Are urodynamic studies really needed during bladder augmentation follow-up?

ObjectiveWe assessed clinical and urodynamic outcomes, over a minimum 10-year follow-up period, of neuropathic bladder patients treated with a bladder augmentation (BA) to determine if periodic urodynamic studies are needed.Material and methodsThirty-two patients with poorly compliant bladders underwent BA at a mean age of 11 years (2.5–18). Mean follow-up was 12 years (10–14.5) and mean patient age at the end of the study was 22 years (12.2–33). During follow-up all patients were controlled at regular intervals with urinary tract imaging, serum electrolyte and creatinine levels, cystoscopy and urodynamic studies. Preoperative, 1-year post-BA and latest urodynamic studies results were compared.ResultsUrodynamic studies at 1-year post-BA showed a significant increase in bladder capacity and a decrease in end-filling detrusor pressure compared with preoperative values (396 vs 106 ml; 10 vs 50 cm H₂O, P < 0.0001). The increase in bladder capacity was more significant at the end of the study than after 1 year (507.8 vs 396 ml, P < 0.002). Thirteen patients had phasic contractions after 1 year and 11 at the end (not significant, NS), and these contractions were more frequent with colon than with ileum (NS). At the end of follow-up, phasic contraction pressure had decreased while trigger volume had increased (35 vs 28 cm H₂O; 247 vs 353 ml, NS). All patients are dry and have normal renal function, except one who had mild renal insufficiency before BA.ConclusionBA improves bladder capacity and pressure, and these changes are maintained over time (although phasic contractions do not disappear). Repeated urodynamic studies are only necessary when upper urinary tract dilatation or incontinence does not improve.     

Plasma renin activity for monitoring vesicoureteric reflux therapy: mid-term observations

ObjectiveTo study plasma renin activity (PRA) as an early marker for monitoring treatment of vesicoureteric reflux (VUR).Patients and methodsFifty-nine children (35 males and 24 females), mean age 43.3 ± 26.5 (range 4.5–89) months, with various grades (I–V) of primary VUR were enrolled. PRA, renal scars, split renal function (SRF), glomerular filtration rate (GFR), serum creatinine, blood pressure and episodes of breakthrough urinary tract infection were monitored at regular intervals. Surgery was performed as per currently accepted criteria. PRA values were used for post-hoc analysis of results.ResultsThirty-eight children (64.4%) underwent anti-reflux surgery during the mean follow up of 17.1 ± 3.1 months; 21 (35.6%) continued on non-operative follow up. Rise in PRA up to the time of surgery was seen in all patients. It normalized after surgery in 86%, and reduced but plateaued at a higher level than normal in 13.8% in the surgical group. While improvement in SRF and GFR was seen only in 2/38 (5.2%) and 12/38 (31.6%), respectively, blood pressure stabilized in 30.7% and serum creatinine showed inconsistencies. In non-operatively managed cases, improvement in SRF was seen in only one case and GFR in 14.2% of cases. However, 80.9% children showed a progressive rise in PRA throughout the period of non-operative follow up.ConclusionCurrent end points of non-operative management already cause irreversible renal damage by the time surgery is indicated. Our results suggest that serial measurement of plasma renin activity may help in better stratification of patients with moderate to high grade (III–V) VUR with respect to management and prognosis.     

Total endoscopic management (TEM approach) of children with non-compliant neuropathic bladder: a preliminary report

PurposeWe prospectively evaluated the efficacy and durability of a combination of intradetrusor botulinum-A toxin (BTX-A) and endoscopic treatment of vesicoureteric reflux (VUR) to manage children with myelomeningocele (MMC) and non-compliant refluxing bladders who were not responding to standard conservative therapy. We also evaluated whether this combined therapy can lower intravesical pressure, increase bladder capacity, gain social continence and protect the upper tract from recurrent urinary tract infection.Material and methodsA total of 10 patients with a mean age of 5.9 ± 3.6 years (range 2–12 years) with MMC (eight females and two males) were prospectively involved in the study. All patients were fully compliant to clean intermittent catheterization, and all were non-responders (failed to gain continence and/or poor compliance) to the maximum tolerable dose of anticholinergics and catheterization. All patients were subjected to cystoscopic intradetrusor injection of 12 U/kg (maximum 300 U) of BTX-A in an infection-free bladder. They all had VUR (16 refluxing ureters, six patients with bilateral VUR) and did not show resolution in the pretreatment voiding cystourethrogram; accordingly, submucosal injection of Deflux^® was performed either with the second BTX-A treatment (initial four patients) or with the first BTX-A treatment (the other six patients). The grade of reflux was G III, IV and V in three, seven and six ureters, respectively.ResultsThe maximum bladder capacity increased significantly from 79 ± 49 to 155 ± 57 ml (p < 0.022), and the maximum detrusor pressure decreased significantly from 55 ± 16 to 37 ± 11 cm H₂O (p < 0.001). Fifteen out of 16 (93.75%) refluxing ureters were completely resolved (one of them on second attempt), and one (6.25%) (GV reflux) remained unchanged despite of two attempts. Of six incontinent patients, five reached complete dryness between catheterizations and one showed partial improvement.ConclusionsA combination of BTX-A and endoscopic correction of VUR is a simple and effective way to overcome the increased risk of high intravesical pressure and recurrent UTI. This treatment decreases the incidence of renal damage in children on whom conservative management fails to help, in a minimally invasive way.     

The role of the Lich-Gregoir procedure in refluxing duplicated collecting systems: experience from long-term follow up of 45 children

ObjectiveThe need for surgical correction of vesicoureteral reflux (VUR) is increased in duplicated systems. The aim of this study was to evaluate the outcome of the Lich–Gregoir procedure (LG) with regard to VUR persistence, contralateral de-novo VUR, hydronephrosis, preservation of split renal function, urinary tract infections (UTI) and postoperative side effects.Patients and methodsBetween 1993 and 2007, 45 children (mean age 3.2 years) underwent a unilateral common sheath LG. A combined number of at least 75 episodes of febrile UTI had occurred in 39 children prior to surgery. VUR grades I to V were present in two, nine, 16, 16 and two children, respectively. Hydronephrosis was present in 18 children. Mean split renal function was 44.03% (range 15–63%). Indications for surgery were febrile breakthrough UTI in 11 children and abscessing pyelonephritis in two. The remainder underwent surgery due to renal scars, reduced split renal function (<45%), VUR persistence and/or parental desire.ResultsPersistent ipsilateral and de-novo contralateral VUR were detected in three children (ipsilateral in one, contralateral in one, bilateral in one), resulting in a 4.4% rate of persistent ipsilateral VUR. One year post surgery, low-grade hydronephrosis persisted in six patients without impact on split renal function. Mean split renal function remained stable at 44.06% (range 15–68%). During follow up (mean 41 months), six febrile UTIs occurred in five girls (92.4% risk reduction, P < 0.00000005). Neither urinary retention nor any other side effect was observed.ConclusionPerformed unilaterally, common sheath LG is a safe and effective technique to cure VUR, prevent febrile UTI and maintain split renal function in duplicated systems with otherwise uncomplicated anatomy.     

Post-pyelonephritic renal scars are not associated with high voiding pressures

ObjectiveThe aim of this study was to evaluate whether renal scars and vesicoureteral reflux (VUR) are associated with bladder dysfunction in children after first clinical pyelonephritis.Patients and methodsSixty-four children were evaluated with urodynamics and voiding cystourethrography at a median of 8 weeks after their first episode of clinical pyelonephritis. All patients had ultrasonography and dimercaptosuccinic acid (DMSA) scintigraphy during the infection. After 2 years, DMSA scintigraphy was repeated in 58 patients. Re-infections were recorded.ResultsOveractive detrusor was found in 27 (42%) patients. There was no significant difference in the incidence of overactive detrusor between boys and girls. The maximal voiding pressure was higher in boys (median 92.5, range 48–191 cmH₂O) than in girls (median 82, range 37–150 cmH₂O) (P = 0.0117). Thirty-one (48%) patients had renal defects in scintigraphy during the infection. Ultimately, 12 patients (21%) developed renal scars; 11 patients (17%) had VUR. Renal defects in DMSA scintigraphy and the presence of VUR were not associated with overactive detrusor or high voiding pressures.ConclusionOveractive detrusor is a common finding after first episode of pyelonephritis. The dysfunction may explain the development of urinary tract infections in some children. There were no differences in the incidence of overactive detrusor or voiding pressures in patients with and without VUR, or in those with and without renal defects on DMSA scintigraphy. Urodynamic study is not a primary investigation in pyelonephritic children.     

Transvesicoscopic cross-trigonal ureteroneocystostomy in children: a single-center experience

PurposeTo evaluate the safety and efficacy of transvesicoscopic ureteric reimplantation in children.Patients and methodsSeventeen ureteric units in 11 patients underwent a transvesicoscopic ‘Cohen’ ureteroneocystostomy in 2003–2007 and the results were retrospectively analyzed. There were four boys and seven girls. All patients had vesicoureteric reflux (VUR), except for one with paraostial diverticula. Six patients underwent bilateral and five unilateral transvesicoscopic reimplantation (a total of 17 units).ResultsThe procedure was successfully completed in all patients. Mean operation time was 217 min in unilateral cases and 306 min in bilateral cases without perioperative complications, except for pneumoperitoneum development in two cases. In the early postoperative period, two patients developed macroscopic hematuria. Mean hospital stay was 3.8 days (3–5 days), except for one patient who suffered from urinary tract infection and needed longer hospitalization. Mean follow-up period was 4.5 years (3–7 years). One patient with bilateral VUR had passive unilateral grade I VUR on postoperative cystogram, giving a success rate of 91% (94% of ureters). This patient was followed conservatively. One patient had recurrent urinary tract infections without reflux.ConclusionTransvesicoscopic cross-trigonal ureteroneocystostomy can be safely performed with a high success rate in children.     

Transvesicoscopic Cohen ureteric reimplantation for vesicoureteral reflux in children: A single-centre 5-year experience

PurposeTo evaluate our results with a new method of intravesical ureteric reimplantation using laparoscopic pneumovesicum in children.Materials and methodsSeventy-two patients (mean age 4.2 years, range 0.5–20 years) with primary vesicoureteral reflux (VUR) underwent a laparoscopic transtrigonal ureteric reimplantation with CO₂ pneumovesicum. Ports were inserted suprapubically – 5 mm for the camera and two 3–5-mm working ports. Having mobilized the ureter(s) intravesically, a submucosal tunnel is created and ureteric reimplantation performed with 5/0 and 6/0 absorbable sutures. Bladder drainage was maintained for 2–3 days postoperatively. Patients were followed up with clinical assessment and renal ultrasonography ± voiding cystourethrogram.ResultsNinety percent had VUR grade ≥3. A total of 113 ureters were reimplanted. The mean operative time was 82 min for unilateral and 130 min for bilateral reimplantation. Four cases (6%) were converted. Three patients presented with temporary ureteric dilatation without symptoms on follow-up renal ultrasound. Seven patients had postoperative urinary tract infection without persistent reflux on cystography. Follow-up cystogram was performed in 50 patients (81 ureters). Reflux persisted in four patients (8%).ConclusionsLaparoscopic ureteric reimplantation with CO₂ pneumovesicum is technically feasible with a high success rate (92%). The role of this new technique in the treatment of VUR remains to be determined.     

Long-term upper and lower urinary tract functions in children with posterior urethral valves

ObjectiveSeveral factors have been identified as predictive of future renal function in children with posterior urethral valves (PUV). Our aim was to analyse upper and lower urinary tract outcome in patients with PUV, and determine any factors from the period of early management that were predictive of future renal function.MethodsThe charts of 67 boys (mean age 2.4 years) diagnosed with PUV were reviewed. The most common presenting symptom was dribbling in 43.2% and UTI in 28.3%. Twenty-three (34.8%) patients developed end-stage renal disease aged 1–15 years. The mean time of renal survival was calculated as 7.8 (SEM = 0.73) years.ResultsIncontinence in patients over 5 years old, lower urinary tract dysfunction, serum creatinine level in first year or at the time of diagnosis, and presence of vesicoureteral reflux and high-grade bilateral reflux were significant risk factors for occurrence of renal failure in the future. Lower tract dysfunction was seen in 58.6% of patients. Postnatal diagnosis and presence of high-grade reflux were significant risk factors for the future occurrence of lower urinary tract dysfunction.ConclusionIt is important to recognize that PUV have consequences not only during childhood or before treatment, but also during or after the treatment period. Long-term assessment and follow-up of upper and lower urinary tract functions after valve ablation is necessary.     

Urodynamic changes in patients with anterior urethral valves: before and after endoscopic valve ablation

PurposeTo retrospectively review a series of children with anterior urethral valves (AUV), with emphasis on patterns of urodynamic change and long-term outcome of endoscopic treatment.Patients and methodsWe reviewed the medical records of eight patients who had undergone thorough radiological and urodynamic exams before and after treatment. The diagnosis of AUV was based on radiological imaging and confirmed by urethrocystoscopy. The valves were ablated through either transurethral fulguration or resection. The upper urinary tracts were studied by renal scan and ultrasonography before and after the procedure. Bladder function was assessed urodynamically 3 months after surgery. Uroflowmetry was performed as soon as the children were toilet trained.ResultsEndoscopic ablation of AUV was successful in all cases and no surgical complications occurred. The initial symptoms resolved in all boys. VUR disappeared in two out of three patients, and five children had bladder trabeculation that was resolved after surgery. The final outcome was successful in seven patients (88%). The major urodynamic dysfunction was bladder hypercontractility that resolved following valve ablation. The mean maximum voiding detrusor pressure (P_detmax) decreased from 213.2 ± 17.9 cmH₂O to 80.7 ± 9.9 cmH₂O, 6 months after treatment (P < 0.001). None of the patients had low-compliant bladder, detrusor instability or myogenic failure. The voiding pattern in all toilet-trained patients was staccato and of an interrupted shape prior to surgery, but changed to a normal bell-shaped voiding pattern following valve ablation.ConclusionAUV should be considered in the differential diagnosis of patients presenting with infravesical obstruction. We recommend endoscopic valve ablation as the treatment of choice.     

Button vesicostomy: a continent urinary stoma

ObjectiveA modified technique of vesicostomy is described using a gastrostomy button, which could be used as a continent urinary stoma in children with incomplete voiding.Patients and methodsFrom 1998 to 2005, 21 children aged between 4 days and 16 years underwent insertion of button vesicostomy to permit bladder drainage. They had incomplete bladder emptying and clean intermittent urethral catheterization (CIC) could not be established. In six of 23 procedures, the button was placed through a classical vesicostomy (3) or via a suprapubic catheter tract (3). In 17, a standardized technique of button vesicostomy stoma formation was used. The median follow up was 2.5 years (0.75–8 years).ResultsPatients were selected on the basis of clinical need. Idiopathic hypotonic bladder was the most common indication (9), followed by anorectal malformation (5), neuropathic bladder and posterior urethral valves (2 each), traumatic rupture of urethra (1), visceral myopathy (1) and posterior urethritis (1). Granuloma formation around vesicostomy button was observed in five patients. Local infection was observed in three patients and urinary tract infection in four. No peri-button leakage occurred in the standardized button stomas but was seen in all three of the buttons placed in classical vesicostomies, and transient leakage occurred in one of the three patients with a button placed via a suprapubic catheter tract. The median duration of use of vesicostomy button was 11 months (2–30 months). In eight patients, bladder function improved and intermittent drainage was no longer required. Three patients are still using the button, four progressed to Mitrofanoff, four started CIC per urethra, and two reverted to continuous drainage.ConclusionsButton vesicostomy is a useful addition to the options available for a catheterizable continent urinary stoma in children in the short or medium term. The risk of major complications was low although minor complications were common, and the technique was well accepted by patients and parents.     

The role of laparoscopic surgery for urinary tract reconstruction in infants weighing less than 10 kg: a comparison with open surgery

PurposeTo compare the outcome of laparoscopic urinary tract reconstruction (LUTR) in children weighing 10 kg or less with a weight-matched cohort undergoing open urinary tract reconstruction surgery (OUTR).Materials and methodsWe conducted a retrospective chart review of patients weighing 10 kg or less at the time of surgery who underwent open or laparoscopic pyeloplasty, transuretero-ureterostomy and ipsilateral uretero-ureterostomy between January 2000 and May 2007. The following information was recorded: body weight, age, sex, diagnosis, type of procedure, operative time, estimated blood loss, pre- and postoperative hemoglobin levels, length of hospitalization, length of follow up, use of drains and stents, intraoperative and postoperative analgesic requirement, need for readmission, subsequent procedures, costs, complications and reoperations.ResultsThere were 52 patients divided into two groups: LUTR (n = 23) and OUTR (n = 29). Median weight (range) and follow up was 6.2 kg (3.9–10) and 9 months and 6.5 kg (4.7–9.6) and 31 months for LUTR and OUTR, respectively. Mean operative time for LUTR (including cystoscopy and stent placement) was 237 min and for OUTR 128 min (P < 0.01). There were no differences in blood loss, intra- or postoperative analgesic requirement, results or complications. Mean hospitalization time was shorter for LUTR than OUTR (2 and 3 days, respectively). There were no differences in hospital costs between the two groups.ConclusionsLaparoscopic reconstructive surgery for congenital urological anomalies is safe and effective in small infants and can be performed with outcomes comparable to that of open surgery.     

The modified Barry technique to prevent vesicoureteric reflux in paediatric renal transplant recipients: initial recipient outcomes

ObjectiveWe present the initial clinical results of the ‘modified Barry technique’ for the prevention of VUR in paediatric renal transplant grafts. Ours is the only centre in the UK using this technique, as confirmed in a questionnaire developed in our department.Patients and methodsWe retrospectively analysed data of 15 paediatric renal transplant patients (operated June 2006–November 2009) who had their vesicoureteric anastomosis performed using the modified Barry technique with a 2-cm submucosal anti-reflux tunnel. The original Barry technique involved the creation of a 4-cm tunnel; this was modified by us to reduce the risk of ureteric stenosis.ResultsAt a median follow up of 23.7 months (6.3–39.4), the incidence of VUR was 7% (1/15). There was no evidence of postoperative urological complications, such as urinary leak, primary ureteric obstruction including anastomotic stricture/stenosis, transplant graft renal calculi and chronic rejection. At current follow up, graft and patient survival are 100%.ConclusionWith the introduction of the modified Barry technique, the incidence of VUR in our series fell 10-fold to 7%, compared with our earlier study (P < 0.0001), without any urological complications. Although the initial results are encouraging, larger patient numbers and longer follow up are required to validate this technique further.     

Leech in urinary bladder causing hematuria

ObjectiveTo estimate efficacy of normal saline in the management of hematuria caused by accidental entry of a leech per urethra into the urinary bladder.MethodsAn intervention study was carried out in the Department of Pediatric Surgery of Sylhet MAG Osmani Medical College between January 1998 and December 2003. A total of 43 boys (mean age 8 years, SD ± 2.6) were enrolled. In all cases, a leech had entered the urinary bladder through the urethra causing hematuria. All patients were equipped with a self-retaining Foley catheter. They were managed by infusing 50 ml of normal saline into the urinary bladder through the catheter that was then clamped for 3 h.ResultsAfter removing the catheter, in all cases the whole leech was spontaneously expelled intact, dead or alive, within 2–24 h during the subsequent act of micturition. Hematuria gradually diminished to a clear flow within the next 6 h in 27 cases, 12 h in 14 cases and 24 h in two cases. All patients were followed up for 2 weeks, and none developed recurrent hematuria.ConclusionCatheterization and irrigation of the urinary bladder with normal saline is a relatively simple, safe and inexpensive method of removing the leech and controlling hematuria.     

Diagnostic accuracy of color Doppler sonographic study of the ureteric jets in evaluation of hydronephrosis

ObjectiveHydronephrosis (HN) and obstruction are closely associated, but upper urinary tract dilatation can occur without significant obstruction. Despite some pitfalls, conventional ultrasonography and diuretic renography (DR) are the main means of evaluation of HN in children. Recent reports have demonstrated color Doppler ultrasonography (CDUS) to reliably identify ureteric jets in the bladder. The aim of this study was to evaluate this method as a diagnostic tool to distinguish obstructive from non-obstructive dilatations of the upper tract.MethodsWe evaluated 51 patients (37 boys and 14 girls), aged 3 months to 14 years (median 4 years), who presented with unilateral grade III and IV hydronephrosis with suspicion of pyeloureteral junction obstruction. All patients underwent DR and evaluation of ureteric jets by transverse CDSG of the bladder within a maximum of 2 weeks. Obstruction was considered in the DR when the hydronephrotic unit showed a differential renal function of less than 40%, or when symptomatic intermittent renal colic was present in older children. The number of ureteric jets was counted over a 5-min period and the frequency calculated for each ureteral orifice. Relative jet frequency (RJF) was defined as frequency of the hydronephrotic side divided by total ureteric jet frequency. Receiver-operating characteristic (ROC) plots were constructed to determine the best cut-off for RJF, in order to identify renal units with obstructive hydronephrosis.ResultsTwenty-three (45.1%) hydronephrotic units were considered obstructed. The mean RJF differed between obstructive (0.09 ± 0.15) and non-obstructive hydronephrosis (0.42 ± 0.11) (p < 0.001). ROC analysis revealed that RJF <0.25 was the best threshold, and correctly discriminated obstruction in 91.2% of the children with a sensitivity of 87% (95% CI 78.6–98.2%) and specificity of 96.4% (95% CI 87.8–99%). The positive likelihood ratio was 24.3 and the area under the ROC curve was 0.92 (95% CI 0.86–0.98).ConclusionsRJF <25% was found to be a good indicator of obstruction in children with unilateral hydronephrosis. CDUS evaluation of ureteric jets is an easy and non-invasive method that can be used as an initial diagnostic tool, and in follow-up cases, to differentiate obstructed from non-obstructed hydronephrosis in the pediatric population.     

Could prophylactic antibiotics be stopped in patients with history of vesicoureteral reflux?

ObjectiveTo compare the incidence and type of urinary tract infection (UTI) in patients with primary vesicoureteral reflux (VUR) diagnosed after a febrile UTI while they were on prophylactic antibiotics (PA) and after stopping PA.Materials and methodsCriteria to discontinue PA were: no UTI during 12+ or more months on PA, old enough to communicate UTI symptoms, potty trained and absence of risk factors for UTI. Patients with at least 1 year of follow up without PA were included (n = 77). We recorded: age at which PA was indicated and stopped, time on and off PA, incidence and type of UTI (cystitis vs acute pyelonephritis (APN)), and renal scan results.ResultsPA was started and stopped at a mean age of 18.5 and 61 months, respectively. Mean time on PA was 39 months (range 12–95): 25 patients had 44 UTI episodes (0.17 episodes/patient/year), and 31 (70%) of them were APN. Mean time of antibiotics was 44.5 months (range 12–162): 13 patients had 24 UTI episodes (0.08 episodes/patient/year), eight (33%) of which were APN (P < 0.05). A renal scan was performed in 71 patients after the index infection and repeated in 12. Two patients lost renal function while still on PA.ConclusionDiscontinuing PA in patients with history of VUR is a safe practice and should be considered as a management option.     

Urethral length in girls with lower urinary tract symptoms and forme fruste of female epispadias

PurposeTo investigate systematically the length of the urethra in girls with lower urinary tract symptoms.Materials and methodsIn a group of 121 consecutive girls presented at a tertiary referral clinic for urinary incontinence or recurrent urinary tract infections, urethral length was measured by perineal ultrasound. The urethra was measured with the patient in supine position without anesthesia. Mean age of the patients was 7.8 (0–15) years.ResultsAverage urethral length was 26 mm. Minimum length was 12 mm, measured in a 5-year-old girl with dribbling incontinence. Maximum measured length was 40 mm in a 15-year-old girl. In four girls (3.3%), aged 1–10 years (mean 6.3), a short urethra was detected, with measured lengths of 12 and 14 mm. All four had normal genitalia, and were referred with therapy-resistant urinary incontinence or urinary tract infections. A gradual increase in average urethral length was measured from 23 mm at birth to 32 mm at 15 years.ConclusionUrethral length can be measured accurately by ultrasound. Although a short urethral length is rarely detected by ultrasound in girls with incontinence, it may be associated with therapy-resistant incontinence. In such cases, different treatment options are available.     

Risk factors for progression to end-stage renal disease in children with posterior urethral valves

ObjectiveTo identify the variables which affect long-term renal outcome in children with posterior urethral valves (PUV).Materials and methodsRetrospective analysis of 260 children with PUV who underwent ablation of valves in 1992–2008 at our tertiary care center. The following risk factors for progression to end-stage renal disease (ESRD) were analyzed: nadir serum creatinine greater than 1.0 mg/dl, bilateral grade 3 or higher VUR at diagnosis, recurrent febrile UTIs, and severe bladder dysfunction. Patients were divided into two groups: those who developed ESRD (group 1) and those who did not (group 2).ResultsForty (17.62%) patients had nadir serum creatinine >1 mg/dl. At time of initial presentation, high-grade VUR was seen in 63.1% and 33.5% of groups 1 and 2, respectively (P = 0.002). Overall, 77 (34%) of the boys developed breakthrough urinary tract infections: 37.03% and 33.5% in groups 1 and 2, respectively (P = 1). Fifty-nine (26%) patients were found to have severe bladder dysfunction: 77.8% and 19% in groups 1 and 2, respectively (P < 0.0001). Twenty-seven (11.89%) patients progressed to ESRD, at mean age of 11.21 years (5–16). On univariate analysis, the risk-predicting variables were: nadir serum creatinine value greater than 1 mg/dl (P < 0.0001), bilateral high-grade VUR (P = 0.002) and severe bladder dysfunction (P < 0.0001). On multivariate logistic regression analysis, nadir serum creatinine greater than 1 mg/dl (OR 23.79; CI 8.20–69.05) and severe bladder dysfunction (OR 5.67; CI 1.90–16.93) were found to be independent risk factors predictive of ultimate progression to ESRD.ConclusionsNadir serum creatinine and bladder dysfunction are the main factors affecting long-term renal outcome in cases of PUV. Early identification and treatment of bladder dysfunction may thus be beneficial.     

Long-term follow up (37–69 years) of patients with bladder exstrophy treated with ureterosigmoidostomy: Uro-nephrological outcome

ObjectiveTo describe the urological and nephrological long-term outcome of patients born with classical bladder exstrophy treated with bilateral ureterosigmoidostomies in early childhood.Patients and methodOut of 42 patients born with bladder exstrophy in Switzerland between 1937 and 1968, 25 participated in this study; seven had died, seven were lost to follow up and three refused consent. Assessment included chart review, clinical examination, and assessment of renal function and morphology.ResultsAfter a follow-up period of 37–69 years ((mean 50 years), 13 of the 25 participants (52%) had their ureterosigmoidostomy still in place. All others had different forms of urinary diversions. Fifteen (60%) patients had normal renal function or mild chronic kidney disease as assessed by estimated glomerular filtration rate. Three patients were on renal replacement therapy. MRI (n = 16) showed 10 morphologically normal kidneys. One patient suffered from adenocarcinoma of the colon, five had benign colonic polyps, one urethral papillary carcinoma and 18 no evidence of tumor.ConclusionThe majority of our patients have normal or mildly impaired renal function and a well functioning ureterosigmoidostomy. This is remarkable, given the fact that ureterosigmoidostomies are considered to be refluxing high-pressure reservoirs at risk of renal injury and malignancy.     

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

PurposeTo 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 methodsForty 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).ResultsMean 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.ConclusionThe presence of D allele is associated with progression of renal scarring and reduced GFR in PUV patients.     

Single-stage repair of bladder exstrophy in older children and children with failed previous repair

AimEvaluation of cosmetic and functional outcome of single-stage exstrophy–epispadias complex repair in older children and those with previously failed repair.Materials and methodsThis study comprised 15 children (12 boys and 3 girls) with classic bladder exstrophy and a mean age at repair of 8.6 months (range 2–24 months). Eight children had a previously failed repair. All children underwent complete primary repair using the single-stage Mitchell technique. Half of the boys had complete penile disassembly, while in the others a modified Cantwell–Ransley technique for epispadias repair was used. Anterior iliac osteotomy was performed and hip spica used for immobilization in all children.ResultsOne child had urethral stricture treated by endoscopic visual urethrotomy. Three children had penopubic fistulae that closed spontaneously. No bladder dehiscence or prolapse was encountered. Vesicoureteral reflux was present in 20 renal units but ureteral reimplantation was not performed. Average bladder capacity after closure was 134 cm³ (range 110–160 cm³) with only two partially continent and six incontinent children. Mean follow-up period is 2 years (range 1–3 years).ConclusionsSingle-stage repair was performed in children with previously failed repair and those presenting at an older age with satisfactory results. Acceptable bladder and genital anatomy and function were achieved together with preservation of renal function. The impact of this technique on continence is not encouraging, but needs to be determined in a longer follow-up period.