Transitioning young adults with neurogenic bladder-Are providers asking too much?

INTRODUCTION: Significant numbers of young adults with chronic health conditions fail to transition. OBJECTIVE: The aim of the study was to evaluate how ready transitioned urologic patients were for that process. Owing to the cognitive impairments frequently seen with spina bifida (SB), it is hypothesized that these individuals will be less prepared to transition their medical care to adult providers compared with their healthy counterparts. METHODS: Participants included consecutive patients in the transitional SB clinic at the study institution and controls (college students without obvious physical disability or interest in healthcare-related fields aged 18-25 years). Both groups were administered the Transition Readiness Assessment Questionnaire (TRAQ) over a nine-month period. Five TRAQ domains assess 20 skills necessary to transition. Likert scale responses range from 1 "no, I do not know how" to 5 "yes, I always do this when I need to" (which the authors considered appropriate for transitioned patients). Demographics and the number of daily medications taken were collected. Patients and healthy controls were compared using (1) total and domain TRAQ scores, (2) the proportion of non-transitioned skills ("1"), and (3) fully transitioned skills ("5"). Non-parametric statistics were used. RESULTS: Forty-three unique SB patients (30.8% shunted, 46.5% female) and 100 controls were enrolled. Patients with SB were older than controls (21 vs 20 years, p < 0.001). There was no gender difference between groups (p = 0.33). Transitioned patients and college students were fully transitioned only in the "Talking with Providers" domain (Figure). College students performed significantly better than patients in the domains of "Appointment Keeping" (p = 0.04) and "Tracking Health Issues" (p = 0.02). Transitioned patients were less likely to be interested in learning how to perform skills in the domains of "Appointment Keeping" and "Tracking Health Issues" (p < 0.001 for both domains). DISCUSSION: The transition readiness of young adults with SB compared to healthy controls and other youths with chronic health conditions is described. The limitations include the small sample size, potentially limiting generalizability, and cross-sectional nature. CONCLUSION: "Transitioned" patients with SB had lower TRAQ scores in some domains compared to healthy college students, who themselves had scores indicating that they were not fully ready for transition. Increased attention to transition readiness in people with SB is necessary, as even healthy young adults struggle with these tasks and are poorly prepared for transition.

Initial collection of an inadequate 24-hour urine sample in children does not predict subsequent inadequate collections.

INTRODUCTION: Approximately half of adult stone formers submit specimens that are either under or over collections as determined by 24-h creatinine/kg. Previously identified predictors of inadequate collection in adults include female sex, older age, higher body mass index (BMI), vitamin D supplementation, and weekday collection. OBJECTIVE: The objective of this study is to determine risk factors for inadequate 24-h urinary specimen collection in the pediatric population. STUDY DESIGN: A retrospective analysis of all children (<18 years of age) with renal and/or ureteral calculi evaluated at the study tertiary care pediatric center from 2005 to 2015 was performed. Those who had at least one 24-h urinary metabolic profile after a clinical visit for kidney and/or ureteral stones were included; children with bladder stones were excluded. Adequate collections had a urine creatinine of 10-15 mg/kg/24 h. A bivariate analysis of potential factors associated with inadequate collection of the initial urinary metabolic profile, including child demographics, parental socio-economic factors, history of stone surgery, and weekday vs. weekend urine collection, was performed. A mixed-effects logistic regression, controlling for correlation of specimens from the same patient, was also performed to determine whether an initial inadequate collection predicted a subsequent inadequate collection. RESULTS: Of 367 patients, 80 had an adequate collection (21.9%): median age, 13 years (interquartile range, 8-16); 61.1% female; 93.5% white; 19.5% obese; and 13.0% overweight. No parental or child factors were associated with inadequate collection (Summary Table). Of inadequate collections, more than 80% were over collections. In the 175 patients with more than one 24-h urinary specimen collection, the effect of an initial inadequate collection on subsequent inadequate collections was not significant after controlling for the correlation of samples from the same patient (p = 0.8). DISCUSSION: Any parental or child factors associated with the collection of inadequate 24-h urine specimens in children were not found. An initial inadequate collection does not predict subsequent inadequate collections. It was surprising that >80% of the inadequate collections were over collections rather than under collections. Possible explanations are that children collected urine samples for longer than the 24-h period or that stone-forming children produce more creatinine per 24-h period than healthy children due to hyperfiltration. CONCLUSION: Inadequate collections are very common, and the risk factors for them are unclear. A repeat collection would be suggested if the first is inadequate. Further studies must be planned to explore barriers to accurate specimen collection using qualitative research methodology.

The ability of a limited metabolic assessment to identify pediatric stone formers with metabolic abnormalities.

INTRODUCTION: American Urological Association guidelines recommend a urinary metabolic evaluation after the first stone event in all pediatric stone patients. Prior studies identified hypercalciuria and urine hypovolemia as the most common abnormalities in children with urolithiasis. Recent data suggest that hypocitraturia is most prevalent. It was hypothesized that a limited evaluation would detect the majority of clinically significant metabolic abnormalities in pediatric stone formers. MATERIAL AND METHODS: A retrospective analysis of all children (<18 years of age) with renal/ureteral calculi evaluated at the study institution from 2005 to 2015 was performed. Children with ≥ one 24-h urinary metabolic profile after a clinical visit for renal/ureteral calculi were included. Those with bladder stones and those with undercollection or overcollection or missing urinary creatinine were excluded. Demographics and data from the first urinary metabolic profile and stone analyses were collected. The sensitivity, specificity, and positive and negative predictive value (NPV) of a limited urinary metabolic evaluation consisting of four parameters (24-h calcium, citrate, and oxalate and low urinary volume) were compared to a complete urinary metabolic profile. The number and type of metabolic abnormalities that would have been missed with this limited evaluation weredetermined. RESULTS: Of 410 patients, 21 were excluded for age ≥18 years, 13 for bladder stones, 248 for overcollections, 38 for undercollections, and 10 for missing creatinine. This left 80 patients for inclusion: median age 11.4 years, 60% female, and 96.3% white. Of the entire cohort, 69.6% had hypocitraturia, 52.5% had low urine volume, and 22.5% had hypercalciuria. Sensitivity was 87.5%. Specificity could not be calculated because no patients had a normal complete metabolic evaluation. The NPV was zero, and the positive predictive value was 100%, but these are artifacts resulting from the absence of patients with a normal complete metabolic evaluation. Of the 80 patients, 10 had at least one abnormality missed by a limited metabolic evaluation (Table 1). The missed abnormalities were high pH (n = 6), abnormal 24-h phosphorus (low in 1 patient and high in 1 patient), low 24-h magnesium (n = 3), low 24-h potassium (n = 3), and high 24-h sodium (n = 4). DISCUSSION: A limited urinary metabolic evaluation would have detected the vast majority of clinically significant metabolic abnormalities in the study sample. Approximately two-thirds of the study patients submitted inadequate 24-h urine specimens. CONCLUSIONS: A simplified approach to metabolic evaluation in first-time stone formers with a stone analysis available was proposed. This streamlined approach could simplify the metabolic evaluation and reduce health care costs.

Incidence of pathologic postobstructive diuresis after resolution of ureteropelvic junction obstruction with a normal contralateral kidney.

INTRODUCTION: Postobstructive diuresis (POD) after unilateral pyeloplasty or percutaneous nephrostomy (PCN) tube insertion for ureteropelvic junction obstruction (UPJO) in patients with a normal contralateral kidney is not well described. OBJECTIVE: The objective of this study was to determine the incidence and characteristics of POD after relief of unilateral UPJO in patients with a normal contralateral kidney. STUDY DESIGN: Children who underwent a unilateral pyeloplasty or PCN for UPJO from 2010 to 2017 with a normal contralateral kidney were retrospectively reviewed. Postobstructive diuresis was defined as urine output (UO) of >300% of expected UO. Patients with a solitary kidney or those who underwent bilateral pyeloplasty or bilateral PCN tube placement were excluded. RESULTS: Out of 396 children meeting inclusion criteria, seven (1.8%) developed POD (4 after pyeloplasty and 3 after PCN tube placement). Median age at intervention was 1.7 years (range 11 days-18 years); median weight was 11.4 kg (range 3.7-54.2 kg). Postobstructive diuresis was more likely to occur in patients with grade 4 hydronephrosis (3.0%) and larger kidneys and if a PCN tube was placed before pyeloplasty. There was no significant difference in age, gender, kidney laterality, or function between those who developed POD and those who did not. Postobstructive diuresis was managed with additional intravenous fluids and electrolyte monitoring. Median initial postprocedure UO was 5.9 mg/kg/hr (range 3.2-10.0 mg/kg/hr). In five children who underwent PCN in whom UO could be differentiated between kidneys, median initial postprocedure UO was 6.1 mg/kg/hr (range 2.5-9.1 mg/kg/hr) from the affected side and 0.8 mg/kg/hr (range 0.4-0.9 mg/kg/hr) from the unaffected side. The median length of time to resolution of POD was 3 days (range 2-4 days). One patient developed significant acidosis and lethargy that improved with intravenous fluid management. Mild hyponatremia developed in two, hypokalemia in one, hypophosphatemia in one, acidosis in one, and hypoglycemia in 1 patient. DISCUSSION: A low but clinically significant risk of POD occurring after relief of unilateral UPJO in children with a normal contralateral kidney is described. Limitations include retrospective analysis and small sample size due to the rarity of the condition. CONCLUSION: Postobstructive diuresis after decompression of UPJO in patients with a normal contralateral kidney is a rare event (1.8%). However, POD does occur, and patients should be carefully monitored after these procedures given the potential for significant dehydration and electrolyte disturbances.

Early readmission and reoperation characteristics of ambulatory hypospadias repair.

INTRODUCTION AND BACKGROUND: Hypospadias is a common birth defect. It is present in about 34.2 in 10,000 live births in North America. However, few studies have evaluated the immediate reoperation and readmission rates following elective repair. OBJECTIVE: This study aimed to define the rates of readmission and reoperation following elective hypospadias repair, to improve pre-operative counseling on risks of the procedure. STUDY DESIGN: The Pediatric Health Information System (PHIS) was interrogated from 2004 to 2015 for all elective hypospadias repairs (ICD-9-CM code 58.45) performed in the ambulatory surgical setting. The following were then determined: age at initial operation, insurance status, race, presence or absence of readmission or reoperation within 30 days, presence of repeat hypospadias repair (same ICD-9 code), and presence or absence of another urethral operation (ICD-9 CM code 58.XX, excluding 58.45). Mixed effects logistic regression were then performed with dependent variables of 30-day repeat encounter, 30-day emergency department (ED) visit, 30-day readmission, or 30-day reoperation; and independent variables of age, race, ethnicity, and insurance status. RESULTS: The study identified 45,264 hypospadias repairs during 2004-2015 performed in 43 hospitals. Within 30 days of the procedures, 2826 (6.2%) had additional encounters in the ED at the same facilities, and 546 (1.2%) had readmissions. A total of 105 (0.2%) underwent second anesthetic within the first 30 days. With regards to a 30-day repeat encounter, odds of repeat encounter were significantly increased in patients aged <5 years, Black and Asian patients, and those with Medicaid. Of the 4882 repeat encounters, 954 (19.5%) had discharge ICD-9 codes related to the penis, or to postoperative complications in general. DISCUSSION: This study described the epidemiology of clinical events occurring at the same tertiary children's hospital within the first 30 days following more than 45,000 hypospadias repairs. Limitations included a cohort generated from a single set of ICD-9 codes. CONCLUSIONS: Elective hypospadias repair had a low rate of readmission (1.2%) and reoperation (0.2%) within the first 30 days. Patients aged <5 years, of non-white race, Hispanic ethnicity, and on Medicaid had significantly higher odds of 30-day repeat encounters.

A case of base rate bias, or are adolescents at a higher risk of developing complications after catheterizable urinary channel surgery?

OBJECTIVE: Adolescents are considered to be at high risk of developing complications after lower genitourinary tract reconstruction. This perception may be due to base rate bias, where clinicians favor specific information (adolescents with complications), while ignoring more general information (number of total adolescents being followed). The goal of this study was to assess whether age was a true risk factor for subfascial and stomal revisions after continent catheterizable urinary (CCU) channel procedures. MATERIALS AND METHODS: Consecutive patients aged <21 years and who underwent appendicovesicostomy and Monti surgery at the present institution were retrospectively reviewed; demographic and surgical data were collected. Time to subfascial or stomal revision was stratified by age at initial surgery (child: <8, preteen: 8-12, adolescent: 13-17, adult: ≥18 years old) and analyzed with Cox proportional-hazards regression. Secondary analyses included: different age categories at initial surgery (<8, 8-11, 12-15, 16-19, ≥20 years), analyzing age as a continuous and a time-varying covariate. RESULTS: Of the 510 patients with CCU channels (median age at surgery: 7.9 years), 63 (12.4%) had subfascial and 53 (10.4%) had stomal revision (median follow-up: 6.8 years). Median age at subfascial and stomal revision was 11.3 and 10.3 years, respectively. Preteens contributed 33.0% and adolescents contributed 29.3% of the total follow-up time (3263.9 person-years). Over 80% of revisions occurred within 5 years of surgery, regardless of age at initial surgery (P ≥ 0.57) (Summary table). On multivariate analysis, age at initial surgery was not associated with undergoing subfascial (P ≥ 0.62) or stomal revisions (P ≥ 0.69). Montis were 2.1 times more likely than appendicovesicostomies to undergo a subfascial revision (P = 0.03). No other variables were associated with the risk of subfascial or stomal revision (P ≥ 0.11). Secondary analyses provided similar results. DISCUSSION: Since the median age at surgery was 8 years old and most complications occurred within the first 5 years of follow-up, it is not surprising that most revisions occurred in 8-13 year olds. Pediatric urologists appear to base their impression of adolescents being "high risk" on specific information (adolescents having complications), while subconsciously ignoring more general information (adolescents represent a large proportion of patients in follow-up). This study had several limitations: channel complications treated non-surgically (e.g. prolonged catheterization) were not included. The findings may not be generalizable to other genitourinary reconstructive procedures or clinical settings. CONCLUSIONS: While complications were twice as high in Monti channels than appendicovesicostomies, no single age group was at increased risk. The impression that adolescents are a high-risk group appears to represent a base rate bias.

Mobility, hydrocephalus and quality of erections in men with spina bifida.

INTRODUCTION: Little is known about erectile dysfunction (ED) among men with spina bifida (SB). The goal of this study was to determine quality of erections and possible factors affecting erections in this population. It was hypothesized that men who ambulate and do not have a ventriculoperitoneal shunt (VPS) are more likely to have erections sufficient for intercourse. METHODS: An online survey was administered over an 18-month period to men aged ≥18 years and with SB. Participants were recruited through local, national and international SB organizations via social media. Exclusion criteria were: poor English proficiency, not completing the questionnaire or missing information regarding erections, VPS or ambulation. Data were collected on demographics, previous surgeries and function. Ambulatory status was classified using the Hoffer classification (Hoffer et al., 1973). Those able to walk at least at home, with or without crutches/braces, were classified as "ambulators." Erections were assessed using a single question from the validated Expanded Prostate Cancer Index Composite questionnaire (e.g. normal: "firm enough for intercourse"). Logistic regression was used for statistical analysis. RESULTS: The median age of 122 participants was 33 years, 53.3% were ambulators and 70.5% had a VPS. Overall, 41.0% reported normal erections. Ambulators were more likely to report normal erections than non-ambulators (63.1% vs 15.8%, P < 0.001) (Table). Those with and without a VPS reported similar rates of normal erections (37.9% vs 48.6%, P = 0.32). On multivariate analysis, ambulators were more likely to have normal erections (OR ≥8.65, P ≤ 0.001) after correcting for age and VPS status. Age and VPS status were not correlated with normal erections on multivariate analysis (P ≥ 0.32 and P = 0.62, respectively). DISCUSSION: Approximately 59% of men with SB reported ED, with ambulators being far more likely to have normal erections. This suggests that ambulatory status, similar to neurological lesion level, is a confounder of erectile function in the SB population. A limitation of the study was that a single item was used to assess erectile function. Rather than performing a comprehensive analysis of sexual health, the study aimed to gauge the prevalence of normal erections and assess possible risk factors. It did not assess sexual activity, erection duration or ED treatments. However, this is the largest study, to date, on SB and erectile quality with international participants. CONCLUSION: About 40% of men with SB reported normal erections. Ambulatory status, rather than hydrocephalus, appeared to be the primary factor associated with erectile function. Approximately 2/3 of ambulators and 1/6 of non-ambulators reported normal erections.

Ambulatory patients with spina bifida are 50% more likely to be fecally continent than non-ambulatory patients, particularly after a MACE procedure.

INTRODUCTION: While fecal incontinence (FI) affects many patients with spina bifida (SB), it is unclear if it is associated with ambulatory status. OBJECTIVE: To determine if ambulatory status is associated with FI, and a potential confounding variable, in patients with and without a Malone antegrade continence enema (MACE). STUDY DESIGN: This study retrospectively reviewed of patients aged ≥8 years with SB who were enrolled in an international quality of life study at outpatient visits (January 2013 to September 2015). Patients reported FI over the last 4 weeks (strict criteria: any FI/accidents vs no FI). Patients unable to self-report FI due to developmental delay were excluded. Those who were ambulating outdoors with/without braces/crutches were considered community ambulators. Non-parametric tests and logistic regression were used for analysis. RESULTS: A total of 115 patients with a MACE and 57 without a MACE were similar in gender (P = 0.99), ventriculoperitoneal status (P = 0.15) and age (16.0 vs 15.4 years, P = 0.11). Median ages at MACE procedure and follow-up were 7.0 and 8.2 years, respectively, and all used the MACE ≥3x/week. They were less likely to be ambulators (54.8 vs 71.9%, P = 0.03). In patients with a MACE, 64 (55.7%) had total fecal continence, compared with 29 (50.9%) without a MACE (P = 0.62). In the MACE group, ambulators were more likely to be continent compared with non-ambulatory patients (65.1 vs 44.2%, P = 0.04) (Table). Although not statistically significant, a similar difference was observed in the non-MACE group (56.1 vs 37.5%, P = 0.25). In the MACE group, continent and incontinent patients, regardless of ambulatory status, had similar rates of MACE use, additive use and time for MACE completion (P ≥ 0.43). MACE ambulators were more likely to be continent than MACE non-ambulators on multivariate analysis (OR 3.26, P = 0.01). DISCUSSION: This study reported higher than typical FI rates since: (1) it used a stringent definition of total fecal continence; (2) patients without FI were perhaps less likely to participate; and (3) it relied on patient-reported rather than clinician-reported outcomes. This cross-sectional study should not be interpreted as "MACE procedure is ineffective;" this would require a longitudinal study. The present findings may not apply to young children or those with significant developmental delay (patients excluded from the study). CONCLUSIONS: Ambulatory patients with SB are 50% more likely to have total fecal continence on long-term follow-up, particularly after a MACE procedure. Ambulatory status is a significant confounder of FI and should be considered in future analyses.

Long-term follow-up of composite bladder augmentation incorporating stomach in a multi-institutional cohort of patients with cloacal exstrophy.

INTRODUCTION: Composite bladder augmentation, incorporating gastric and bowel segments, has the theoretical advantage of metabolic neutrality while potentially avoiding the morbidities of gastrocystoplasty, such as hematuria-dysuria syndrome. The most common indication for this operation is a paucity of bowel, such as in cloacal exstrophy. Despite several early descriptive studies of this technique, there are no reports, to date, of long-term follow-up in this population. OBJECTIVE: To describe the outcomes of composite bladder augmentation utilizing stomach in a cohort of cloacal exstrophy patients. MATERIALS AND METHODS: A retrospective review of cloacal exstrophy patients who underwent composite bladder augmentation from 1984 to 2006 at two institutions was performed. The incidence of mortality and morbidities related to augmentation was evaluated. RESULTS: Eleven patients with cloacal exstrophy underwent composite bladder augmentation. Median age at initial augmentation was 6.4 years (interquartile range (IQR) 4.4-9.1). Median follow-up was 13.2 years (IQR 11.2-24.6). The Summary table describes the types of composite bladder augmentations. Of the three patients with pre-operative metabolic acidosis, two improved with composite bladder augmentation and one developed metabolic alkalosis. Three developed hematuria-dysuria syndrome: one improved with staged ileocystoplasty, and two had persistent symptoms successfully treated with H2 receptor blockers. Two of 11 developed symptomatic bladder stones. There were no reported bladder perforations, bladder malignancies, conversions to incontinent urinary diversions, or deaths. CONCLUSION: With long-term follow-up, very few patients developed metabolic acidosis/alkalosis after composite bladder augmentation. The composite bladder augmentation will continue to be used in patients with cloacal exstrophy, in order to minimize the impact on the pre-existing short gut in these patients.

Long-term outcomes of the Kropp and Salle urethral lengthening bladder neck reconstruction procedures.

INTRODUCTION: The Kropp and Salle procedures have shown good short-term outcomes for managing neuropathic urinary incontinence. However, few studies have assessed their long-term results. OBJECTIVE: This study aimed to evaluate the long-term outcomes of Kropp and Salle procedures, including: dryness, secondary interventions for incontinence or complications, upper urinary tract changes, and use of urethral catheterizations. STUDY DESIGN: Consecutive patients undergoing Kropp and Salle procedures at the present institution (1983-2012) were retrospectively reviewed. Patients with <1-year follow-up or prior bladder neck (BN) continence procedures were excluded. Data were collected on postoperative dryness per urethra at 4-hourly and 3-hourly intervals, secondary interventions, lab tests, and imaging. Non-parametric tests were used for statistical analysis. RESULTS: Thirty-eight patients had Kropp (K: 30 boys) and 12 had Salle procedures (S: 8 boys). Patients underwent surgery at similar median ages (K: 7.4 vs S: 8.7 years old, P = 0.51) and had similar median follow-up (6.9 vs 10.3 years, P = 0.10). Most patients had myelomeningocele, a prior/concomitant bladder augmentation (K: 73.7%, S: 58.3%) and catheterizable channel (K: 81.6%, S: 50.0%). Differences in all outcomes between Kropp and Salle procedures were statistically non-significant. The majority of patients did not have additional BN procedures for dryness (K: 84.2%, S: 66.7%). Of this group, K: 81.3% and S: 75.0% were dry for ≥4 h, K: 93.8% and S: 87.5% were dry for ≥3 h between catheterizations (Table). Of the minority of patients who underwent additional BN procedures for dryness (K: 15.8%, S: 33.3%), most achieved dryness for ≥4 h (K: 66.7%, S: 100%) and ≥3 h (100% for both). Among patients without an initial catheterizable channel, 57.1% had one subsequently created after a Kropp procedure, and 33.3% after a Salle. Among patients without bladder augmentation, approximately half underwent delayed augmentation (K: 50.0%, S: 40.0%). Ultimately, most patients required a secondary intervention under anesthesia for incontinence or complications (K: 79.0%, S: 66.7%). Few patients developed worsening hydronephrosis, vesicoureteral reflux or renal function (K: 2.6%, S: 0.0%). At the end of follow-up, few patients catheterized per urethra (K: 10.5%, S: 33.3%). DISCUSSION: This was a retrospective study without urodynamic data. Originating from a tertiary center, the results may not apply to other clinical settings. CONCLUSIONS: At the present institution the Kropp and Salle procedures attained similar dryness in 75-81% at 4-hourly intervals and 88-94% at 3-hourly intervals without additional BN procedures. Few patients required subsequent BN procedures to achieve dryness. However, the overall need for secondary procedures during long-term follow-up was high for both procedures.

Is renal scintigraphy necessary after heminephrectomy in children?

INTRODUCTION: Heminephrectomy remains an excellent option for a poorly functioning moiety in a duplicated collecting system. A primary concern during heminephrectomy is the potential for a significant functional loss in the remaining ipsilateral moiety. As the gold standard for the assessment of differential renal function, renal scintigraphy is often used in the postoperative evaluation of children undergoing heminephrectomy. However, this imaging modality is costly, invasive, and associated with exposure to radiation. Doppler renal ultrasound (RUS) avoids these concerns and is able to evaluate for structural and functional abnormalities. OBJECTIVE: The present study sought to compare Doppler RUS to renal scintigraphy in determining the viability of the remaining ipsilateral moiety in children who underwent heminephrectomy for a poorly functioning moiety in a duplicated collecting system. MATERIALS AND METHODS: The institutional database of children who underwent open heminephrectomy for a poorly functioning moiety in a duplicated collecting system between 2006 and 2013 was reviewed. Only children who underwent both a postoperative Doppler RUS and renal scan were included. A blinded pediatric radiologist independently reviewed all Doppler RUS. Vascular flow on Doppler RUS was correlated with the preservation of renal function in the remaining ipsilateral moiety on renal scintigraphy. RESULTS: A total of 29 children were identified for inclusion. Demographic and operative data are provided in Table. The average pre-operative and postoperative differential renal function in the ipsilateral kidney was 41.6% and 38% on renal scintigraphy, respectively, for an average decrease of 3.6% (-18% to +12%). Doppler RUS demonstrated the presence of vascular flow to the remaining ipsilateral moieties of all children after heminephrectomy. Renal scintigraphy confirmed the viability of these moieties in all children. DISCUSSION: The first study comparing Doppler RUS to renal scintigraphy was performed to determine the viability of the remaining ipsilateral moiety after heminephrectomy. While no cases of complete functional loss were observed, an average decrease of 3.6% in the ipsilateral renal function favorably compared with other series of children undergoing open heminephrectomy. The limitations of the study included its retrospective design at a single institution. The interpretation of Doppler RUS by an individual pediatric radiologist may also have lead to interobserver variability and impacted the reproducibility of the study, while the absence of any cases of complete functional loss may have impacted its generalizability. CONCLUSIONS: Doppler RUS is an accurate imaging modality for determining the viability of the remaining ipsilateral moiety after heminephrectomy and may obviate the need for renal scintigraphy.

Current opinions regarding care of the mature pediatric urology patient.

INTRODUCTION: With continued improvements in pediatric urology care of patients with complex congenital genitourinary conditions, many survive into adulthood. This fact has created a challenging situation of transitioning from pediatric to adult care. Establishing long-term follow-up with appropriate specialists is a critical part of a successful transition to adulthood for this population. OBJECTIVE: This study sought to elucidate current practices and opinions regarding the management of adult complex genitourinary patients by pediatric urologists, in order to determine if a consensus for adult care exists. STUDY DESIGN: An anonymous, 15-question online survey was created to address practice patterns and opinions regarding the transition of care of complex genitourinary patients. An invitation to participate was distributed via email to 200 pediatric urologists who were members of the American Urological Association. Complex genitourinary patients were defined broadly as those with a history of: spina bifida, bladder exstrophy, cloacal exstrophy, cloacal anomalies, posterior urethral valves or disorders of sex development. Fisher's exact test was used for analysis. RESULTS: The response rate was 31.0% (62/200). Two-thirds (67.7%) cared for adults with complex genitourinary conditions. Overall, 51.6% of pediatric urologists felt that general urologists best follow adult patients, but only 6.5% recommended this for patients with prior complex genitourinary reconstruction (P < 0.001). Instead, the majority (80.6%) felt that a pediatric or adult urologist with an interest and training in adolescent/transitional urology who routinely performs such procedures would provide optimal care. Follow-up by a primary care physician alone was not recommended. Recommendations did not change if patients had developmental delay or lived independently (P = 0.47 and P = 0.72, respectively). Overall, 69.4% would refer mature complex genitourinary patients to a urologist with interest and training in adolescent/transitional urology, if one was available. However, only 45.2% had such an individual available in their practice (P < 0.001). DISCUSSION: In the present study, the opinions of pediatric urologists regarding optimal providers of long-term follow-up for mature complex genitourinary patients were presented. While the results may not represent the views of the entire pediatric urology community, responses from motivated individuals with a particular interest in transition care may be especially valuable. Although the present study did not outline a mechanism for improving transitional care, it offered valuable information on prevailing opinions in this area. Finally, the opinions of mostly North American Pediatric Urologists were presented, which may not apply to other healthcare settings. CONCLUSIONS: Pediatric urologists appeared to be virtually unanimous in recommending that urologists provide the most appropriate long-term follow-up of patients with congenital genitourinary conditions. Specifically, 80% recommended that patients with prior complex surgical reconstruction be followed by a urologist with specific interest, training and experience in the area of transitional urology. The data suggest that this may be an unmet need of these specialists and may signify the need for specific training in the care of such patients.

A comparison of the Monti and spiral Monti procedures: A long-term analysis.

INTRODUCTION/BACKGROUND: The Monti ileovesicostomy provides an excellent substitution for an appendicovesicostomy when the appendix is unavailable or suitable for use. The spiral Monti is a useful modification to the traditional Monti as it allows creation of a longer channel when needed. In 2007, the short-term outcomes were reported; they compared traditional and spiral Monti in 188 patients with an average follow-up of 43 months. In the present population, a total of 25 subfasical revisions were performed in 21 patients: nine (8.3%) subfascial revisions in the traditional Monti (TM) patients and 12 (15.2%) subfascial revisions in the spiral Monti (SM) patients. The study found an increased risk of subfascial revisions of either TM or SM when the stoma was located at the umbilicus versus right lower quadrant (16.8% vs 6.3%, P < 0.05). On subgroup analysis, this increased subfascial revision rate appeared to be driven by SM channels to the umbilicus rather than other stomal locations, but this trend was not statistically significant. OBJECTIVE: It was hypothesized that with longer follow-up, the spiral Monti would require more subfascial revisions due to progressive lengthening of the channel. STUDY DESIGN: A retrospective chart review was performed for all patients undergoing a traditional Monti (TM) or spiral Monti (SM) procedure at the present institution (1997-2013). Patient demographics, bowel segment used, stomal location, channel or stomal revisions, number of anesthetic endoscopic procedures performed, and indications for revision were reviewed. Kaplan-Meier analysis and Cox proportional hazards modeling was used for analysis. RESULTS: Of the 296 patients identified, 146 had Monti procedures and 150 had spiral Monti procedures (median follow-up 7.7 years). Median age at surgery was 10.6 years. Myelomeningocele was the most common underlying cause of neuropathic bladder, totaling 169 (57.1%) patients. Stomas were located at the umbilicus (106, 35.8%), right lower quadrant (183, 61.8%) and left lower quadrant (seven, 2.4%). Median follow-up for the entire cohort was 7.7 years (range: 1 month-15.7 years). Stomal stenosis rate was 7.4%, and 96.6% of the channels were continent. A total of 87 revisions were performed in 74 patients (25.0%). Of these, 55 were subfascial revisions in 49 patients (16.6%). The umbilical spiral Monti on univariate and multivariate analysis was found to be over twice as likely to undergo subfascial revision. DISCUSSION: The majority of patients with a Monti channel had durable results and did not require further channel surgery with long-term follow-up. Spiral Monti channels to the umbilicus were more than twice as likely to undergo subfascial revision compared to all other Monti channels. Overall, one in three umbilical SM channels required a subfascial revision at 10 years after the initial surgery, compared to one in six of all other Monti channels. The study was limited by being a retrospective, single-center series; however, it does represent the largest series of pure SM and TM patients. It focused only on surgical interventions, thus was likely to underestimate the overall risk of complications, as some complications were managed conservatively. As in all studies, some patients were lost to follow-up and inevitably some of these may have had complications. Correction for this was attempted through survival analysis. CONCLUSION: The present study reported durable and reliable long-term results with Monti and spiral Monti procedures based on a large patient cohort. Spiral Monti to the umbilicus was more than twice as likely to require a subfascial revision.

TNF-alpha neutralization ameliorates obstruction-induced renal fibrosis and dysfunction.

Upper urinary tract obstruction results in tubulointerstitial fibrosis and a progressive decline in renal function. Although several inflammatory mediators have been implicated in the pathophysiology of renal obstruction, the contribution of TNF-alpha to obstruction-induced fibrosis and renal dysfunction has not been thoroughly evaluated. To study this, male Sprague-Dawley rats were subjected to left unilateral ureteral obstruction vs. sham operation. Rats received either vehicle or a pegylated form of soluble TNF receptor type 1 (PEG-sTNFR1) every 84 h. The kidneys were harvested 1, 3, or 7 days postoperatively, and tissue samples were analyzed for TNF-alpha expression (ELISA), macrophage infiltration (ED-1 staining), transforming growth factor-beta(1) expression (ELISA, RT-PCR), collagen I and IV activity (Western Blot, immunohistochemistry), alpha-smooth muscle actin accumulation (immunohistochemistry, Western blot analysis), and angiotensinogen expression (Western blot). In a separate arm, the glomerular filtration rate (inulin clearance) of rats subjected to unilateral ureteral obstruction in the presence of either vehicle or PEG-sTNFR1 was determined. Renal obstruction induced increased tissue TNF-alpha and transforming growth factor-beta(1) levels, collagen I and IV activity, interstitial volume, alpha-smooth muscle actin accumulation, angiotensinogen expression, and renal dysfunction, whereas treatment with PEG-sTNFR1 significantly reduced each of these markers of renal fibrosis. These results demonstrate that TNF-alpha mediates obstruction-induced renal fibrosis and identify TNF-alpha neutralization as a potential therapeutic option for the amelioration of obstruction-induced renal injury.

What is the need for additional bladder surgery after bladder augmentation in childhood?

PURPOSE: Bladder augmentation has revolutionized the care of children with a neuropathic bladder but it remains a major surgical procedure. However, the need for subsequent bladder surgery has not been well defined in a large series with long-term followup. MATERIALS AND METHODS: We retrospectively reviewed the records of the first 500 bladder augmentations performed from 1978 to 2003 at our institution. Charts were reviewed for complications requiring additional surgery, including malignancy, bladder perforation, repeat augmentation, bowel obstruction and bladder calculi. Mean and median followup was 13.3 years. RESULTS: Complications occurred in 169 patients (34%) resulting in a total of 254 surgeries. The cumulative risk of further surgery at the bladder level was 0.04 operations per patient per year of augmentation. Three patients (0.6%) had transitional cell carcinoma, of whom all presented with metastatic disease and died. Bladder perforation occurred in 43 patients (8.6%) with a total of 53 events. Of the patients 16 (3.2%) required laparotomy for bowel obstruction and 47 (9.4%) required repeat augmentation. Bladder stones were treated in 75 patients (15%), who required a total of 125 surgeries. CONCLUSIONS: Bladder augmentation provides immeasurable improvements in quality of life but it requires lifelong dedication from the patient, family and health care providers. While the requirements for additional surgery are not trivial, 66% of our patients have not required any further surgery in the augmented bladder.

Spontaneous bladder perforations: a report of 500 augmentations in children and analysis of risk.

PURPOSE: The spontaneous perforation of an augmented bladder is an uncommon but serious complication. To our knowledge our institution has the largest reported series of bladder augmentations. We examined our data to determine the incidence of spontaneous bladder perforation and to delineate associated risk factors. MATERIALS AND METHODS: We performed a retrospective chart review of 500 bladder augmentation procedures performed during the preceding 25 years with a minimum followup of 2 years. RESULTS: Spontaneous perforations occurred in 43 patients (8.6%), for a total of 54 events. The calculated risk was 0.0066 perforations per augmentation-year at risk. Approximately a third of the cases had perforated within 2 years of surgery, a third between 2 and 6 years postoperatively, and a third at more than 6 years after augmentation. Patients who underwent augmentation between 1997 and 2003 had a higher rate of perforation within 2 years of surgery than those operated on between 1978 and 1987. Increased risk of perforation was observed with the use of sigmoid colon and bladder neck surgery. A decreased risk was associated with the presence of a continent catheterizable channel. CONCLUSIONS: We believe that this large and comprehensive series gives valuable insight into this serious complication. The delineation of these potential risk factors serves as a guide for further discussion and investigation.

TNF-alpha mediates obstruction-induced renal tubular cell apoptosis and proapoptotic signaling.

Obstruction of the upper urinary tract induces a progressive loss in renal mass through apoptotic renal cell death. Although TNF-alpha has been implicated in ischemia-reperfusion-induced apoptotic renal cell death, its role in obstructive renal cell apoptosis remains unknown. To study this, male Sprague-Dawley rats were subjected to left unilateral ureteral obstruction vs. sham operation. Twenty-four hours before surgery and every 84 h thereafter, rats received either vehicle or a pegylated form of soluble TNF receptor type 1 (PEG-sTNFR1). The kidneys were harvested 1, 3, or 7 days postoperatively, and tissue samples were subsequently analyzed for TNF-alpha (ELISA, RT-PCR), Fas ligand (RT-PCR), apoptosis (TUNEL, ELISA), and caspase 8 and 3 activity (Western blot). Renal obstruction induced increased tissue TNF-alpha and Fas ligand mRNA levels, TNF-alpha protein production, apoptotic renal tubular cell death, and elevated caspase 8 and 3 activity, whereas treatment with PEG-sTNFR1 significantly reduced obstruction-induced TNF-alpha production, renal tubular cell apoptosis, and caspase activity. PEG-sTNFR1 did not significantly alter Fas ligand expression. These results demonstrate that TNF-alpha mediates obstruction-induced renal tubular cell apoptosis and proapoptotic signaling and identify TNF-alpha neutralization as a potential therapeutic option for the amelioration of obstruction-induced renal injury.

Radiologic, pathologic and molecular attributes of two types of papillary renal adenocarcinomas.

OBJECTIVE: Most papillary renal tumors are not as aggressive as clear cell carcinomas and thus carry a better prognosis. However, several reports in the literature have demonstrated a subset of patients with papillary tumors that have a more aggressive biology and advanced stage at presentation. We compared several parameters of these subsets of renal tumors in an effort to characterize these lesions. PATIENTS AND METHODS: We reviewed 391 cases of nephrectomies that were performed for cancer over a 20-year period from four institutions. Of these, 41 were documented as papillary adenocarcinomas. We reviewed these cases with respect to stage at presentation, size, vascularity on (computerized tomography) CT scan, histology, and cytokeratin immunohistology. RESULTS: Thirty-two of the lesions presented in the fifth, sixth, seventh and eighth decades of life (Type I), while most of the remaining 9 tumors (Type II) presented in the fourth decade of life, and in more advanced stages. Tumor volumes ranged from 84 cm3 to 1660 cm3. Type I tumors had an average size of 515 cm3 and an enhancement on CT of 36 +/- 4 Hounsfield units, compared with Type II tumors which had an average size of 164 cm3 and an enhancement on CT of 92 +/- 8 Hounsfield units. Type II tumors also had a higher mean Fuhrman score of nuclear pleomorphism than Type I, and a greater expression of cytokeratin. CONCLUSIONS: We found that the more common Type I variant of papillary renal adenocarcinoma was less vascular on CT scan, larger in size, and had a lower amount of nuclear pleomorphism as well as decreased expression of cytokeratin 7. The more aggressive biological variant, Type II, presented in the earlier decades of life, with a smaller, but more vascular, cancer and had a greater nuclear pleomorphism. Nuclear pleomorphism still appears to have the best prognostic assessment. However, other molecular and genetic parameters of these tumors, as well as long-term survival data will be necessary to determine the significance of these findings.

The adolescent varicocele. II: the incidence of hydrocele and delayed recurrent varicocele after varicocelectomy in a long-term follow-up.

OBJECTIVE: To determine, in adolescent boys after varicocelectomy, the incidence of hydroceles, when they develop and whether the development is procedure-related. PATIENTS AND METHODS: The records were retrospectively reviewed of 77 boys who underwent 95 varicocelectomies and had an examination at > or = 6 months after surgery. Fifty-six patients underwent a standard Palomo procedure (45 left and 11 bilateral) and 21 a modified Ivanissevich repair (14 left and seven bilateral). The mean (range) age of the patients at surgery was 14.1 (10-22) years and the mean follow-up 25.5 (6-84) months. RESULTS: Of the 67 Palomo varicocelectomies 19 (24%) were complicated by hydroceles after surgery, compared with four of 28 (14%) Ivanissevich procedures (P = 0.034). Of the Ivanissevich repairs, none of the 14 unilateral repairs developed hydroceles. Three hydroceles (one bilateral and one left) developed in two of seven patients after bilateral varicocelectomy. Of the Palomo repairs, 12 of 45 unilateral repairs were complicated by hydroceles, and seven developed in five of 11 patients after bilateral varicocelectomy. Although more hydroceles developed after bilateral varicocelectomy, there was no significant difference from the unilateral group. However, patients who developed hydroceles after bilateral varicocelectomy were more likely to require hydrocelectomy (P = 0.013, Fisher's exact test), implying that hydroceles developing after bilateral repair tend to be larger. Of the hydroceles, two were detected in the first 6 months after surgery, nine at 6-12 months, three at 13-18 months, five at 19-24 months and four at > 2 years after surgery. Three patients had late varicocele recurrence, i.e. 15, 37 and 76 months after surgery; these patients had not had varicoceles on palpation after surgery at 3, 14 and 63 months, respectively. CONCLUSION: Hydroceles are detected infrequently within 6 months of varicocelectomy, with most occurring after 6 months and even appearing after 3 years. They occur significantly more often after a Palomo repair. More hydroceles develop after bilateral repair regardless of the technique used, but not significantly so. Because hydroceles often develop, a lymphatic-sparing procedure should be used, especially for bilateral repair. Recurrent varicoceles may appear as late as 76 months after varicocelectomy in patients where none had been detected at a mean of 27 months after surgery.