Search

Posterior urethral valves (PUV) are the most common cause of bladder outlet obstruction in children. They occur in 1/5000–25,000 live births and constitute 10% of urinary obstruction diagnosed in utero [1–4]. PUV are associated with high fetal and neonatal mortality (30%) and considerable lifelong morbidity. The morbidity is related to the congenital obstruction of the urinary tract at the critical time in organogenesis which may have a profound and lifelong effect on kidney, ureter, and bladder function [4]. In severe cases, the disorder can lead to anhydramnios and pulmonary dysplasia during the canalicular phase of lung development. Mortality is related to ongoing renal damage in children.

Background: Continent diversions have since been performed with increasing frequency since the 1950s, with some providers espousing the use of orthotopic diversions as being the new gold standard. However, patients must be counseled to take great care in the maintenance of their diversions because of complications such as metabolic abnormalities, pouch stones, and mucus retention. Case Report: A 21-year-old male with a history of posterior urethral valves underwent a continent catheterizable diversion as a child. He is undocumented and without health insurance and as a result unable to follow-up with an urologist for >10 years. He subsequently develops pyocystis with cutaneous fistulization and pouch stones necessitating percutaneous management. Conclusion: Patients may benefit greatly from a continent urinary diversion, however, selection must include patients who are able to maintain their pouch and follow-up with their providers to avoid major complications that may occur up to years following the procedure.

Objectives: Posterior urethral valves (PUV) is the commonest obstructive uropathy with varied consequences. Though valve fulguration is the treatment of choice, appropriate bladder management modifies outcome and includes rational use of anticholinergics. Here, we aim to evaluate the effects of oxybutynin on the bladder and urinary tract morphology and function. Concurrently, we document adverse effects encountered, patient compliance, and medication adherence. Methods: A retrospective study of children below 5 years of age (2012–2017) post fulguration and on oxybutynin for at least 6 months. Patient demographics, clinical features, renal ultrasound, micturating cystourethrogram, dimercaptosuccinic acid scan, adverse effects, and pill count for medication adherence were collated. Results: 48 children below the age of 5 years were included, and 12 were excluded either due to the presence of concomitant problems or were not on oxybutynin. Of the 36, four were lost to follow-up and one had died due to an unrelated condition. Thus, a total of 31 children were analyzed. At follow-up, 28/31 patients were asymptomatic, two had daytime incontinence, and one had recurrent urinary tract infection. All patients except one have preserved renal function tests. On ultrasonography, hydroureteronephrosis worsened in only 1/25 children and two showed significant post void residues. The resolution of vesicoureteral reflux was noted in almost 50%. 4/31 renal units had progressive scars. Only two parents defaulted medication. Urodynamic study done in half of these children showed stable bladder pressures except in two. Conclusion: Oxybutynin therapy following adequate valve fulguration aids upper tracts preservation by stabilizing deranged bladder dynamics. The medication is well tolerated with minimal or no side effects.

The aim of this study is to investigate the performance and reliability of urethral valve driven by ultrasonic-vaporized steam. The performance model of urethral valve is established to analyze the driving and opening/closing performances of urethral valve. The reliability model of urethral valve is obtained, and the reliability simulation algorithm is proposed to calculate the reliability index of urethral valve. The numerical simulation and experimental results show that urethral valve has a good opening/closing performance, the driving performance can be improved by increasing ultrasonic intensity, radiation area and ultrasonic frequency, and the corrosion and aging of driving bags are the weak links of urethral valve.

Objective To evaluate prenatal sonographic signs that distinguish male fetuses with posterior urethral valves (PUV) from those with vesicoureteral reflux (VUR). Methods Antenatal data were retrospectively retrieved from all consecutive women delivering between 2003 and 2012 of a male newborn with a postnatal diagnosis of PUV or VUR. Prenatal parameters included fetal bladder characteristics, identification of a dilated posterior urethra, and change in shape and size in the fetal renal pelvis or ureter. Results 136 women gave birth to a male newborn with a postnatal diagnosis of PUV (n = 49) or VUR (n = 87). The presence of posterior urethral dilatation (21 [42.9%] fetuses versus 0 [0%], p = 0.000), a thickened fetal bladder wall (37 [75.5%] versus 4 [4.6%], p = 0.000) and anhydramnios (14 [28.6%] versus 0, p = 0.000) were strongly associated with the postnatal diagnosis of PUV. Change in shape and size in the fetal renal pelvis or ureter was observed in 15 (17.2%) of 87 children with VUR versus 1 (2.0%) out of 49 with PUV (p = 0.010). Conclusions Prenatal ultrasound may differentiate with reasonable accuracy male fetuses with a postnatal diagnosis of PUV from those with VUR.

Posterior urethral valves are the most common cause of partial bladder outlet obstruction in the pediatric population. However, to our knowledge the etiology and the detailed mechanisms underlying pathological changes in the bladder following partial bladder outlet obstruction remain to be elucidated. Recent findings suggest that hypoxia and associated up-regulation of HIFs (hypoxia-inducible factors) have a key role in partial bladder outlet obstruction induced pathology in the bladder. We examined the effects of pharmacological inhibition of HIF pathways by 17-DMAG (17-(dimethylaminoethylamino)-17-demethoxygeldanamycin) in pathophysiological phenotypes after partial bladder outlet obstruction.Partial bladder outlet obstruction was surgically created in male C57BL/6J mice. The animals received oral administration of 17-DMAG or vehicle daily starting from the initiation of obstruction up to 5 days. Sham operated mice served as controls. Bladders were harvested from each group 2, 4 and 7 days postoperatively, and analyzed for histological and biochemical changes. Bladder function was assessed by in vitro muscle contractility recordings.Partial bladder outlet obstruction caused a significant increase in the bladder mass accompanying enhanced collagen deposition in the bladder wall while 17-DMAG treatment suppressed those increases. Treatment with 17-DMAG attenuated the degree of up-regulation of HIFs and their target genes involving the development of tissue fibrosis in obstructed bladders. Treatment with 17-DMAG improved the decreased responses of obstructed bladder strips to electrical field stimulation and KCl.In vivo 17-DMAG treatment decreased partial bladder outlet obstruction induced pathophysiological changes in the bladder. HIF pathway inhibition has a potential clinical implication for the development of novel pharmacological therapies to treat bladder pathology associated with partial bladder outlet obstruction.

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.

Conditions impairing bladder function in children and adults, such as myelomeningocele, posterior urethral valves, bladder exstrophy or spinal cord injury, often need urinary diversion or augmentation cystoplasty as when untreated they may cause severe bladder dysfunction and kidney failure. Currently, the gold standard therapy of end-stage bladder disease refractory to conservative management is enterocystoplasty, a surgical enlargement of the bladder with intestinal tissue. Despite providing functional improvement, enterocystoplasty is associated with significant long-term complications, such as recurrent urinary tract infections, metabolic abnormalities, stone formation, and malignancies. Therefore, there is a strong clinical need for alternative therapies for these reconstructive procedures, of which stem cell-based tissue engineering (TE) is considered to be the most promising future strategy. This review is focused on the recent progress in bladder stem cell research and therapy and the challenges that remain for the development of a functional bladder wall.

PURPOSE : Bladder augmentation is an effective surgical procedure for increasing bladder capacity and reducing pressure on the urinary system. It is indicated for patients with anomalies such as spina bifida, myelomeningocele, urethral valve and bladder exstrophy, who progress with low tolerance of medication. CASES : This was a retrospective study conducted on pediatric patients submitted to bladder augmentation from 2000 to 2011. RESULTS : 34 patients aged 4 to 17 years were submitted to bladder augmentation, 30 of them with an ileal loop and 4 with a ureter.A continent urinary shunt was performed in 16 patients, the Mitrofanoff conduit was associated in 15, and the Macedo technique was used in one. Mean follow-up was 34.35 months (1 to 122 months). Mean creatinine was 1.5 ng/ml (0.4 to 7.5 ng/ml) preoperatively and 1.78 ng/ml postoperatively. Three patients required a renal transplant during follow-up. There was improvement or resolution of vesicoureteral reflux in 83.5% of the kidneys on the right and in 75% on the left. Bladder capacity increased, on average, from 152.5 ml to 410 ml. The main complications were vesical lithiasis in 3 patients and conduit perforation in one. CONCLUSION : Bladder augmentation showed good results in this series, preserving renal function in most of the patients.

Clinical problem We diagnosed 8 patients with late-stage posterior urethral valves (PUV) between 1 and 14 years of age. Five patients complained of symptoms related to voiding dysfunction. The other 3 patients required urethrocystoscopy for other reasons (hypospadias fistulae, difficulty with catheterisation and high-grade vesicoureteral reflux [VUR]). A second review of the first 2 patients’ medical history showed voiding dysfunction symptoms. All patients underwent preoperative ultrasonography: 3 patients had normal results and 5 had renal or vesical disorders. The diagnosis was reached through voiding cystourethrogram (VCUG), and 4 patients underwent urodynamic studies. The diagnosis was confirmed by urethrocystoscopy, performing valve electrofulguration. We performed urethrocystoscopy during the check-ups at 3–6 weeks and observed no stenosis. The symptoms disappeared for all patients after 20 months of follow-up. The patient with VUR was cured. The ultrasounds showed no progression of the renal involvement and showed improvement in the vesical involvement. The velocimetries during check-ups presented curves within normal ranges. Discussion Most children with PUV are diagnosed through ultrasound during the neonatal period. Some patients present PUV at later ages with diverse symptoms, which impedes its diagnosis. We should suspect PUV in male patients with symptoms of voiding dysfunction, either when they have normal or pathological results from ultrasounds or VCUG. We recommend performing urethrocystoscopy to rule out urethral obstruction.

Background: Evaluation of our 10 year’s experience and analyze the prognostic factors affecting renal outcome in children with posterior urethral valves (PUV).Methods: We retrospectively reviewed the medical records of 129 patients diagnosed and managed as posterior urethral valves from 2009 till 2019 in our institution age of presentation, signs and symptoms, anthropometry, surgical interventions, and biochemical investigations were recorded and data were analyzed on an excel spreadsheet.Results: Mean age of presentation was 3 years and mean follow up was 4 years. 99 underwent primary valve ablation and 30 patients underwent diversion as primary treatment, the most common presenting symptom was poor urinary stream (90), enuresis (88), and fever (60). Correlation of resolution of symptoms pre and postoperatively was statistically not significant. 26% patients developed chronic renal failure (CRF) and 8.5% patients progressed to end stage renal disease (ESRD). Correlation of mean nadir serum creatinine >1 mg/dl (p=0.030), presence of protenuria (p=0.020), bladder dysfunction (p=0.029), bilateral high grade vesicoureteric reflux (VUR) (p=0.019), hypertension (p=0.01) was statistically significant. Correlation of age of presentation (p=0.341), outcome for patients treated with diversion or valve ablation in relation to CRF (p=0.239), incontinence (p=0.209), malnutrition (p=0.194) was statistically insignificant.Conclusions: Nadir serum creatinine >1 mg/dl, presence of protenuria, bladder dysfunction, bilateral high grade VUR are significant prognostic factors affecting long term outcome in PUV. Early detection and timely management may lead to a better outcome in these patients.

Summary Background Anterior urethral valves (AUV) and associated anterior urethral diverticula (AUD) are a rare cause of congenital lower urinary tract obstruction. They occur 25–30 times less frequently than posterior urethral valves (PUV) and historically tend to have a less aggressive presentation and outcome. However, due to the low incidence, little is known about management and long-term prognosis. Objective We aim to evaluate the outcomes after AUV valve ablation and compare this group to a previously studied PUV cohort. Study design In this IRB-approved study, we retrospectively identified all patients from 2002 to 2017 undergoing valve ablation using CPT code 52400. Charts were manually reviewed to identify AUV patients, their presenting symptoms, timing of diagnosis, pre and postnatal imaging findings, age at presentation and valve ablation, creatinine, recurrences, additional surgeries and follow-up. The AUV group was then compared to a previously studied PUV cohort of 104 patients from our institution. Results Nine AUV patients were identified. Only four (44%) patients were diagnosed prenatally. Three (33%) patients were diagnosed neonatally, one for weak stream/dribbling and two for febrile UTIs. The remaining two patients were diagnosed at ages 3 and 4 due to dysuria and penile swelling with micturition. Patients presenting later in life had no evidence of renal dysfunction or VUR. All patients underwent primary TUR-AUV (1 laser, 4 cold knife, 4 cautery), but 55% had residual valves and/or diverticula identified on routine postoperative imaging in two patients and due to development of clinic symptoms in three patients. All five patients with recurrences underwent repeat endoscopic evaluation, and thereafter two patients required urethral reconstruction. In contrast, only 15% (16/104) of PUV patients required re-TUR (p = 0.01). At an average follow-up time of 4.24 years (range 0.6–11.4 years), 44% of patients had no evidence of CKD, and two patients (22%) had progressed to CKD of at least stage IIIA, compared to 21.4% in the PUV cohort at a mean follow-up of 2.3 years. Discussion The overall incidence of AUV is low, making it difficult to characterize these patients definitively. However, despite a milder phenotype and later presentation in most AUV patients, they do require more aggressive surgical treatment for complete resolution of the AUV. Furthermore, the long-term renal outcomes appear more severe than previously reported. Conclusions The poorer outcomes of AUV patients both with respect to recurrence and long-term CKD indicate that close urologic follow-up is essential in this group. Download : Download high-res image (447KB) Download : Download full-size image Extended Summary Figure . Anterior Urethral Valve Diagnosis and Treatment

This paper presents a new urethral valve to deal with severe urinary incontinence (UI) caused by sphincter injury or dysneuria. This valve consists of a wireless power transfer system and a valve body structure driven by SMA (shape memory alloy) spring. The mechanical model of SMA spring and coupling model of wireless energy transfer are derived. Special attention is also given to the biological effects of electromagnetic filed as the proposed device is powered by wireless power transfer system. This valve is validated by simulation experiment and the models are used to investigate the influence of SMA spring parameters, control parameters and coil parameters on the driving characteristics of the proposed urethral valve.

A 4-year-old boy with posterior urethral valves and end stage kidney disease on peritoneal dialysis presented with high pressure non-compliant bladder with left ureterohydronephrosis. Inability to perform hemodialysis due to patient's weight exclusion, imposed the necessity to preserve peritoneal dialysis. A bilateral nephrectomy by retroperitoneoscopy with extraperitoneal augmentation ureterocystoplasty using left ureter and pelvis associated with continent diversion using right ureter as umbilical stoma was performed followed by kidney transplantation. An excellent outcome with voluntary voiding without CIC is reported eighteen months later. This treatment modality is the best option to manage End Stage Kidney Disease on peritoneal dialysis in those patients.

Summary We describe use of an improvised light source to perform cystoscopy and PUV resection while working in a resource poor setting. The light emitted from a mobile telephone LED (iPhone 6) was sufficient to perform the procedure and there was an excellent surgical outcome. We hope that this report may prove to be helpful to colleagues working in similar circumstances with limited resources.

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.

Objective The aim was to establish the reliability of the SWRD score as a predictor of both renal and bladder outcomes in posterior urethral valves. This retrospective study included 67 patients with PUVs at King Abdul-Aziz University Hospital. The score was calculated from voiding cystourethrogram before and after the relief of obstruction, and estimated glomerular filtration rates (eGFRs) were calculated as well. Results Based on Spearman correlations, both baseline eGFRs and SWRD scores can be possible predictors of long-term renal outcomes, as a significant positive correlation between the baseline eGFRs and the last eGFRs was found (p = 0.005). A significant negative correlation was also found between the SWRD score calculated before the intervention and the last eGFRs (p = 0.02). Additionally, the baseline SWRD scores can be possible predictors of short-term bladder outcomes, as the correlation analysis showed a positive relationship between the baseline SWRD scores and the SWRD scores calculated within 2 months after the intervention (p

Summary Background Neuropathic bladder, voiding dysfunction, and posterior urethral valves may cause a great challenge in children. Preserving the kidney function is the main aim in all of these patients which can be achieved by cutaneous vesicostomy. Objective The objective of this study is to evaluate the long-term outcomes of patients who have undergone cutaneous vesicostomy in an 11-year period at the study center. Study design In this retrospective study, the authors evaluated the long-term treatment results and complications of cutaneous vesicostomy on children with bilateral severe hydronephrosis, bilateral vesicoureteral reflux (VUR), and urosepsis who were operated at our center from 2007 to 2018. Results There were 64 (80%) boys and 16 (20%) girls. Their mean of age was 15.27 months old when they underwent vesicostomy. Twenty-three (28.75%) of them had neurogenic bladder and 17 (21.25%) of them had intact neuronal pathway defined as dysfunctional voiding. Twenty-five (31.25%) boys had posterior urethral valves. Fifteen (18.75%) of them were younger than six months old with primary bilateral high-grade VUR and urosepsis. Mean of follow-up time was 65.34 ± 37.82 months (11.5 months–10.5 years). Cure rate was 95% in urinary tract infection, 80.7% in secondary VUR, and 40% in primary VUR. Creatinine level was significantly reduced after vesicostomy and during follow-up (P Discussion A number of 29 of 66 patients with closed vesicostomy needed another surgery: three modified Gil-Vernet antireflux surgeries, one ureteral reimplantation, two endoscopic Deflux injections, 13 valve ablations, six ileocystoplasties, and four Botox injections. The 37 (56.06%) remaining patients did not require any other surgery after closure of vesicostomy. Conclusion Vesicostomy should be considered in children with neuropathic bladder or bladder outlet obstruction in case first-line therapies fail. This simple and reversible procedure can reduce febrile urinary tract infections, protect the upper urinary tract, and reduce the need for a major surgery without decreasing the bladder capacity. Table . The diagnoses of the studied children who underwent vesicostomy. Primary diagnoses Number Percentage Neurogenic bladder 23 28.75 Dysfunctional voiding 17 21.25 Posterior urethral valve 25 31.25 Primary high-grade VUR 15 18.75 Total 80 100 VUR, vesicoureteral reflux.

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

Background The incidence of posterior urethral valve (PUV) is estimated at 1:5000–1:8000 males. It is the most common paediatric urologic urgency and the most common cause of male obstructive uropathy and chronic renal failure in children. The study aimed to describe the experience of Yaoundé gynaeco-obstetrics and paediatric hospital in the management of PUV. Methods Retrospectively, medical records were retrieved over a ten year period and all data recorded and analyzed for study objectives. Patients were called and evaluated for outcomes regarding morbidity and mortality. Results A total of 18 patients all males were managed over the ten year period, given prevalence of 13 cases/100,000 admissions and an admission rate of 2 per annum. The median age at presentation was 22 months and 13 (72.2%) participants presented late. Voiding urethrocystogram was done in all the participants where it showed dilated and elongated posterior urethral valves in 16 (88.9%) of the cases. Endoscopic valve ablation resulted in the relief of obstruction in all but 3 (16.7%) participants that had residual valves and 2 (11.2%) participants that had urethral stenosis. Type I valves were most common in 14 (78.0%) participants. The mean duration of follow up was 34.56 ± 21.47 months. Complications at final follow up were: 10 (55.6%) chronic renal failure, 2 (11.2%) end-stage renal failure. The case fatality rate was 5.6%. Conclusion Many patients present late in our setting with already established complications. There is the need to counsel parents/guardians on the importance of long-term follow up after relief of obstruction.

Neurogenic bladder occur as a consequence of several conditions, most commonly posterior urethral valves syndrome, spina bifida, tethered cord, sacral agenesis, and Arnold-Chiari malformation. It is characterized by diminished bladder capacity and/or reduced compliance, associated with high-pressure voiding that can lead to deterioration of renal function if left untreated. When medical management fails, bladder reconstruction with bowel (aumentation ileocystoplasty) becomes the treatment of choice for these patients. In most cases, it is accompanied by the creation of a continent catheterizable channel, with the appendix being the most commonly used conduit (Mitrofanoff appendicovesicostomy). Conventional open surgery has proven to be an efficient and safe approach in these patients. However, robot-assisted surgery not only offers a cosmetic advantage, but also results in a shorter hospital stay, less postoperative pain, and even decreased number of adhesions (as shown in porcine models). Nevertheless, the complexity of this technique has limited the wide adoption of this approach. We seek to provide a technical guide to robot-assisted laparoscopic ileocystoplasty and Mitrofanoff appendicovesicostomy in pediatric patients, as well as a critical review of literature about the perioperative care of these patients.

The purpose of this review is to bring the reader up to date on the current risk factors for the development of renal deterioration in the boys with posterior urethral valves (PUV) and approaches to modify this risk. Renal bladder ultrasound (RBUS) is routinely performed in boys with PUV and recent advancements allow imaging processing that can more accurately quantify renal parenchyma and correlate this with risk for renal loss. Refinement of urine studies may improve our ability to stratify patients into renal loss categories. Use of videourodynamics (VUDS) allows refined assessment of the valve bladder to identify those who might benefit from secondary procedures and/or the addition of targeted pharmacotherapy to improve bladder emptying or dangerous storage pressures. All boys with a history of PUV are at a significant long-term risk of renal deterioration. The literature suggests that several technical advances have improved our ability to predict this risk, although there needs to be further refinement and validation before widespread use. Utilization of close follow-up, VUDS, pharmacotherapy, and bladder drainage provide the best methods to improve care to this group of patients and if more studies confirm their utility, adoption of these as part of standard of care protocols may be warranted.

N/A

Purpose To describe our experience in the management of children with urine retention at the Konfo Anokye Teaching Hospital in Kumasi Methods From 1 st April to 30 th November, 2012, data on children presenting with urine retention at the Komfo Anokye Teaching Hospital, Kumasi was obtained. The data collected was entered, cleaned, validated and analyzed using SPSS 16.0 for Windows. Results Thirteen male children presented with urine retention during the study period. Their mean age was 5.9±5.1 (range1-14) years. The causes of urine retention were; posterior urethral valves 9(69.2%), neurological disorder 2(15.4%) and one each of phimosis and bladder calculus. Conclusion Posterior urethral valve was the main cause of urine retention among children who presented to urologist in Kumasi. Immediate relief of urine retention was varied requiring the use of urethral catheterization, suprapubic cystostomy, or vesicostomy. Definitive management was varied and tailored to the underlying cause.

Urinary ascites is most commonly seen in fetuses and newborns with obstructive uropathy. The majority of cases are associated with lower urinary tract obstruction, such as posterior urethral valves. In this report, we present a unique case of neonatal urinary ascites caused by congenital supravesical obstruction. To date, neonatal urinary ascites secondary to bilateral mid-ureteral stenosis without a history of antenatal diagnosis has not been reported.

The most common cause of fetal bladder outlet obstruction is posterior urethral valves. Ultrasound examination shows a dilated, thick-walled bladder, often with dilatation of the proximal urethra—the “keyhole” sign. Renal findings may include hydronephrosis, cortical cysts, or kidneys that are small and echogenic. Ureteral dilatation is also common. Oligohydramnios before midgestation confers an extremely poor prognosis and prompts consideration for fetal vesicoamniotic shunt therapy. Important factors before vesicoamniotic shunt placement are that the anomaly appears isolated, that the fetus is male, that the chromosomal microarray (or karyotype) results are normal, and urinary analyte values obtained via serial vesicocentesis suggest relatively preserved renal function. Families need to be counseled about the high rates of acute and chronic morbidity associated with this condition.

Posterior urethral valves (PUV) are the most common cause of urinary obstruction in male. This condition only affects the boys as the etiology is congenital and related to opening of the ejaculatory ducts into the posterior urethra. The pathology often involves the urinary tract above the obstruction that determines the outcome.

Purpose To determine whether there is a difference in the outcome of renal transplantation (RT) in patients with posterior urethral valves (PUV) and children with non-uropathy related end stage renal disease. Methods Data were acquired retrospectively. We analyzed possible factors that influence the function of renal allografts and graft survival. Between 1995 and 2016 there were 149 RT. Out of them, there were 27 boys with PUV, who received 29 kidneys. Thirty patients, who received a total of 31 renal grafts due to a non-uropathic (NU) diagnosis, served as control group. Mean follow-up was 7.4 to 10.2 years. Results There was no difference in estimated graft survival between patients with PUV and NU patients. Graft failure occurred in 23.1% of PUV patients and 34.5% patients of the NU group. There was no statistically significant disparity in graft function between the two groups. Age at transplantation and donor age were the only factors that had a significant impact on renal function. There was a higher incidence of UTI in the PUV group (96%) than in the NU group (67%). Vesicostomy was the favourable intervention in regards of graft function. Conclusions RT in PUV patients is successful with the same outcome as in NU patients. Bladder dysfunction may not have a major impact on graft function and graft survival. It seems that the type of pre-transplant surgical procedures may influence outcome. Therefore, these interventions -if necessary- should be limited to a minimum. Type of Study Retrospective Comparative Study Level of Evidence Level III

Posterior urethral valves (PUV) are the most common cause of lower urinary tract obstruction in male infants. Due to improved technique and availability of prenatal imaging, diagnosis is typically suspected prenatally and confirmed postnatally. Prenatal intervention is performed in select centers with vesicoamniotic shunting or with fetal cystoscopy and antegrade ablation of the PUV when detected. Prenatal intervention has been associated with an improvement in neonatal survival; however, evidence that renal function is indeed improved remains scant. The mainstay of treatment remains early diagnosis with early establishment of drainage and subsequent management of bladder dysfunction with behavioral therapy, medication, clean intermittent catheterization, and occasionally with bladder augmentation. Other measures such as nephrectomy can be implemented to manage polyuria. Ultimately, 30 % of patients born with PUV develop end-stage renal disease and require renal transplantation.

Posterior urethral valves (PUVs) are the most common cause of infravesical obstruction in children, whereas anterior urethral valves and/or diverticula (AUV/D) are less commonly encountered. Concomitant AUV/D and PUV is believed to be very rare and may be missed on the initial evaluation. In this review, we provide brief information on embryology of AUV/D and PUV to explain the concurrent presence of these anomalies. We also summarize the information on published cases of concomitant AUV/D and PUV in the English literature with a special focus on diagnosis and the importance of both voiding cystourethrography and careful urethrocystoscopy.

Objective To report our 12-year experience with endoscopic management of patients with concomitant anterior and posterior urethral valves. Methods We retrospectively reviewed the charts of patients referred to us for management of urethral valves from 2000 to 2012 to find cases with concomitant anterior and posterior valves. The diagnosis of valves was first suspected on voiding cystourethrography and confirmed by urethrocystoscopy. We collected available data on patients' age at diagnosis, clinical presentations, ultrasound and urodynamic findings, and surgical treatments. The final outcome at last follow up was also recorded. Results From 38 cases with anterior urethral valve, six (15.8%) presented concomitant anterior and posterior valves. The age at diagnosis in these patients ranged from antenatal diagnosis to 13 years. Initial presenting symptoms were recurrent urinary tract infection, incontinence, urosepsis and poor urinary stream. All valves were ablated by transurethral fulguration/resection using small-sized urethrocystoscopes. Among those with concomitant anterior and posterior valves, four patients had vesicoureteral reflux at presentation that resolved in two patients after valve ablation. One patient progressed to renal failure and required dialysis. Bladder hypercontractility and detrusor overactivity were the main urodynamic patterns in these patients. Conclusions Concomitant anterior and posterior valves seem to be more prevalent than previously assumed, and might be missed on initial assessment. Oblique view voiding cystourethrography with full-length delineation of the urethra is of paramount diagnostic importance when obstruction is suspected. A meticulous urethrocystoscopy should follow for confirming the diagnosis and endoscopic ablation/resection of the valves.

Objective: To report the results of fetal cystoscopic laser ablation of posterior urethral valves (PUV) in a consecutive series in two referral centers. Methods: Twenty pregnant women with a presumptive isolated PUV were treated with fetal cystoscopy under local anesthesia. Identification and fulguration of the PUV by one or several firing-contacts with diode laser were attempted. Perinatal and long-term outcomes were prospectively recorded. Results: The median gestational age at procedure was 18.1 weeks (range 15.0-25.6), and median operation time was 24 min (range 15-40). Access to the urethra was achieved in 19/20 (95%) cases, and postoperative, normalization of bladder size and amniotic fluid was observed in 16/20 (80%). Overall, there were 9 (45%) terminations of pregnancy and 11 women (55%) delivered a liveborn baby at a mean gestational age of 37.3 (29.1-40.2) weeks. No infants developed pulmonary hypoplasia and all were alive at 15-110 months. Eight (40% of all fetuses, 72.7% of newborns) had normal renal function and 3 (27.3%) had renal failure awaiting renal transplantation. Conclusion: Fetoscopic laser ablation for PUV can achieve bladder decompression and amniotic fluid normalization with a single procedure in selected cases with anyhydramnios. There is still a significant risk of progression to renal failure pre or postnatally.

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.

These types of pathology do not need conservative surgery and segmentectomy. The only exception is Ask-Upmark kidney or segmental hypoplasia. This maldevelopment forms cortical scars, sinking, and wrinkling of the underlying pyramids, and dilation of relative calyces. Histologic examination of this abnormal metanephric differentiation shows microcysts and the absence or glomerular paucity. Urethral valves and vescicorenal reflux, with or without infection, frequently coexist. This demonstrates the acquired maturation of the lesions, although already present in intrauterine life. Obviously conservative surgery and segmentectomy could be considered when the segmentary form of the hypoplasia is associated with normal unaffected parenchyma. It is also obvious that prior to renal conservative surgery, any other malformations (urethral, ureteral) must be corrected, if present [104-109].