Predicting Risk of Chronic Kidney Disease in Infants and Young Children at Diagnosis of Posterior Urethral Valves: Initial Ultrasound Kidney Characteristics and Validation of Parenchymal Area as Forecasters of Renal Reserve.

PURPOSE: There is paucity of validated objective early imaging markers to help predict future renal deterioration in infants with posterior urethral valves. We evaluated the prognostic value of total renal parenchymal area, renal echogenicity and corticomedullary differentiation regarding future development of chronic kidney disease. MATERIALS AND METHODS: We analyzed initial postnatal ultrasonographic images from serial posterior urethral valve cases seen at a single tertiary referral center using National Institutes of Health sponsored image processing software. Echogenicity and corticomedullary differentiation were objectively measured as ratios relative to the adjacent liver or spleen and between cortex and medulla. The primary study outcome, renal function at last followup, was dichotomized based on glomerular filtration rate and/or need for renal replacement therapy (dialysis or renal transplantation, stage 5 chronic kidney disease). RESULTS: A total of 75 patients were evaluated, of whom 16 had progression to stage 5 chronic kidney disease after a mean ± SD followup of 64.2 ± 38.9 months. Mean renal parenchymal area was 21.41 cm(2) in patients without and 16 cm(2) in patients with stage 5 chronic kidney disease (p <0.001), and mean corticomedullary differentiation was 1.77 and 1.21, respectively (p <0.001). Bilateral echogenic kidneys were significantly associated with development of stage 5 chronic kidney disease (p = 0.004). The performance of corticomedullary differentiation in predicting stage 5 chronic kidney disease was statistically significant (AUROC 0.881, 95% CI 0.776-0.987, p <0.001). CONCLUSIONS: Estimates of renal parenchyma quantity (total renal parenchymal area) and quality (corticomedullary differentiation and renal echogenicity) measured on initial postnatal ultrasound carry prognostic value in determining future risk of stage 5 chronic kidney disease in patients with posterior urethral valves. These data are promising for developing tools to risk stratify patients, counsel parents and customize monitoring protocols.

Inappropriate Use of Ultrasound in Management of Pediatric Cryptorchidism.

BACKGROUND AND OBJECTIVES: There is a limited role for ultrasound in the management of an undescended testicle (UDT). We hypothesized that ultrasound remains overused by referring physicians. Our goal was to characterize the trends, patterns, and impact of ultrasound use for UDT and to reaffirm its limited diagnostic value for this indication. METHODS: The records of boys aged 0 to 18 years with UDT in Ontario, Canada, between 2000 and 2011 were reviewed by using health administrative data housed at the Institute for Clinical and Evaluative Sciences (ICES). A second review of boys referred to our institution with UDT between 2007 and 2011 was conducted to complement the health administrative data. Trends in frequency, distribution, and costs of ultrasound use were assessed. Time delays between diagnosis and definitive management were compared between the ultrasound and non-ultrasound groups. Using our institutional data, we analyzed demographic patterns of ultrasound use and compared its diagnostic accuracy by using surgical findings as the gold standard. RESULTS: Ultrasound was used in 33.5% of provincial referrals and 50% of institutional referrals. Children who underwent ultrasound experienced an approximate 3-month delay in definitive surgical management. Ultrasound correctly predicted physical examination findings in only 54% of patients. Physicians in community practice, and those with fewer years in practice, were more likely to order ultrasound. CONCLUSIONS: Ultrasound has limited value for the management of UDT but remains widely overused, with an increasing trend over time. This practice has negative implications for access to care and cost-containment. Widespread educational efforts should be undertaken, targeting current and future referring physicians.

Decisional regret after distal hypospadias repair: single institution prospective analysis of factors associated with subsequent parental remorse or distress.

PURPOSE: Hypospadias repair is a commonly performed procedure. Little is known about decisional regret in parents who agree to proceed with this surgical reconstruction. We present data on this previously underexplored issue. MATERIALS AND METHODS: We performed followup analysis of 100 couples prospectively evaluated after counseling for surgical correction of distal hypospadias in their son with assessment of complications and decisional regret 1 year after surgery. Findings were contrasted with baseline demographics, hypospadias knowledge and decisional conflict at the time of counseling. RESULTS: Decisional regret was found in 116 parents, including mild regret in 41.4% and moderate to severe regret in 8.6%. There was no statistically significant difference in paired regret analysis between mothers and fathers. Complications were strongly associated with decisional regret (p <0.001). On regression analysis postoperative complications (OR 14.7, 95% CI 1.6-131.6), parental desire to avoid circumcision (OR 7.4, 95% CI 1.1-49.4) and initial decisional conflict level (OR 1.06, 95% CI 1.02-1.09) were statistically significant predictors of moderate to strong decisional regret. These findings remained robust after imputation strategies to address missing data. The impact of decisional conflict and preference for circumcision were significant even after excluding families who experienced complications. CONCLUSIONS: To our knowledge this is the first study demonstrating parental decisional regret after providing consent for surgical correction of distal hypospadias in their son. Based on the described risk factors efforts aimed at minimizing complications and counseling about foreskin preservation techniques may be prudent to ameliorate decisional regret. The novel association between decisional conflict and regret suggests that conflict assessment during counseling may help screen families at risk for postoperative regret.

Evaluating practice patterns in postnatal management of antenatal hydronephrosis: a national survey of Canadian pediatric urologists and nephrologists.

OBJECTIVE: To ascertain practice patterns of prescribing continuous antibiotic prophylaxis (CAP) and obtaining a voiding cystourethrogram for infants with antenatal hydronephrosis (AHN) by pediatric nephrologists and urologists across Canada. METHODS: A previously piloted online survey was distributed to members of the Canadian pediatric nephrology and urology associations. Summarized confidential responses were stratified by specialty, AHN grade, and laterality. RESULTS: A total of 88 of 139 responses were received (response rate, 63.3%; 95% confidence interval, 55.0%-71.0%): 46 nephrologists, 39 urologists, and 3 undisclosed. Only 17 of 88 (19.32%; 95% confidence interval, 12.4%-28.8%) reported following standardized AHN protocols. Concern surrounding the development of urinary tract infections was the main deciding factor for prescribing CAP (nephrology, 65.4%; urology, 71.4%). Almost a third of nephrologists (29.6%) recommend CAP for bilateral low-grade AHN compared with 11.4% of urologists (P = .02); in contrast, 73% of nephrologists and 38.2% of urologists (P = .02) offer CAP in the presence of isolated high-grade AHN. In regards to indications for voiding cystourethrogram, 31% of pediatric nephrologists would recommend this test for patients with unilateral low-grade AHN compared with 7.7% of urologists (P < .01), although almost all nephrologists (96.6%) and 69.2% of urologists (P = .02) would obtain this test for patients with unilateral high-grade isolated AHN. CONCLUSION: Our results show important practice variability between pediatric nephrologists and urologists in the management of children with AHN, which are partially explained by laterality and degree of dilation. This survey reflects the lack of treatment guidelines and supports efforts to obtain high-level evidence to develop management protocols for this common condition.

Impact of penile degloving and proximal ventral dissection on curvature correction in children with proximal hypospadias.

INTRODUCTION: Penile degloving is an important step in orthoplasty. Although its role in correcting mild curvature in distal and midshaft hypospadias has been previously reported, its impact on ventral curvature (VC) correction in proximal defects warrants further investigation. Therefore, we sought to document the effect of degloving and proximal urethral dissection on VC correction in children with proximal hypospadias. METHODS: We retrospectively reviewed the records of 137 patients who underwent proximal hypospadias repair between 1998 and 2006. VC, defined as mild (<30%), moderate (30%-45%), and severe (>45%), was recorded before penile degloving and after erection test. Percent improvement in VC and need for further treatment (beyond degloving and proximal dissection) based on preoperative degree of curvature were assessed. ANOVA test was used to compare improvement among the 3 groups. RESULTS: Mean age at repair was 14 months (range: 6-24). Penile degloving associated with proximal urethral dissection when necessary was responsible for the improvement in the degree of curvature in 7 of 9 (77%) patients with mild VC, 23 of 44 (52%) with moderate and 35 of 84 (40%) with severe VC. Additionally, degloving alone was sufficient for VC correction in 7 of 9 (77%) mild cases, 14 of 44 (30%) moderate and only 2 of 84 (2%) severe cases. The difference among these 3 groups was statistically significant (p < 0.001). CONCLUSIONS: Penile degloving alone can correct VC. The percentage of improvement depends on the preoperative degree of curvature, with severe VC cases showing the least improvement.

Bilateral epididymitis in a child with undiagnosed posterior urethral valves.

Posterior urethral valves are most commonly detected in the early neonatal period, the diagnosis often antenatally suspected from ultrasound findings. Nevertheless, some cases might go undetected and become manifest later in life with lower urinary tract symptoms. We describe the unusual case of a 5-year-old boy with a 2-month history of bladder distension, urinary dribbling, and epididymitis. Cystourethrography revealed posterior urethral valves with reflux into the seminal vesicles, vas deferens, and epididymis, bilaterally. A review of the published data is provided in the context of this unusual presentation pattern.

Early postoperative ultrasound after open pyeloplasty in children with prenatal hydronephrosis helps identify low risk of recurrent obstruction.

PURPOSE: Prediction of successful pyeloplasty can be challenging, particularly in asymptomatic children treated for worsening prenatally diagnosed hydronephrosis. We evaluated early noninvasive objective predictors of success in this population. MATERIALS AND METHODS: We reviewed patients who underwent pyeloplasty for asymptomatic, prenatally detected, worsening hydronephrosis treated between 2000 and 2010 with followup greater than 1 year. For all patients renal pelvis anteroposterior diameter in a mid transverse view and Society for Fetal Urology hydronephrosis grade were evaluated preoperatively and 3 to 4 months postoperatively. Aside from subjective evaluation based on change in hydronephrosis grade, we estimated the percentage of improvement in anteroposterior diameter (preoperative diameter--postoperative diameter/preoperative diameter). Repeat intervention after pyeloplasty arbitrarily defined failure. Patients were categorized into 3 groups, ie no failure on followup ultrasound (group 1), no failure with postoperative nuclear scan to document success (group 2) and failed pyeloplasty (group 3). ROC curves were plotted to correlate 4 variables with failure, namely preoperative anteroposterior diameter, postoperative anteroposterior diameter, percent improvement in anteroposterior diameter and subjective change in grade. RESULTS: Of 229 patients 192 (84%) who met inclusion criteria had ultrasound at 3 to 4 months postoperatively available. Mean age at surgery was 19 months and mean ± SD followup was 49.9 ± 27.7 months. Percent improvement in anteroposterior diameter and postoperative anteroposterior diameter measure were the most reliable variables to predict failure (AUC 0.88 and 0.86, respectively, p <0.0001), whereas preoperative anteroposterior diameter and subjective changes in grade were not good predictors (AUC 0.52, p >0.05). A percent improvement in anteroposterior diameter of 38% or greater or postoperative anteroposterior diameter of 11.5 mm or less was associated with success, with sensitivity of 100% and specificity of 61% and 55%, respectively. CONCLUSIONS: Percent improvement in anteroposterior diameter and postoperative pelvic anteroposterior diameter measure can provide objective guidance as to which patients need closer monitoring during followup, and can help select children at low risk for repeat intervention after pyeloplasty.

Comparative analysis of detorsion alone versus detorsion and tunica albuginea decompression (fasciotomy) with tunica vaginalis flap coverage in the surgical management of prolonged testicular ischemia.

PURPOSE: Recent data suggest that testicular torsion may include an element of the compartment syndrome that improves with decompression. In 2009 we instituted tunica albuginea incision with tunica vaginalis flap coverage as an alternative in cases in which the torsed testis continued to appear ischemic after detorsion. MATERIALS AND METHODS: The medical records of 65 boys who underwent scrotal exploration for testicular torsion between 2000 and 2010 were reviewed. There were 6 patients excluded from study due to lack of followup. Of the remaining 59 patients 31 (52.5%) showed improvement in testicular appearance after detorsion and underwent orchiopexy, whereas 28 (47.5%) did not show evidence of recovery after detorsion. Of these patients 11 underwent tunica albuginea incision with tunica vaginalis flap coverage and 17 underwent orchiectomy. Demographic data, duration of symptoms and rate of testicular salvage were analyzed. RESULTS: Mean patient age was 11.8 years (detorsion plus orchiopexy), 10.1 years (tunica albuginea incision plus tunica vaginalis flap coverage) and 10.1 years (detorsion plus orchiectomy). Average followup was greater than 6 months in all groups. Mean duration of torsion was 13.4 hours (detorsion plus orchiopexy), 31.2 hours (tunica albuginea incision plus tunica vaginalis flap coverage) and 67.5 hours (detorsion plus orchiectomy). Before tunica albuginea incision with tunica vaginalis flap coverage was offered, the rate of orchiectomy was 35.9% (14 of 39) vs 15% (3 of 20) after this technique was introduced (p <0.05). The rates of testicular salvage were 62.5% (detorsion plus orchiopexy), 54.6% (tunica albuginea incision plus tunica vaginalis flap coverage) and 0% (detorsion plus orchiectomy). Although the numbers are limited, it is likely that without tunica albuginea incision with tunica vaginalis flap coverage 6 of 11 testes would have been removed. CONCLUSIONS: This preliminary experience suggests that tunica albuginea incision with tunica vaginalis flap coverage is a promising option for the management of clinically marginal torsed testes, enhancing salvageability after prolonged ischemia. We recommend considering this maneuver before performing orchiectomy in selected cases of testicular torsion.

Analysis of decisional conflict among parents who consent to hypospadias repair: single institution prospective study of 100 couples.

PURPOSE: Although obtaining informed consent for distal hypospadias repair is common practice, little is known about the uncertainty or conflict between consenting parents faced with this decision. We systematically evaluated decisional conflict between parents who elected to have their child undergo hypospadias surgery. MATERIALS AND METHODS: A total of 100 couples who were counseled about treatment options agreed to participate. Using a validated questionnaire, the Decisional Conflict Scale, we prospectively collected data on decisional conflict demographics, preference for circumcision, education level and prior knowledge about hypospadias. RESULTS: All parents elected surgical repair. Evidence of decisional conflict was encountered in 28% of participants (score less than 25 in 72%, 25 to 37.5 in 23.5%, greater than 37.5 in 4.5%). No statistically significant differences among parents were noted for total score (mean ± SD 16.1 ± 12 in mothers and 18.3 ± 12.6 in fathers) or subscales, except the informed subscale (mean ± SD 16.7 ± 14.3 in mothers and 21.1 ± 16.6 in fathers). Parental self-report of prior knowledge about hypospadias and preference for neonatal circumcision correlated with lower Decisional Conflict Scale scores (p = 0.02 and p <0.01, respectively). No statistical association was found between score and parental education level (p = 0.7) or expertise of the counselor (staff vs pediatric urology fellow, p = 0.4). CONCLUSIONS: These data describe the level of decisional conflict in couples agreeing to proceed with hypospadias repair, with no evidence of significant discrepancy between them. The novel description of factors related to decreased decisional conflict might help focus efforts aimed at minimizing difficulties encountered during the decision making process.

Newborn with persistent cloaca presenting with accessory phallic urethra and ambiguous genitalia.

Persistent cloaca is a rare urogenital anomaly that can pose significant challenges for adequate diagnosis and management. We describe the case of an infant girl with a cloacal malformation, having a single perineal orifice and an accessory phallic urethra, who presented with ambiguous genitalia at birth. The distal part of the accessory phallic urethra was used to create a mucosa-lined vestibule as a part of the total urogenital sinus mobilization. This technical maneuver allowed a more natural looking, and possibly functioning, vaginal introitus, improving the final cosmetic result.

Can we spare removing the adrenal gland at radical nephrectomy in children with wilms tumor?

PURPOSE: In patients with Wilms tumor indications for adrenalectomy are not well-defined. Following the rationale for preserving the adrenal gland in cases of other renal malignancies we determined predictors of adrenal involvement and the impact of adrenalectomy on retroperitoneal recurrence. MATERIALS AND METHODS: We retrospectively reviewed the record of patients who underwent surgical resection as primary treatment for Wilms tumor between 1990 and 2008 at 2 Canadian pediatric centers. Patient and tumor characteristics were reviewed to determine potential links to adrenal involvement. Recurrence was evaluated as a time dependent variable based on followup duration. RESULTS: Of 180 patients diagnosed with Wilms tumor 95 underwent initial radical nephrectomy. Mean ± SD age at diagnosis was 46 ± 38 months and mean survival followup was 189 ± 8.3 months. Disease was stage 1 to 4 in 28, 34, 23 and 4 patients, respectively. Adrenalectomy was done in 58 patients (61%). Only 1 adrenal gland was reportedly positive for tumor invasion while peri-adrenal fat involvement was noted in 3 patients. No studied patient or tumor characteristics predicted involvement. No statistically significant difference in retroperitoneal recurrence was found between the groups in which the adrenal gland was removed vs preserved. CONCLUSIONS: Adrenal involvement in patients with Wilms tumor is rare and difficult to predict. Preserving the adrenal gland was not associated with an increased risk of local recurrence. Thus, it seems prudent to avoid adrenalectomy at radical nephrectomy when technically feasible, instead attempting to otherwise remove all peri-adrenal fat with the specimen.

Outcome of antibiotic prophylaxis discontinuation in patients with persistent vesicoureteral reflux initially presenting with febrile urinary tract infection: time to event analysis.

PURPOSE: The use of antibiotic prophylaxis for preventing urinary tract infections has recently been called into question. Some studies support discontinuation of antibiotic prophylaxis in selected groups of children with vesicoureteral reflux. We report on the outcome of this practice in a cohort of patients assembled based on initial presentation with a febrile urinary tract infection. MATERIALS AND METHODS: We retrospectively reviewed records of patients with persistent vesicoureteral reflux without symptoms suggestive of dysfunctional elimination who discontinued antibiotic prophylaxis after being toilet trained. Exclusion criteria consisted of secondary reflux and previous surgery for vesicoureteral reflux. End points included development of febrile urinary tract infections, renal abnormalities on followup ultrasound and need for further interventions. Infection-free survival was analyzed using the Kaplan-Meier method and compared using the log rank and Cox's tests. RESULTS: We evaluated 84 girls and 26 boys with a mean age of 5.4 years. Febrile urinary tract infections developed in 10 girls and 1 boy at an average of 17.2 months after discontinuation of antibiotic prophylaxis. In a time to event analysis group comparison showed no significant differences when patients were stratified by gender (p = 0.22), age at antibiotic prophylaxis discontinuation (p = 0.14) or disease laterality (p = 0.23). However, a significant difference was found in number of patients with high grade vesicoureteral reflux (III to V, p = 0.05) and development of symptoms suggestive of bladder/bowel dysfunction (p <0.01). CONCLUSIONS: Our data support antibiotic prophylaxis discontinuation in the majority of patients with persistent vesicoureteral reflux who initially present with a febrile urinary tract infection, once their elimination habits have been optimized. Those with high grade reflux appear to be at increased risk for recurrent urinary tract infections. Development of dysfunctional elimination symptoms appears to be a risk factor amenable to treatment.

Bilateral ureteral reimplantation at primary bladder exstrophy closure.

PURPOSE: We present the outcomes of children who underwent concurrent complete primary repair of bladder exstrophy and bilateral ureteral reimplantation vs those undergoing bladder exstrophy repair alone, focusing on the rate of postoperative febrile urinary tract infections. MATERIALS AND METHODS: We performed complete primary repair of bladder exstrophy with bilateral ureteral reimplantation using a cephalotrigonal technique in 15 patients (group 1) and without bilateral ureteral reimplantation in 23 patients (group 2). Postoperative assessment included ultrasound and voiding cystourethrogram in all patients. Outcome measurements included postoperative febrile urinary tract infections, hydronephrosis and presence of vesicoureteral reflux. RESULTS: Mean followup was 34 months (range 6 to 54) for group 1 and 70 months (23 to 117) for group 2. Median age at surgery was 3 days for both groups (range 1 to 140). There were 10 boys and 5 girls in group 1, and 11 boys and 12 girls in group 2. Two of 15 patients (13%) in group 1 had hydronephrosis postoperatively compared to 10 of 23 (43%) in group 2 (p = 0.05). One patient in group 1 (7%) had a febrile urinary tract infection vs 11 (48%) in group 2 (p = 0.01). No patients in group 1 had postoperative vesicoureteral reflux compared to 17 (74%) in group 2 (p = 0.04). There were no complications related to ureteral reimplantation. CONCLUSIONS: Bilateral ureteral reimplantation can be safely and effectively performed during primary closure of bladder exstrophy in newborns, potentially reducing postoperative febrile urinary tract infections and hydronephrosis by early correction of vesicoureteral reflux.

Placebo-controlled trials in pediatric urology: a cautionary view from an ethical perspective.

The ethical dispute regarding placebo-controlled trials is discussed in this review. Important issues, such as clinical equipoise, fiduciary obligation and middle ground theory, are examined in the context of pediatric urology clinical research. After reviewing the literature, the authors summarize specific indications for placebo-controlled trials in pediatric urology, and emphasize that physicians have ethical and moral obligations to patients, in the sense that one should carefully plan and conduct such trials in order to gain clinically important information without exposing children to undue risks.

Comparison of flank, dorsal lumbotomy and laparoscopic approaches for dismembered pyeloplasty in children older than 3 years with ureteropelvic junction obstruction.

PURPOSE: Selecting a surgical approach for correction of ureteropelvic junction obstruction in children is mostly driven by surgeon preference, and includes laparoscopy, flank incision and dorsal lumbotomy. Comparative analyses focusing on operative time, hospital stay and complications are lacking. MATERIALS AND METHODS: We retrospectively reviewed the charts of 41 consecutive patients older than 3 years who underwent laparoscopic pyeloplasty by a single surgeon between 2005 and 2008. We compared these cases to 67 age matched cases managed by flank incision (42) or dorsal lumbotomy (25). We evaluated age at surgery, operative time, performance of retrograde pyelogram, length of hospitalization and complication/failure rates. RESULTS: Mean patient age was 7.3 to 8.1 years. Mean operative time was significantly longer for laparoscopy (178 minutes) compared to flank incision (144) and dorsal lumbotomy (148, p = 0.01). Mean hospital stay was significantly shorter for laparoscopy (2.3 days) compared to flank incision (3.6) and dorsal lumbotomy (3.3, p = 0.01). Complications occurred in 4 laparoscopic, 2 flank incision and 2 dorsal lumbotomy procedures. Patients who underwent laparoscopic pyeloplasty had a significantly shorter mean followup (28 months) compared to flank incision (49) and dorsal lumbotomy (47, p = 0.02). CONCLUSIONS: Overall complication rates were not significantly different for the 3 pyeloplasty approaches. For the evaluated outcomes our data do not favor one particular surgical access over others in children older than 3 years.

Concurrent ureteropelvic and ureterovesical junction obstruction in children: the value of retrograde pyelography.

Ureteropelvic junction obstruction with concomitant ureterovesical junction obstruction is a rare condition whose treatment remains challenging [1-3]. Renal ultrasonography and diuretic renogram are universally utilized to assess a patient prior to pyeloplasty; however, the role of retrograde pyelography at the time of pyeloplasty is still debatable [5-7]. Herein, we describe two cases where the use of retrograde pyelography preoperatively helped to avoid pyeloplasty failure by allowing the surgeon to visualize a concurrent ureterovesical junction obstruction, which would not have been visible in the surgical field had the procedure been carried out without intraoperative imaging.

Assessment of urethral plate appearance through digital photography: do pediatric urologists agree in their visual impressions of the urethral plate in children with hypospadias?

OBJECTIVE: There is a paucity of literature on a definition or set criteria evaluating urethral plate (UP) quality. We sought to determine whether pediatric urologists are in agreement in their assessment of UP quality. MATERIALS AND METHODS: A cohort of 21 pediatric urologists attending a national annual meeting were surveyed with a questionnaire inquiring about practice patterns and perceived impressions of UP quality. Nineteen sequentially projected, standardized, digital photographs, depicting plates with variable meatal locations, were presented. Intra- and inter-rater agreement among pediatric urologists was estimated by calculating the Kappa statistic (kappa). RESULTS: Thirty percent of respondents had more than 15years of practice and one-third repair 10 or more hypospadias per month. Measurement of level of agreement in the impression of UP quality of the 18 projected photographs revealed poor inter-rater agreement (kappa=0.06, P=0.0003), which was not improved by focusing on particular meatal locations or surgeon experience. CONCLUSIONS: Despite the inherent shortcomings of digital photography, this study highlights the potential subjectivity and lack of agreement on UP quality. Disagreement seems to be present irrespective of level of the defect or surgeon experience. Setting standards and improving agreement is likely to enhance reporting and interpretation of data in hypospadias studies.