Federal research funding and academic productivity in pediatric urology: from early career to research independence.

INTRODUCTION: Federal grants from the National Institutes of Health (NIH) or Agency for Healthcare Research and Quality (AHRQ) are crucial for early career physician-scientists. Federal funding success has been explored in other surgical specialties, but has not been evaluated in pediatric urology. OBJECTIVE: To characterize factors associated with receipt of federal grants, hypothesizing that pediatric urologists who were have advanced research degree(s) were more likely to be federally funded. STUDY DESIGN: All pediatric fellowship graduates from 1985-2016 listed on the Societies for Pediatric Urology and institutional websites were queried using the NIH Research Portfolio Online Reporting Tools. Primary outcomes were successful receipt of federal grants and transition from mentor-based to independent funding. The secondary outcome was publication rate on PubMed as of November 2017. Covariables included advanced degree(s) (eg, PhD, MPH, MSc, etc), sex, and year of fellowship graduation (1985-2006 versus 2007-2016). RESULTS: Of 445 pediatric urologists (73% male), 36 (8%) were federal grant recipients. Of 18 mentor-based awardees, 9 (50%) transitioned to independent awards. After adjusting for sex and year of fellowship graduation, having an advanced degree(s) was associated with funding success for mentor-based awards (hazard ratio [HR] 3.83 [95% confidence interval, 1.21-12.14], p = 0.02; Summary Table) and independent awards (HR 3.11 [1.21-8.02], p = 0.02), and with higher publication rates (incident rate ratio [IRR] 2.03 [1.43-2.87], p < 0.001). Recent training (2007-2016) was also associated with higher publication rates (IRR 2.70 [2.16-3.37], p < 0.001). DISCUSSION: Among fellowship-trained pediatric urologists in North America between 1985 and 2016, the prevalence of federal grant recipients was 8%. Pediatric urologists who had an advanced educational degree were more likely to be a federally funded grant recipient and have a higher publication rate. CONCLUSIONS: Fellowship programs should consider adding opportunities for self-selected applicants to pursue additional research training and degrees.

Uncharted territory: navigating the pediatric urology job market.

INTRODUCTION: There is not a structured algorithm, timeline, or resource on how, when, and which programs are looking for a new staff pediatric urologist. We hypothesized that current and future pediatric urology fellows could benefit from the experiences of recently graduated fellows on navigating the urology job market. OBJECTIVE: The purpose of this study was to survey current and recent pediatric urology fellowship graduates about the process of finding their first job. STUDY DESIGN: A Research Electronic Data Capture (REDCap) online survey was designed and distributed through email to current fellows and 2012-2017 graduates. Results were evaluated using STATA. RESULTS: 153 participants were emailed, and 94 (61%) completed the survey. The plurality of applicants (44%) began the job search at the Spring American Urological Association meeting, 14 months before finishing (Figure). Of those who started 14 months before finishing, 33% wished they started earlier and 61% would have started at the same point. The median number of programs contacted for a position was 3-4 and the median number of programs visited and offers received was two. After the offer, 40% reported having >8 weeks to decide. Less than half (38.5%) hired an attorney for contract negotiation. Of those who hired an attorney, 68% felt it provided benefit. Regarding contract negotiation, 22% did not negotiate and 35% negotiated for salary. Unsurprisingly, 28% of those who took academic jobs thought negotiating for protected research/educational time was most important compared with only 4% of those who took non-academic jobs (P = 0.02). When asked how they learned about the job they accepted: 28% were contacted by the program, 25% cold called the program, 30% accepted where they did residency or fellowship, and 18% learned through society websites. The plurality (50%) thought the number of desirable positions during the process were as they expected. 41% however, thought the number of desirable positions were expectedly or surprisingly low. Regarding quality of life and satisfaction with job/career choice, 98% stated that they would still choose to subspecialize in pediatric urology. CONCLUSIONS: The results from this survey should provide guidance to fellows on how to approach the job search with respect to timing, expectations, contract negotiation, and initial job satisfaction.

The changing gender landscape of pediatric urology fellowship: results from a survey of fellows and recent graduates.

INTRODUCTION: Women are entering the subspecialty of pediatric urology at an accelerated rate. Gender differences affecting fellowship and job selection have been identified in other fields of medicine. OBJECTIVE: The objective of this study was to understand gender differences in pediatric urology fellowship and job selection and how they may affect the workforce. STUDY DESIGN: A 47-question electronic survey consisting of questions regarding demographics, residency training, and factors influencing fellowship and job selection was distributed to current fellows and recent graduates in pediatric urology in May 2017. RESULTS: A total of 111 recent and current fellows were contacted, and 72% completed the survey (55% female [F] and 45% male [M]; 61% current fellows and 39% recent fellows). Respondents rated factors important in choosing pediatric urology on a scale of 1-5 (1, not important and 5, extremely important), and the top three for both genders were 1-working with children, 2-influential mentors, and 3-bread and butter cases such as inguinal orchiopexy. During residency, 93% of respondents reported having influential mentors in pediatric urology. However, mentorship was more important in fellowship choice for males than females (3.6 F, 4.1 M; P-value = 0.048), and 45% reported having only male mentors. Rating factors important in job choice on a scale of 1-5, respondents reported the top factors as 1-rapport with partners/mentorship (4.5), 2-geography/family preferences (4.3), and 3-participation in mentoring/teaching (3.8). Although most job selection criteria were rated similarly between genders, females rated call schedule higher than males (3.5 F, 2.9 M, P-value = 0.009). Although most females and males (79% of F, 78% of M, P-value = 0.868) sought primarily academic positions, a smaller proportion of females accepted academic positions (52% of F, 72% of M, P-value 0.26), and females reported lower satisfaction regarding the availability of jobs on a scale of 1-5 (1, very dissatisfied and 5, very satisfied; 3.1 F, 3.7 M; P-value = 0.034), particularly in academic positions (3.1 F, 3.7 M; P-value = 0.06). This difference was more pronounced in current fellows than recent graduates and may represent a worsening trend. CONCLUSION: Although significant gender differences in fellowship and job selection may exist in other fields, we found that women and men choose pediatric urology fellowships and jobs using similar criteria, which include work-life balance. Gender differences exist in the influence of mentors, indicating a need for more female mentors. While men and women sought similar types of jobs, women were less satisfied with the availability of jobs, particularly academic jobs, than men, which warrants further investigation.

Improving surgical training by identifying the most common feedback trainees receive for index cases.

BACKGROUND: To make surgical training more effective, a proven method is needed to provide feedback to residents on their surgeries. Residency programs may make up for limited training time in the operating room by improving feedback that trainees receive about cases. OBJECTIVE: The goals of this study were (1) to determine if an online tool to communicate feedback for attendings and trainees shows face validity and (2) to use an online tool to identify the most common feedback trainees receive after performing orchiopexy and hypospadias repair by survey. METHODS: In 2016, determining whether an online tool to provide pediatric urology trainees feedback after surgery shows face validity begun. The tool was launched at the authors' institutions. Then, attendings, fellows, and postgraduate year 4-5 trainees of 65 resident training programs were surveyed for their observations on preparing for and performing orchiopexy and hypospadias repair using the study tool to identify common feedback. RESULTS: The results of using the tool to provide feedback shows face validity are as follows: feedback was exchanged between attendings and trainees on orchiopexy (n = 28) and hypospadias (n = 22). Anecdotally, the tool was easy to use. The results of using the tool to identify the most common feedback trainees receive by survey are as follows: from a pool of 65 institutions, 37 attendings and 28 trainees were enrolled who made 219 observations. Most trainees prepare using undocumented online resources (17/28, 67%) instead of speaking with their attendings or cotrainees (11/28, 33%). For orchiopexy, most respondents reported that trainees need to improve skills for hernia ligation (observations: attending = 28/45, 62%; and trainee = 17/26, 65%) and strategies for hernia exposure (observations: attending = 17/27, 62%; and trainee = 7/12, 58%). For hypospadias, most respondents reported that trainees need to improve skills for neourethroplasty (observations: attending = 31/53, 58%; and trainee = 10/16, 62%) and strategies for repair choice (observations: attending = 15/22, 68%; and trainee = 12/18, 67%) (chi-squared, all P = NS). DISCUSSION: It was shown that both trainees and attendings agree on the areas of surgical strategy and execution which require improvement. With this study, it is also shown that the online feedback tool developed shows face validity in allowing attendings and trainees to communicate before and after surgery. CONCLUSIONS: The most common feedback pediatric urology trainees receive for routine pediatric urology surgery is identified. Online tools that emphasize remediations to address a trainee's specific feedback needs are to be built, so that they will be able to improve their skills at their next case.

Preliminary report: Surgical outcomes following genitoplasty in children with moderate to severe genital atypia.

INTRODUCTION: Prior studies of outcomes following genitoplasty have reported high rates of surgical complications among children with atypical genitalia. Few studies have prospectively assessed outcomes after contemporary surgical approaches. OBJECTIVE: The current study reported the occurrence of early postoperative complications and of cosmetic outcomes (as rated by surgeons and parents) at 12 months following contemporary genitoplasty procedures in children born with atypical genitalia. STUDY DESIGN: This 11-site, prospective study included children aged ≤2 years, with Prader 3-5 or Quigley 3-6 external genitalia, with no prior genitoplasty and non-urogenital malformations at the time of enrollment. Genital appearance was rated on a 4-point Likert scale. Paired t-tests evaluated differences in cosmesis ratings. RESULTS: Out of 27 children, 10 were 46,XY patients with the following diagnoses: gonadal dysgenesis, PAIS or testosterone biosynthetic defect, severe hypospadias and microphallus, who were reared male. Sixteen 46,XX congenital adrenal hyperplasia patients were reared female and one child with sex chromosome mosaicism was reared male. Eleven children had masculinizing genitoplasty for penoscrotal or perineal hypospadias (one-stage, three; two-stage, eight). Among one-stage surgeries, one child had meatal stenosis (minor) and one developed both urinary retention (minor) and urethrocutaneous fistula (major) (Summary Figure). Among two-stage surgeries, three children developed a major complication: penoscrotal fistula, glans dehiscence or urethral dehiscence. Among 16 children who had feminizing genitoplasty, vaginoplasty was performed in all, clitoroplasty in nine, external genitoplasty in 13, urethroplasty in four, perineoplasty in five, and total urogenital sinus mobilization in two. Two children had minor complications: one had a UTI, and one had both a mucosal skin tag and vaginal mucosal polyp. Two additional children developed a major complication: vaginal stenosis. Cosmesis scores revealed sustained improvements from 6 months post-genitoplasty, as previously reported, with all scores reported as good or satisfied. DISCUSSION: In these preliminary data from a multi-site, observational study, parents and surgeons were equally satisfied with the cosmetic outcomes 12 months after genitoplasty. A small number of patients had major complications in both feminizing and masculinizing surgeries; two-stage hypospadias repair had the most major complications. Long-term follow-up of patients at post-puberty will provide a better assessment of outcomes in this population. CONCLUSION: In this cohort of children with moderate to severe atypical genitalia, preliminary data on both surgical and cosmetic outcomes were presented. Findings from this study, and from following these children in long-term studies, will help guide practitioners in their discussions with families about surgical management.

Lipomyelomeningocele for the urologist: Should we view it the same as myelomeningocele?

INTRODUCTION: The primary urologic objectives for lipomyelomeningocele (LMM) and myelomeningocele (MM) are preserving renal integrity and achieving continence. Due to this common ground, LMM and MM are urologically treated the same. However, unlike MM, LMM may present with no evident functional concerns. Indications for and timing of tethered cord release (TCR) in LMM are therefore controversial. Long-term urologic outcomes are not well defined. OBJECTIVE: Expectations for continence and potential for intermittent catheterization (CIC) following TCR in LMM are important for realistically counseling families regarding future needs. The present study aimed to identify prognostic factors for continence and need for CIC in LMM. STUDY DESIGN: The present study retrospectively identified 143 patients from the multidisciplinary clinic who underwent TCR for LMM between 1995 and 2010. Concomitant anorectal/genitourinary anomalies, filar lipoma, fatty filum, previous TCR, and follow-up <1 year were excluded. Analysis was limited to those toilet trained or aged ≥6 years at latest follow-up. Lipomyelomeningocele was classified as dorsal, distal, transitional or chaotic. Pre- and post-TCR urologic status was assessed. Ability to achieve urinary continence, with or without CIC, was the primary outcome, and need for CIC was the secondary outcome of interest. RESULTS: A total of 56 patients met inclusion criteria. Median age at TCR was 4.4 months (range 1.0-224.0) with a median follow-up of 10.7 years (range 1.3-19.1); 68% were asymptomatic at presentation. Clinical symptoms were urologic in 7%. At the latest follow-up, 86% of patients were continent spontaneously or with CIC (Summary Fig.). Of the four patients who presented with urologic symptoms, all were continent, but three required CIC. Overall, 23% of patients required CIC. Median age at CIC initiation was 7.6 years (range 1.6-17.4). Long-term continence was not associated with any demographic, anatomic, surgical or functional variable. Need for CIC at latest follow-up was associated with symptomatic presentation, partial TCR, transitional lipoma, and high-risk pre-operative urodynamics. DISCUSSION: In this series of primary TCR for LMM, where 93% of patients were urologically asymptomatic before TCR, prospects for continence were excellent. No studied parameter clearly impacted continence; however, need for CIC was associated with multiple variables. CONCLUSIONS: Clear predictors for continence after TCR will require additional long-term patient outcomes. Families can anticipate 23% likelihood of CIC, which is considerably less than in MM, but long-term urologic follow-up is still strongly recommended.

Prospective assessment of cosmesis before and after genital surgery.

INTRODUCTION: Little data exist about the surgical interventions taking place for children with disorders of sex development (DSD). Most studies that have evaluated cosmetic outcomes after genitoplasty have included retrospective ratings by a physician at a single center. OBJECTIVE: The present study aimed to: 1) describe frequency of sex assignment, and types of surgery performed in a cohort of patients with moderate-to-severe genital ambiguity; and 2) prospectively determine cosmesis ratings by parents and surgeons before and after genital surgery. STUDY DESIGN: This prospective, observational study included children aged <2 years of age, with no prior genitoplasty at the time of enrollment, moderate-to-severe genital atypia, and being treated at one of 11 children's hospitals in the United States of America (USA). Clinical information was collected, including type of surgery performed. Parents and the local pediatric urologist rated the cosmetic appearance of the child's genitalia prior to and 6 months after genitoplasty. RESULTS: Of the 37 children meeting eligibility criteria, 20 (54%) had a 46,XX karyotype, 15 (40%) had a 46,XY karyotype, and two (5%) had sex chromosome mosaicism. The most common diagnosis overall was congenital adrenal hyperplasia (54%). Thirty-five children had surgery; 21 received feminizing genitoplasty, and 14 had masculinizing genitoplasty. Two families decided against surgery. At baseline, 22 mothers (63%), 14 fathers (48%), and 35 surgeons (100%) stated that they were dissatisfied or very dissatisfied with the appearance of the child's genitalia. Surgeons rated the appearance of the genitalia significantly worse than mothers (P < 0.001) and fathers (P ≤ 0.001) at baseline. At the 6-month postoperative visit, cosmesis ratings improved significantly for all groups (P < 0.001 for all groups). Thirty-two mothers (94%), 26 fathers (92%), and 31 surgeons (88%) reported either a good outcome, or they were satisfied (see Summary Figure); there were no significant between-group differences in ratings. DISCUSSION: This multicenter, observational study showed surgical interventions being performed at DSD centers in the USA. While parent and surgeon ratings were discordant pre-operatively, they were generally concordant postoperatively. Satisfaction with postoperative cosmesis does not necessarily equate with satisfaction with the functional outcome later in life. CONCLUSION: In this cohort of children with genital atypia, the majority had surgery. Parents and surgeons all rated the appearance of the genitalia unfavorably before surgery, with surgeons giving worse ratings than parents. Cosmesis ratings improved significantly after surgery, with no between-group differences.

Is glans penis width a risk factor for complications after hypospadias repair?

INTRODUCTION: Recent studies have suggested that a smaller glans penis size may be associated with a higher likelihood of complications after hypospadias repair. Accurate identification of risk factors other than the well-understood variable of meatal location would allow development of better prognostic models and individualized risk stratification. OBJECTIVE: To test the hypothesis that a smaller width of the glans penis predicts adverse outcomes after hypospadias surgery. METHODS: Prospectively recorded clinical data were reviewed from a single-institution registry of primary hypospadias repairs performed between 2011 and 2014. Follow-up records were examined for occurrence of complications. Urethroplasty complications were defined to include meatal stenosis, dehiscence, urethrocutaneous fistula, urethral stricture, and/or urethral diverticulum. The subset of meatal stenosis and dehiscence were regarded as glanular complications. Regression analyses were performed to determine association between glans width and occurrence of complications. Because pre-operative androgen stimulation is known to increase glans penis size, separate subgroup analyses were included of patients with and without pre-operative use of testosterone cream. RESULTS: A total of 159 patients met criteria for inclusion in the study cohort: 140 patients underwent a single-stage repair, while 19 patients had a two-stage repair. The median glans penis width was 15 mm (range 10-22). Eighty-four patients (53%) received testosterone cream pre-operatively and had a significantly wider glans penis than the 75 patients who did not (median 15.5 vs 14 mm; P < 0.001). Median clinical follow-up was 7 months (IQR 1-12), with a minimum time elapsed since surgery of 10 months at the time of chart review. Twenty-four patients (15%) had one or more urethroplasty complications, including 11 (7%) with glanular complications. Overall, there was no statistically significant association between glans width and urethroplasty complications (P = 0.26) or glanular complications (P = 0.90) (Summary Table). Subgroup analyses of patients with and without pre-operative testosterone also revealed no significant associations between glans width and complications. CONCLUSIONS: Glans penis width was not a risk factor for complications after hypospadias repair. This finding differs from the results of other recent studies and encourages further research into the value of measuring penile parameters in patients undergoing hypospadias repair.

Uroflowmetry with pelvic floor electromyography: inter-rater agreement on diagnosis of pediatric non-neurogenic voiding disorders.

INTRODUCTION: Uroflowmetry with electromyography (uroflow-EMG) is commonly used for evaluation of lower urinary tract (LUT) function in children. Diagnostic criteria based largely on uroflow-EMG findings have previously been proposed for several conditions collectively termed non-neurogenic voiding disorders (NNVDs). These include dysfunctional voiding (DV), idiopathic detrusor overactivity disorder (IDOD), detrusor underutilization disorder (DUD), and primary bladder neck dysfunction (PBND). It is unknown whether practitioners with varying levels of training and experience can apply the diagnostic criteria for these conditions with a high level of consistency. OBJECTIVE: To assess inter-rater agreement on diagnosis of NNVDs using uroflow-EMG studies. STUDY DESIGN: Six raters performed post hoc evaluation of 84 uroflow-EMG studies and associated clinical data from children with symptoms of LUT dysfunction and no evidence of neurologic or anatomic abnormalities. Each rater was asked to categorize the uroflow-EMG studies as being consistent with DV, IDOD, DUD, PBND, or normal/unclassifiable. A consensus diagnosis was noted for studies on which at least four raters agreed. Inter-rater agreement was assessed via calculation of unweighted Fleiss' kappa statistics. RESULTS: Overall inter-rater agreement on NNVD diagnoses was moderate (kappa 0.46, 95% CI 0.38-0.54). Agreement between individual raters ranged from 0.33 (fair) to 0.74 (substantial) (Figure). There was no consensus on diagnosis for 20 patients (24%). DISCUSSION: Several factors may contribute to inter-rater disagreement on diagnosis of NNVDs. These include instances where patients satisfy one criterion for a particular diagnosis while missing others - or have findings consistent with more than one diagnosis. Strategies to address this may involve simplifying the diagnostic criteria, developing a clear algorithm that prioritizes certain criteria, and/or allowing assignment of multiple diagnoses. Practitioners could also benefit from standardized education regarding the diagnostic criteria for NNVDs. Potential limitations of this analysis included the use of just one uroflow-EMG study per patient in almost all cases. Also, the raters had variable levels of previous experience using the diagnostic criteria for NNVDs, and it is possible that they were not always applied as originally intended. If this were the case, it would support development of a standardized education tool to facilitate practitioner understanding and application of the criteria. CONCLUSIONS: Uroflow-EMG has shown promise for improving clinical management of NNVDs associated with pediatric LUT dysfunction. However, inter-rater agreement on NNVD diagnoses using current criteria is suboptimal. Various mechanisms should be explored to improve consistency in practitioners' diagnosis of NNVDs.

Tap water and the Malone antegrade continence enema: a safe combination?

PURPOSE: The Malone antegrade continence enema provides independence and improved quality of life in patients with fecal incontinence or intractable constipation. However, isolated reports of fatal hypernatremia after irrigation with normal saline have raised safety concerns about frequent colonic irrigation in children. Significant electrolyte abnormalities have also been reported with hypertonic phosphate and high colonic tap water enemas. Because our patients routinely use tap water for Malone antegrade continence enema irrigations, we examined the safety profile of this practice MATERIALS AND METHODS: In the last 3.5 years 71 patients at our institution have used antegrade tap water enemas for managing fecal incontinence or intractable constipation. Standard serum electrolytes were measured RESULTS: We obtained 101 sets of serum electrolyte measurements in 71 patients at a mean of 8.4 months postoperatively (range 1 to 33). A girl who presented with severe hyponatremia and hypochloremia had not used the Malone antegrade continence enema for several days. The most interesting finding was significantly elevated sodium and chloride in 1 case 6 weeks after surgery that was associated with tap water treated with a home softening system. Electrolytes reverted to normal 1 week after using untreated tap water CONCLUSIONS: We did not detect significant hyponatremia or hypochloremia in any patient using tap water for Malone antegrade continence enema irrigation. Although dangerous electrolyte abnormalities are rare, potential morbidity in those cases warrants periodic evaluation. Due to the elevated sodium content in softened tap water families should be alerted to use untreated tap water for preparing enemas.

p38 MAPK mediates renal tubular cell TNF-alpha production and TNF-alpha-dependent apoptosis during simulated ischemia.

Ischemia causes renal tubular cell loss through apoptosis; however, the mechanisms of this process remain unclear. Using the renal tubular epithelial cell line LLC-PK(1), we developed a model of simulated ischemia (SI) to investigate the role of p38 MAPK (mitogen-activated protein kinase) in renal cell tumor necrosis factor-alpha (TNF-alpha) mRNA production, protein bioactivity, and apoptosis. Results demonstrate that 60 min of SI induced maximal TNF-alpha mRNA production and bioactivity. Furthermore, 60 min of ischemia induced renal tubular cell apoptosis at all substrate replacement time points examined, with peak apoptotic cell death occurring after either 24 or 48 h. p38 MAPK inhibition abolished TNF-alpha mRNA production and TNF-alpha bioactivity, and both p38 MAPK inhibition and TNF-alpha neutralization (anti-porcine TNF-alpha antibody) prevented apoptosis after 60 min of SI. These results constitute the initial demonstration that 1) renal tubular cells produce TNF-alpha mRNA and biologically active TNF-alpha and undergo apoptosis in response to SI, and 2) p38 MAPK mediates renal tubular cell TNF-alpha production and TNF-alpha-dependent apoptosis after SI.

Shunt infection and malfunction after augmentation cystoplasty.

PURPOSE: Maintenance of a sterile intraperitoneal environment is critical in patients with ventriculoperitoneal shunts. Recent series have reported a broad discrepancy in the rate of shunt infection (0% to 20%) following augmentation cystoplasty. The need for distal shunt revision has not been well defined. We report the incidence of shunt infection and revision at our institution after bladder augmentation. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients with myelodysplasia and a ventriculoperitoneal shunt who underwent augmentation cystoplasty since August 1990. All patients included in the study had a minimum of 12 months of followup. RESULTS: A total of 55 patients with a ventriculoperitoneal shunt secondary to myelodysplasia required augmentation cystoplasty for management of a neuropathic bladder. Standard perioperative intravenous and oral antibiotic preparation, mechanical bowel preparation and intraoperative shunt isolation were used. Mean postoperative followup was 60.4 months (range 12 to 111). One patient presented with an extruded peritoneal shunt tip and positive cultures from cerebrospinal fluid and urine. Bladder perforation occurred in 2 patients and the shunt was empirically externalized. Revision was required for 5 (9%) distal shunt obstructions, including 1 cerebrospinal fluid pseudocyst. CONCLUSIONS: The incidence of shunt infection after augmentation cystoplasty is low (less than 2% in this large series), and presence of a ventriculoperitoneal shunt should not preclude bladder augmentation. Meticulous perioperative and intraoperative preparation contributes to the low rate of adverse events. Although the rate of distal revision after augmentation is significant, it does not exceed the reported distal failure rate for ventriculoperitoneal shunts in children without a history of urological surgery.

How well do patients with exstrophy actually void?

PURPOSE: Achieving continence and preserving renal function are goals in the care of patients with bladder exstrophy. The Young-Dees-Leadbetter bladder neck reconstruction should ideally provide continence and normal voiding dynamics without the need for intermittent catheterization. We review our experience with bladder neck reconstruction in this population with emphasis on voiding dynamics among those patients doing well. MATERIALS AND METHODS: We retrospectively analyzed all patients with the exstrophy-epispadias complex seen at our institutions since 1985. We reviewed staged reconstruction in 53 patients, including 31 with classic bladder exstrophy, 4 with exstrophy variants and 18 with incontinent epispadias. Patients with additional neurogenic dysfunction were excluded from study. Subjective and objective data regarding voiding function and complications were collected. RESULTS: Complete reconstruction for continence was performed in 38 cases, of which 11 that required bladder augmentation with bladder neck reconstruction or who had a different primary continence procedure were excluded from study. The remaining 27 patients treated with the Young-Dees-Leadbetter bladder neck reconstruction had 2 or more years of followup (mean 5.9). Dry intervals of at least 2 hours were achieved by 18 patients and all were considered by parents to void well. Despite near or total subjective continence and "good" voiding, 13 of these 18 patients (72%) have clinical problems related to emptying, which include recurrent urinary tract infections in 10, epididymitis in 2 and bladder calculi in 4. Objective urodynamic parameters confirm poor voiding in most patients. CONCLUSIONS: Bladder neck reconstruction in patients with exstrophy can achieve continence without intermittent catheterization. In our experience patients who achieve these goals have an alarming frequency of clinical and urodynamic problems related to emptying. One must question the normalcy of the voiding pattern and price to achieve continence among patients with exstrophy.

Y-to-I wrap: use of the distal spongiosum for hypospadias repair.

PURPOSE: Rather than resecting the distal spongiosum lateral to the open urethra for hypospadias, we mobilized this tissue with the urethral plate away from the corpora cavernosa, subsequently wrapping it around various types of urethroplasty to prevent fistula formation. MATERIALS AND METHODS: The distal spongiosum was preserved and used for coverage in 25 hypospadias repairs in 1 year. We initially applied it in this manner when the distal spongiosum persisted as a pillar of healthy erectile tissue but later when the distal tissue appeared more fibrous in nature. The wrap was used to cover various types of urethroplasty, including advancement in 6 cases, tubularization in 10, flip-flap repair in 6 and an island onlay pedicle graft in 3. RESULTS: All patients have at least 1 year of followup. There has been no fistula formation or residual chordee. In 1 patient minor meatal retraction did not require a secondary procedure. Cosmetic results have been good. CONCLUSIONS: A distal wrap of corpus spongiosum may be used to avoid fistula formation without causing residual or recurrent curvature. It re-creates a nearly normal urethra in some cases.

Coronal cuff: a problem site for buccal mucosal grafts.

PURPOSE: Urethral reconstruction of complex hypospadias or epispadias continues to present a significant challenge. Buccal mucosa as an onlay or tube graft is an excellent option when faced with paucity of penile skin. We identified the factors that lead to success or failure in these repairs. MATERIALS AND METHODS: During 6 years we placed 34 buccal mucosal grafts in 31 patients to repair complex hypospadias and epispadias. Penile skin was preferentially used for urethroplasty but a free buccal mucosal graft was used for reconstruction due to lack of adequate penile skin. The cases were complicated with an average of 5 previous unsuccessful repairs each in 16. Grafts ranged from 1.5 to 10 cm. and 10 patients required pieced grafts. We created 1 combination, 16 onlay and 17 full tube grafts. Buccal mucosa was used with a Thiersch-Duplay urethroplasty in 8 patients. RESULTS: Anastomotic stricture in 5 patients was proximal in 4. Fistula was a complication in 13 grafts that generally developed on the distal shaft, particularly at the coronal cuff. Of the 7 patients who underwent proximal Thiersch-Duplay urethroplasty with a distal buccal graft 6 had a coronal fistula. Fistula was more common with tube and pieced than with onlay grafts. CONCLUSIONS: Fistula is overwhelmingly the most common complication after buccal mucosal graft urethroplasty. Most fistulas develop at the coronal cuff, and we suspect that skin coverage and potential blood supply have not been good in that region. Anticipation of this problem during stage 1 of hypospadias repair would allow more advantageous distribution of the existing penile skin. Good distal skin coverage cannot be compromised in these complex cases.

Does every patient with prenatal hydronephrosis need voiding cystourethrography?

PURPOSE: Prenatal ultrasound has allowed early identification of urinary tract abnormalities that may require urological followup or early intervention. While all children with prenatal hydronephrosis should undergo ultrasound within the first few weeks of life, we believe that there is a subset of postnatal hydronephrosis for which voiding cystourethrography can be avoided if careful observation is continued. MATERIALS AND METHODS: For 5 years 175 infants with a history of prenatal hydronephrosis were evaluated by ultrasound. Of 60 infants with less than Society for Fetal Urology grade II unilateral or bilateral hydronephrosis 44 underwent voiding cystourethrography as part of the early evaluation and 16 were observed without voiding cystourethrography. Four infants for whom we would routinely obtain voiding cystourethrography were excluded from study due to severe prenatal hydronephrosis, renal duplication, hydroureter, ipsilateral small or echogenic kidney and grade II or higher hydronephrosis. RESULTS: Voiding cystourethrography was positive in 6 of the 40 infants (15%) with less than grade II hydronephrosis. Of these cases 3 had grade III or higher vesicoureteral reflux and 1 with high grade reflux required reimplantation. None of the 16 patients followed without voiding cystourethrography has required further evaluation or intervention. In all patients with negative or no voiding cystourethrography parenchyma was preserved and hydronephrosis stabilized or resolved. CONCLUSIONS: Prenatal and postnatal ultrasound in infants should be used to guide further urological evaluation. Among infants with less than grade II hydronephrosis postnatally 15% had reflux on voiding cystourethrography, which is significantly higher than that reported among normal children (approximately 1%). However, none of the 16 infants observed without voiding cystourethrography on short-term antibiotic prophylaxis had deleterious renal events with 6 months to 4.5 years of followup. Therefore, we question the actual significance of the reflux detected in the first cohort of infants. Voiding cystourethrography can provide a definitive answer. However, we also believe that it is not absolutely mandatory based on the outcome in the observed group. With careful counseling and followup most patients with less than grade II hydronephrosis can be observed without urological sequela.

Angiotensin type 2 receptor is important in the normal development of the ureter.

In humans, the actions of angiotensin II are transduced through the AT1 and AT2 receptors which have recently been implicated in renal organogenesis. Polymorphisms in the human angiotensin II receptor genes have been linked to cardiovascular and nephrological disorders. In this study we evaluated 35 patients with either primary obstructive megaureter or posterior urethral valves. Each was genotyped for the A1166 AT1 polymorphism and the recently described A-1332G AT2 transition. The incidence of these genetic variants was also evaluated in normal controls without any ultrasonographic urological abnormalities. Similar to our previous findings in congenital urological abnormalities, the AT1 receptor genotype distribution did not differ between patients with either primary obstructive megaureter or posterior urethral valves versus controls. In contrast, compared with normal controls, there was a dramatic increase in the occurrence of the AT2 A-1332G transition in patients with primary obstructive megaureter (75.0% vs. 41.9% in controls, P<0.025). In patients with posterior urethral valves, there was no difference in the occurrence of the transition versus controls (36.9%, P=NS). Thus, there is no correlation between the AT1 receptor gene polymorphism and urological abnormalities. However there is an increased incidence in the AT2 genetic variant in patients with primary obstructive megaureter.

Angiotensin II, type 2 receptor in the development of vesico-ureteric reflux.

OBJECTIVE: To investigate if mutation of the angiotensin II (Ang II) receptors AT2 is involved in primary vesico-ureteric reflux (VUR) in humans. PATIENTS AND METHODS: Genetic polymorphisms in the AT1 and AT2 receptors was evaluated in 23 patients having the most common congenital urological abnormality, namely primary congenital VUR. The occurrence of the A1166C transition in the AT1 receptor gene and the A-1332G transition in the AT2 receptor gene were evaluated and compared with the incidence in normal controls with no urological abnormalities. RESULT: The distribution of the AT1 receptor genotypes was no different between patients with VUR and healthy controls. Furthermore, 10 of 23 (44%) patients with VUR and seven of 19 (42%) controls carried the AT2 receptor gene variation. These results contrast with our previous finding of an association between the A-1332G transition in the AT2 receptor gene and primary obstructive megaureter, and pelvi-ureteric junction obstruction. CONCLUSIONS: We propose that while the AT2 receptor is crucial for the normal development of the ureter, it does not contribute to the processes which culminate in VUR, which is primarily an abnormality in the bladder trigone.