Posterior Urethral Valve Patients Research Articles

Residual valve and stricture after posterior urethral valve ablation: How to evaluate?

ObjectiveTo investigate the clinical and radiological parameters of posterior urethral valve (PUV) patients with residual valve or strictures after primary valve ablation. Patients and methodsA total of 127 PUV patients were treated in our clinic between 1986 and 2009. We retrospectively reviewed the records of 101 patients, who had at least 1 year of follow-up data, regarding the presence of valve remnants or urethral strictures after PUV ablation. ResultsA total of 21 patients (20.8%) underwent repeat-urethroscopy and, of these, residual valve leaflets or stricture were detected in 10 patients (10/101, 9.9%). In 2 of these 10 (20%), the urethra had been found to be normal on the first voiding cystourethrogram following ablation. However, these two boys underwent re-urethroscopy due to persistent vesicoureteral reflux in one and persistent hydroureteronephrosis in the other, and valve remnants were detected. The remaining 8 cases had radiological signs consistent with persistent infravesical obstruction in the early period. Obstruction was due to urethral stricture and residual valve remnants in 2 and 6 cases, respectively. ConclusionsThere was clinical suspicion of residual valve in about 20% of the cases and in half of these the urethra was found to be normal on urethroscopy. The possible presence of residual valve remnants after primary valve ablation should be confirmed by careful clinical, radiological and endoscopic evaluation.

Risk factors for renal insufficiency in children with urethral valves

Posterior urethral valves (PUV) associated with renal dysplasia are one of the most common causes of end stage kidney disease (ESKD) in childhood. In order to identify risk factors for the progression of this condition to early renal failure, we have retrospectively analyzed the clinical course, renal function, and first postnatal renal ultrasound in a sample of 42 young male patients with PUV, who were followed at a single center. Twelve (28.6%) were diagnosed with ESKD at a median age of 11.3 years. Our comparison of PUV patients without decreased estimated glomerular filtration rate (eGFR) (group A; K/DOQI CKD stage 0-1) with PUV patients showing a decreased eGFR (group B; K/DOQI CKD stage 2-5) revealed the following significant risk factors for loss of eGFR: renal volume <3rd percentile (P < 0.001), elevated echogenicity (P = 0.001), pathologic corticomedullary differentiation (P < 0.001), >3 febrile urinary tract infections (P = 0.012), and decreased eGFR at 1 year of age (P < 0.001). Receiver operating characteristic curve analysis in the cohort confirms that patients showing a renal volume >88.2 ml/m(2) body surface area (BSA) are not at risk to develop K/DOQI CKD stage 5 (sensitivity 75%, specificity 77.3%, positive/negative predictive value 37.5/94.4%). Ultrasound promises to be a valuable tool for identifying endangered patients.

Posterior urethral valve

Posterior urethral valve (PUV) is a significant cause of morbidity, mortality and ongoing renal damage in children. It accounts for end-stage renal disease in a proportion of children. This article aims at highlighting the current trend in the management of boys with posterior urethral valve. PubMed/Medline and bibliographic search for posterior urethral valve was done. Relevant literatures on presentation, pathology, evaluation, management and outcomes of PUV were reviewed. PUV which is increasingly diagnosed prenatally presents a spectrum of severity. The varied severity and degree of obstruction caused by this abnormality depend on the configuration of the obstructive membrane within the urethra. The decision to intervene prenatally is dependent on gestational age, amniotic volume, and renal function of fetal urine aspiration. Identification of the patients who may benefit from early intervention remains inconclusive. Endoscopic ablation of the valve is the gold standard of treatment but use of Mohan's valvotome and other modalities are invaluable in developing countries where endoscopic facilities are limited. Proximal urinary diversion may result in poor bladder compliance and should be reserved for patients with persisting or increasing upper urinary tract dilatation, increasing serum creatinine or inappropriate instruments. The behavior of the bladder and its subsequent management after valve ablation may influence the long-term renal outcome in PUV patients. The care of children with PUV continues to improve as a result of earlier diagnosis by ultrasound, developments in surgical technique and meticulous attention to neonatal care. The ultimate goal of management should be to maximize renal function, maintain normal bladder function, minimize morbidity and prevent iatrogenic problems.

Lower Urinary Tract Symptoms in Adults Treated for Posterior Urethral Valves in Childhood

Purpose Lower urinary tract symptoms (LUTS) are common in children with posterior urethral valves (PUV). In the present study we compared the prevalence of LUTS between adult patients treated for PUV in childhood and age-matched population-based cohort. Material and Methods Information on LUTS was assessed using the Danish Prostatic Symptom Score (DAN-PSS) questionnaire. Out of 108 PUV patients, 69 (64%) returned the questionnaire. Age-matched-cohort of 1,242 men (randomly identified from the population register) was used as a control. Results The median age of the 69 patients and controls was 38 years (age range: 17-57 for patients and 18 -57 for controls). Both any urgency and any stress incontinence were more common in the patients than in the controls (14.5% and 11.6% vs. 6.3% and 3.6%, p=0.02 and p= Fifteen of the 69 patients (22%) had originally had a temporary bilateral ring-type ureterocutaneostomy because of severe disease and seven patients (10%) had later undergone kidney transplantation. The incidence of LUTS did not differ between these groups and those without primary stoma and kidney transplantation. Conclusions In adulthood PUV patients have more LUTS than normal population.

Augmentation of Non-Compliant Bladder with Associated Vesicouretral Reflux (VUR): is Concomitant Antireflux Surgery Mandatory?

Purpose We studied VUR resolution and risk of pyelonephritis following bladder augmentation. Material and Methods Between February 2005 and March 2009, 42 patients underwent ileocystoplasty for non-compliant bladder at a mean age of 9 years (range 4-17). Patients included 29 boys and 13 girls. The indications are neuropathic bladder (NB) in 19, posterior urethral valves (PUV) in 12, and bladder exstrophy (BE) in 11. Bladder neck reconstruction was performed for 21 patients (50%). VUR was low-grade in 15 (34%), high-grade in 27 (64%), unilateral in 18 (43%), and bilateral in 24 (57%). All patients were kept on oral antibiotic prophylaxis. Patients were followed up with renal/bladder ultrasound and voiding cystourethrography at 3 months postoperatively and annually thereafter. Results Mean follow-up duration was 20 months (range 8-48). VUR resolved in 21 patients (50%). During follow-up 6 patients (14%) had documented one or more attacks of break-through pyelonephitis. For PUV patients, VUR resolution rate was 83% and pyelonephritis rate was 8%. For BE, VUR resolution rate was 18% and pyelonephritis rate was 9%. For NB, VUR resolution rate was 47% and pyelonephritis rate was 21%. For patients with initial low-grade VUR, resolution rate was 33% and rate of pyelonephritis was 0%. Although patients with initial high-grade VUR had higher resolution rate of 59%, the rate of pyelonephritis was 22% which is of statistical significance (p Conclusions At time of bladder augmentation, concomitant anti-reflux surgery should be performed for all patients with initial high grade VUR regardless of etiology. If left uncorrected, the risk of pyelonephritis and subsequent need for a difficult anti-reflux surgery is 22%.

Association of angiotensin converting enzyme and angiotensin type 2 receptor gene polymorphisms with renal damage in posterior urethral valves

To examine the association with renal damage in patients with posterior urethral valves (PUV) of two renin-angiotensin system gene polymorphisms: angiotensin converting enzyme insertion/deletion (ACE I/D) and angiotensin type 2 receptor (AT2R A1332G), PATIENTS AND METHODS: In 120 patients with PUV, after stabilization, transurethral fulguration or a Blocksom vesicostomy was performed. Records were reviewed for age at diagnosis, biochemical renal function at diagnosis, results of urine cultures, voiding cystourethrograms, radiologic, sonographic and nuclear medicine scan findings, and follow-up data. ACE I/D genotypes were determined by the polymerase chain reaction using allele specific primers. The frequency of the ACE DD genotype was significantly higher in patients with chronic kidney disease (P=0.02) and renal scarring (P=0.05). These genotypes were also associated with a statistically higher incidence of vesicoureteral reflux, diurnal incontinence, proteinuria and hypertension. A significantly higher frequency of the AT2R GG genotype was found in PUV patients as compared to healthy unrelated control subjects (P=0.001), and in PUV patients with scarring (P=0.02). The ACE DD and AT2R GG genotypes are associated with chronic kidney disease and scarring in PUV patients. The GG genotype incidence is higher among PUV patients compared to the control population, and further studies in this area may help understanding of the genetic basis of PUV.

Clinical and Radiological Changes after use of Alpha-1 Blocker Therapy in Patients of Posterior Urethral Valve with Bladder Neck Hypertrophy

Background: We evaluated the effects of alpha-1 blocker therapy on clinical and radiological abnormalities in patients of posterior urethral valve with bladder neck hypertrophy.&#x0D; Materials and Methods: A total of 74 patients with posterior urethral valves were seen at our department between 2003 and 2007. Out of these 24 had radiological evidence of bladder neck hypertrophy. Those patients with bladder neck hypertrophy who were seen before June 2006 and did not receive alpha-1 blocker (prazocin) therapy after valve ablation were assigned to group 1 (n=10). Group 2 consisted of 14 age matched patients with bladder neck hypertrophy and comparable prognostic factors who received alpha-1 blocker therapy after valve ablation (n=14). Micturating cystourethrography (MCU) was done periodically and a novel method used to calculate bladder neck hypertrophy. Trends in symptoms and radiological changes were evaluated throughout follow-up.&#x0D; Results: Mean patient age at presentation was 3.02±2.68 years in group 1 and 3.12±3.4 years in group 2. Bladder neck hypertrophy decreased from 2.3±1.0 to 2.0±0.5 in a mean time of 52.0 (34-52) weeks in group 1 where as it decreased from 2.2±0.5 to 1.6±0.3 in a mean time of 32.0 (22-52) weeks. Symptomatically patients in group 2 who received alpha-1 blocker therapy after valve ablation were better and had quick resolution of bladder neck hypertrophy.&#x0D; Conclusions: Use of alpha-1 blocker therapy in patients of posterior urethral valve with bladder neck hypertrophy helps in quick resolution of bladder neck hypertrophy. J Med Sci 2009;12(1):3-7.

Posterior Urethral Valves and Cryptorchidism

PURPOSE To evaluate the incidence of cryptorchidism in patients with a posterior urethral valve (PUV) and to analyse if the patient characteriscs are different in patients with and without cryptorchidism. MATERIAL AND METHODS Two hundred PUV patients, treated during years 1953-2003, were identified from hospital database. Patient records were retrospectively reviewed. In 191 patients the state of testicular descent was recorded. Patient characteristics were analyzed in this patient cohort. RESULTS Thirty-one out of 191 patients (16%) had cryptorchidism, which was bilateral in 6 (26%). The patients with cryptorhidism had a more severe form of PUV. Serum creatine levels were higher in cryptorchid group and poor functioning kidneys were detected more often in these patients. At the time of diagnosis serum creatinine was 154± 120umol/L in the cryptorchid boys and 123± 109umol/L in noncryptorchid boys, p=0.234. After 6 months follow-up the serum creatine levels were 149± 159umol/L in the cryptrorchid group and 70± 85umol/L in boys with normal testes, p=0.013. Cryptorchidism was also more common in those who had neonatal diagnosis compared to those with diagnosis at later age (10 out of 32 patients (31%) vs. 18 out of 128 patients (14%) respectively, p=0.035). CONCLUSIONS The incidence of cryptocrchidism requiring surgery is 16% in patients with PUV. The patients with cryptorchidism appear to have a more severe form of PUV.

Results of Prospective Clinical Trial Comparing Concurrent Valve Ablation/Bladder Neck Incision (BNI) with Simple Valve Ablation in Children with Posterior Urethral Valve (PUV)

Purpose To evaluate whether BNI along with PUV ablation can improve the final outcome. Patients and methods From 1998 to 2003, 46 PUV patients entered our prospective clinical trial. In group 1, 22 patients underwent simultaneous valve ablation and BNI (at 6 o'clock position). In group 2, 24 age-matched patients with comparable risk factors underwent simple valve ablation. Trends of renal function tests, urodynamics, and changes in the upper urinary tracts were evaluated throughout the follow-up. Results The mean age of patients at presentation was 1.6 and 1.8 years in group 1 and 2, respectively. The mean (range) duration of follow-up in both groups was 4.5 (2 to 7) years. Preoperatively, all patients had hypercontractile bladders and comparable high Pdetmax in both groups. Six months after procedure, mean Pdetmax, number of patients with bladder hypercontractility, and detrusor instability were significantly lower in group 1 versus 2. At the end of our follow-up, in group 1 no patient had bladder hypercontractility or detrusor instability and mean Pdetmax was 53 ± 15 cmH2O; whereas in group 2, 9 patients had bladder hypercontractility, 6 had detrusor instability, and mean Pdetmax was 87 ± 45 cmH2O (P Conclusions Combination of BNI and PUV ablation confers better bladder and renal function outcomes compared with simple valve ablation. Our findings propose bladder neck discoordination as an imperative part of PUV pathophysiology. The modified BNI technique seems to minimize the possibility of future retrograde ejaculation.

Angiotensin-converting enzyme gene polymorphism in North-west Indian children with posterior urethral valves

Purpose To investigate the association of angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism and other risk factors with renal scarring in patients with posterior urethral valves (PUV). Materials and methods Forty consecutive patients from North-west India were treated for PUV in 1997–2004. The patients were divided into group 1 (no renal scarring, n = 12) and group 2 (renal scars present, n = 28) based on dimercato-succinic acid scans. ACE I/D polymorphism was determined by polymerase chain reaction in PUV patients and unrelated healthy controls ( n = 100). Results Mean age at presentation was 23.7 ± 37.2 months and mean follow up was 4.8 ± 1.5 years. Preoperative mean serum creatinine levels for group 1 (non-scarred) and group 2 (scarred) were 1.1 ± 1.6 mg/dl and 1.7 ± 1.6 mg/dl, respectively. One year after treatment, the serum creatinine levels had decreased to 0.6 ± 0.1 mg/dl and 0.8 ± 0.3 mg/dl in group 1 and group 2, respectively. ACE genotype distribution in children with PUV was no different from that of controls. The occurrence of D allele was significantly ( p = 0.04) higher in patients of group 2. Multivariate logistic regression analysis showed that D allele had a significant impact on renal scar formation, introducing a 4.6-fold risk (odds ratio 4.6, 95% confidence interval 1.03–20.38, p = 0.04). A highly significant correlation between the occurrence of renal scarring and presence of breakthrough urinary tract infection (odds ratio = 7.5, 95% confidence interval 1.60–35.07, p = 0.006) and serum creatinine at follow up (odds ratio = 0.6, 95% confidence interval 0.47–0.81, p = 0.03) was observed. The mean values for glomerular filtration rate (GFR) after 1 year of treatment ( p = 0.006) and at follow up ( p = 0.027) were significantly different between the patients with II genotype and ID/DD genotype. Hypertension was observed in 13 patients and proteinuria in nine patients with no significant difference between the patients having II/I D/DD genotypes. Conclusion The presence of D allele is associated with progression of renal scarring and reduced GFR in PUV patients.

Significance of age-specific creatinine levels at presentation in posterior urethral valve patients

Objective Determination of prognostic factors is of great importance in decision making on therapies and informing parents about the probable outcome of disease. The present study aims to evaluate and assess the prognostic factors in posterior urethral valve (PUV) patients. Materials and methods The data of 68 patients treated between 1996 and 2004 were evaluated retrospectively. The mean age at diagnosis was 36.4 months. The serum creatinine levels were standardized using the age-specific creatinine reference (ASCR) levels. The age at diagnosis, clinical presentation, initial serum creatinine, method of treatment, presence of vesicoureteral reflux (VUR) and hydroureteronephrosis (HUN), all of which may effect the prognosis, were evaluated. Results The analysis revealed that, of the 36 patients whose initial serum creatinine levels were within ASCR, only one (2.8%) had a final serum creatinine higher than ASCR values. In contrast, of the 32 patients whose initial serum creatinine levels were not within ASCR, 19 (59.4%) had final serum creatinine levels higher than ASCR ( P < 0.001). The distribution of final serum creatinine according to treatment method, presence of UTI before treatment and age at diagnosis was not statistically significant; however, the difference in distribution for initial serum creatinine ( P < 0.001), mode of clinical presentation (0.016), presence of HUN (0.019) and state of VUR (0.037) before treatment was statistically significant. Conclusion Final outcome with regard to renal function in PUV patients is affected by bilateral VUR and most significantly by serum creatinine levels higher than ASCR.

Effectiveness of Oral Desmopressin Therapy in Posterior Urethral Valve Patients with Polyuria and Detection of Factors Affecting the Therapy

Objectives: The present study aims to evaluate the effect of desmopressin treatment on urine output, density and glomerular filtration rate (GFR) in patients with posterior urethral valve (PUV) and the factors affecting the response to this treatment. Methods: A total of 68 PUV patients who were followed-up after valve ablation were examined with the fluid intake, urine output and GFR. Sixteen patients who were polyuric (a urine output more than 30 ml/kg/day) and had hypoosmolar urine (urinary density of 1015 or lower) included in the study. Blood chemistry and serum ADH level were studied. Following 5 days of observation, patients were given DDAVP perorally with a dosage of 0.4 mg/day, two equal doses per day. After 7 days and after a 3 month period of treatment, voiding characteristics, day-time and night-time urine densities and also GFR have been re-evaluated. Results: The mean age was 6.8 years (range 2 to 11 years). The mean age at valve ablation was 20.7 months (range 5 months to 6 years). The mean daily urine output during first week and at the third month of the treatment had decreased significantly ( p = 0.004 and p = 0.006). There was increase in night-time and day-time urine density in 10 patients (62%) and in 13 patients (81%) respectively at the third month evaluation. Increments in urine density were statistically significant for the third month evaluation. Nine (56%) patients had ADH levels within normal (<7 pcg/ml) levels and 7 patients had higher levels. There was no statistically significant difference between pretreatment and posttreatment micturation characteristics. However patients with voiding dysfunction responded better to DDAVP treatment. Conclusions: Desmopressin treatment improves polyuria in PUV patients. The responses are better particularly in PUV patients with significant bladder dysfunction. This supports the harmful role of polyuria on bladder dysfunction. The DDAVP treatment improves the day-time and night-time in PUV patients. Combination of DDAVP treatment with overnight catheterization may be a good alternative that needs to be evaluated by further prospective randomized studies.

Cutaneous Vesicostomy Revisited - the Second 15 Years

The aim of this paper was to review the authors' experience with cutaneous vesicostomy (CV) over the last 15 years including indications, results, and complications of CV. The records of 31 patients treated by CV between 1987 and 2002 were reviewed. There were 20 boys and 11 girls. The two main primary pathologies were neuropathic bladder (19 patients) and posterior urethral valve (PUV) (7 patients). All patients underwent a Blocksom-type operation at a mean age of 23 months (range 14 days-9 years). Pre- and postoperative conventional uromanometry was performed in 18 patients (58%) and bladder function was assessed. In 23 patients (74%) the CV provided a successful diversion with improvement of the upper urinary tract and/or stabilization of the renal function. In 5 patients (16%) with PUV, the improvement was temporary. In 3 patients (10%) the CV did not result in an improvement. Twenty-four patients underwent CV closure after a mean duration of 23 months (range, 1 month-7 years) of diversion. In 2 patients with myelomeningocele (MMC) and severe somato-mental developmental delay, CV was not closed and is being considered as a permanent treatment option. Urodynamic studies in 5 PUV patients showed impaired compliance and high intravesical pressure following a successful valve ablation and closure of CV. In the neuropathic bladder group the bladder function improved following closure of CV and commencement of anticholinergic medication and clean intermittent catheterization (CIC). Our augmentation ratio in the neuropathic bladder group was 22%. Complications of CV included: stenosis in 7 patients (22%), prolapse in 2 (6%), and cellulitis in 2 (6%). The revision rate was 16%. In young infants CV had a less favourable result in the PUV patients than in cases with high-pressure neuropathic bladder with upper tract dilatation and severe urinary tract infection (UTI), where CV provided decompression and prevented deterioration of the renal function. Cutaneous vesicostomy has stood the test of time in our changing paediatric urological practice and it remains a valuable weapon in the armoury of paediatric urologists in selected patients.

Hormonal analysis in post-pubertal patients with posterior urethral valves.

Infertility in patients with posterior urethral valves (PUV) is known but the exact incidence and etiology is still speculative; it may be partly due to dysfunction of genital organs. The aim of this study is to analyse the hormonal profile of post-pubertal patients with PUV. All post-pubertal patients with posterior urethral valves (age > 16 years) visiting the follow-up urology clinic of our department from 1985 to December 1999 were contacted and requested to participate in the study; 9 patients agreed and form the study group. The medical records were reviewed. Physical examination included examination of the genitalia and secondary sexual characteristics. Blood samples were taken for estimation of lutenizing hormone (LH), follicle-stimulating hormone (FSH), prolactin and testosterone. Ages ranged from 16 - 21 years (mean age = 17.5 years). Physical examination was normal for the entire study group except for 2 patients; one patient had hypertension and the other had unilateral undescended testis at the time of initial presentation. Secondary sexual characteristics were normal in all patients. At the time of the study, one patient had chronic renal failure; in the others the renal function tests were normal. LH and testosterone were normal in all patients including the patient with unilateral undescended testis, and one patient had marginally elevated FSH. Hyperprolactinemia was noted in 5 of 9 patients. In the present study, hyperprolactinemia was seen in 62 % (5 of 9) patients. Hyperprolactinemia is known to cause slow ejaculation and can have an impact on the subsequent fertility status of these patients. The bearing of these findings on subsequent sexual and fertility functions of PUV patients requires further investigation.

Urinary Tract Infections in Children with Posterior Urethral Valves After Kidney Transplantation

The records of 14 boys with posterior urethral valves who had renal failure and subsequently underwent renal transplantation were reviewed to determine the postoperative incidence of urinary tract infection relative to that of 29 male transplant children without valves, who served as controls. There were no significant differences between the posterior urethral valve patients and controls with regard to age, donor source, immunosuppression, followup after transplantation or mean calculated creatinine clearance. Vesicoureteral reflux was found in 1 child with posterior urethral valves and 3 of the children in the control group (p not significant). A total of 15 urinary tract infections occurred in 5 children (36%) with posterior urethral valves, for a rate of 1 per 30 patient-months of followup, and 6 urinary tract infections occurred in 2 controls (7%), for a rate of 1 per 216 patient-months of followup (p <0.05). However, only 1 of 26 controls (4%) without vesicoureteral reflux had urinary tract infection, for a rate 1 per 1,144 patient-months (p <0.01). Conversely, the rate of urinary tract infections in controls with vesicoureteral reflux was similar to that of children with posterior urethral valves. Of the 5 children with posterior urethral valves 4 had the initial urinary tract infection within 2 months of transplantation and 10 of 15 episodes occurred within the first 4 months. Antimicrobial prophylaxis did not appear to decrease the rate of infection in children with posterior urethral valves. A history of posterior urethral valves increases the frequency of urinary tract infection after renal transplantation but the usefulness of antimicrobial prophylaxis and the relationship to long-term graft function remain to be determined. Urinary tract infection rarely develops in other transplanted boys without vesicoureteral reflux.

Posterior urethral valves: Functional classification

Among 37 boys with posterior urethral valves (PUV), the clinical courses could be divided into three functional levels of severity which did not always correspond to the traditional anatomic classification of valves. Children with PUV presented at all ages, had varying degrees of renal impairment, and manifested a variety of voiding symptoms. Eleven patients progressed to uremia, but the progression did not correlate with the presence of vesicoureteral reflux or the extent of the voiding dysfunction. Two patients with PUV fathered children eighteen and twenty-one years, respectively, after recognition of their valves. A functional classification of PUV patients is suggested as a way to overcome long-standing bias about the levels of kidney function attainable after primary valve treatment.