Parental perspectives on decision-making about hypospadias surgery.

INTRODUCTION: Many parents who choose hypospadias repair for their son experience decisional conflict and regret. The utilization of a shared decision-making process may address the issue of decisional conflict and regret in hypospadias repair by engaging both parents and physicians in decision-making. OBJECTIVE: The objective of this study was to develop a theoretical framework of the parental decision-making process about hypospadias surgery to inform the development of a decision aid. STUDY DESIGN: We conducted semistructured interviews were conducted with parents of children with hypospadias to explore their role as proxy decision-makers, inquiring about their emotions/concerns, informational needs, and external/internal influences. Interviews were conducted until no new themes were identified, analyzing them iteratively using open, axial, and selective coding. The iterative approach entails a cyclical process of conducting interviews and analyzing transcripts while the data collection process is ongoing. This allows the researcher to make adjustments to the interview guide as necessary based on preliminary data analysis in order to explore themes that emerge from early interviews with parents. Grounded theory methods were used to develop an explanation of the surgical decision-making process. RESULTS: Sixteen mothers and one father of seven preoperative and nine postoperative patients (n = 16) with distal (8) and proximal (8) meatal locations were interviewed. Four stages of the surgical decision-making process were identified: (1) processing the diagnosis, (2) synthesizing information, (3) processing emotions and concerns, and (4) finalizing the decision (Extended Summary Figure). Core concepts in each stage of the decision-making process were identified. Primary concerns included anxiety/fear about the child not waking up from anesthesia and their inability to be present in the operating room. Parents incorporated information from the Internet, medical providers, and their social network as they sought to relieve confusion and anxiety while building trust/confidence in their child's surgeon. DISCUSSION: The findings of this study contribute to our understanding of decision-making about hypospadias surgery as a complex and multifaceted process. The overall small sample size is typical and expected for qualitative research studies. The primary limitation of the study, however, is the underrepresentation of fathers, minorities, and same-sex couples. CONCLUSIONS: This study provides an initial framework of the parental decision-making process for hypospadias surgery that will inform the development of a decision aid. Future stages of decision aid development will focus on recruitment of fathers, minorities, and same-sex couples in order to enrich the perspectives of our work.

Community engagement of adolescents in the development of a patient-centered outcome tool for adolescents with a history of hypospadias repair.

INTRODUCTION: Hypospadias may lead to long-term issues with urination, sexual function and psychosocial well-being. Limited evidence exists regarding the healthcare communication preferences of male adolescents regarding sensitive topics. OBJECTIVE: The purpose of this qualitative study was to explore the healthcare communication preferences of male adolescents regarding sensitive topics (e.g. urinary and sexual issues) and engage them in the initial stages of development of a patient-centered outcome tool for adolescents with a history of hypospadias repair. STUDY DESIGN: A multidisciplinary team with communication design expertise, pediatric urology experts, and health services researchers developed a self-reported toolkit for adolescent patients who had hypospadias repair as children. The toolkit featured short writing/diagramming exercises and scales to facilitate participant reflections about genital appearance, urination, sexual function, and psychosocial well-being. We recruited students from two local high schools for two focus groups to obtain feedback about the usability/acceptability of the toolkit's appearance/content. We inquired about language preferences and preferred format and/or setting for sharing sensitive information with researchers. The focus groups were audio recorded, professionally transcribed, checked for accuracy, and analyzed by two coders using qualitative content analysis. Major themes and subthemes were identified, and representative quotes were selected. RESULTS: We conducted two focus groups in January 2018 with 33 participants, aged 14-18 years. Participants preferred language that would make patients feel comfortable and serious, clinical language rather than slang terms/sexual humor (Extended Summary Table). They recommended avoidance of statements implying that something is wrong with a patient or statements that would pressure the patient into providing answers. They suggested fill-in-the-blank and open-ended responses to encourage freedom of expression and colorful graphics to de-emphasize the test-like appearance of the toolkit. Most participants preferred a toolkit format to a one-on-one interview to discuss sensitive topics such as urinary or sexual issues. Participants would prefer either a male interviewer or would like to have a choice of interviewer gender for individual qualitative interviews, and they recommended a focus group leader with a history of hypospadias repair. DISCUSSION: This study provides a rich description of a group of male high school students' experiences with healthcare providers and researchers. Its qualitative design limits generalizability, and our findings may not be similar to those of adolescents with a history of hypospadias repair. CONCLUSION: We used focus group feedback on the toolkit prototype to refine the tool for use in a future study of adolescents with a history of hypospadias repair.

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.

Pediatric sacral nerve stimulator explanation due to complications or cure: a survival analysis.

INTRODUCTION: Historically, there have been few treatment options for children with severe refractory bladder and bowel dysfunction (BBD). Sacral neuromodulation (SNM) continues to show promising results in this challenging pediatric population with recalcitrant lower urinary tract symptoms. At the authors institution, they have begun offering explantation to those with persistent improvement after >6 months of having device turned off. The authors hypothesized that (1) SNM explantation for cure increases with extended follow-up and (2) those explanted for cure would have improved symptoms and quality of life when compared to those explanted for complication. MATERIALS & METHODS: The authors retrospectively reviewed all consecutive patients aged <18 years who underwent SNM placements at their institution (2012-2017). They excluded those without the second stage procedure. Reasons for device explantation were categorized as cure (resolution of symptoms with the device turned off for at least 6 months) or a complication (e.g. infection, need for magnetic resonance imaging, or pain). Non-parametric tests and survival analysis were used for analysis to account for differential follow-up time. Of those explanted, surveys were electronically sent to assess BBD severity and overall quality of life. RESULTS: Of 67 children who underwent a first stage procedure, 62 (92.5%) underwent a second stage procedure. 61 met inclusion criteria (68.9% female, 29.5% with previous filum section, median age at implantation 10.3 years). During follow-up (median 2.3 years), 12 patients (19.7%) had the SNM exchanged/revised because of lead fracture/breakage and return of urinary symptoms. To date, 50 patients remain with their SNM implanted, and 11 have been explanted. Adjusting for follow-up time, the risk of explantation was 6.5% at 2 years (2.2% for cure, 4.3% for complications) (Figure 1). Explantation increased to 24.5% at 3 years (16.5% for cure, 8.0% for complications) and 40.4% at 4 years (32.4% for cure, 8.0% for complications). Questionnaires were collected on patients after explant (median 2.2 years), with improvement in those explanted for cure compared to complication (Figure 2). DISCUSSION: Sacral neuromodulation explantation for cure is a novel concept previously not described in the literature. Limitations of this study include the relatively small numbers and lack of objective data in the cohort that remains with SNM device implanted. CONCLUSION: Sacral neuromodulation is a safe, viable option for the pediatric patient with refractory bladder dysfunction. Furthermore, SNM explantation for cure is an option with increasing likelihood after 2 years.

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.

What about my daughter's future? Parental concerns when considering female genital restoration surgery in girls with congenital adrenal hyperplasia.

PURPOSE: The parental decision-making process regarding female genital restoration surgery (FGRS) for girls with congenital adrenal hyperplasia (CAH) is controversial and poorly understood. The aim of the study aim was to evaluate parental concerns related to their child's future and parental plans about disclosure prior to FGRS. MATERIALS AND METHODS: The authors performed an online survey of consecutive parents presenting at a tertiary referral center for consultation regarding FGRS for their daughter with CAH before 3 years of age (2016-2018). Twenty issues initially identified by three families and six clinicians were rated on a 6-point Likert scale of importance ('not at all' to 'extremely'). RESULTS: Sixteen consecutive families participated (Prader 3/4/5: 43.8%/43.8%/12.5%). Fourteen girls (87.5%) subsequently underwent FGRS at a median age of 8 months. Most issues (19/20, 95.0%) were ranked 'quite a bit' to 'extremely' important (Table). Top issues were not surgical: Normal physical/mental development, adrenal crisis and side-effects of medications. Surgery-related and self-image concerns followed in importance. Least prioritized issues were multiple genital exams ('quite a bit' important) and the child not being involved in the decision to proceed with FGRS ('somewhat' important). On average, no issues were considered 'not at all' or 'a little' important. Disclosure of FGRS to their daughter was the 15th prioritized issues. Almost all families (93.8%, 1 unsure) planned to disclose the surgery to their daughter, although many were unsure when and how to do it (33.3% and 37.5%, respectively). COMMENT: Initial efforts to understand the complex process of parental decision-making regarding FGRS in the context of CAH, a complex, multifactorial disease, are presented. Parents of infant girls with CAH simultaneously weigh multiple life-threatening concerns with a decision about FGRS. While issues of genital ambiguity and surgery are important, they are not overriding concerns for parents of girls with CAH. Parents report significant uncertainty about appropriate timing and approach to disclosing FGRS to their daughters. Unfortunately, best practice guidelines for this process are lacking. The findings are not based on actual history of disclosure but on parents' anticipated behavior. Further data are need from parents, children, and women with CAH about successful disclosure. Being a single-center series, these data may not correspond to the wider CAH community. CONCLUSIONS: Parental decision-making regarding FGRS is multifactorial. Even when considering FGRS, parents' largest concerns remain focused on the life-threatening and developmental effects of CAH and side-effects of its medical treatment. The disclosure process deserves further attention.

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.

Is surgical antibiotic prophylaxis necessary for pediatric orchiopexy?

INTRODUCTION: Surgeons frequently use surgical antibiotic prophylaxis (SAP), despite limited evidence to support its efficacy. Potential adverse events associated with antibiotic use include allergic reaction (including anaphylaxis), Clostridium difficile infection, and selecting for resistant bacteria. Surgical site infections (SSI) are very rare in patients undergoing clean pediatric urologic procedures. Current guidelines are unclear about the efficacy of surgical antibiotic prophylaxis for prevention of SSI in the pediatric population. OBJECTIVE: It was hypothesized that children who received SAP prior to orchiopexy would have no reduction in surgical site infection (SSI) risk but an increased risk of antibiotic-associated adverse events. METHODS: A retrospective cohort study was conducted of all males aged between 30 days and 18 years who underwent an orchiopexy (ICD-9 CM 62.5) in an ambulatory or observation setting from 2004 to 2015 using the Pediatric Health Information System database. Inpatients and those with concomitant procedures were excluded. Chi-squared or Fisher's exact tests were used to determine the association between SAP and allergic reaction (defined as a charge for epinephrine or ICD-9 diagnosis code for allergic reaction on the date of surgery) and any of the following within 30 days: SSI, hospital readmission or any repeat hospital encounter. Mixed effects logistic regression was performed, controlling for age, race, and insurance, and clustering of similar practice patterns by hospital. RESULTS: A total of 71,767 patients were included: median age was 4.6 years, 61.4% were white, and 49.3% had public insurance; 33.5% received SAP. Of these participants, 996/71,767 (1.4%) had a perioperative allergic reaction and <0.1% were diagnosed with an SSI. On mixed effects logistic regression, those who received SAP had 1.2 times the odds of a perioperative allergic reaction compared with those who did not receive SAP (P = 0.005). Surgical antibiotic prophylaxis was not associated with decreased rates of SSI, lower hospital readmission, nor a lower chance of a repeat encounter within 30 days. CONCLUSIONS: In patients undergoing orchiopexy, it was found that SAP did not reduce the risk of postoperative SSI, readmissions, or hospital visits. Patients who received SAP had significantly increased odds of perioperative allergic reaction. This demonstrated that the risks of SAP outweigh the benefits in children undergoing orchiopexy.

Do anterior catheterizable urinary channels have fewer complications than posterior channels? An international cohort study.

OBJECTIVE: Appendicovesicostomy (APV) and Monti ileovesicostomy (Monti) are durable catheterizable channels. While subfascial revision rates vary by channel type, a channel implanted in the anterior (vs posterior) aspect of the bladder may have a lower subfascial revision risk, due to decreased channel mobility and better fascial fixation. The present study aimed to compare long-term durability of anteriorly compared to posteriorly implanted APV and Monti channels in a large international cohort. MATERIALS AND METHODS: A retrospective cohort study was conducted on patients aged ≤21 years and who underwent APV or Monti surgery with an open technique at three high-volume centers (1990-2015). The following were noted: patient demographics, stomal and subfascial revisions, stomal location, channel placement (anterior/posterior), and channel type - APV, spiral Monti to umbilicus (SMU), other Monti channels. Survival analysis and Cox proportional hazards regression were used to separately examine the three channel groups. RESULTS: Of the 675 patients who met inclusion criteria, 387 had an APV (71.3% anterior), 53 had an SMU (13.2% anterior) and 235 had other Monti channels (42.1% anterior). Median age at surgery was 8.8 years for APV (median follow-up: 5.5 years), 9.2 years for other Monti (follow-up: 6.6 years) and 7.9 years for SMU (follow-up: 9.0 years). Patients originated from the USA (67.9%), Argentina (26.4%) and Chile (5.8%). Overall, 76 stomal and 77 subfascial revisions occurred. Risk of stomal revision was 9.3-12.0% at 5 years of follow-up, and was similar between channel types or location (P = 0.57). Risk of subfascial revision at 5 years was 7.4% for APV, 12.7% for all other Monti channels and 25.9% for SMU (P = 0.001). On survival analysis, stomal and subfascial revision rates were similar between anterior and posterior channels for APV (P ≥ 0.16), other Monti channels (P ≥ 0.62) and SMU (P ≥ 0.43) (Summary Fig.). On multivariate regression, channel configuration was not associated with stomal or subfascial revision for APV (P ≥ 0.18) or other Monti channels (P ≥ 0.64). Sex, age, diagnosis, country and stomal location were not associated with revision risk (P ≥ 0.06). DISCUSSION: Contrary to the hypothesis, subfascial revision rates were no different between anterior and posterior channels. Given that many reported outcomes related to genitourinary reconstruction occur rarely and require prolonged follow-up, collaborative research in this area should be encouraged. CONCLUSIONS: The study demonstrated durable long-term results with the APV and Monti techniques in an international cohort. Risks of stomal and subfascial complications were not significantly different between anteriorly and posteriorly implanted channels. As previously reported, Monti channels, particularly SMU, were more prone to undergoing subfascial revisions.

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.

Ultrasound classification of solitary renal cysts in children.

INTRODUCTION: Solitary renal cysts are typically incidentally found in children who have undergone renal ultrasound (US). The main concern is a cystic tumor. There is no US-based grading system for children to guide management. OBJECTIVE: To evaluate a US-based, modified Bosniak grading system in order to differentiate between simple (grade I or II) and complex (grade II or IV) renal cysts and guide management in children. STUDY DESIGN: This was a retrospective (2003-2011) study of 212 children (114 females), age range one day to 17 years (mean 8.4 years), with solitary renal cysts diagnosed by US. Two radiologists, who were independent and blinded to clinical information, graded the cysts using the modified Bosniak classification system. In children with more than one year of follow-up US, the change (>10%) in cyst diameter was evaluated. Inter-observer variability (Kappa) was calculated. RESULTS: Radiologists one and two saw simple renal cysts in 96.2-96.6% (204-205/212) of the children. Ten children had complex renal cysts, as rated by either of the radiologists. There was good inter-observer agreement (kappa = 0.65) for simple versus complex cysts. In 20.2% (18/89) of the children, the cysts increased in size. A definitive diagnosis was obtained in 8.5% (18/212) of the children. A cystic tumor (multilocular cystic nephroma) was found in one child (Figure) with a complex cyst (graded III by both radiologists). DISCUSSION: The use of a modified Bosniak classification system to grade renal cysts was found to have good inter-observer variability (kappa = 0.65) in differentiating between simple and complex renal cysts. Using this classification, few (<4%) renal cysts were classified as complex. Cystic tumors are rare and the only cystic tumor (multilocular cystic nephroma) was classified as complex renal cysts by the two radiologists. Growth of simple, solitary renal cyst is common (20.2%) and, therefore, if not associated with other imaging findings, is not an indication for a cystic tumor. There were limitations inherent in the retrospective nature of the study and because only one child had a cystic tumor. CONCLUSION: The modified Bosniak classification system demonstrated good inter-observer agreement, and identified the single tumor as a complex cyst. The vast majority of solitary renal cysts in children are simple and if asymptomatic, they require no other imaging evaluation. Complex renal cysts are uncommon and should be evaluated with a pre-intravenous and postintravenous contrast CT scan to exclude a tumor.