Long-term results of continent catheterizable urinary channels in adults with non-neurogenic or neurogenic lower urinary tract dysfunction.

Objectives: To evaluate the long-term results after the construction of a Continent Catheterizable Urinary Conduit (CCUC) in adults. Methods: This study retrospectively reviewed the charts of 41 adults from two tertiary centers who received a CCUC. The demographics, underlying diseases, indications for a CCUC and outcomes such as the reoperation rate and the occurrence of complications were extracted. The patient reported outcome was measured with the Patient Global Impression of Improvement (PGI-I) scale and four additional questions about continence, leakage and stomal problems. Results: Twenty-nine patients were women. The median age at surgery was 32 years, with a median follow-up of 52 months. Twenty-six patients had a neurogenic bladder. The reoperation rate was 48.8%, with a median of 10.5 months after constructing the CCUC. Superficial stomal stenosis was the most common registered complication (20 times) and stoma revision was the most often performed reoperation (12 times). Twenty-four patients completed the PGI-I; the mean improvement rating was 2 (=much better). Conclusion: The construction of a CCUC in adults is associated with a high complication and reoperation rate. The high reoperation rate is in accordance with the sparse literature. Despite this, patients reported 'much better' on the PGI-I.

Urotherapy in children with dysfunctional voiding and the responsiveness of two condition-specific questionnaires.

AIMS: We sought to establish the responsiveness of the Dutch Vancouver Symptom Score for Dysfunctional Elimination Syndrome (VSSDES) and Pediatric urinary incontinence Quality of life (PinQ) questionnaires. Secondary, we evaluated the outcome of urotherapy extended for children with dysfunctional voiding (DV). METHODS: This cross-sectional multicenter study was done in one tertiary and two community hospitals. Children with DV were included, also when refractory to previous urotherapeutic treatment. The questionnaires were completed before and after urotherapy. The primary outcome measure was the responsiveness of the Dutch VSDESS and PinQ. Secondary outcome was the initial success (defined by the International Children's Continence Society) of extended urotherapy. RESULTS: Between June 2014 and May 2016, 64 children (median age 7 years, IQR 6-10) received urotherapy (median 18 weeks, IQR 11-28). In contrast to the VSSDES, the PinQ showed good responsiveness. For children and parents, respectively, the area under the ROC-curve was 0.79 (P = 0.01) and 0.72 (P = 0.03) for the PinQ and 0.50 (P = 0.98) and 0.55 (P = 0.62) for the VSSDES. Fifty children received extended urotherapy, 27 had complete, and 14 had partial response. Sixteen children had been refractory to previous treatment; four showed complete, and six showed partial response. CONCLUSION: The PinQ is able to detect clinically important changes in continence-specific quality of life after treatment. We support the use of the VSSDES questionnaire in addition to the current diagnostics for the diagnosis of DV. Extended urotherapy showed to be a successful treatment for children with DV, also for those who had received previous unsuccessful treatment.

Long-term effect of conservative treatment versus low threshold endoscopic desobstruction on urine incontinence and urgency in boys with persistent overactive bladder symptoms: A cohort study.

AIMS: To assess the long-term effects of two treatment strategies (low threshold endoscopic desobstruction vs. conservative treatment) on urinary incontinence (UI) and urgency-frequency in boys. METHODS: Boys with persistent overactive bladder symptoms treated in two tertiary referral centers between 2006 and 2009 were included. Treatment strategy in center 1 was urethrocystoscopy (UCS) and in case of obstruction urethral desobstruction and in center 2 conservative. The primary outcome was time to being dry during daytime, secondary outcomes were being dry both day and night and presence of urgency-frequency, using the "provisional" International Consultation on Incontinence Questionnaires Children's Lower Urinary Tract Symptoms (LUTS) questionnaire. RESULTS: Median age at start of treatment was 8.0 (IQR 6.4-9.4) years in center 1 and 8.4 (IQR 6.0-10.1) years in center 2. At baseline daytime incontinence was present in 100/104 children (96%, center 1) and 37/44 (84%, center 2). In center 1, UCS was performed in 98 (93%) boys, with desobstruction in 93 (88%), while in center 2 these numbers were 16 (36%), and 5 (11%). There were no differences between groups after a mean follow-up of 5 years concerning dryness at daytime (HR 0.86, 0.56-1.30), dryness day and night (HR 0.72, 0.51-1.14), and presence of urgency-frequency (HR 0.67, 0.38-1.25). CONCLUSIONS: The benefit of a strategy including low-threshold UCS and endoscopic desobstruction in boys with urge incontinence and suspected infravesical obstruction to prevent LUTS and incontinence on the longer term could not be confirmed.

20 years experience with appendicovesicostomy in paediatric patients: Complications and their re-interventions.

AIMS: To evaluate the long-term outcome of appendicovesicostomies and to present the frequency and timing of complications needing re-intervention. METHODS: In this retrospective study we included patients in whom an appendicovesicostomy was created at our institution between 1993 and 2011. Patients with a follow-up less than 1 year were excluded. Patient characteristics and conduit-related complications requiring re-intervention were collected. RESULTS: One hundred and twenty-eight patients were included with mean age at initial surgery of 10.1 ± 3.9 years. Two thirds of the children had underlying neurogenic disease. The mean follow-up was 10.1 ± 4.8 years. All but one patient continued to use the catheterizable channel. Re-intervention for conduit-related complications was necessary in 32.0% of the patients. A second, third, and fourth re-intervention was required in respectively 10.9%, 2.3%, and 1.6%. The commonest complications were cutaneous/fascial stenosis in 14.8%, stenosis at conduit-bladder level in 9.4%, and stomal incontinence in 6.3% of the patients. The most performed re-interventions were stoma revision (in 16.4% of the patients), conduit revision (10.2%), and dilatation of a stenotic tract (4.7%). 63.3% of the re-interventions was superficial and/or endoscopic. The peak incidence of re-interventions was in the 1st year after conduit construction and decreased yearly. CONCLUSIONS: Our study gives an overview of patients and their conduits developing from prepubertal children to young adults. During a mean follow-up of 10.1 years, roughly one third of the patients needed a re-intervention. We conclude that an appendicovesicostomy is an effective and durable treatment for whom transurethral clean intermittent catheterization is not feasible. Neurourol. Urodynam. 36:1325-1329, 2017. © 2016 Wiley Periodicals, Inc.

Vancouver Symptom Score for Dysfunctional Elimination Syndrome: Reliability and Validity of the Dutch Version.

PURPOSE: We sought to establish the reliability and validity of the Dutch version of the Vancouver Symptom Score for Dysfunctional Elimination Syndrome for children with dysfunctional voiding and their parents. MATERIALS AND METHODS: For this cross-sectional multicenter study the Vancouver Symptom Score for Dysfunctional Elimination Syndrome was translated and cross-culturally adapted to Dutch following a standardized process. Patients 16 years or younger with dysfunctional voiding and their parents were recruited at pediatric, pediatric urology and pelvic floor physical therapy outpatient clinics. The reference group consisted of children 6 to 16 years old without dysfunctional voiding and their parents. All groups completed questionnaires. The evaluated measurement properties included discriminative ability, internal consistency, test-retest reliability, interrater agreement, criterion validity using the Pediatric Incontinence Questionnaire and construct validity. A cutoff value for diagnosis of dysfunctional voiding was determined. RESULTS: A total of 50 patients and 60 references and their parents were included in the study. The Vancouver Symptom Score for Dysfunctional Elimination Syndrome showed good discriminative ability. A moderate internal consistency was found (Cronbach alpha 0.37-0.55). Test-retest reliability was moderate to good, and interrater agreement demonstrated good correlation between children and parents (ICC 0.85, 95% CI 0.79-0.89). A weak correlation with the Pediatric Incontinence Questionnaire was found in patients and construct validity was confirmed. Cutoff scores for dysfunctional voiding were 11 and 9 for patients and parents, respectively. CONCLUSIONS: The Dutch Vancouver Symptom Score for Dysfunctional Elimination Syndrome displayed moderate to good reliability and validity properties for the patient and parent versions. Use of this instrument in clinical practice will support the assessment of dysfunctional voiding and facilitate international reporting of research results.

Neurogenic lower urinary tract dysfunction in the early disease phase of paediatric multiple sclerosis.

Neurogenic lower urinary tract dysfunction (LUTD) in multiple sclerosis (MS) is highly prevalent in adults, but has not previously been described in paediatric MS. A total of 24 consecutive children with newly diagnosed MS were prospectively assessed for bladder and bowel problems early after diagnosis. Five of 24 children (21%) showed LUTD during assessment. One of these patients did not report voiding complaints. This high prevalence of LUTD indicates that all recently diagnosed patients with paediatric MS should be evaluated early in their disease and treated for urinary problems in order to prevent potential damage to the upper urinary tract.

Higher Incidence of Hypospadias in Monochorionic Twins.

BACKGROUND: Hypospadias is associated with twinning. The incidence of hypospadias in monochorionic and dichorionic male twins is, however, yet to be determined. METHODS: All medical records of monochorionic and dichorionic twins admitted to our neonatal nursery between January 2004 and August 2013 were reviewed for the presence of hypospadias. RESULTS: A total of 350 monochorionic and 303 dichorionic male twins were included in the study. The incidence of hypospadias in monochorionic and dichorionic groups was 4% (14/350) and 1% (3/303) (p = .016) respectively. In 11 of the 15 twin couples, hypospadias occurred in the twin with the lowest birth weight. The rate of hypospadias in twin infants small-for-gestational-age group was 10% (6/60) compared with 2% (11/593) in the appropriate-for-gestational-age group (p = .002). In a multivariate analysis, both monochorionicity and small-for-gestational-age were independently associated with hypospadias, odds ratio 4.1 (95% confidence interval (CI): 1.1-14.7) and 6.1 (95% CI: 2.2-17.2) respectively. CONCLUSIONS: The incidence of hypospadias is four-fold higher in monochorionic twins compared with dichorionic twins. Hypospadias is also independently associated with small-for-gestational-age.

Effects of reconstructive surgery on bladder function in patients with anorectal malformations.

PURPOSE: Bladder dysfunction is common in patients with anorectal malformations and can be congenital or acquired as a consequence of surgery. We investigated the effects of surgical correction of anorectal malformations on bladder function. MATERIALS AND METHODS: The charts of all 341 patients who underwent surgery at our center between 1990 and 2010 were retrospectively analyzed for preoperative and postoperative videourodynamics. A total of 52 patients were eligible for study inclusion. Each assessment was scored according to International Children's Continence Society standards. RESULTS: Urodynamic study indicated normal bladder function preoperatively in 36 patients (69%) and postoperatively in 37 (71%). Median bladder emptying efficiency and relative bladder capacity changed significantly after posterior sagittal anorectoplasty. Bladder function according to International Children's Continence Society standards did not change postoperatively in 43 patients (83%). In 1 of 4 patients with deterioration of bladder function the deterioration could be attributed solely to surgery. Clinical outcome was available in 38 patients and showed complete urinary continence with spontaneous voiding in 24 (63%). Seven of 25 patients (28%) with preoperative videourodynamics indicating normal bladder function demonstrated dysfunctional voiding at latest followup. CONCLUSIONS: Urodynamic and clinical outcomes after anorectal malformation repair are good, with 63% of patients being continent of urine. Urodynamic studies are of limited value in preoperative settings in these patients. Current techniques of reconstructive surgery for anorectal malformations seem to preserve bladder function in the majority of patients.

Laparoscopic transperitoneal pyeloplasty in children from age of 3 years: our clinical outcomes compared with open surgery.

OBJECTIVE: To report clinical outcomes for laparoscopic pyeloplasty (LP) in children compared with open pyeloplasty (OP) and literature findings. PATIENTS AND METHODS: In a prospective study, the outcomes of 57 consecutive transperitoneal LP in children from the age of 3 years were analyzed and compared with a matched historic control group of OP and with series of LP in the literature. Successful result was defined as resolution of symptoms, no conversion or re-operation, improved hydronephrosis, and/or improved renographic drainage. RESULTS: Mean operative time was 177 (SD 50.5)min in the LP group and 108 (SD 25.6)min in the OP group (p < 0.001). Mean hospital stay was 1.2 (SD 0.46) days in the LP and 6.7 (SD 1.2) days in the OP group. Improvement in renographic drainage was observed more often after LP than after OP (98% vs 83%; p = 0.010). A successful result was reported in 56 (98%) LP and 54 (95%) OP (p = 0.298) patients. Our LP series demonstrates a high success rate compared to literature data. CONCLUSIONS: Our LP has a similar success rate and more often improved renographic drainage in comparison to OP. Furthermore, our LP demonstrates a shorter hospital stay and favorable outcomes compared to the literature. We thus regard LP as standard treatment for repair of ureteropelvic junction obstruction in children from the age of 3 years.

Genes in the ureteric budding pathway: association study on vesico-ureteral reflux patients.

Vesico-ureteral reflux (VUR) is the retrograde passage of urine from the bladder to the urinary tract and causes 8.5% of end-stage renal disease in children. It is a complex genetic developmental disorder, in which ectopic embryonal ureteric budding is implicated in the pathogenesis. VUR is part of the spectrum of Congenital Anomalies of the Kidney and Urinary Tract (CAKUT). We performed an extensive association study for primary VUR using a two-stage, case-control design, investigating 44 candidate genes in the ureteric budding pathway in 409 Dutch VUR patients. The 44 genes were selected from the literature and a set of 567 single nucleotide polymorphisms (SNPs) capturing their genetic variation was genotyped in 207 cases and 554 controls. The 14 SNPs with p<0.005 were included in a follow-up study in 202 cases and 892 controls. Of the total cohort, ~50% showed a clear-cut primary VUR phenotype and ~25% had both a duplex collecting system and VUR. We also looked for association in these two extreme phenotype groups. None of the SNPs reached a significant p-value. Common genetic variants in four genes (GREM1, EYA1, ROBO2 and UPK3A) show a trend towards association with the development of primary VUR (GREM1, EYA1, ROBO2) or duplex collecting system (EYA1 and UPK3A). SNPs in three genes (TGFB1, GNB3 and VEGFA) have been shown to be associated with VUR in other populations. Only the result of rs1800469 in TGFB1 hinted at association in our study. This is the first extensive study of common variants in the genes of the ureteric budding pathway and the genetic susceptibility to primary VUR.

Cystic nephroma, cystic partially differentiated nephroblastoma and cystic Wilms' tumor in children: a spectrum with therapeutic dilemmas.

BACKGROUND: Cystic renal tumors are a diagnostic and therapeutic challenge. Cystic nephroma (CN), cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms' tumor (CWT) are a spectrum with CN at the benign end, CWT at the malignant end and CPDN in the intermediate position. CN and stage 1 CPDN are often treated with surgery alone. International Society of Pediatric Oncology (SIOP) protocols for Wilms' tumor (WT) advocate preoperative chemotherapy, which may be unnecessary and potentially harmful in CN and in stage 1 CPDN. There are difficulties in differentiating the three types using imaging techniques. Therefore, controversies exist regarding the optimal treatment. METHODS: We describe 6 children, who each had a postoperative diagnosis of CN, CPDN or CWT, and we retrospectively evaluate the treatment strategies. RESULTS: The three types cannot be differentiated using imaging techniques, although the presence of solid components in the tumor is indicative of WT. CONCLUSIONS: Surgery as first-line therapy should be seriously considered in children who have a cystic renal tumor, but further collaborative studies are needed since the distinction line between CPDN and CWT is not always clear.

Prolonged follow-up after paediatric pyeloplasty: are repeat scans necessary?

OBJECTIVE: To establish the value of repeat renal scans to assess the relative renal function after successful paediatric pyeloplasty. PATIENTS AND METHODS: Renal scans were assessed from 138 patients after successful pyeloplasty; the relative renal function before and immediately after pyeloplasty were compared, as was split renal function at 3.5 and 5.5 years after surgery. RESULTS: With a few exceptions the relative renal function before and after pyeloplasty was stable. Repeat renal scans 3.5 and 5.5 years after surgery also showed stable split renal function, even if the renal function was already diminished. CONCLUSIONS: Repeat renal scans are not necessary after successful pyeloplasty in children, as most operated renal units showed stable renal function for 5-7 years. Further studies are needed to investigate whether renal function changes further after puberty.

A standardised mini pig model for in vivo investigations of anticholinergic effects on bladder function and salivation.

PURPOSE: The objective was to validate an in vivo model for evaluation of pharmacological effects on bladder function taking the most predominant anticholinergic side effect (hyposalivation) into account. Therefore, two anticholinergic properties (propiverine hydrochloride and tolterodine-L(+)-tartrate) were used to test the in vivo model. Sacral anterior root stimulation (SARS) was performed to induce reproducible and standardized bladder contractions. To evaluate hyposalivation standardised salivavary flow measurements by stimulating the lingual nerve was performed in addition to SARS. MATERIALS AND METHODS: 10 male mini pigs were anaesthetised. The carotid artery was cannulated for blood pressure measurement and the jugular vein for administration of propiverine 0.4 mg kg(-1) b.w. and tolterodine 0.06 mg kg(-1) b.w. For stimulation-induced salivary flow measurements both lingual nerves were exposed and a cuff electrode was placed around the nerves. The bladder was exposed and a cystostomy catheter was inserted to performed cystometrographic measurements during SARS. RESULTS: In all experiments, for each animal reproducible intravesical pressure values (pves) and salivary flow rates were elicited during electrostimulation before administration of the drug. Bladder pressure: After administration of propiverine, neurostimulation-induced rise in pves had fallen by 60% from the initial value. After administration of tolterodine pves had fallen by about 50%. After additional administration of atropine pves decreased to about 15% of the initial value for both drugs. Salivation: After propiverine salivary flow had fallen by 61%. Inhibition of salivary flow under tolterodine was about 56%. Additional administration of atropine led in both drugs to a nearly complete blockade of salivation. Heart rate (HR) and blood pressure (BP): Directly following intravenous administration of both drugs, a short-term and reversible period of mild but significant fluctuations in HR was observed. There was also a slight but non-significant rise in blood pressure. CONCLUSIONS: This model allows comparative investigations of various drugs with bladder inhibitory properties in terms of acute efficacy and side effects.