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.

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.

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.

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.

Testicular microlithiasis in children and associated testicular cancer.

PURPOSE: To evaluate the prevalence of testicular microlithiasis (TM) in children who have undergone scrotal ultrasonography (US) and their association with testicular tumors. MATERIALS AND METHODS: This HIPAA-compliant study with waiver of informed consent was approved by the institutional review board. From 2003 to 2012, all patients with scrotal US and report mentioning calcifications or microlithiasis and all patients with testicular tumors from pathology database were identified. US studies were evaluated for the type of TM (classic ≥5 microliths or limited <5 microliths in a single view) and change in follow-up studies if available. Medical charts were reviewed for US indication, underlying medical conditions, and pathologic abnormalities, when available. Fisher exact test was used to analyze the association of testicular tumors and TM. RESULTS: A total of 3370 boys had scrotal US, 83 (2%) of whom had TM or microcalcifications in the report. TM was usually bilateral (n = 62, 75%) and classic (n = 59, 71%) type.TM was significantly less common in those younger than 2 years of age than in older age groups (0.1% vs 3.1%, P < .0001). The most common indication for US was scrotal pain (40 of 83 patients, 48%), and the most common associated medical condition was cryptorchidism (nine of 83 patients, 11%). Testicular tumor was significantly more likely in boys with TM (12% vs 0.3%, P < .01). Five (83%) of six patients with premalignant or benign tumors had a premalignant condition (cryptochydism in two and Peutz-Jeghers syndrome in three). Four patients with TM had malignant testicular tumors, all diagnosed after the age of 16 years. CONCLUSION: TM has a prevalence of 2% in boys who undergo scrotal US. It is most commonly bilateral, classic type, and stable at follow-up studies. There is a significant association of TM and testicular tumors. Malignant tumors were seen only in adolescent boys.

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.

Parental decision regret after pediatric urologic surgeries compared to decisions of everyday life.

INTRODUCTION: Parents are at risk of decision regret (DR) for decisions affecting their children. The Decision Regret Scale (DRS) measures medical DR but lacks context outside of healthcare. OBJECTIVE: To compare parental DR 1) between common pediatric urologic surgeries and everyday decisions and 2) with preference to make a different choice. METHODS: We conducted a cross-sectional online survey of randomly selected parents >1year (y) after their children underwent: orchiopexy (males ≤10y), open ureteral reimplant (OUR, females 2-6y), open pyeloplasty (OP, ≤2y), or robotic pyeloplasty (RP, 5-17y) (2017-2021). Higher DRS scores indicate increased DR (none: 0, mild: 1-25, moderate: 30-50, strong: 55-75, very strong: 80-100). Parents completed DRS on four decisions: their child's surgery, most recent/current romantic relationship, most recent leased/purchased car, and most recent purchased meal. Parents reported if they would make the same choice (yes/no). Nonparametric statistics were used. RESULTS: We surveyed 191 parents (orchiopexy n = 52, OUR n = 50, OP n = 51, RP n = 38). The median parent age was 36y (mothers: 86%). Some DR was reported for all decisions, but with significant differences in DR severity. The lowest median DRS score was seen with surgery (orchiopexy 0 [IQR 0-10], OUR 0 [IQR 0-5], OP 0 [IQR 0-0], RP 0 [IQR 0-0]), with no difference between surgery groups (p = 0.78). This was followed by relationship (0, IQR 0-20), car (15, IQR 0-25), and meal (20, IQR 0-30, p < 0.001). Most parents did not report any DR regarding surgery (orchiopexy 69%, OUR 74%, OP 76%, RP 76%, with no difference between surgery groups p = 0.85, Summary Figure). Comparatively, 59% of parents did not have any regret about their relationship, 37% their car, and 28% their meal (p < 0.001). All surgical DR was mild or moderate. No parent (0%) would have chosen differently for their child's surgery versus 4-12% for non-surgical decisions (p < 0.001). Overall, increasing DR corresponded to increasing desire to have made a different choice (DRS≤10: 0%, DRS 45-50: 32%, DRS 55-60: 66%, DRS≥75: 100%, p < 0.001). CONCLUSION: Parental DR varied between urological surgical and non-surgical decisions. It was lowest after surgery. Some regret was reported after every decision, but the subset of parents with regret was smallest after surgical decisions. Positive DRS scores do not necessarily correspond to parents wishing they made a different choice.

Multi-stakeholder opinion statement on the care of individuals born with differences of sex development: common ground and opportunities for improvement.

BACKGROUND: In the last 15 years, the care provided for individuals born with differences of sex development (DSD) has evolved, with a strong emphasis on interdisciplinary approaches. However, these developments have not convinced some stakeholders to embrace the current model of care. This care model has also paid insufficient attention to socio-cultural differences and global inequalities. SUMMARY: This article is an opinion statement, resulting from in-depth discussions and reflection among clinicians, patients, and family support organizations based in the US and Europe, where we seek areas of common ground and try to identify opportunities to further develop resources. The product of these conversations is summarized in 10 panels. The corresponding sections provide additional discussion on some of the panel items. KEY MESSAGES: Participants identified areas of agreement and gained a deeper understanding of the reasons behind disagreements on certain matters and identified the necessary steps to foster future consensus. We offer preliminary recommendations for guiding clinical management and resource allocation. By promoting a broader consensus, we aim to enhance the quality of care and well-being for individuals of all ages who have a DSD.

People with spina bifida use their MACE on long-term follow-up: A single institutional retrospective cohort study.

OBJECTIVE: While the Malone antegrade continence enema (MACE) facilitates bowel movements in patients with spina bifida (SB) and neuropathic bowel, little is known about its long-term use. We aimed to assess long-term MACE use and potential risk factors for disuse. METHODS: All patients with SB who underwent MACE procedures at our institution were retrospectively reviewed. Main outcome was MACE disuse (no longer catheterizing the MACE for antegrade enemas) based on self-report on a clinic questionnaire, or medical record for patients last seen before introducing the questionnaire 5 years ago. Survival analysis used two timeframes: time after surgery (Analysis 1) and chronological age: accounting for older children reaching adulthood earlier (Analysis 2). RESULTS: Overall, 411 patients (54% female, 78% shunted, 65% augmented) underwent a MACE procedure at median 7.9 years old (median follow-up: 8.4 years). Thirty-three (8%) patients no longer used their MACE. Most common reasons for doing so were channel/stomal stenosis (61%) and excision at colostomy or other abdominal surgery (12%). Bowel management afterwards included oral agents ± enemas (55%), Chait tube (30%), colostomy (12%). After correcting for differential follow-up, 90% of participants used their MACE at 10 years and 87% at 15 years after surgery. Based on chronological age, 97% used their MACE at 15 years old, 92% at 20 and 81% at 30 (Summary Figure). On multivariate analysis, umbilical MACEs were 2.4 times more likely to be disused than right lower quadrant MACEs (p = 0.04). Without correcting for chronological age (Analysis 1), patients undergoing MACE surgery at older ages were more likely to stop MACE use (p = 0.03). However, after accounting for chronological age (Analysis 2), patients undergoing a MACE procedure at older ages were no more likely to stop its use (p = 0.47, Figure). Gender, SB type, shunt status, mobility status, bladder augmentation or a urinary catheterizable channel were not associated with stopping MACE use (p ≥ 0.10). COMMENT: Participants were regularly followed in multi-disciplinary SB clinics. We did not assess continence, satisfaction or long-term urinary channel use, making it premature to recommend optimal stomal locations. CONCLUSIONS: Most patients with SB followed by a multi-disciplinary team continue using their MACE; 1% stopped MACE use annually, particularly after adolescence. This strongly suggests it is an effective bowel management method and transitioning to self-care plays a role in maintaining long-term MACE use. Umbilical MACEs may be at high risk of disuse, but all people with a MACE can benefit from support as they transition to adult care.

Age-based risk of end-stage kidney disease in patients with myelomeningocele.

OBJECTIVE: We aimed to quantify end-stage kidney disease (ESKD) risk after infancy in individuals with myelomeningocele (MMC) followed by urology in the modern medical era and to assess if ESKD risk was higher after surgery related to a hostile bladder. METHODS: We retrospectively reviewed patients with MMC followed by urology at our institution born ≥ 1972 (when clean intermittent catheterization was introduced) past 1 year of age (when mortality is highest, sometimes before establishing urology care). ESKD was defined as requiring permanent peritoneal/hemodialysis or renal transplantation. Early surgery related to hostile bladder included incontinent vesicostomy, bladder augmentation, detrusor Botulinum A toxin injection, ureteral reimplantation, or nephrectomy for recurrent urinary tract infections. Survival analysis and proportional hazards regression were used. Sensitivity analyses included: risk factor analysis with only vesicostomy, timing of surgery, including the entire population without minimal follow-up (n = 1054) and only patients with ≥ 5 years of follow-up (n = 925). RESULTS: Overall, 1029 patients with MMC were followed for a median of 17.0 years (49% female, 76% shunted). Seven patients (0.7%) developed ESKD at a median 24.3 years old (5 hemodialysis, 1 peritoneal dialysis, 1 transplantation). On survival analysis, the ESKD risk was 0.3% at 20 years old and 2.1% at 30 years old (Figure). This was ∼100 times higher than the general population (0.003% by 21 years old, p < 0.001). Patients who underwent early surgery for hostile bladder had higher ESKD risk (HR 8.3, p = 0.001, 6% vs. 1.5% at 30 years). On exploratory analyses, gender, birth year, shunt status and wheelchair use were not associated with ESKD risk (p ≥ 0.16). Thirty-year ESKD risk was 10% after early vesicostomy vs. 1.4% among children without one (p = 0.001). Children undergoing bladder surgery between 1.5 and 5 years old had a higher risk of ESKD. No other statistically/clinically significant differences were noted. COMMENT: Patients with MMC remain at risk of progressive renal damage throughout life. We relied on the final binary ESKD outcome to quantify this risk, rather than imprecise glomerular filtration rate formulas. Analysis was limited by few people developing ESKD, inconsistent documentation of early urodynamic findings and indications for bladder-related surgery. CONCLUSIONS: While ESKD is relatively uncommon in the MMC population receiving routine urological care, affecting 2.1% of individuals in the first 3 decades, it is significantly higher than the general population. Children with poor bladder function are likely at high risk, underlining the need for routine urological care, particularly in adulthood.

Sexual activity and function of adult men with spina bifida.

OBJECTIVE: Data on sexual function of men with spina bifida (SB) is limited. We aimed to assess sexual activity and erectile dysfunction (ED) in a large international sample of men with SB. METHODS: Men with SB (≥18yo) were recruited in an international online survey via clinics and social media. We collected data on demographics, ambulation (Hoffer classification), penile rigidity (Erection Hardness Score), sexual activity and ED (International Index of Erectile Function). Non-parametric tests were used. RESULTS: A total of 162 men (median age 35, 62% shunted, 38% community ambulators) reported sexual desire similar to the general population (p = 0.82), but 55% were dissatisfied with their sex life (Summary Table). Overall, 36% reported full penile rigidity with erections, more commonly with better ambulation (p = 0.01), 69% had ever experienced orgasm and 84% ejaculated. In 44 men (27%) attempting sexual intercourse in the last 4 weeks, 59% had ED (11% severe, 7% moderate, 14% mild-moderate, 27% mild). In this group, 91% of men reporting less than full penile rigidity had ED, compared to 30% with full penile rigidity (p = 0.001). Overall, partnered non-genital contact in the last 3 months was reported by 56%, solo masturbation: 62%, partnered intercourse: 48% (31% vaginal). Of 54 men who used phosphodiesterase type 5 inhibitors (PDE5I), 80% reported improved erections, 56% improved intercourse. Overall, 40% reported non-genital erogenous zones as most pleasurable, especially with poorer ambulation (p = 0.002, chest/nipples: 73%). COMMENT: Strengths of this study include anonymous, voluntary, online participation maximizing participation of a heterogenous, international population. Whenever available, we compared findings to published values for the general population. Since romantic and sexual activity is a complex intersection of interest, opportunity and ability, a more comprehensive assessment was beyond the study's scope. Future work will focus on the interplay with issues like incontinence. CONCLUSIONS: ED was frequent among men with SB, especially in men with poorer ambulation. PDE5 inhibitors may be beneficial. Partnered sexual activity was reported by half of the men, although it may not involve penetrative intercourse.

Interleukin-18 stimulates a positive feedback loop during renal obstruction via interleukin-18 receptor.

PURPOSE: Interleukin-18 is a proinflammatory cytokine that is an important mediator of obstruction induced renal tubulointerstitial fibrosis independent of tumor necrosis factor-α and ß1 activity. We hypothesized that interleukin-18 stimulates a positive feedback loop during obstruction via interleukin-18 receptor to increase interleukin-18 gene expression and protein production. MATERIALS AND METHODS: Male C57BL6 interleukin-18 receptor knockout (The Jackson Laboratory, Bar Harbor, Maine) and control wild-type mice underwent unilateral ureteral obstruction or sham operation and were sacrificed 1 week after surgery. Renal cortical tissue samples were harvested and analyzed for interleukin-18 protein by enzyme-linked immunosorbent assay, and for interleukin-18 and interleukin-18 receptor gene expression by quantitative polymerase chain reaction. The specific cellular localization of interleukin-18 and interleukin-18 receptor expression during obstruction was assessed using dual labeling immunofluorescence staining. RESULTS: Renal interleukin-18 receptor expression increased significantly in wild-type mice in response to obstruction but remained at sham operation levels in interleukin-18 receptor knockout mice. Similarly while interleukin-18 protein and gene expression were significantly increased in wild-type mice in response to obstruction, interleukin-18 levels and gene expression were significantly decreased during obstruction in knockout mice. Obstruction induced interleukin-18 and interleukin-18 receptor production were localized predominantly to tubular epithelial cells and to a lesser extent to the renal interstitium. CONCLUSIONS: Results reveal that interleukin-18 stimulates a positive feedback loop via interleukin-18 receptor during renal obstruction to stimulate interleukin-18 production and gene expression. The predominant cellular source of interleukin-18 production during renal obstruction appears to be tubular epithelial cells rather than infiltrating macrophages.

Nephrogenic adenoma in the augmented bladder.

PURPOSE: Nephrogenic adenoma is an uncommon, benign urothelial lesion. Risk factors for nephrogenic adenoma include trauma, chronic inflammation, immunosuppression and radiation. We characterized nephrogenic adenoma in the pediatric augmented bladder. MATERIALS AND METHODS: We reviewed the records of patients diagnosed with nephrogenic adenoma from January 2000 to March 2010. Those with prior bladder augmentation were studied further. Data were retrospectively obtained on pathological characteristics, cystoscopic findings, recurrence patterns and presentation. RESULTS: Ten patients with ileal bladder augmentation and nephrogenic adenoma were identified. The underlying pathological condition was myelodysplasia in 7 patients, sacral agenesis in 2 and bladder exstrophy in 1. Concomitant procedures were a continent channel in 9 patients, ureteroneocystostomy in 5 and bladder neck reconstruction in 7. Mean time to the discovery of nephrogenic adenoma was 9.2 years. During that time a total of 221 surveillance cystoscopies were performed in patients with bladder augmentation. The diagnosis was made by surveillance cystoscopy in 7 cases, at surgery for bladder stone in 2 and by cystoscopy for urinary incontinence in 1. Nephrogenic adenoma was identified along the floor in 5 cases, near the channel entrance in 3 and adjacent to the enteric anastomosis in 2. Six patients returned for surveillance with recurrence identified in 2. The longest recurrence free interval was 27 months. CONCLUSIONS: Nephrogenic adenoma is not uncommon in the augmented bladder and it is often asymptomatic. The lesions tend to develop at sites that may be prone to chronic catheterization injury. Recurrent lesions are not unusual.

Extravesical implantation of a continent catheterizable channel.

PURPOSE: Extravesical ureteral reimplantation provides results equivalent to those of the open technique with the advantage of less postoperative morbidity from a large cystotomy. Surgical series describing the technique and efficacy of extravesical implantation of continent catheterizable channel are lacking. We reviewed our results to determine the efficacy of this technique with an emphasis on continence and the need for revision. MATERIALS AND METHODS: We reviewed the records of 394 patients who underwent a bladder continent catheterizable channel procedure from 1999 to 2009. Operative records describing an extravesical technique were noted. Briefly, a 3 to 6 cm incision is made in the detrusor and seromuscular flaps are created. The continent catheterizable channel is laid in the tunnel and the flaps are brought anterior to the channel and sutured to each other. Fixation of the bladder wall to the abdominal wall preserves tunnel length and minimizes the risk of angulation. The type of continent catheterizable channel, stomal continence and the need for revision were recorded. RESULTS: The extravesical implantation technique of a continent catheterizable channel was done in 84 of 394 patients (21%). The channel was an appendix in 47 cases and Monti ileovesicostomy in 37. Stomal continence was achieved in 79 of 84 cases (94%). At a mean followup of 45 months 22 patients (26%) required a total of 30 surgical revisions, of which most were skin level or endoscopic procedures at a mean of 26 months after channel creation. CONCLUSIONS: The extravesical technique for continent catheterizable channel implantation is effective. If intravesical surgery is not necessary, avoidance of a large cystotomy and longer operative time may expedite postoperative recovery when using an extravesical implantation technique to create a continent catheterizable channel.