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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.

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

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.

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.

Congenital anomalies of the urethra are insufficiently covered in the literature, except for hypospadias and epispadias. Therefore, there is a significant gap in information on the diagnosis, classification and management of patients with such anomalies among practising physicians and researchers. Also, the urgency of studying this problem is due to the complexity of these malformations. This fact dictates the need for a very careful and correct approach to timely surgical treatment and subsequent rehabilitation, which will minimize the negative medical and social consequences of these anomalies. Taking these circumstances into account, key data on the most common congenital anomalies of the urethra were summarized in the form of a lecture material based on modern scientific advances. The following malformations of the urethra are considered as the main options: hypospadias; epispadias; urethral valves, obliterations, strictures, diverticula and cysts; Guerin's sinus; hypertrophy of verumontanum; agenesis of the urethra; urethral duplication; megalourethra; urethral fistulas; urethral polyp. Moreover, all types of anomalies are presented with illustrations of real clinical examples. The information from this study can be successfully used in the daily work for practising physicians of various profiles, scientific and pedagogical activities when teaching students at medical universities, residency training, postgraduate studies and in the courses of advanced training and professional retraining, as well as in conducting scientific research in this direction.

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.

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

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

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.

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

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.

The clinical presentation of the progression of the posterior urethral valve with the development of vesi-coureteral reflux has been described. The authors have accentuated on an early surgical treatment of the valve of the posterior urethra in children with a view of avoiding grave complications in children. The possibilities and advantages of the said defects in children have been demonstrated, namely high efficacy and minimal terms of inpatient treatment., Описано клинику прогрессирования клапана задней уретры с развитием пузырно-мочеточникового рефлюкса. Сделан акцент на раннем хирургическом лечении клапана задней уретры у детей относительно предупреждения грозных осложнений. Показаны возможности и преимущества эндоскопической коррекции названных аномалий у детей, а именно высокую эффективность и минимальные сроки стационарного лечения., Описано клініку прогресування клапана задньої уретри з розвитком міхурово-мискового рефлюксу. Акцентовано на ранньому хірургічному лікуванні клапана задньої уретри в дітей щодо запобігання грізним ускладненням. Показано можливості та переваги ендоскопічної корекції названих вад у дітей, а саме високу ефективність та мінімальні терміни стаціонарного лікування.

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...

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

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

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.

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.

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.