Contrast-enhanced voiding urosonography (CEVUS) as a safe alternate means of assessing vesicoureteral reflux in pediatric kidney transplant patients.

BACKGROUND: Although voiding cystourethrogram (VCUG) is currently the gold standard in VUR evaluation, there is ionizing radiation exposure. Contrast-enhanced voiding urosonography (CEVUS) uses ultrasound contrast agents to visualize the urinary tract and has been reported to be safe and effective in VUR evaluation in children. CEVUS application has yet to be specifically described in VUR evaluation in the pediatric kidney transplant population. The purpose of this study was to report the use of CEVUS and VCUG in evaluating and managing VUR in pediatric renal transplant patients. METHODS: Retrospective review was conducted for pediatric kidney transplant patients (18 years and younger) who underwent VCUG or CEVUS to assess for transplant VUR from July 2019 through June 2021. Demographic information, reason for VUR evaluation, fluoroscopy time, and postimaging complications were evaluated. Costs of imaging modalities were also considered. RESULTS: Eight patients were evaluated for transplant VUR during the study period. Of the 3 patients who underwent VCUG, all 3 had VUR (median grade 3). Median fluoroscopy time was 18 s and dose-area product was 18.7 uGy*m2 . Of the 5 patients who underwent CEVUS, 4 had VUR (median grade 4). There were no complications for either modality. Based on clinical and radiographic findings, patients were recommended no intervention, behavioral modification, or ureteral reimplantation. The total cost of CEVUS was $800 less than that of VCUG. CONCLUSION: CEVUS can provide an alternate means of safely evaluating VUR in kidney transplant patients with similar outcomes, potentially lower costs, and no exposure to ionizing radiation.

Solo and Partnered Sexual Behavior Among an International Sample of Adults With Spina Bifida.

BACKGROUND: Spina bifida (SB) may differentially impact adults' participation in solo and partnered sexual behaviors, but little research investigates this topic. AIM: Describe solo and partnered sexual behaviors among an international sample of adult men and women with SB. MAIN OUTCOME MEASURES: Ever participated (no/yes) and recent participation (>1 year ago/within last year) in solo masturbation, cuddled with a partner, held hands with a partner, kissed a partner, touched a partner's genital, had genitals touched by a partner, gave a partner oral sex, received oral sex from a partner, vaginal sex, anal sex, and sex toy use. METHODS: Data were drawn from a larger cross-sectional, internet-based survey assessing the sexual behaviors of an international sample of men and women with SB. We used logistic regression to examine the impact of background (gender, age, independent living, and relationship status) and health (shunt status, ambulation, and genital sensation) factors on each outcome. RESULTS: The sample consisted of 345 respondents aged 18-73 years from 26 nations. Very few (<3%) had no lifetime experience with any solo or partnered behaviors; 25.0% reported participating in all behaviors at some point in their lives. The median number of past year sexual behaviors (of 16 total) was 7. Lifetime and recent participation were associated with demographic and health factors. CLINICAL IMPLICATIONS: Despite impairment, adults with spina bifida do participate in solo and partnered sexual behaviors. Medical personnel who work with this population should include discussions about sexuality as part of routine care. STRENGTHS & LIMITATIONS: Although this research measured solo and partnered sexual behavior in large international sample of adults with spina bifida, it is limited by its cross-sectional retrospective design and non-clinical convenience sample. CONCLUSION: Despite disability, many adults with SB participate in solo and partnered sexual behavior. Medical and psychosocial supports are needed to help adults in this population enjoy sexuality in a healthy and safe manner. Hensel DJ, Misseri R, Wiener JS, et al. Solo and Partnered Sexual Behavior Among an International Sample of Adults With Spina Bifida. J Sex Med 2022;19:1766-1777.

Short term reoperation rates after artificial urinary sphincter placement in pediatric patients.

INTRODUCTION: Artificial urinary sphincters (AUS) have demonstrated good functional outcomes in pediatric populations. We sought to examine the nationwide short term reoperation rates in pediatric patients after AUS placement. MATERIALS AND METHODS: An observational cohort study was designed utilizing claims from the Truven MarketScan Commercial Claims and Encounters database from 2007 to 2018. Patients under 18 years of age undergoing an AUS procedure were identified using CPT and ICD9/10 codes. Reoperations included any removal, replacement, or AUS placement codes which occurred after the initially identified placement code. Follow up time was the amount of time between AUS placement and the end of MarketScan enrollment. RESULTS: From 2007-2018, we identified 57 patients under the age of 18 who underwent AUS placement and after excluding 8 for concurrent AUS complication procedure codes and 4 for follow up < 60 days, the final cohort included 45 patients. The median age was 13 years (IQR 9-16 years) at the time of AUS placement, and the median follow up time after AUS placement was 787 days (IQR 442-1562 days), approximately 2.2 years. Total reoperation rate was 22%. Reoperations included 40% device removals (4/10) and 60% replacements (6/10). Neither gender (p = 0.70) nor age (p = 0.23) was associated with need for reoperation. Patients who had a concurrent bladder surgery had a higher rate of undergoing reoperation (50% vs. 12%, p = 0.007). CONCLUSIONS: The rate of reoperation after AUS placement approached 1 in 4 in pediatric patients. These data may be instrumental for providers and parents in counseling and decision-making regarding risks of prosthetic implantation.

Additional Surgeries after Bladder Augmentation in Patients with Spina Bifida in the 21st Century.

PURPOSE: We determined the long-term risks of additional surgery after bladder augmentation in a modern spina bifida cohort accounting for differential followup. MATERIALS AND METHODS: We retrospectively reviewed patients with spina bifida who were born after 1972 and were followed at our institution after augmentation surgery performed between 1979 and 2018. Outcomes included diversion, bladder stones, perforation, reaugmentation, laparotomy for bowel obstruction, and benign and malignant bladder tumors. Survival analysis was used for the entire cohort and the modern cohort (detubularized and reconfigured ileocystoplasty beginning in 2000). RESULTS: A total of 413 patients were included in the study. At a median followup of 11.2 years 80.9% of the patients had undergone ileocystoplasty and 44.1% had undergone 370 additional surgeries. Ten-year risk of any reoperation was 43.9%, with 17.4% of patients undergoing 2 or more and 9.9% undergoing 3 or more additional surgeries. Outcomes included conversion to a diversion (2.7% at 10-year followup) and bladder stones (28.2% with recurrence in 52.4%) irrespective of detubularized reconfigured status (p ≥0.20). Bladder perforation risk was 9.6% for patients undergoing vs 23.7% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.01). Similarly reaugmentation rate was 5.3% for patients undergoing vs 15.2% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.001). Finally, 10-year reperforation risk was 32.1% for patients undergoing vs 73.8% for those not undergoing detubularized reconfigured ileocystoplasty (p=0.053). Other risks included bowel obstruction (4.5% with recurrence in 15.8%), nephrogenic adenoma (2.2% with regrowth in 48.2%) and malignancy (0.0% at 20 years). For 222 patients in the modern cohort (median followup 9.1 years) 10-year risk of any reoperation was 46.0%, which consisted of diversion in 4.0%, stones in 32.9% (recurrence in 44.5%), perforation in 8.8% (recurrence in 42.2%), reaugmentation in 4.3%, obstruction in 4.9% (recurrence in 10.0%), adenoma in 4.7% (regrowth in 40.0%) and cancer in 0.0%. CONCLUSIONS: Bladder augmentation is long-lasting. While benefiting continence and renal outcomes, this operation frequently requires additional surgeries, necessitating close followup. Since survival analysis based risks of alternative management options such as incontinent diversion are unavailable, comparisons with augmentation are unfeasible.

Epidural Analgesia Decreases Narcotic Requirements in Patients with Low Level Spina Bifida Undergoing Urological Laparotomy for Neurogenic Bladder and Bowel.

PURPOSE: Concerns regarding anatomical anomalies and worsening neurological symptoms have prevented widespread use of epidural catheters in patients with low level spina bifida. We hypothesize that thoracic epidural placement in the T9 to T10 interspace is safe and decreases narcotic requirements following major open lower urinary tract reconstruction in patients with low level spina bifida. MATERIALS AND METHODS: We reviewed consecutive patients with low level spina bifida who underwent lower urinary tract reconstruction and received epidurals for postoperative pain control. Controls were patients with low level spina bifida who received single injection transversus abdominis plane blocks and underwent similar procedures. Complications of epidural placement, including changes in motor and sensory status, were recorded. Opioid consumption was calculated using equivalent intravenous morphine doses. Mean and maximum pain scores on postoperative days 0 to 3 were calculated. RESULTS: Ten patients with low level spina bifida who underwent lower urinary tract reconstruction with epidural were matched to 10 controls with low level spina bifida who underwent lower urinary tract reconstruction with transverse abdominis plane block. Groups were demographically similar. All patients had full abdominal sensation and functional levels at or below L3. No epidural complications or changes in neurological status were noted. The epidural group had decreased opioid consumption on postoperative days 0 to 3 (0.75 mg/kg vs 1.29 mg/kg, p = 0.04). Pain scores were similar or improved in the epidural group. CONCLUSIONS: Thoracic epidural analgesia appears to be a safe and effective opioid sparing option to assist with postoperative pain management following lower urinary tract reconstruction in individuals with low level spina bifida.

Risk of Reaugmentation after Enterocystoplasty Using a Reconfigured Bowel Segment in Patients with Spina Bifida: A Bi-Institutional Cohort Study.

PURPOSE: Reaugmentation cystoplasty rates vary in the literature but have been reported as high as 15%. It is likely that bladders augmented with detubularized and reconfigured bowel are less likely to require reaugmentation. We assessed the incidence of reaugmentation among patients with spina bifida at 2 high volume reconstruction centers. MATERIALS AND METHODS: We retrospectively reviewed medical records of patients with spina bifida who underwent enterocystoplasty before age 21 years (1987 to 2017). Those who did not undergo augmentation with a detubularized and reconfigured bowel segment were excluded from analysis. Data on demographic and surgical variables were collected. Reaugmentation was the main outcome. One analysis was performed using the entire cohort and another analysis was restricted to patients with ileocystoplasty performed in the last 15 years (2002 to 2017). Survival analysis was used. RESULTS: A total of 289 patients were identified. Enterocystoplasty was performed in patients at a median age of 8.1 years (median followup 11.3, IQR 5.2-14.9). Most initial augmentations were performed using ileum (93.4%), followed by sigmoid (6.2%). Seven patients underwent reaugmentation, including 6 with initial augmentation using ileum and 1 with initial augmentation using sigmoid. On survival analysis risk of reaugmentation was 1.1% at 5 years and 3.3% at 10 years after the original surgery. All reaugmentations occurred within the first 9 years of followup. In the more contemporary cohort (162, median followup 7.0 years) only 1 patient underwent reaugmentation at 2.0 years. CONCLUSIONS: The risk of reaugmentation after enterocystoplasty with a detubularized and reconfigured bowel in the spina bifida population is lower than that reported in initial series.

Radiographic abnormalities, bladder interventions, and bladder surgery in the first decade of life in children with spina bifida.

BACKGROUND: Spina bifida (SB) patients are at increased risk for hydronephrosis, bladder storage and emptying problems, and renal failure that may require multiple bladder surgeries. METHODS: We retrospectively reviewed patients born with SB 2005-2009, presenting to our institution within 1 year of birth. Outcomes at 8-11 years old included final renal/bladder ultrasound (RBUS) results, clean intermittent catheterization (CIC) use, anticholinergic use, surgical interventions, and final renal function. We excluded those without follow-up past age 8 and/or no RBUS or fluoroscopic urodynamic images (FUI) within the first year of life. Imaging was independently reviewed by four pediatric urologists blinded to radiologists' interpretation and initial findings compared with final outcomes. RESULTS: Of 98 children, 62 met inclusion criteria (48% male, 76% shunted). Median age at last follow-up was 9.6 years. Upon initial imaging, 74% had hydronephrosis (≥ SFU grade 1), decreasing to 5% at 10 years (p < 0.0001). Initially, 9% had ≥ SFU grade 3 hydronephrosis, decreasing to 2% (p = 0.13). CIC and anticholinergic use increased from 61% and 37% to 87% and 86%, respectively (p = 0.001 and p < 0.0001, respectively). With follow-up, 55% had surgical intervention and 23% had an augmentation. Of children with a serum creatinine/cystatin-C at 8-11 years old, one had confirmed chronic kidney disease (stage 2). CONCLUSIONS: Despite initial high incidence of hydronephrosis, this was low grade and resolved in the first decade of life. Additionally, the 8-11-year incidence of kidney disease and upper tract changes was low due to aggressive medical management.

Variation in Surgical Antibiotic Prophylaxis for Outpatient Pediatric Urological Procedures at United States Children's Hospitals.

PURPOSE: Guidelines recommend surgical antibiotic prophylaxis for clean-contaminated procedures but none for clean procedures. The purpose of this study was to describe variations in surgical antibiotic prophylaxis for outpatient urological procedures at United States children's hospitals. MATERIALS AND METHODS: Using the PHIS (Pediatric Health Information System®) database we performed a retrospective cohort study of patients younger than 18 years who underwent clean and/or clean-contaminated outpatient urological procedures from 2012 to 2014. We excluded those with concurrent nonurological procedures or an abscess/infected wound. We compared perioperative antibiotic charges for clean vs clean-contaminated procedures using a multilevel logistic regression model with a random effect for hospital. We also examined whether hospitals that were guideline compliant for clean procedures, defined as no surgical antibiotic prophylaxis, were also compliant for clean-contaminated procedures using the Pearson correlation coefficient. We examined hospital level variation in antibiotic rates using the coefficient of variation. RESULTS: A total of 131,256 patients with a median age of 34 months at 39 hospitals met study inclusion criteria. Patients undergoing clean procedures were 14% less likely to receive guideline compliant surgical antibiotic prophylaxis than patients undergoing clean-contaminated procedures (OR 0.86, 95% CI 0.84-0.88, p <0.0001). Hospitals that used antibiotics appropriately for clean-contaminated procedures were more likely to use antibiotics inappropriately for clean procedures (r = 0.7, p = 0.01). Greater variation was seen for hospital level compliance with surgical antibiotic prophylaxis for clean-contaminated procedures (range 9.8% to 97.8%, coefficient of variation 0.36) than for clean procedures (range 35.0% to 98.2%, coefficient of variation 0.20). CONCLUSIONS: Hospitals that used surgical antibiotic prophylaxis appropriately for clean-contaminated procedures were likely to use surgical antibiotic prophylaxis inappropriately for clean procedures. More variation was seen in hospital level guideline compliance for clean-contaminated procedures.

All Incontinence is Not Created Equal: Impact of Urinary and Fecal Incontinence on Quality of Life in Adults with Spina Bifida.

PURPOSE: We previously reported that the self-reported amount of urinary incontinence is the main predictor of lower health related quality of life in adults with spina bifida. In this study we sought to determine the impact of fecal incontinence on health related quality of life after correcting for urinary incontinence. MATERIALS AND METHODS: An international sample of adults with spina bifida was surveyed online in 2013 to 2014. We evaluated fecal incontinence in the last 4 weeks using clean intervals (less than 1 day, 1 to 6 days, 1 week or longer, or no fecal incontinence), amount (a lot, medium, a little or none), number of protective undergarments worn daily and similar variables for urinary incontinence. Validated instruments were used, including QUALAS-A (Quality of Life Assessment in Spina bifida for Adults) for spina bifida specific health related quality of life and the generic WHOQOL-BREF (WHO Quality of Life, short form). Linear regression was used (all outcomes 0 to 100). RESULTS: Mean age of the 518 participants was 32 years and 33.0% were male. Overall, 55.4% of participants had fecal incontinence, 76.3% had urinary incontinence and 46.9% had both types. On multivariate analysis fecal incontinence was associated with lower bowel and bladder health related quality of life across all amounts (-16.2 for a lot, -20.9 for medium and -18.5 for little vs none, p <0.0001) but clean intervals were not significant (-4.0 to -3.4, p ≥0.18). Conversely, health related quality of life was lower with increased amounts of urinary incontinence (-27.6 for a lot, -18.3 for medium and -13.4 for little vs none, p <0.0001). Dry intervals less than 4 hours were not associated with lower health related quality of life (-4.6, p = 0.053) but the use of undergarments was associated with it (-7.5 to -7.4, p ≤0.01). Fecal incontinence and urinary incontinence were associated with lower WHOQOL-BREF scores. CONCLUSIONS: Fecal incontinence and urinary incontinence are independent predictors of lower health related quality of life in adults with spina bifida. Health related quality of life is lower with an increasing amount of urinary incontinence. Fecal incontinence has a more uniform impact on health related quality of life regardless of frequency or amount.

Quantity, Not Frequency, Predicts Bother with Urinary Incontinence and its Impact on Quality of Life in Adults with Spina Bifida.

PURPOSE: The effects of urinary incontinence on health related quality of life in adults with spina bifida is poorly understood. We determined which quantification method best captures bother with urinary incontinence. We also quantified the impact of urinary incontinence on health related quality of life. MATERIALS AND METHODS: We surveyed an international sample of adults with spina bifida online from January 2013 through September 2014. We evaluated dry intervals (4 hours or greater considered social continence), quantity of urinary incontinence (a lot, medium, little, none) and number of undergarments worn daily (pads, pull-ups or disposable underwear). A 5-point Likert bother scale was used. We applied validated instruments, including QUALAS-A (Quality of Life Assessment in Spina Bifida for Adults) and the generic WHOQOL-BREF (WHO Quality of Life). We analyzed data using linear regression with a range of 0 to 100 for all outcomes. RESULTS: Mean age of the 461 participants was 32 years and 31.0% were male. Overall 26.5% and 51.8% of participants were dry for less than 4 hours and 4 hours or greater, respectively, while 21.7% were always dry. On multivariate analysis worse bother was predominantly determined by the quantity of urinary incontinence (a lot 32.9 and medium 16.2 vs little, p <0.0001) rather than dry intervals less than 4 hours (7.21, p = 0.08) or number of undergarments (-2.2 to 4.2, p ≥0.43). Bladder and bowel health related quality of life was lower with higher quantities of urinary incontinence (a lot -31.2, medium -23.0 and little -17.2 vs none, p <0.0001) but not for dry intervals less than 4 hours (-4.1, p = 0.14). Use of undergarments regardless of number was associated with lower health related quality of life (-10.2 to -15.4, p ≤0.001). Quantity of urinary incontinence was the main predictor of lower WHOQOL-BREF scores. CONCLUSIONS: We report that adults with spina bifida and urinary incontinence have lower health related quality of life than those who are dry. Self-reported quantity of urinary incontinence was the best predictor of bother and health related quality of life.

Mortality after bladder augmentation in children with spina bifida.

PURPOSE: Renal failure has been a leading cause of death for children with spina bifida. Although improvements in management have increased survival, current data on mortality are sparse. Bladder augmentation, a modern intervention to preserve renal function, carries risks of morbidity and mortality. We determined long-term mortality and causes of death in patients with spina bifida treated with bladder augmentation. MATERIALS AND METHODS: We retrospectively reviewed the records of patients with spina bifida who underwent bladder augmentation between 1979 and 2013. Those born before 1972 or older than 21 years at augmentation were excluded. Demographic and surgical data were collected. Outcomes were obtained from medical records, death records and the Social Security Death Index. Fisher exact and Wilcoxon rank-sum tests and Kaplan-Meier plots were used for analysis. RESULTS: Of 888 patients in our bladder reconstruction database 369 with spina bifida met inclusion criteria. Median followup was 10.8 years. A total of 28 deaths (7.6%) occurred. The leading causes of mortality were nonurological infections (ventriculoperitoneal shunt related, decubitus ulcer fasciitis, etc) and pulmonary disease. Two patients (0.5%) died of renal failure. No patient died of malignancy or bladder perforation. Patients with a ventriculoperitoneal shunt had a higher mortality rate than those without a shunt (8.9% vs 1.5%, p = 0.04). CONCLUSIONS: Previously reported mortality rates of 50% to 60% in patients with spina bifida do not appear to apply in children who have undergone bladder augmentation. On long-term followup leading causes of death in patients with spina bifida after bladder augmentation were nonurological infections rather than complications associated with augmentation or renal failure.

Long-term renal functional outcomes after primary gastrocystoplasty.

PURPOSE: We assessed long-term renal function, morbidities and mortality in 50 patients who had undergone primary gastrocystoplasty at our institution. MATERIALS AND METHODS: We retrospectively reviewed patients 21 years or younger who had undergone primary gastrocystoplasty between 1984 and 2004. Patients who underwent secondary gastrocystoplasty or primary composite augmentation or had cloacal exstrophy were excluded. Primary outcome was progression to end-stage renal disease. Secondary outcomes included mortality, bladder malignancy, hematuria-dysuria syndrome, electrolyte abnormalities and surgical revisions. RESULTS: Of 50 patients who had undergone gastrocystoplasty 35 met inclusion criteria. Median age was 9.4 years and 60% of the patients were male. Median followup was 19 years (IQR 11 to 25). Of the 35 patients 15 (43%) had normal preoperative estimated glomerular filtration rate and 5 (14%) had stage 2, 10 (29%) stage 3 and 5 (14%) stage 4 chronic kidney disease. Five of the 15 patients with stage 3 or 4 chronic kidney disease improved to normal estimated glomerular filtration rate, 1 remained with stage 3 disease and 9 progressed to end-stage renal disease. In 1 patient with normal estimated glomerular filtration rate end-stage renal disease developed following an episode of septic shock due to osteomyelitis. Seven patients in the cohort (20%) died, with 1 each dying of ventriculoperitoneal shunt infection, pneumonia, end-stage renal disease, complications of pregnancy and unknown cause, and 2 patients dying of septic shock due to urinary tract infection. There were no bladder malignancies. Hematuria-dysuria syndrome developed in 9 patients (24%). Eight patients (23%) underwent surgical revision. CONCLUSIONS: The majority of patients had preserved or improved renal function after gastrocystoplasty. There were no deaths attributable to gastric augmentation and no bladder malignancies. Approximately a fourth of patients required surgical revision.

Assessing health related benefit after reconstruction for urinary and fecal incontinence in children: a parental perspective.

PURPOSE: We sought to evaluate health related benefit in children undergoing surgical reconstruction for urinary and fecal incontinence from a parental perspective. MATERIALS AND METHODS: A health related benefit instrument was mailed to the parents or guardians of 300 consecutive patients who had undergone reconstruction for urinary and/or fecal incontinence at our institution between 1997 and 2011. We assessed parent reported health related benefit using the validated Glasgow Children's Benefit Inventory and satisfaction with 6 supplemental questions. One-sample t-tests as well as exploratory univariate and multivariate linear regressions were performed for statistical analysis. RESULTS: Response rate was 40.0% at a mean of 5.5 years (range 0.6 to 13.8) after reconstruction. Spina bifida was the most common primary diagnosis (48 patients, 56.5%). Mean total Glasgow Children's Benefit Inventory score and subscores for each domain were positive, indicating an improved health related benefit after reconstruction (all p <0.0001). Certain patients, possibly based on diagnosis and gender, may particularly benefit from reconstruction (p ≤0.04). Parents believed that the bladder augmentation and/or urinary continent catheterizable channel most changed the life of their child but that achievement of stool continence was most important to themselves. Only 17 families (16.2%) required more than 1 month to become comfortable with catheterizations, and 69 patients (68.8%) required less care or no assistance with daily activities after reconstruction. Only 2 parents (1.9%) would be unwilling to consent to the procedure again. CONCLUSIONS: We observed moderate parental satisfaction and parent reported improvement in health related quality of life for children undergoing surgical reconstruction for urinary and fecal incontinence.

QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A): development and international validation of a novel health-related quality of life instrument.

PURPOSE: Health-related quality of life (HRQOL) is important in spina bifida (SB) management. No clinically useful, comprehensive instrument incorporating bladder/bowel domains exists. We aimed to develop and validate a self-reported QUAlity of Life Assessment in Spina bifida for Adults (QUALAS-A). METHODS: We drafted the 53-question pilot instrument using a comprehensive item generation/refinement process. It was administered to an international convenience sample of adults with SB and controls recruited online via social media and in person at outpatient SB clinics (January 2013-September 2014). Final questions were determined by: clinical relevance, high factor loadings and domain psychometrics in an Internal Validation Sample randomly selected from United States participants (n = 250). External validity was evaluated in United States and International External Validation Samples (n = 165 and n = 117, respectively). Adults with SB completed the validated general WHOQOL-BREF and International Consultation on Incontinence Questionnaire (ICIQ). RESULTS: Mean age of 532 participants was 32 years (32.7 % males, 85.0 % Caucasian), similar to 116 controls (p ≥ 0.08). There were 474 online and 58 clinical participants (61.1 % eligible). Face validity and content validity of the 3-domain, 15-question QUALAS-A were established by patients, families and experts. Internal consistency and test-retest reliability were high for all domains (Cronbach's alpha ≥ 0.70, ICC ≥ 0.77). Correlations between QUALAS-A and WHOQOL-BREF were low (r ≤ 0.60), except for high correlations with Health and Relationships domain (0.63 ≤ r ≤ 0.71). Bladder and Bowel domain had a high correlation with ICIQ (r = -0.70). QUALAS-A scores were lower among adults with SB than without (p < 0.0001). QUALAS-A had good statistical properties in both External Validation Samples (Cronbach's alpha 0.68-0.77). CONCLUSIONS: QUALAS-A is a short, valid HRQOL tool for adults with SB.

Cutting for stone in augmented bladders-what is the risk of recurrence and is it impacted by treatment modality?

PURPOSE: Bladder stones are common after bladder augmentation, often resulting in numerous procedures for recurrence. We sought to determine whether surgical technique and stone fragmentation are significant predictors of bladder stone recurrence after bladder augmentation. MATERIALS AND METHODS: We retrospectively reviewed 107 patients treated for first bladder stones at our institution. Patient demographics, details of surgeries, stone therapy and recurrence were reviewed. Kaplan-Meier survival and Cox proportional hazards analysis were used to determine predictors of time to first stone recurrence. RESULTS: Of 107 patients 55.1% were female and 79.4% had neuropathic bladder. Patients underwent augmentation at a median age of 8.0 years (range 2.4 to 22.8) and were followed for a median of 12.4 years (1.8 to 34). Segments used for augmentation included ileum (72.9% of cases), sigmoid (16.8%), cecum/ileocecum (9.4%) and other (ureter, stomach/ileum, 1.8%). Bladder neck procedures were performed in 63.6% of patients and catheterizable channels in 75.7%. First stone surgery occurred at a median of 3.1 years after augmentation (range 5 months to 21.8 years). Endoscopy was used in 66.4% of cases and open cystolithotomy in 33.6%. Overall 47.7% of stones were fragmented. Bladder stones recurred in 47.7% of patients (median recurrence time 9.5 years, range 3 months to 14.7 years). Recurrence risk was greatest in the first 2 years postoperatively (12.1% per patient per year, p = 0.03). Recurrence risk did not change with technique (endoscopic vs open) or fragmentation, even after controlling for surgical and clinical variables. CONCLUSIONS: Bladder stones recurred in almost half of the patients at 9 years postoperatively independent of treatment technique and patient characteristics. As a high risk group, yearly x-ray of the kidneys, ureters and bladder, and ultrasound of the kidneys and bladder are recommended in these patients.

Multicystic dysplastic kidney: is an initial voiding cystourethrogram necessary?

INTRODUCTION: Traditionally, a voiding cystourethrogram (VCUG) has been obtained in patients diagnosed with multicystic dysplastic kidney (MCDK) because of published vesicoureteral reflux (VUR) rates between 10%-20%. However, with the diagnosis and treatment of low grade VUR undergoing significant changes, we questioned the utility of obtaining a VCUG in healthy patients with a MCDK. We reviewed our experience to see how many of the patients with documented VUR required surgical intervention. MATERIALS AND METHODS: We performed a retrospective review of children diagnosed with unilateral MCDK from 2002 to 2012 who also underwent a VCUG. RESULTS: A total of 133 patients met our inclusion criteria. VUR was identified in 23 (17.3%) children. Four patients underwent ureteral reimplant (3.0%). Indications for surgical therapy included breakthrough urinary tract infections (2 patients), evidence of dysplasia/scarring (1 patient) and non-resolving reflux (1 patient). All patients with a history of VUR who are toilet trained, regardless of the grade or treatment, are currently being followed off antibiotic prophylaxis. To date, none have had a febrile urinary tract infection (UTI) since cessation of prophylactic antibiotics. Hydronephrosis in the contralateral kidney was not predictive of VUR (p = 0.99). CONCLUSION: Routine VCUG in healthy children diagnosed with unilateral MCDK may not be warranted given the low incidence of clinically significant VUR. If a more conservative strategy is preferred, routine VCUG may be withheld in those children without normal kidney hydronephrosis and considered in patients with normal kidney hydronephrosis. If a VCUG is not performed the family should be instructed in signs and symptoms of urinary tract infection.

A novel approach to bladder cycling for defunctionalized bladders prior to pediatric kidney transplantation: A case series.

Ensuring a safe reservoir for pediatric renal transplantation into a defunctionalized bladder is a challenge faced by urologists. We present three patients with defunctionalized bladders who initiated bladder cycling utilizing overnight continuous saline infusion via enteral feeding pump for bladder salvage and subsequent transplantation.

A Surprise During "Routine" Orchidopexy: An Extrarenal Wilms Tumor Case Report.

We present the case of a 12-month-old male diagnosed with an extrarenal Wilms tumor found incidentally at the time of inguinal orchiopexy. He was staged and treated according to Children's Oncology Group (COG) protocol, with no evidence for disease at the end of treatment. We review the patient's presentation and treatment course, followed by a review of current literature on extrarenal Wilms tumor and considerations for management.