The postnatal management of boys in a national cohort of bladder outlet obstruction.

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 < 0.05 was taken as significant. 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 < 0.0001. Polyuria occurred in 23/45(51%), 12/48(25%), 0/28(0%), respectively, p < 0.0001. Initial surgical treatment was TUI in 108/121(89%) and vesicostomy in 2. Two ureterostomies were secondary procedures. Circumcision was performed in 52/121(43%) in antenatal presentation vs. postnatal vs. late 27/45(60%), 20/48(42%), 2/28(7%), respectively, p = 0.01. 69 UTIs occurred in 49 patients. Circumcision was associated with an 86% reduced risk of UTI, p < 0.0001. There was a 66% reduction in UTI risk associated with TUI alone, p < 0.01. There was 1 death due to pulmonary hypoplasia and renal failure, and 2 experienced end-stage renal failure (ESRF). 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.

Current epidemiology and antenatal presentation of posterior urethral valves: Outcome of BAPS CASS National Audit.

AIM: Posterior urethral valves (PUVs) are the most common cause of congenital bladder outlet obstruction (BOO) in boys and end-stage renal failure (ESRF) in childhood. In the 1980s, 1 in 4000 boys had PUV. Presentation was 1/3 antenatal/neonatally, 1/3 postnatal, 1/3 late (>1 year). This study aimed to describe the current proportions in a contemporary cohort. METHODS: A national audit (BAPS CASS) of referrals in the UK and Ireland of boys diagnosed with suspected or confirmed PUV in a year was conducted. National registration data provided the male birth-rate. Data were presented as number (%), analysed by Mann-Whitney U-test and Chi-square test, with P < 0.05 taken as significant. The study was approved by a national ethics committee (NRES Committee South Central Oxford A (12/SC/0416)). RESULTS: Data were collected from 1st October 2014 to 30th September 2015 from 25/26 centres on 121 cases of suspected bladder outlet obstruction (BOO), of which 113 (93%) were because of PUV. The male birth rate during the period was 432,806/year. The calculated incidence of BOO was 1/3580 and for PUV was 1/3800 per-annum. The proportion of PUV presenting according to age was: antenatally (n = 40, 35%), infancy (n = 47, 42%), and late (n = 26, 23%). Plasma creatinine was higher in antenatally-diagnosed BOO vs. postnatal, 54 (39.5-109.5) µmol/l vs. 34(21-47) µmol/l, P = 0.0005. Hydronephrosis and ureteric dilatation were significantly greater in antenatally diagnosed BOO vs. postnatal vs. late. Renal dysplasia (cortical thinning, poor corticomedullary differentiation, or renal cysts) was significantly more likely in antenatally diagnosed BOO. CONCLUSION: Neither the incidence (~1/4000) nor the proportion antenatally diagnosed (~1/3) of boys with PUV appears to have changed in the past 30 years. Those boys who were antenatally diagnosed have significantly higher postnatal plasma creatinine, more hydroureteronephrosis, and renal dysplasia than those diagnosed in infancy or later. It may be hypothesized that this is the reason they are detected antenatally. LEVEL OF EVIDENCE: Prognosis study - Level I - prospective national cohort study.

Bladder dysfunction in Wolfram syndrome is highly prevalent and progresses to megacystis.

AIM: Wolfram syndrome is a rare genetic defect in WFS1 or WSF2(CISD2). It includes diabetes mellitus and insipidis, sensorineural deafness, optic atrophy, but not bladder dysfunction. However, this has appeared a common finding in our national referral clinic, and we sought to quantify this problem. METHODS: Data were collected from a multidisciplinary team managing all Wolfram patients in the UK. The following was analyzed: age, date of non-invasive urodynamics (NIU), symptoms, bladder capacity, voided volume, post-void residual and uroflow pattern. Bladder capacity was given as percentage predicted bladder capacity (PBC). Bladders were divided into normal, overactive (OAB), and underactive (UAB). Symptoms, bladder behavior, and genotyping were correlated. Data were expressed as median (interquartile range). MAIN RESULTS: Forty patients with Wolfram syndrome were identified, and 38 underwent NIU. This showed normal bladder function (n=4), OAB (n=9), UAB (n=25). Symptoms were present in only 11 children. The different patterns of bladder behavior (OAB vs. normal vs. UAB) were significantly associated with different %PBC (36 (29-59)% vs. 105 (93-233)% vs. 100 (77.5-337)%; p<0.001), and percentage emptying (100 (80-100)% vs. 100 (87-100)% vs. 69 (48-93)%; p<0.05). There was no association of genotype, symptoms and bladder behavior. Patients with megacystis were older: [13.4 (9.7-16.1) vs. 15.4 (13.9-18.7) years; p<0.05). CONCLUSION: Bladder dysfunction is very common in Wolfram syndrome (~90%), but most children cope (symptoms ~30%). With time there is a significant progression to megacystis, which may represent an underlying neuropathic myogenic failure and is likely to require intervention in the future. LEVEL OF EVIDENCE: Level II (National cohort study of prognosis).

Prediction of need for intervention in posterior urethral valves: Use of urine osmolality.

AIM: Renal tubular dysfunction (RTD) causing obligate production of hypoosmolar urine in boys with posterior urethral valves (PUVs) has been described. It is not known how clinically significant this is. We hypothesize that a feedback loop is present in many PUV boys who suffer deterioration of their lower urinary tract (LUT). RTD results in hypoosmolar urine, obligate polyuria, and bladder stretch-injury. The increasing back-pressure worsens RTD, thus exacerbating the injury. Coexisting renal dysplasia and acquired renal scarring exacerbate this. We compared the concentrating ability (random clinic urine osmolality) of PUV boys who had no LUT deterioration to those who required intervention, examining the confounding effect of renal impairment with a subgroup analysis comparing those with plasma creatinine ≤80µmol/l. METHODS: A retrospective review of our PUV database was performed. Age, intervention, and highest recorded random clinic urine osmolality (>1year) with concurrent plasma creatinine were recorded (normal urine osmolality 500-850 mOsm/kg). Data are given as median values, analyzed by Mann-Whitney u-test, with P<0.05 deemed significant. MAIN RESULTS: Urine osmolality was available in 77 boys with PUV out of 125 in our series. Of these, 34 required subsequent intervention (e.g., Mitrofanoff procedure, bladder augmentation). Age at testing trended towards being higher in the intervention group [7.9 (4.3-10.9) years vs. nonintervention 6.3 (4-8.4); P=0.06]. Urine osmolality was significantly reduced in the intervention group [411(293-547) vs. 631 (441-805) mOsm/kg; P<0.001]. Subgroup analysis comparing only those with creatinine ≤80µmol/l was respectively 451 (322-567) mOsm/kg (n=22) vs. 645 (469-810) mOsm/kg (n=40), P<0.01. CONCLUSION: This study confirms that hypoosmolar urine is highly associated with progression of LUT dysfunction, requiring intervention. Even boys with normal creatinine values have a greater risk of LUT deterioration if they have a RTD and produce hypoosmolar urine. LEVEL OF EVIDENCE: IV (retrospective service development project).

Extended spectrum beta lactamase (ESBL) producing bacteria urinary tract infections and complex pediatric urology.

AIM OF THE STUDY: Extended spectrum beta lactamase (ESBL) producing bacteria are resistant to most beta-lactam antibiotics including third-generation cephalosporins, quinolones and aminoglycosides. This resistance is plasmid-borne and can spread between species. Management of ESBL is challenging in children with recurrent urinary tract infections (UTIs) and complex urological abnormalities. We aim to quantify the risk in children and specifically in urological patients. METHODS: Retrospective review of a microbiology database (April 2014 to November 2015). This identified urine isolates, pyuria, ESBL growth and patient demographics. Data analysis was by Chi square, Mann-Whitney U-test and ANOVA. A P value of <0.05 was taken as significant. MAIN RESULTS: Analysis of 9418 urine samples showed 2619 with pure isolates, of which 1577 had pyuria (>10×106 WC/L). 136 urine cultures (n=79 patients) grew purely ESBL. Overall, 5.2% of urine isolates were ESBL and 9.5% isolates with pyuria (>100×106 WC/L) had ESBL, whereas only 22/1032 (2.1%) with no pyuria, (P<0.0001). Urology patients had 86/136 (63%) ESBL positive cultures. These represented 86/315 (27%) of all positive cultures for urology patients vs. 50/2267 (2.2%) for all other specialties (P<0.0001). Potential ESBL transmission between organisms occurred in 3 (all on prophylactic antibiotics). Over the study period, there was no significant rise of the monthly incidence between 2014 and 2015 (ANOVA P=0.1). CONCLUSION: This study is the first to document the incidence of ESBL in children (5%), and estimate the frequency of possible plasmid transmission between bacterial species in children. This quantifies the risk of ESBL, especially to urology patients, and mandates better antibiotic stewardship. LEVEL OF EVIDENCE: Level IIc.

Gastrostomy insertion in the 21st century: PEG or laparoscopic? Report from a large single-centre series.

PURPOSE: To determine whether laparoscopic-assisted gastrostomy (LAG) has superseded percutaneous endoscopic gastrostomy (PEG) based on the clinical outcomes. METHODS: A retrospective study was undertaken for the period January 06-December 09. Demographic and clinical outcomes were recorded and the two groups were compared. RESULTS: 164 patients were studied (PEG, n = 107; LAG, n = 57). 93.5 % of PEG patients required two general anaesthetics compared with 8 % of LAG patients. Median time to using the gastrostomy was 24 (range 0-168) h in PEG and 0 (0-96) h in LAG patients (p < 0.001). Major complications occurred in 15/107 (14 %) of PEG and 2/57 (3.5 %) of LAG patients (p = 0.05). Re-operation rate following complications was 18/107 (16.8 %) for PEG and 3/57 (5.2 %) for LAG (p = 0.05). Minor complications arose in 41/107 (38 %) of PEG and 32/57 (56 %) of LAG (p = 0.05). Post-operative hospital stay was 2 (1-40) days for PEG and 2 (0-20) days for LAG (p = 0.01). The day-case rate was 0/107 for PEG and 5/57 (9 %) for LAG. There was no gastrostomy-related mortality in the series. CONCLUSION: LAG requires fewer anaesthetics, is associated with shorter time to feeding, shortened hospital stay and has a reduced risk of major complications. LAG is a very good alternative to the PEG in children.

Congenital bladder diverticulum with benign bladder wall lesion resembling rhabdomyosarcoma.

Congenital bladder diverticula in children are uncommon and rarely present with bladder outlet obstruction. We present a case highlighting an interesting association between a congenital bladder diverticulum and a benign inflammatory bladder wall lesion mimicking a rhabdomyosarcoma. Open surgery was required as different imaging modalities and cystoscopy were insufficient to exclude a malignant process.