blocker, antibiotics, biofeedback, diversion, augmentation cystoplasty and renal transplantation. Only papers written in English were included. The relevant literature was summarized. Despite advances in antenatal intervention for fetal LUTO, it remains associated with considerable fetal and maternal morbidity. Patient selection criteria for antenatal intervention are greatly debated. Fetal intervention has resulted in improved perinatal survival in properly selected cases, with a questionable benefit to postnatal renal function. There is decent evidence supporting the use of anticholinergics in infants and young children following valve ablation, with less robust evidence advocating alpha blockers, overnight catheter drainage and biofeedback. The role of urinary diversion remains contentious. Optimizing bladder dynamics is crucial for successful renal transplantation outcomes. Conclusion While antenatal intervention has failed to improve renal function outcomes of PUV, patient-centered medical and sometimes surgical interventions can help delay the progression of chronic kidney disease. Lifelong monitoring and management of the associated bladder dysfunction is indispensable even after successful renal transplantation.

In a male neonate with bilateral hydroureteronephrosis, the most common surgical diagnosis is posterior urethral valves. This case report describes a male infant with the same presentation, but caused by a very uncommon congenital anomaly. The summation of different imaging modalities allowed a multidisciplinary team of colleagues to define the anatomy: bilateral duplex kidneys draining into separate urinary bladders. Only one of the bladders had an outlet, hence the obstructive uropathy to the right kidney led to total loss of function. The distended tortuous ureters produced a mass effect at presentation. This case acts as a reminder that complex congenital anomalies can mimic the presentation of more common conditions, and that they often require input from various specialists to diagnose the condition and guide its management.

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

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.

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.

Objective: Posterior urethral valve is the most common lower urinary tract obstruction in male children. A high percentage of patients with posterior urethral valve evolve to end‐stage renal disease. Previous studies showed that cytokines, chemokines, and components of the renin–angiotensin system contribute to the renal damage in obstructive uropathies. The authors recently found that urine samples from fetuses with posterior urethral valve have increased levels of inflammatory molecules. The aim of this study was to measure renin–angiotensin system molecules and to investigate their correlation with previously detected inflammatory markers in the same urine samples of fetuses with posterior urethral valve. Methods: Urine samples from 24 fetuses with posterior urethral valve were collected and compared to those from 22 healthy male newborns at the same gestational age (controls). Renin–angiotensin system components levels were measured by enzyme‐linked immunosorbent assay. Results: Fetuses with posterior urethral valve presented increased urinary levels of angiotensin (Ang) I, Ang‐(1‐7) and angiotensin‐converting enzyme 2 in comparison with controls. ACE levels were significantly reduced and Ang II levels were similar in fetuses with posterior urethral valve in comparison with controls. Conclusions: Increased urinary levels of angiotensin‐converting enzyme 2 and of Ang‐(1‐7) in fetuses with posterior urethral valve could represent a regulatory response to the intense inflammatory process triggered by posterior urethral valve. Resumo: Objetivo: A válvula de uretra posterior é a obstrução do trato urinário inferior mais comum em crianças do sexo masculino. Uma alta porcentagem de pacientes com válvula de uretra posterior evolui para doença renal em estágio final. Estudos anteriores mostraram que citocinas, quimiocinas e componentes do sistema renina‐angiotensina contribuem para o dano renal em uropatias obstrutivas. Recentemente, descobrimos que amostras de urina de fetos com válvula de uretra posterior tinham níveis aumentados de moléculas inflamatórias. O objetivo deste estudo foi medir as moléculas de renina‐angiotensina e investigar sua correlação com marcadores inflamatórios previamente detectados nas mesmas amostras de urina de fetos com válvula de uretra posterior. Métodos: Amostras de urina de 24 fetos com válvula de uretra posterior foram coletadas e comparadas com amostras de urina de 22 recém‐nascidos saudáveis de mesma idade gestacional (controles). Os níveis dos componentes de SRA foram medidos por ensaio de imunoabsorção enzimática. Resultados: Os fetos com válvula de uretra posterior apresentaram níveis urinários aumentados de angiotensina (Ang) I, Ang‐(1‐7) e enzima conversora de angiotensina 2 em comparação com os controles. Os níveis de enzima conversora de angiotensina eram significativamente menores e os níveis de Ang II eram semelhantes nos fetos com válvula de uretra posterior em comparação com os controles. Conclusões: O aumento dos níveis urinários de enzima conversora de angiotensina 2 e de Ang‐(1‐7) em fetos com válvula de uretra posterior poderia representar uma resposta regulatória ao intenso processo inflamatório desencadeado pela válvula de uretra posterior. Keywords: Posterior urethral valve, Renin–angiotensin system, Angiotensin II, Angiotensin‐(1‐7), ACE2, Inflammation, Palavras‐chave: Válvula de uretra posterior, Sistema renina‐angiotensina, Angiotensina II, Angiotensina‐(1‐7), ACE2, Inflamação

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.

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.

Background: Posterior urethral valves (PUVs) are usually suspected during antenatal sonograms or by postnatal evidence of bilateral hydronephrosis with enlarged bladder. Gross hematuria as an initial manifestation of PUV with a history of normal antenatal sonogram is very rare. Methods: This is a retrospective chart study. Results: We describe a nine-day-old male neonate who presented with gross hematuria and was later found to have a urinary tract infection (UTI) and severe acute kidney injury (AKI). The mother apparently had normal antenatal sonograms with no evidence of fetal hydronephrosis. The child did not have postnatal renal bladder sonogram done until gross hematuria occurred at Day 9 of life. Sonogram showed bilateral severe hydronephrosis and hydroureter with enlarged bladder. The patient underwent ablation of the PUVs after initial bladder decompression with indwelling urethral catheterization. His AKI resolved after prompt treatment of UTI and PUV ablation. Conclusions: This report emphasizes the importance of a high index of suspicion for obstructive uropathy in a newborn with gross hematuria irrespective of prenatal sonogram findings.

Objective: To identify indicators that predict outcome in posterior urethral obstruction management in a resource-constrained environment with emphasis on the importance of early indicators. Subjects and methods: A retrospective review of all children who were managed for posterior urethral obstruction from 2002 to 2012 was done. Variables studied were: age at diagnosis (AgD) and of ablation, creatinine at diagnosis (CrD), nadir creatinine (CrN), vesicoureteric reflux (VUR), urinary diversion, and urodynamic study (UDS) findings. These were evaluated against two groups of patients—those with end stage renal disease (ESRD)-Group A and those with normal renal function-Group B. Significant variables were entered into a multivariate logistic regression to identify the independent prognostic factors that determine progression to ESRD. The independent factors were further analyzed with the receiver operating characteristics (ROC) to identify cutoff levels. The data is expressed as mean ± SD and median (IQR). A p-value of 0.05 was regarded as significant. Results: Sixty patients had adequate documents for evaluation. ESRD was identified in 30% of the cases. The AgD was 2.5 weeks (1–18 weeks). CrD, CrN, abnormal UDS, and VUR were significant prognostic indicators of ESRD (p = 0.0001), with CrN and CrD being independent factors on regression analysis. CrD value of ≥102 μmol/L had sensitivity of 94.4% and specificity of 68.3%, with a PPV of 56.7% (CI: 37.4–74.5) and a NPV of 96.6% (CI: 82.2–99.9). CrN of ≥89 μmol/L had sensitivity of 27.8% and a specificity of 95.1% with a PPV of 71.4% (CI: 29%–96.3%) and NPV of 75% (CI: 61.1%–86%) in predicting ESRD. Conclusion: In our environment, a child with initial creatinine value of less than 102 μmol/L may be classified as unlikely to develop ESRD. Additionally, a nadir creatinine greater than 89 μmol/L may increase the probability of developing ESRD. While nadir creatinine has consistently been identified as independent indicator of outcome, its value is evaluated after a year of management; however, early indicator like creatinine at diagnosis may provide essential monitoring information at the onset of treatment. Keywords: PUV, ESRD, Nadir creatinine, Creatinine at diagnosis

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

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.

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.

Objective: To investigate the relationship between low birth weight (LBW

Fetal renal pelvis dilation is a common condition, which is observed in 1-4. 5% of pregnancies. In many cases, this finding resolves spontaneously. However, sometimes it may be a signal of significant urinary tract pathologies. The main abnormalities found after birth are uretero-pelvic junction stenosis, primary vesicoureteral reflux, megaureter, duplex kidneys, and posterior urethral valves, with uretero-pelvic junction stenosis and primary vesicoureteral reflux accounting for most of the cases. Diagnosis, management, and prognosis at short and longer term of these conditions will be reviewed in this article., SCOPUS: re.j, info:eu-repo/semantics/published

Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction (BOO) in boys. Cobb's collar, a rare narrowing of the bulbar urethra, is one of the lesser-known causes of congenital urethral obstruction. We present a case of both congenital anomalies occurring concomitantly, in a preterm baby presenting with urinary ascites. Keywords: Cobb's collar, CC, Anterior urethral valve, AUV, Posterior urethral valve, PUV, Bladder outlet obstruction, BOO

Anterior urethral valve (AUV) is a rare cause of obstructive uropathy in children. These are much rarer than posterior urethral valves. We report a case of a child with AUV and high grade bilateral vesicoureteric reflux. He was managed by transurethral laser ablation. The left sided reflux resolved after ablation. The right sided reflux persisted. The child is awaiting ureteric reimplantation.

Aim The most common cause of congenital bladder outlet obstruction (BOO) is posterior urethral valves (PUV). Initial treatment requires decompression, but transurethral incision (TUI) or primary diversion is all described. There is no randomized control trial to guide management. This study aims to describe management, circumcision, and UTI rate in a national cohort of PUV boys. Methods Boys diagnosed with BOO were recruited (via BAPS CASS) over 1 year with ethics committee approval (ref: 12/SC/0416). Data were collected via questionnaire, presented as number (%), analyzed by Mann–Whitney/chi-square/Fisher Exact tests, and p Results BOO presented in 121 boys during 2014–2015, and 113 were PUV. Catheter placement in 87/121(72%) was more likely to happen in antenatal vs. postnatal vs. late(> 1 y) presentations, p Conclusion Standard treatment for BOO and PUV in the current UK cohort is urethral catheterization followed by TUI. Supravesical diversion is a rescue therapy. UTIs are common and reduced by circumcision, with 43% being circumcised. Initial mortality rate was 1%, and 1.6% present in ESRF. Level of Evidence Prognostic study – Level I – Prospective National Cohort Study.

We report a pair of dizygotic twins with type 1 posterior urethral valves. Concomitant and discordant presentation of posterior urethral valves in twins is rare, with a handful of cases in the literature. The occurrence of identical pathology in dizygotic twins probably suggests the possibility of an inherited trait as it does exist in monozygotic twins.Keywords: fraternal twins, identical pathology, inherited trait, posterior urethral valves

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Aims: Study on prognostic significance of antenatal ultrasonography and renin angiotensin system activation in predicting disease severity in posterior urethral valves. Materials and Methods: Antenatally diagnosed hydronephrosis patients were included. Postnatally, they were divided into two groups, posterior urethral valve (PUV) and non-PUV. The studied parameters were: Gestational age at detection, surgical intervention, ultrasound findings, cord blood and follow up plasma renin activity (PRA) values, vesico-ureteric reflux (VUR), renal scars, and glomerular filtration rate (GFR). Results: A total of 25 patients were included, 10 PUV and 15 non-PUV. All infants with PUV underwent primary valve incision. GFR was less than 60 ml/min/1.73 m 2 body surface area in 4 patients at last follow-up. Keyhole sign, oligoamnios, absent bladder cycling, and cortical cysts were not consistent findings on antenatal ultrasound in PUV. Cord blood PRA was significantly higher (P < 0.0001) in PUV compared to non-PUV patients. Gestational age at detection of hydronephrosis, cortical cysts, bladder wall thickness, and amniotic fluid index were not significantly correlated with GFR while PRA could differentiate between poor and better prognosis cases with PUV. Conclusions: Ultrasound was neither uniformly useful in diagnosing PUV antenatally, nor differentiating it from cases with non-PUV hydronephrosis. In congenital hydronephrosis, cord blood PRA was significantly higher in cases with PUV compared to non-PUV cases and fell significantly after valve ablation. Cord blood PRA could distinguish between poor and better prognosis cases with PUV.

Congenital anomalies of the urogenital tract are the most common anomalies found in the foetus, neonates and infants, but anterior urethral valves and diverticula are rare. Here, we present a case with congenital anterior urethral diverticulum associated with patent ductus arteriosus and polydactyly.

Introduction: A significant number of children with posterior urethral valves (PUV) develop chronic renal failure (CRF) due to activation of the renin angiotensin system (RAS). We investigated the role of plasma renin activity (PRA) in these cases and sought to establish a relationship between the accepted criteria of renal damage and PRA. Aims and Objectives: The aim of this study is to establish the relationship between PRA and CRF. Materials and Methods: The records of 250 patients with PUV were reviewed. Multiple linear regression analysis was used to assess correlations between PRA, grade of reflux, presence of scars and raised creatinine and decrease in glomerular filtration rates (GFR). A P < 0.5 was considered as significant. Results: A total of 58 patients were included. Their mean age was 16 years, range 5.3-24.2 years, mean follow-up period was 12.6 ± 3.6 years. At diagnosis, 22/58 (38%) patients were in CRF and 36/58 (62%) patients had normal renal function (RF). The mean PRA after treatment was higher in those who developed CRF than in those with normal RF (12.6 ± 10.2 vs. 34.6 ± 14.2 ng/ml/24 h, P = 0.02). Mean GFR at 1 year of age were 48 ± 9.8 ml/min/1.73 m 2 and 86 ± 12.5 ml/min/1.73 m 2 respectively (P = 0.005). PRA correlated negatively with GFR, t = -2.816, Confidence Interval: P = 0. 007. In the temporal plot over a period of 14 years, a rise in PRA preceded the fall in GFR in patients who developed CRF. Conclusions: This study shows that RAS is activated earlier in kidneys susceptible to damage. PRA could be investigated as a marker for the early detection and prevention of ongoing renal damage.

Summary Background Urethral valves are infravesical congenital anomalies, with the posterior urethral valve (PUV) being the most prevalent one. Anterior urethral valve (AUV) is a rare but a well-known congenital anomaly. AUV and diverticula can cause severe obstruction, whose repercussions on the proximal urinary system can be important. Few cases have been described; both separately and in association with urethral diverticulum. The presentation of such a rare but important case led us to a report with highlighting its classic imaging features. Case Report We present a case report of AUV with lower urinary tract symptoms in a 6-year-old boy with complaints of a poor stream of urine and strain to void. Unique findings were seen on Retrograde Urethrography (RGU) and Voiding Cysto-Urethrography (VCUG), i.e. linear incomplete filling defect in the penile urethra and associated mild dilatation of the anterior urethra ending in a smooth bulge. On cysto-urethroscopy the anterior urethral valve was confirmed and fulguration was done. Conclusions Congenital anterior urethral valve is an uncommon but important cause of infravesical lower urinary tract obstruction that is more common in male urethra. It can occur as an isolated AUV or in association with diverticulum and VATER anomalies. Early diagnosis and management of this rare condition is very important to prevent further damage, infection and vesicoureteral reflux. AUV may be associated with other congenital anomalies of the urinary system; therefore a full evaluation of the urinary system is essential.

Posterior urethral valves (PUV) are now commonly suspected on antenatal ultrasound, but can present with a broad spectrum of severity postnatally. Rarely, the diagnosis is missed until adolescence or adulthood when the patient usually presents with lower urinary tract symptoms. We describe an even rarer case of PUV in a late adolescent who first presented with preserved renal function and urinary incontinence. We review the literature on presentation, natural history and outcomes of both early and late presenting PUV cases.

The most common cause of lower urinary tract obstruction in male infants is posterior urethral valves. Although the incidence has remained stable, the neonatal mortality for this disorder has improved due to early diagnosis and intensive neonatal care, thanks in part to the widespread use of prenatal ultrasound evaluations. In fact, the most common reason for the diagnosis of posterior urethral valves presently is the evaluation of infants for prenatal hydronephrosis. Since these children are often diagnosed early, the urethral obstruction can be alleviated rapidly through catheter insertion and eventual surgery, and their metabolic derangements can be normalized without delay, avoiding preventable infant mortality. Of the children that survive, however, early diagnosis has not had much effect on their long-term prognosis, as 30% still develop renal insufficiency before adolescence. A better understanding of the exact cause of the congenital obstruction of the male posterior urethra, prevention of postnatal bladder and renal injury, and the development of safe methods to treat urethral obstruction prenatally (and thereby avoiding the bladder and renal damage due to obstructive uropathy) are the goals for the care of children with posterior urethral valves[1].

Posterior urethral valves are the commonest cause of lower urinary tract outflow obstruction in male infants with an estimated incidence of 1:5000 male infants and 1:25,000 live births. Despite treatment with fulguration of the obstructing valves, bladder function is abnormal in up to 70% of older children and adolescents. Bladder dysfunction causes morbidity e.g. urinary incontinence and has been implicated in the late deterioration of renal function in this population. A poor understanding and inappropriate management of bladder dysfunction can result in unnecessary morbidity, which can handicap a child for life. Any method that measures function or dysfunction of the lower urinary tract constitutes a urodynamics investigation. Broadly, the investigations can be classified into simple or noninvasive urodynamics and invasive urodynamics. The objective of urodynamics assessments in children is to reproduce the patient's complaints or symptoms. Video urodynamics can provide additional information that may contribute to a further understanding of the problem under investigation. Urodynamics provides a useful tool to test the efficacy of treatment as well as determine any refinements necessary to improve the outcome of such treatment.

Posterior urethral valves (PUV) are the most common cause of lower urinary tract obstruction in male neonates. The course and treatment of the disease can further be complicated if it coexists with Renal rickets. We present a rare case of PUV complicated with Renal rickets, which was managed successfully at our institution.

INTRODUCTION: The lowest serum creatinine (nadir Scr, cut-off 1 mg/dl) during infancy predicts subsequent renal outcome in posterior urethral valve (PUV) infants, but early, neonatal values may be useful to guide care. We aimed to explore correlations between neonatal Scr values and long-term renal outcome. METHODS: Retrospective evaluation of records of 39 PUV patients, treated in the University Hospitals Leuven (2001-2011). Scr measurements were collected and associations (Mann-Whitney U, Spearman) to predict unfavorable renal outcome [GFR

Congenital obstruction of the male urethra is usually caused by posterior urethral valves. Anterior urethral valves (AUV) represent a rare anomaly with a wide spectrum of presentation varying from mild voiding difficulties to end-stage renal disease. Prompt diagnosis and appropriate treatment is essential to prevent renal impairment. We report the case of a 13 month-old boy who presented with deterioration of kidney function caused by unrecognized AUV disorder. Temporary cutaneous vesicostomy was necessary to protect the upper urinary tract from further damage and to stabilize renal function. Even though a voiding cystourethrogram (VCUG) demonstrated obstruction of distal urethra, AUV were initially overlooked but finally diagnosed on additional VCUG followed by urethroscopy.

The purpose of this article is to review normal developmental bladder physiology in infants and bladder dysfunction in conditions such as neurogenic bladder, posterior urethral valves and high grade vesicoureteric reflux. We contrast the classical concept that bladder function in nontoilet-trained children is thought to be ‘reflexive’ or ‘uninhibited’, with the results of more recent research showing that infants most commonly have a stable detrusor. The infant bladder is physiologically distinct from the state seen in older children or adults. The voiding pattern of the infant is characterized by an interrupted voiding stream due to lack of proper urinary sphincter relaxation during voiding. This is called physiologic detrusor sphincter dyscoordination and is different from the pathologic ‘detrusor sphincter dyssynergy’ seen in patients with neurogenic bladder. Urodynamic abnormalities in neonates born with spina bifida are common and depend on the level and severity of the spinal cord malformation. Upper neuron lesions most commonly lead to an overactive bladder with or without detrusor sphincter dyssynergy while a lower neuron lesion is associated with an acontractile detrusor with possible denervation of the external urinary sphincter. In infants with neurogenic bladder, the role of ‘early prophylactic treatment (clean intermittent catheterization and anticholinergics)’ versus initial ‘watchful waiting and treatment as needed’ is still controversial and needs more research. Many urodynamic-based interventions have been suggested in patients with posterior urethral valves and are currently under scrutiny, but their impact on the long-term outcome of the upper and lower urinary tract is still unknown. Cumulative data suggest that there is no benefit to early intervention regarding bladder function in infants with high-grade vesicoureteric reflux.

The broad range of renal and urinary tract abnormalities associated with Down syndrome are not well known. We present two cases from a single institution of posterior urethral valves associated with Down syndrome. The cases illustrate the potential for delayed diagnosis and the management challenges. The literature is reviewed and a discussion of the need to screen Down syndrome children for urinary tract anomalies is presented. Keywords: Posterior urethral valves; Down syndrome

BackgroundPosterior urethral valves (PUV) may cause subtle to severe obstruction of the urethra, resulting in a broad clinical spectrum. PUV are the most common cause of chronic renal disease in boys. Our purpose was to report the incidences of kidney and bladder dysfunction in boys treated with endoscopic valve resection for PUV.MethodologyWe searched MEDLINE and EMBASE databases until 1st of July 2011, to identify original papers that described outcome of endoscopic valve resection (EVR) in boys. We extracted information on (1) patient characteristics and clinical presentation of PUV related to outcomes and (2) the post-treatment absolute risks for kidney and bladder dysfunction.Principal findingsThirty-four studies describing renal function, vesicoureteral reflux (VUR), incontinence, and urodynamic bladder function after EVR in 1474 patients were retrieved. Patients treated for PUV show high percentages of chronic kidney disease (CKD) or end stage renal disease (ESRD), 22% (0-32%) and 11% (0-20%), respectively. Elevated nadir serum creatinine was the only independent factor associated with renal failure. Before treatment, VUR was present in 43% of boys and after EVR, VUR was present in 22%. Post treatment, 19% (0-70%) was reported to suffer from urinary incontinence. Urodynamic bladder dysfunction was seen in many patients (55%, 0-72%) after treatment of PUV.ConclusionsThe reported cumulative incidence of renal and bladder dysfunction in patients with PUV after endoscopic PUV treatment varies widely. This may reflect a broad clinical spectrum, which relates to the lack of a standardised quantification of obstruction and its severity. Moreover, the risk of bias is rather high, and therefore we put little confidence in the reported estimates of effect. We found elevated nadir serum creatinine as a predictor for renal dysfunction. In order to be able to predict outcomes for patients with PUV, an objective classification of severity of obstruction is mandatory.

Delayed presentation of patients with posterior urethral valve with complications like severe urosepsis, uremia, and anemia are seen in our setting. Renal replacement therapy which should have been offered to these patients is not readily available for children in our country. The aim of this study is to determine the pattern of late presentation and outcome of management of posterior urethral valve in a resource-limited setting. A descriptive retrospective study (1997–2009) was conducted. Data including pattern of presentation, duration of symptoms, complications, and outcome of initial management were analyzed. Twenty-one patients were seen. The median age was 3 years (2 days–13 years). The mean duration of symptoms before presentation was 2.6 years. Nineteen patients (91%) presented with urosepsis while 8 patients (36%) presented with significant renal insufficiency. Laboratory findings varied from-mild-to marked elevation in serum creatinine. Radiological findings confirmed the diagnosis of posterior urethral valve. We concluded that late presentation is common in our setting. This is associated with high morbidity and mortality rates. Efforts at improving awareness and early diagnosis among the health team should be made to stem the tide.

Anterior urethral valves (AUVs) are uncommon congenital anomalies causing urethral obstruction in boys. Medical records of 13 children were reviewed retrospectively. Each patient (pt) was evaluated with voiding cysto-urethrogram (VCUG) and renal ultrasonography. Pts older than 5 years performed an uroflowmetry. Serum creatine was determined in all children. All pts had difficulty in voiding and eight had recurrent urinary tract infection. Renal function was normal in all pts. VCUG showed filling of Cowper’s duct in a pt. In a case, a vesicoureteral reflux was discovered. Uroflowmetry showed a flat trace in all cases. Transurethral endoscopic resection of the valves was carried out in 11 children while open resection was necessary in two children. At serial follow-up, all pts remain symptom-free and uroflowmetry documented regular pattern. Anterior urethral valves are rare lesions that may create problems at different stages. Some AUVs may result from the obstruction distal lip of a ruptured syringocele. Improvements in endoscopic equipment allow for minimally invasive transurethral resection. Large diverticula are best managed with open diverticulectomy and reconstruction. In newborns with severe altered urinary tract drainage, particularly in low birth-weight infant, a vesicostomy may be necessary.

We report a case of posterior urethral valve (PUV) in a six year old boy with nocturnal eneuresis and failure to thrive as the only presenting symptoms. The clinically occult lesion was only unravelled when micturating cystourethrogram revealed a dilated posterior urethra with a distal narrow stream of opacified urine, bilateral hydronephrosis and hydroureters, thus confirming an obstructive uropathy due to posterior urethral valves. Eneuresis and haematuria are both documented symptoms of PUV which are barely mentioned at clinical rounds. In an entity where delay in diagnosis could result in irreversible renal damage, the need to raise the index of suspicions for, eneuresis and haematuria (especially microscopic haematuria), as important sysmptoms and sign in PUV is emphasized. The need to remember genitourinary infections in the search for causes of recurrent PUO in the paediatric patient is readdressed. West African Journal of Radiology Vol. 13(1) 2006: 26-28

The association between monozygotic twins and posterior urethral valves (PUV) in postnatal life has been thoroughly described. In the fetus, the prenatal recognition of PUV is feasible. However, it has been repeatedly reported in singletons but never in monochorionic twins. We describe two cases of early prenatal diagnosis of concordant PUVs in monochorionic twins. In one of the sets, the expression of the disease was different for each twin.

Urethral syringoceles are cystic dilatations of paired bulbourethral glands of Cowper. They can cause voiding dysfunction in male children and usually occur in isolation. We report a rare association of Cowper's syringocele with posterior urethral valves in a 4-year-old child, which was successfully managed endoscopically.

Posterior urethral valve and vesical calculus are individually among the most common causes of obstructive lower urinary complaints in children. There are very few reports of association between posterior urethral valves and bladder calculus. We report three such cases. This association of the vesical calculi with posterior urethral valves may lead to a delay in the diagnosis of the posterior urethral valves. The diagnosis of posterior urethral valves should be suspected in all children with vesical or urethral calculi who have persistence of symptoms even after removal of the calculi.

Symptomatic presentation of orthotopic ureterocele in infancy is very rare and its association with posterior urethral valves has not been reported till date. The first such case and a review of the literature on anomalies in association with posterior urethral valves is presented.