Bladder bowel dysfunction scoring system (BBDSS): A novel, illustrated questionnaire for evaluation of voiding dysfunction in children.

INTRODUCTION: We sought to evaluate the reliability and validity of a new, illustrated questionnaire, the bladder bowel dysfunction symptom score (BBDSS) in the assessment of overactive bladder (OAB) and bladder bowel dysfunction (BBD). METHODS: The BBDSS questionnaire consisted of 12 structured questions. This pilot study was designed with two principal groups of questions: one group to assess bladder symptoms and the other to assess bowel dysfunction during the preceding month. Each question had three possible answers, with each answer being assigned a severity score. We prospectively collected previously untreated patients referred to our voiding dysfunction clinic for the first time. A control group of healthy children was recruited to assess the reliability of the BBDSS questionnaire. The provisional diagnosis was collected from patients' charts at the time of presentation. RESULTS: The questionnaire was administered to 92 children (44 in the affected group and 48 in the control group). The age at presentation was similar in both groups (17 months or nine years, nine months). The mean total score for the affected group was 8.7 (3-14) while it was 1.19 (0-5) for the control group (p<0.001). There was a strong correlation, between the total BBDSS score and both groups (r=0.88, p<0.001). Using the ROC curve, the BBDSS was found to be an excellent tool in differentiating normal from affected patients (area under the curve [AUC]=0.98, p<0.001). When the total BBDSS score was ≥6, the positive predictive value was 1, with a negative predictive value of 0.89. The defecation part of the BBDSS was a good tool in differentiating OAB from BBD patients (AUC=0.89, p<0.001). No patient with OAB had a bowel score >3. CONCLUSIONS: The BBDSS is a reliable and valid instrument in the diagnosis of voiding dysfunction. The questionnaire was easily administered by parents or children. Moreover, it can differentiate between OAB and BBD.

Voiding efficiency in children is significantly impaired by the presence of the urethral catheter during pressure-flow studies.

BACKGROUND: Little is known about the effect of catheterization during pressure-flow studies (PFS) on voiding efficiency in children. Our objective was to determine the effect of urethral catheterization on Qmax and flow index (FI) during PFS compared to the free flow of uroflowmetry (UF). METHODS: We retrospectively reviewed 63 consecutive children who underwent UF and PFS at our center on the same day (2019-2022). Voiding data was available for 46 patients. Patients first underwent a UF with full bladder, then PFS after urethral catheter insertion. Patients with urethral pathologies (n = 6), on clean intermittent catheterization (CIC) (n = 2) and with major comorbidities (n = 2) were excluded. Indications for UF/PFS were LUTS, recurrent UTIs, incontinence or neurosurgical pre-operative evaluation. Data was collected from the UF and the PFS and compared using paired t-test. The idealized Qmax and flow index (FI) were calculated for UF and PFS using the formulas described by Franco et al.: Male Qmax = 11.26 + 0.0701(TBC [total bladder capacity]) - 0.0000513(TBC); Female Qmax = 10.723 + 0.073(TBC) - 0.0000423(TBC), FI = Actual Qmax/Expected Qmax (Franco and et al., 2016; Franco et al., 2018; Franco and et al., 2016). RESULTS: Median age was 7 years old (IQR 5-11). Twenty-one (40%) patients were male and 32 (60%) patients were female. Of the 53 patients, 3 boys and 4 girls (n = 7; 13%) were unable to void with the catheter in place during PFS but able to void after its removal. Of the remaining 46 cases, the Qmax during PFS was 5 mL/s slower than the Qmax recorded on the UF without catheter, representing a decrease of 29% (12.3 vs 17.3 mL/s; p < 0.0001). The impact of urethral catheter during PFS was more important in males vs females (Qmax decreased by 7.7 vs 3.3 mL/s, or 45 vs 19%). The mean FI during PFS was 44%, which was a 30% reduction compared to the 74% FI obtained with UF (p < 0.00001). In males, the FI decreased by 37% on PFS, whereas it decreased 26% in females, similar to the Qmax decrease. CONCLUSIONS: Voiding efficiency, as assessed by Qmax and FI, is decreased during PFS compared to uroflow studies. Our data documents for the first time the impact of urethral catheterization on pediatric voiding efficiency. Abnormal flow rates and elevated PVRs should be used to guide patient management only if obtained by uroflow. Prospective validation comparing free flow with PFS studies will help characterize the impact of urethral catheter relative to bladder pathology, age, gender and catheter size used.

How can we measure the renal pelvic anteroposterior diameter in postnatal isolated hydronephrosis?

INTRODUCTION: The anteroposterior diameter (APD) of the renal pelvis is a commonly used ultrasound parameter in the evaluation and management of hydronephrosis. It has been established that an APD value associated with pyeloplasty is around 25 mm. Some believe the APD should be measured at the innermost part of the renal pelvis while others suggest that it should be done at the renal contour. However, there is no consensus on the optimal APD measurement technique including whether it should be measured supine or prone. This study compared six different techniques of APD measurements, in both supine and prone positions, and further evaluated their association with pyeloplasty. METHODS: Data was obtained by retrospectively reviewing patients' charts that had initial high-grade hydronephrosis (HGH) from 2008 to 2014. We recorded the patients' demographics, ultrasound data and management choice. In the mid-renal transverse plane, the APD was measured by 2 blinded investigators, at the intra-renal, renal contour and extra-renal regions of the renal pelvis in supine and prone positions (Figure A). We compared the six APD measurements based on the outcome of management (pyeloplasty vs. conservative management). The ROC curve obtained was then used to assess the ability of various APD measurements in predicting surgical intervention. The cutoff value chosen that predicts pyeloplasty was the lowest diameter with 100% specificity. RESULTS: We included 129 patients (134 renal units). Forty-four renal units (42 patients) underwent pyeloplasty whereas 90 renal units (87 patients) were managed conservatively. Patients' demographics were grouped by both SFU grade and clinical outcome. Regardless of grade, the APD measurements were different in all 6 techniques. All APD measurement techniques showed good inter-rater reliability. Based on the ROC curve, all APD measurements were associated with pyeloplasty with an AUC from 0.89 to 0.91. The supine extra-renal APD measurement of 24 mm was the most sensitive cutoff value. The cutoff values ranged from 18 to 27 mm when including patients from all grades of hydronephrosis. The median APD measurements were significantly less for SFU grade 3 than grade 4 hydronephrosis in all positions (P < 0.001 for all measurements), yet the predictive cutoff value of 24 mm for the supine extra-renal was similar for both grades. CONCLUSION: APD measurements differ based on the technique, but they are all equally associated with the clinical outcome of pyeloplasty. The inter-rater reliability of all techniques were excellent. Though the median APD measurements are smaller in SFU grade 3, it appears that the cutoff for a predictive renal pelvic APD does not differ between SFU grades 3 and 4 for the supine extra-renal technique. We conclude that the technique for measuring the APD needs to be specified in studies of hydronephrosis and in any grading systems.

Persistent urinary leak after partial nephrectomy in a child: Definitive therapy with percutaneous glue.

Urinary fistulas are usually managed with diversion, and persistent fistulas may require nephrectomy. For refractory cases in adults, rare reports have described percutaneous glues as an alternative to nephrectomy. We present the case of a child with persistent urinary leak after partial nephrectomy for recurrent microabscesses. In order to spare the child nephrectomy, the fistula was treated percutaneously with the application of cyanoacrylate glue and a vascular occlusion plug. This is the first report of percutaneous glue treatment with an adjunct vascular occlusion device for a urinary leak, and the first report of percutaneous management of a pediatric urinary fistula.

Von Hippel-Lindau disease and rapidly progressing pheochromocytomas in siblings.

Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited condition with a predisposition to the development of a variety of tumors including pheochromocytomas. A number of cancer surveillance protocols for patients with VHL have been developed, all of which are based on expert opinion. We report a case of two brothers with a strong family history of VHL type 2 due to a pathogenic germline VHL variant, specifically, a surface missense substitution, with a rapidly progressive clinical course that both presented with a large adrenal mass. Both brothers presented with large pheochromocytomas, the earliest presentation being at age 7, despite routine screening. The rapid progression and early presentation of these patients raises an important discussion around the commonly used surveillance protocols for pheochromocytoma in pediatric patients with VHL and missense mutations. We conclude that a more accelerated surveillance protocol may be adequate for VHL families with a high pheochromocytoma risk.

Validation of modified diuretic drainage times criteria in congenital hydronephrosis.

INTRODUCTION AND OBJECTIVE: The value of diuretic renography drainage times in congenital hydronephrosis (AHN) decision making is controversial. Recently, a group suggested a modification to the classically described diuretic drainage time cut-off values. They found that a drainage half-time (T1/2) < 5 min was normal whereas a T1/2 exceeding 75 min predicted pyeloplasty. In addition, they reported on the benefit of a delayed drainage image obtained with gravity assistance. We sought to evaluate the ability of these modified T1/2 criteria to predict pyeloplasty, alone or in combination with a delayed drainage image referred to as Global Washout (GWO). METHODS: We retrospectively reviewed 113 patients, including consecutive pyeloplasties for AHN from 2004 to 2018. Patients who underwent pyeloplasty due to low differential renal function (DRF) < 30% or infection were excluded. The control group comprised high grade AHN managed non-operatively. The initial renal ultrasound and MAG 3 Lasix renogram were reviewed for grade, differential renal function (DRF), T1/2 and GWO. A ROC curve was used to evaluate the T1/2 and GWO cut-off points that can predict pyeloplasty, using a p-value of less than 0.05. RESULTS: The pyeloplasty group consisted of 62 patients and the control group consisted of 51 patients. Two patients (3%) in the pyeloplasty group had a T1/2 < 5 min whereas 21 (34%) had T 1/2 > 75 min (p < 0.001). In the control group, 25 patients (49%) had T 1/2 < 5 min and none had T1/2 >75 min (P < 0.001). The ROC curve for T1/2 < 5 min demonstrated 94% sensitivity and 51% specificity whereas a T1/2 >75 min demonstrated 100% specificity and 34% sensitivity. Analysis of the GWO using a ROC curve revealed that a cut-off of 50% GWO has 100% specificity and 52% sensitivity for pyeloplasty. Overall, a T1/2 > 75 min or GWO <50% predicted 53% of pyeloplasties (Fig A) and was absent in all conservatively managed cases. DISCUSSION: The limitations of the present study include its retrospective nature. Secondly, the lack of a gold standard diagnostic test for uretero-pelvic junction obstruction hampers objective quantification of diagnostic test utility performance. CONCLUSIONS: We confirm the utility of the modified diuretic half-time criteria and delayed gravity assisted imaging. A T1/2 > 75 min or GWO <50% are indicators of severity whereas a T1/2 of <5 min or GWO >90% is reassuring. Gravity assisted delayed imaging can be especially helpful in cases with indeterminate T 1/2 times and should be included in the standard assessment of hydronephrotic kidneys. These parameters can be used to tailor the frequency and invasiveness of imaging within observation protocols.

Does the presence of non-refluxing hydroureter impact the management and outcome of high-grade hydronephrosis?

INTRODUCTION: We aimed to evaluate the impact of non-refluxing hydroureter on the initial management of high-grade hydronephrosis (HGH) management. Moreover, we evaluated the occurrence of febrile urinary tract infection (fUTI) and surgical intervention for conservatively managed units. METHODS: Patients' charts with postnatal hydronephrosis from 2008-2014 were retrospectively reviewed. We included patients who presented in the first year of life. All included patients had HGH (Society of Fetal Ultrasound [SFU] grades 3 and 4) and all were non-refluxing using voiding cystourethrogram (VCUG). We categorized renal units into two groups: with hydroureteronephrosis (HUN) and without hydroureter (isolated hydronephrosis [IH]). We recorded the initial management. We evaluated the impact of non-refluxing hydroureter on hydronephrosis fate, fUTI, and change to surgery for those managed conservatively. RESULTS: We included 169 patients (180 units). IH was diagnosed in 146 units (137 patients), whereas 34 units (32 patients) had HUN. Median followup was 42.9 months. A total of 25.3% (37/146) of IH units had initial surgical management in comparison to 5.1% (2/34) of HUN units (p=0.01). During conservative management, nine HUN patients (30%) and 11 IH patients (10.7%) experienced fUTI (p=0.009). Surgical intervention after failed conservative management was indicated for 12 IH units (11%) and six HUN units (18.8%) (p=0.25). Anteroposterior diameter (APD) worsening was significantly associated with the change to surgery in IH group (p=0.003). More than half (52.3%) of IH units resolved during conservative management in comparison to 18.7% of HUN (p<0.001). HUN had longer time to resolution (log rank=0.004). CONCLUSIONS: IH units had more initial surgical interventions. The fUTI rate was much higher in association with dilated ureter even under antibiotic coverage. HUN was associated with less and slower resolution rate.

Children with vesicoureteric reflux have joint hypermobility and occasional tenascin XB sequence variants.

INTRODUCTION: To consider alternative mechanisms that give rise to a refluxing ureterovesical junction (UVJ), we hypothesized that children with a common heritable urinary tract defect, vesicoureteric reflux (VUR), may have a defect in the extracellular matrix composition of the UVJ and other tissues that would be revealed by assessment of the peripheral joints. Hypermobile joints can arise from defects in the extracellular matrix within the joint capsule that affect proteins, including tenascin XB (TNXB). METHODS: We performed an observational study of children with familial and non-familial VUR to determine the prevalence of joint hypermobility, renal scarring, and DNA sequence variants in TNXB. RESULTS: Most children (27/44) exhibited joint hypermobility using the Beighton scoring system. This included 15/26 girls (57.7%) and 12/18 boys (66.7%), which is a significantly higher prevalence for both sexes when compared to population controls (p<0.005). We found no association between joint hypermobility and renal scarring. Seven of 49 children harbored rare pathogenic sequence variants in TNXB, and two also exhibited joint hypermobility. No sequence variants in TNXB were identified in 25/27 children with VUR and joint hypermobility. Due to the observational design of the study, there was missing data for joint hypermobility scores in six children and for dimercaptosuccinic acid (DMSA) scans in 17 children. CONCLUSIONS: We observed a high prevalence of VUR and joint hypermobility in children followed within a tertiary care pediatric urology clinic. While mutations in TNXB have been reported in families with VUR and joint hypermobility, we identified only two children with these phenotypes and pathogenic variants in TNXB. We, therefore, speculate that VUR and joint hypermobility may be due to mutations in other extracellular matrix genes.

Influence of postnatal hydroureter in determining the need for voiding cystourethrogram in children with high-grade hydronephrosis.

OBJECTIVE: To evaluate the utility of hydroureter (HU) to identify high-grade vesico-ureteric reflux (VUR) in patients with high-grade postnatal hydronephrosis (PH). PATIENTS AND METHODS: We retrospectively reviewed patients' charts that had antenatal hydronephrosis from 2008 to 2014. Patients were excluded if they presented with febrile urinary tract infection (fUTI), neurogenic bladder, posterior urethral valve, multi-cystic dysplastic kidney, and multiple congenital malformations. We reviewed postnatal ultrasonography images and patients with Society of Fetal Urology (SFU) Grades 3 and 4 hydronephrosis with a renal pelvic antero-posterior diameter of ≥10 mm were included. The ureter was assessed and considered dilated if the ureteric diameter was  ≥4 mm. The voiding cystourethrogram (VCUG) studies, fUTI incidence, and surgical reports were reviewed. RESULTS: Of the 654 patients reviewed, we included 148 patients (164 renal units) of whom 113 (76.4%) were male and 35 (23.6%) female. SFU Grade 3 PH was identified in 49% of the renal units, with the remaining 51% being SFU Grade 4. HU was found in 50/164 renal units and was not detected in the remaining 114 units. VUR was diagnosed in four units (3.5%) without HU (low-grade VUR); whilst it was detected in 19 units (38%) with HU (72.7% were high-grade VUR) (P < 0.001). VUR was diagnosed on the contralateral side in four/105 patients with PH without HU and diagnosed in 10/43 patients with PH with HU (P < 0.001). During a median follow-up of 25.9 months, none of the renal units that had VUR without HU developed UTI or had surgeries. CONCLUSION: Low-grade uncomplicated VUR was diagnosed in 3.5% of renal units without HU. Our results support limiting the use of VCUG to renal units with PH if associated with HU.

Is the renal pyramidal thickness a good predictor for pyeloplasty in postnatal hydronephrosis?

OBJECTIVES: We evaluated the feasibility and value of renal pyramidal thickness (PT) as a predictor of pyeloplasty in high-grade postnatal hydronephrosis. PATIENTS AND METHODS: We retrospectively reviewed the charts of patients who presented with postnatal hydronephrosis from 2008 to 2013. Included cases had grade 3 or 4 hydronephrosis. We included only units diagnosed as ureteropelvic junction obstruction. Gender, laterality, hydronephrosis side, renogram data, and follow-up data were recorded. Two investigators reviewed all patients' ultrasounds images. We measured PT and pelvic anteroposterior diameter (APD) in the last ultrasound before surgery. For those managed conservatively, measurements were obtained from the ultrasound with worst hydronephrosis. PT was measured in supine position in the middle third of the sagittal plane (Figure). We assessed the reliability of PT measurement using the intraclass correlation coefficient (ICC). Univariate and multivariate analyses were used to correlate the collected parameters to pyeloplasty incidence. Receiver operating characteristic curve was used to evaluate the cutoff value of PT that predicts pyeloplasty. RESULTS: The total included cases were 155 patients (165 units). One hundred and fourteen units had grade 3 hydronephrosis and 51 units had grade 4 hydronephrosis. Fifty-two cases (55 units) underwent pyeloplasty. The median follow-up period was 37.6 months. PT measurement was reliable (ICC = 0.94). Univariate analysis revealed that SFU grading, APD, PT, T1/2, and MAG-3 curves were associated with surgery. Multivariate analysis showed that PT was a single independent predictor for pyeloplasty. PT ≤ 3 mm had 98.1% sensitivity and 89.7% specificity in predicting pyeloplasty. DISCUSSION: PT is the first portion of renal parenchyma that is affected in high-grade hydronephrosis. Moreover, it changes little over the first 9 years of life. PT measurement in hydronephrosis was not previously evaluated. We found that PT was easily measured in most kidneys with high negative predictive value. The PT value as an indicator for pyeloplasty should undergo extensive assessment by other institutions with different protocols. CONCLUSION: Being a slowly growing part of the parenchyma, PT can be a good measurable parameter to predict pyeloplasty. Measurement of PT in hydronephrosis is reliable. PT ≤ 3 mm can predict pyeloplasty with 98.1% sensitivity and 89.7% specificity.

Heterozygous loss-of-function mutation in Odd-skipped related 1 (Osr1) is associated with vesicoureteric reflux, duplex systems, and hydronephrosis.

Odd-skipped related 1 (Osr1) is a transcriptional repressor that plays critical roles in maintaining the mesenchymal stem cell population within the developing kidney. Here, we report that newborn pups with a heterozygous null mutation in Osr1 exhibit a 21% incidence of vesicoureteric reflux and have hydronephrosis and urinary tract duplications. Newborn pups have a short intravesical ureter, resulting in a less competent ureterovesical junction which arises from a delay in urinary tract development. We describe a new domain of Osr1 expression in the ureteral mesenchyme and within the developing bladder in the mouse. OSR1 was sequenced in 186 children with primary vesicoureteric reflux, and 17 have single nucleotide polymorphisms. Fifteen children have a common synonymous variant, rs12329305, one child has a rare nonsynonymous variant, rs3440471, and one child has a rare 5'-UTR variant, rs45535040 The impact of these SNPs is not clear; therefore, the role of OSR1 in human disease remains to be elucidated. Osr1 is a candidate gene implicated in the pathogenesis of vesicoureteric reflux and congenital abnormalities of the kidney and urinary tract in mice.

A case of epidermoid median raphe cyst traversing the corpora cavernosa.

Median raphe cysts are congenital lesions that typically have a superficial appearance. We present a very unusual case of a deep perineal mass in a six-year-old boy. The lesion extends into the corpus cavernosum, suggesting that the anomaly was an early embryological event. Histopathological features are consistent with an epidermoid type of median raphe cyst.

Antibiotic prophylaxis for prevention of urinary tract infections in prenatal hydronephrosis: An updated systematic review.

INTRODUCTION: While continuous antibiotic prophylaxis (CAP) is currently recommended to prevent urinary tract infections (UTIs) in infants with prenatal hydronephrosis (HN), this recommendation is not evidence-based. The objective of this study was to systematically determine whether CAP reduces UTIs in the HN population. METHODS: Applicable trials were identified through an electronic search of MEDLINE (1946-2015), EMBASE (1980-2016), CINAHL (1982-2016), and CENTRAL (1993-2016) and through a hand search of American Urological Association (AUA) (2012-2015) and European Society for Pediatric Urology (ESPU) (2012-2015) abstracts, as well as reference lists of included trials. The search strategy was not limited by language or year of publication. Eligible studies compared CAP to no CAP in patients with antenatal HN, <2 years of age, and reported development of UTI and HN grades. Two independent reviewers performed title and abstract screening, full-text review, and quality appraisal. RESULTS: Of 1518 citations screened, 11 were included, contributing 3909 patients for final analysis. Of these, four (36%) were considered high-quality when assessed by the Newcastle Ottawa Scale. Meta-analysis of the non-randomized trials (n=10) provided similar pooled UTI rates, regardless of CAP use: 9.9% (95% confidence interval [CI] 8.4-11.4%) for CAP and 7.5% (95% CI 6.4-8.6%) for no CAP. CONCLUSIONS: This systematic review and meta-analysis suggests there may be value in providing CAP to infants with high-grade HN; however, due to the very low-quality data from non-randomized studies, important clinical variables, such as circumcision status, were unable to be assessed.

Laparoscopic pyeloplasty: Initial experience with 3D vision laparoscopy and articulating shears.

INTRODUCTION: Laparoscopic reconstructive surgery is associated with a steep learning curve related to the use of two-dimensional (2D) vision and rigid instruments. With the advent of robotic surgery, three-dimensional (3D) vision, and articulated instruments, this learning curve has been facilitated. We present a hybrid alternative to robotic surgery, using laparoscopy with 3D vision and articulated shears. OBJECTIVE: To compare outcomes of children undergoing pyeloplasty using 3D laparoscopy with articulated instruments with those undergoing the same surgery using standard laparoscopy with 2D vision and rigid instruments. STUDY DESIGN: Medical charts of 33 consecutive patients with ureteropelvic junction obstruction who underwent laparoscopic pyeloplasty by a single surgeon from 2006 to 2013 were reviewed in a retrospective manner. The current 3D cohort was compared with the previous 2D cohort. Data on age, weight, gender, side, operative time, dimension (2D = 19 patients, 3D = 8 patients), presence of a crossing vessel, length of hospital stay, and complication rate were compared between the two groups. Articulating shears were used for pelvotomy and spatulation of the ureter in the 3D group. Statistical tests included linear regression models and chi square tests for trends using STATA software. RESULTS: Operative time per case was decreased by an average of 48 min in the group undergoing 3D laparoscopic pyeloplasty compared with the group undergoing 2D laparoscopic pyeloplasty (p = 0.02) (Figure). Complication rate and length of hospital stay were not significantly affected by the use of 3D laparoscopy. DISCUSSION: These favorable results are in accordance with previous literature emphasizing the importance of 3D vision in faster and more precise execution of complex surgical maneuvers. The use of flexible instruments has also helped overcome the well-described delicate step of a dismembered pyeloplasty, namely the pelvotomy and ureteral spatulation. Limitations of this study are those inherent to the retrospective study design. CONCLUSION: The use of 3D vision endoscopy with articulating instruments blurs the distinction between current robotic-assisted and conventional laparoscopic technology, and provides a hybrid alternative deserving further attention.

Long-term functional outcomes after bladder exstrophy repair: A single, low-volume centre experience.

INTRODUCTION: In this study, we present our experience managing bladder exstrophy (BE) in a low-volume centre over 24 years. METHODS: Charts of patients with BE between 1990 and 2014 were retrospectively reviewed. Patients with BE closure and ≥5 years followup were included. BE closure was carried out in the first two days of life using either complete primary repair (CPRE) or modern-staged repair (MSRE). Daytime urinary continence (UC) was evaluated by the age of five years. Patients were considered continent if completely dry for ≥3 hours using no or one pad/day. Incontinent patients with bladder capacity (BC) ≥100 ml underwent bladder neck reconstruction (BNR) and bilateral ureteric reimplantation (BUR), while patients with BC <100ml underwent simultaneous augmentation cystoplasty (ACP). RESULTS: Sixteen (16) patients met our inclusion criteria with a mean followup time of 18±5 years. Ten (10) underwent CPRE, while six underwent MSRE. Four surgeons were involved in patients' management. Two surgeons had previous experience in BE surgery while working in other institutions. Complications included dehiscence in five patients, vesicocutanous fistula in three and breakthrough UTI in eight. Continence was achieved in 15/16 patients: two after BE closure only, seven with BNR, and six who required ACP and BNR. CONCLUSIONS: Despite the small number of patients and the reterospective nature of the study, some observations are noteworthy. Although continence rate post-primary BE closure was initially low, it rose to 93.8% after auxiliary continence procedures. This might be at the cost of urethral voiding, which was achieved in 60% of patients. Our small cohort did not show clear advantage of CPRE vs. MSRE. Our outcomes may not be different from high-volume centres due to the fact that two exstrophy-experienced surgeons performed most primary or subsequent surgeries. For this reason, we recommend assigning designated centres for BE repair for both new and repeat cases.

Evaluation of Urinary Tract Dilation Classification System for Grading Postnatal Hydronephrosis.

PURPOSE: We assessed the reliability and validity of the Urinary Tract Dilation classification system as a new grading system for postnatal hydronephrosis. MATERIALS AND METHODS: We retrospectively reviewed charts of patients who presented with hydronephrosis from 2008 to 2013. We included patients diagnosed prenatally and those with hydronephrosis discovered incidentally during the first year of life. We excluded cases involving urinary tract infection, neurogenic bladder and chromosomal anomalies, those associated with extraurinary congenital malformations and those with followup of less than 24 months without resolution. Hydronephrosis was graded postnatally using the Society for Fetal Urology system, and then the management protocol was chosen. All units were regraded using the Urinary Tract Dilation classification system and compared to the Society for Fetal Urology system to assess reliability. Univariate and multivariate analyses were performed to assess the validity of the Urinary Tract Dilation classification system in predicting hydronephrosis resolution and surgical intervention. RESULTS: A total of 490 patients (730 renal units) were eligible to participate. The Urinary Tract Dilation classification system was reliable in the assessment of hydronephrosis (parallel forms 0.92). Hydronephrosis resolved in 357 units (49%), and 86 units (12%) were managed by surgical intervention. The remainder of renal units demonstrated stable or improved hydronephrosis. Multivariate analysis revealed that the likelihood of surgical intervention was predicted independently by Urinary Tract Dilation classification system risk group, while Society for Fetal Urology grades were predictive of likelihood of resolution. CONCLUSIONS: The Urinary Tract Dilation classification system is reliable for evaluation of postnatal hydronephrosis and is valid in predicting surgical intervention.

Comprehensive analysis of the clinical and urodynamic outcomes of secondary tethered spinal cord before and after spinal cord untethering.

OBJECTIVE: Secondary tethering of the spinal cord (TSC) occurs in 3-30% of patients with history of spinal cord dysraphism repair. As many patients with spinal cord dysraphism already have symptoms, it might be difficult to diagnose secondary TSC. Regular clinical/urodynamic (UDS) evaluation is therefore advised to pick-up changes early before they progress. This study aimed at presenting clinical/UDS outcomes for patients with secondary TSC who underwent spinal cord untethering (SCU). STUDY DESIGN: Charts of patients with secondary TSC between 1998 and 2010 were reviewed retrospectively. Patients who underwent pre/post SCU clinical/UDS evaluation were included. All patients were followed-up for a minimum of 5 years for proper clinical outcomes assessment. Urologic, neuro-orthopedic, and UDS outcomes were collected and studied. Continence status was assessed in children aged ≥5 years. Patients were considered continent if they were dry for ≥4 h or socially continent if they stayed dry most of the day using maximum 1-2 pads/day. Categorical data were compared using the Fisher-Exact test and continuous variables were compared using the Wilcoxon Signed Rank test. A p-value < 0.05 was considered significant. RESULTS: Twenty-three patients met our inclusion criteria. The median age at time of SCU was 8.8 (range 2.3-16.2) years. The median age at time of UDS follow-up after SCU was 8.8 (range 2.9-17) years. The median follow-up time was 5 (range 5-13.4) years. Urological symptoms were reported in 56.5% of patients before SCU and improved in 61.5% post SCU. Neuro-orthopedic symptoms were reported in 87% of patients before SCU and improved in 65% post SCU. Continence was achieved in 73.9% of the entire cohort on long-term follow-up, while 26.1% had socially acceptable continence. UDS outcomes are summarized in the Table. CONCLUSIONS: Patients with secondary TSC are expected to have progression of their symptoms over time if not untethered. From here comes the importance of closely performing clinical/UDS evaluation to pick-up changes early before they progress. Surprisingly, clinical outcomes after SCU with detailed description of the auxiliary management are sparse in the literature. Most studies focus on UDS outcomes. We provided in the current study a detailed discussion of the clinical outcomes, auxiliary-managements used to achieve continence, and the UDS outcomes. Careful periodic clinical/UDS evaluation is recommended for early pick-up of changes suggestive of TSC. SCU could improve existing symptoms, and prevent worsening or development of new symptoms. UDS parameters that showed most improvement were intravesical pressure at TCBC and bladder compliance.

First case of invasive squamous cell carcinoma in a stoma of a Monti ileovesicostomy.

We report a very rare case of invasive squamous cell carcinoma (SCC) in the abdominal stoma of a Monti ileovesicostomy. Our patient underwent an uncomplicated Monti ileovesicostomy at age 16 for a neurogenic bladder. She presented 10 years later with difficulty catheterizing the stoma. A biopsy of peristomal tissue showed moderately differentiated SCC. A cystoscopy did not reveal any bladder tumours or suspicious lesions. A computed tomography (CT) scan of the abdomen and pelvis did not demonstrate metastasis. The patient underwent a complete en bloc resection of the stomal site, the Monti, a partial cuff of bladder, and 2 loops of bowel that were adherent to the Monti. Final pathology revealed pure invasive SCC arising around the stoma and negative surgical margins. Six months later, a follow-up CT scan showed no evidence of malignancy, while a cystoscopy revealed a small erythematous area in the posterior bladder wall. Urinary cytology was positive for SCC. Transurethral resection of the erythematous lesion with random bladder biopsies showed SCC in situ in the erythematous lesion and right lateral bladder wall. Staging workup was negative. The patient subsequently underwent a radical cystectomy and ileal conduit diversion with bilateral pelvic lymph node dissection. Final pathology on cystectomy specimen was SCC in situ without evidence of invasive carcinoma. The patient has remained in remission at the 3-year follow-up.