Long-term Urological Outcomes in Pelvic Genitourinary Rhabdomyosarcoma: A 48-Year Single-center Experience.

PURPOSE: Multimodal therapy has improved survival in genitourinary rhabdomyosarcoma, a rare pediatric cancer. However, little is reported regarding postoperative complications and long-term urinary and sexual function and quality of life. MATERIALS AND METHODS: We reviewed records from 1970-2018 to identify patients with genitourinary rhabdomyosarcoma of the bladder, prostate, pelvis, vagina, and uterus. We assessed modes of therapy, and if surgical, the type of resection, reconstruction, and reoperation. Primary outcomes included urinary continence, urinary tract infection occurrence, and stone formation. We also surveyed patients older than 18 years for urinary and sexual function. RESULTS: Fifty-one patients were identified for the post-treatment outcomes cohort. All received chemotherapy, 46 (90.2%) underwent surgery, and 34 (67%) received radiation. Twenty-nine patients (56.9%) received trimodal therapy, 17 (33.3%) received chemotherapy/surgery, and 5 (9.8%) received chemotherapy/radiation. Twenty-six had up-front radical surgery (with staged continence mechanism creation); these patients had higher rates of continence, similar rates of urinary tract infection, and higher rates of stone formation compared to those who were organ-spared. A third (4/12) of organ-spared patients underwent additional corrective surgery. Thirty patients with genitourinary rhabdomyosarcoma were surveyed and 14 responded to questionnaires. Overall, urinary complaints were mild, but both male and female respondents reported significant sexual dysfunction. CONCLUSIONS: Organ-sparing treatment was more likely to predispose patients to high rates of additional reconstructive surgery due to compromised urological function. In survey results, both men and women reported poor sexual function, but the majority of patients remained satisfied with their urinary function.

The duplicated collecting system of the urinary tract: embryology, imaging appearances and clinical considerations.

Duplication anomalies of the urinary collecting system are common and can be discovered and characterized with multiple imaging modalities. The embryology, imaging manifestations and clinical ramifications of duplicated ureters and renal collecting systems vary from a normal anatomical variant to urological pathology and are discussed and illustrated in this review.

Management of Proximal Hypospadias with 2-Stage Repair: 20-Year Experience.

PURPOSE: We describe our experience with 2-stage proximal hypospadias repair. We report outcomes, and patient and procedure characteristics associated with surgical complications. MATERIALS AND METHODS: We retrospectively studied patients with proximal hypospadias who underwent staged repair between January 1993 and December 2012. Demographics, preoperative management and operative technique were reviewed. Complications included glans dehiscence, fistula, meatal stenosis, nonmeatal stricture, urethrocele/diverticula and residual chordee. Cox proportional hazards model was used to evaluate the associations between time to surgery for complications and patient and procedure level factors. RESULTS: A total of 134 patients were included. Median patient age was 8.8 months at first stage surgery and 17.1 months at second stage surgery, and median time between surgeries was 8 months. Median followup was 3.8 years. Complications were seen in 71 patients (53%), with the most common being fistula (39 patients, 29.1%). Reoperation was performed in 66 patients (49%). Median time from urethroplasty to surgery for complication was 14.9 months. Use of preoperative testosterone decreased risk of undergoing surgery for complication by 27% (HR 0.73, 95% CI 0.55-0.98, p = 0.04). In addition, patients identified as Hispanic were at increased risk for undergoing surgery for complications (HR 2.40, 95% CI 1.28-4.53, p = 0.01). CONCLUSIONS: We review the largest cohort of patients undergoing 2-stage hypospadias repair at a single institution. Complications and reoperation rates were approximately 50% in the setting of complex genital reconstruction.

Use of buccal mucosa grafts for urethral reconstruction in children: a retrospective cohort study.

BACKGROUND: The use of buccal mucosa grafts (BMG) for urethral reconstruction has increased in popularity over the last several decades. Our aim was to describe our institutional experience with and outcomes after BMG urethroplasty. METHODS: We conducted a retrospective cohort study of boys undergoing BMG urethral reconstruction. Preoperative and perioperative characteristics and postoperative outcomes were evaluated. RESULTS: Twenty-nine patients (median age 8.2 years) underwent BMG urethroplasty from 1995-2012. Of the 10 patients undergoing 1-stage repairs, 6 had tubularized grafts, the last of which was performed in 2000 due to an unacceptably high revision rate (100%). A 2-stage approach was elected for 19 patients (median follow-up 21.3 months). Complications including stricture, fistula, or chordee were seen in 60% of patients completing both stages and 32% required ≥1 revision. However, 71% of 2-stage patients were free of significant problems at last follow-up. CONCLUSIONS: We found BMG to be a reasonable option for use in complex pediatric urethral reconstruction. Tubularized grafts had poor results, and we no longer use them. We favor a 2-stage approach for all patients except those with "simple" non-hypospadiac strictures. Although revision procedures were not uncommon, the majority of patients were ultimately free of long-term problems.

New contralateral vesicoureteral reflux after unilateral ureteral reimplantation: predictive factors and clinical outcomes.

PURPOSE: Although unilateral ureteral reimplantation for vesicoureteral reflux is highly successful, new contralateral reflux will develop postoperatively in some patients. We examined predictors and clinical outcomes of postoperative contralateral vesicoureteral reflux. MATERIALS AND METHODS: We reviewed patients who underwent nontapered unilateral reimplantation for primary vesicoureteral reflux graded on a 3-point scale at our institution from January 1990 to December 2002, and identified those with subsequent contralateral vesicoureteral reflux. We analyzed the association of patient/procedure characteristics with incidence, and time to resolution of contralateral reflux and postoperative urinary tract infection. Multivariable models controlled for variables associated with incidence and time to resolution of contralateral reflux. RESULTS: A total of 395 patients (77.2% female, median age 5.3 years) underwent ureteral reimplantation for vesicoureteral reflux. Preoperative reflux was grade 1 in 2.8% of patients, grade 2 in 56.6% and grade 3 in 40.6%. Technical success was 95.4%. After reimplantation 39 patients (9.9%) had new contralateral reflux (grade 1 in 7, grade 2 in 27 and grade 3 in 5). Median followup was 51.8 months. On multivariate analysis younger age (less than 6 years, OR 3.7, p = 0.006) and low observed bladder capacity as percent of predicted bladder capacity (less than 50% of predicted capacity, OR 6.3, p = 0.02) were significant predictors of contralateral reflux. Contralateral reflux resolved in 21 of 27 patients (77.8%) on subsequent cystography at a median of 21.5 months. Two patients underwent reimplantation for persistent contralateral reflux. Four of 39 patients (10.3%) with contralateral reflux had postoperative febrile urinary tract infections at a median of 26 months, with spontaneous resolution of contralateral reflux in all. CONCLUSIONS: Younger patients and those with low observed vs predicted bladder capacity may be at increased risk for postoperative contralateral vesicoureteral reflux. A majority of contralateral reflux will resolve spontaneously, and the clinical course is typically benign.

Long-term incidence of urinary tract infection after ureteral reimplantation for primary vesicoureteral reflux.

OBJECTIVE: To determine the incidence of urinary tract infection (UTI) after ureteral reimplantation (UR) for primary vesicoureteral reflux (VUR). MATERIALS AND METHODS: In this retrospective review, the pyelonephritis-free survival of patients with primary VUR who underwent open UR from January 1990 to December 2002 was assessed using a Cox proportional hazards analysis. RESULTS: 1076 patients underwent open UR for primary VUR. 73.0% were female; median age was 4.7 years. 80.1% presented with UTI. Clinical success rate for non-tapered UR was 96.5%. Median follow-up was 2.9 years. 21.8% had at least one postoperative UTI. 6.5% had postoperative pyelonephritis (POP) at a median of 21 months postoperatively. On multivariate survival analysis female gender (OR 9.97, 95% CI 3.07-32.34), preoperative VUR grade ≥ 3 (2.14, 1.25-3.69), breakthrough preoperative UTI (2.00, 1.22-3.25), and preoperative renal scarring (1.86, 1.15-2.99) were associated with POP. CONCLUSION: POP is rare on long-term follow-up, suggesting that UR is effective in reducing pyelonephritis in this population.

B7-h1 as a biomarker for therapy failure in patients with favorable histology Wilms tumor.

PURPOSE: A minority of children with Wilms tumor will experience tumor recurrence. In a previous pilot study we found an association between expression of an immune costimulatory molecule, B7-H1, and tumor recurrence in favorable histology Wilms tumor. We sought to verify the prognostic value of B7-H1 as a biomarker in favorable histology Wilms tumor. MATERIALS AND METHODS: We performed a nested case-control study of tumors from the Fifth National Wilms Tumor Study. We randomly selected 44 children unsuccessfully treated (cases) and 49 who were successfully treated for favorable histology Wilms tumor (controls). Cases and controls were matched based on tumor stage, and the analysis was restricted to children who underwent initial resection. We excluded patients with stage IV or V disease and those treated with chemotherapy or radiation. Tumor specimens were stained for B7-H1 expression. RESULTS: Of the 93 total samples analyzed 60 (65%) demonstrated B7-H1 staining, with staining diffusely present in 13 (22%) and blastema predominant in 34 (57%). B7-H1 expression was associated with failure of initial therapy (p = 0.006). Patients with tumors showing less than 20% B7-H1 positive cells were at lower risk for treatment failure, while those with tumors exhibiting greater than 60% B7-H1 positive cells were at greater risk for treatment failure. This association appeared to be independent of tumor stage. CONCLUSIONS: B7-H1 expression by favorable histology Wilms tumor is associated with an increased risk of failure of initial therapy.

Development and initial validation of a scoring system to diagnose testicular torsion in children.

PURPOSE: Testicular torsion is a surgical emergency requiring prompt intervention. Although clinical diagnosis is recommended, scrotal ultrasound is frequently ordered, delaying treatment. We created a scoring system to diagnose testicular torsion, decreasing the indication for ultrasound. MATERIALS AND METHODS: We prospectively evaluated 338 patients with acute scrotum, of whom 51 had testicular torsion. Physical examination was performed by a urologist, and all patients underwent scrotal ultrasound. Univariate analysis and logistic regression were performed, and a scoring system for risk stratification of torsion was created. Retrospective validation was performed with 2 independent data sets. RESULTS: The scoring system consisted of testicular swelling (2 points), hard testicle (2), absent cremasteric reflex (1), nausea/vomiting (1) and high riding testis (1). Cutoffs for low and high risk were 2 and 5 points, respectively. Ultrasound would be indicated only for the intermediate risk group. In the prospective data set 69% of patients had low, 19% intermediate and 11.5% high risk. Negative and positive predictive values were 100% for cutoffs of 2 and 5, respectively (specificity 81%, sensitivity 76%). Retrospective validation in 1 data set showed 66% of patients at low, 16% intermediate and 17% high risk. Negative and positive predictive values for cutoffs of 2 and 5 were 100% (specificity 97%, sensitivity 54%). The second retrospective data set included only torsion cases, none of which was misdiagnosed by the scoring system. CONCLUSIONS: This scoring system can potentially diagnose or rule out testicular torsion in 80% of cases, with high positive and negative predictive values for selected cutoffs. Ultrasound orders would be decreased to 20% of acute scrotum cases. Prospective validation of this scoring system is necessary.

Postnatal longitudinal evaluation of children diagnosed with prenatal hydronephrosis: insights in natural history and referral pattern.

OBJECTIVES: Clinical significance and management of prenatal hydronephrosis (PNH) are sources of debate. Existing studies are flawed with biased cohorts or inconsistent follow-up. We aimed to evaluate the incidence of pathology in a large cohort of PNH and assess the biases and outcomes of this population. METHODS: We reviewed 1034 charts of fetuses with PNH. Records of delivered offspring were reviewed at a pediatric center and analyzed with respect to prenatal and postnatal pathology and management. RESULTS: Prenatal resolution of hydronephrosis occurred in 24.7% of pregnancies. On first postnatal ultrasound, some degree of dilatation was present in 80%, 88% and 95% of mild, moderate and severe PNH cases, respectively. At the end of follow-up, hydronephrosis persisted in 10%, 25% and 72% of children, respectively. Incidence of vesicoureteral reflux did not correlate with severity of PNH. Children with postnatal workup had more severe PNH than those without. CONCLUSIONS: Despite prenatal resolution totalizing 25%, pelvic dilatation persisted on first postnatal imaging in most cases, thus justifying postnatal ultrasound evaluation. Whereas most mild cases resolved spontaneously, a quarter of moderate and more than half of severe cases required surgery. Patients with postnatal imaging and referral had more severe PNH, which could result in overestimation of pathology.

A novel method of evaluating ureteropelvic junction obstruction: dynamic near infrared fluorescence imaging compared to standard modalities to assess urinary obstruction in a swine model.

PURPOSE: Dynamic near infrared fluorescence imaging of the urinary tract provides a promising way to diagnose ureteropelvic junction obstruction. Initial studies demonstrated the ability to visualize urine flow and peristalsis in great detail. We analyzed the efficacy of near infrared imaging in evaluating ureteropelvic junction obstruction, renal involvement and the anatomical detail provided compared to conventional imaging modalities. MATERIALS AND METHODS: Ten swine underwent partial or complete unilateral ureteral obstruction. Groups were survived for the short or the long term. Imaging was performed with mercaptoacetyltriglycine diuretic renogram, magnetic resonance urogram, excretory urogram, ultrasound and near infrared imaging. Scoring systems for ureteropelvic junction obstruction were developed for magnetic resonance urogram and near infrared imaging. Physicians and medical students graded ureteropelvic junction obstruction based on magnetic resonance urogram and near infrared imaging results. RESULTS: Markers of vascular and urinary dynamics were quantitatively consistent among control renal units. The same markers were abnormal in obstructed renal units with significantly different times of renal phase peak, start of pelvic phase and start of renal uptake. Such parameters were consistent with those obtained with mercaptoacetyltriglycine diuretic renography. Near infrared imaging provided live imaging of urinary flow, which was helpful in identifying the area of obstruction for surgical planning. Physicians and medical students categorized the degree of obstruction appropriately for fluorescence imaging and magnetic resonance urogram. CONCLUSIONS: Near infrared imaging offers a feasible way to obtain live, dynamic images of urine flow and ureteral peristalsis. Qualitative and quantitative parameters were comparable to those of conventional imaging. Findings support fluorescence imaging as an accurate, easy to use method of diagnosing ureteropelvic junction obstruction.

Variations in management of mild prenatal hydronephrosis among maternal-fetal medicine obstetricians, and pediatric urologists and radiologists.

PURPOSE: There are no current guidelines for diagnosing and managing mild prenatal hydronephrosis. Variations in physician approach make it difficult to analyze outcomes and establish optimal management. We determined the variability of diagnostic approach and management regarding prenatal hydronephrosis among maternal-fetal medicine obstetricians, pediatric urologists and pediatric radiologists. MATERIALS AND METHODS: Online surveys were sent to mailing lists for national societies for each specialty. Participants were surveyed regarding criteria for diagnosing mild prenatal hydronephrosis and recommendations for postnatal management, including use of antibiotic prophylaxis, followup scheduling and type of followup imaging. RESULTS: A total of 308 maternal-fetal medicine obstetricians, 126 pediatric urologists and 112 pediatric radiologists responded. Pediatric urologists and radiologists were divided between Society for Fetal Urology criteria and use of anteroposterior pelvic diameter for diagnosis, while maternal-fetal medicine obstetricians preferred using the latter. For postnatal evaluation radiologists preferred using personal criteria, while urologists preferred using anteroposterior pelvic diameter or Society for Fetal Urology grading system. There was wide variation in the use of antibiotic prophylaxis among pediatric urologists. Regarding the use of voiding cystourethrography/radionuclide cystography in patients with prenatal hydronephrosis, neither urologists nor radiologists were consistent in their recommendations. Finally, there was no agreement on length of followup for mild prenatal hydronephrosis. CONCLUSIONS: We observed a lack of uniformity regarding grading criteria in diagnosing hydronephrosis prenatally and postnatally among maternal-fetal medicine obstetricians, pediatric urologists and pediatric radiologists. There was also a lack of agreement on the management of mild intermittent prenatal hydronephrosis, resulting in these cases being managed inconsistently. A unified set of guidelines for diagnosis, evaluation and management of mild intermittent prenatal hydronephrosis would allow more effective evaluation of outcomes.

Clinical outcomes and long-term resolution in patients with persistent vesicoureteral reflux after open ureteral reimplantation.

PURPOSE: Success rates of ureteral reimplantation for primary vesicoureteral reflux are high. Few studies document the natural history of children with persistent vesicoureteral reflux. We reviewed their clinical outcomes and long-term resolution. MATERIALS AND METHODS: We performed a retrospective review of all children with persistent vesicoureteral reflux (grade 1 or greater) into the reimplanted ureter(s) on initial cystogram after reimplantation for primary vesicoureteral reflux at our institution from January 1990 to December 2002. We evaluated subsequent cystograms (graded on the 3-point radionuclide cystogram scale), surgery and urinary tract infection. We performed survival analyses of time to resolution of persistent (grade 1 or greater) and clinically significant (grade 2 or greater) vesicoureteral reflux in patients with more than 1 postoperative cystogram. RESULTS: Of 965 patients 59 (94 ureters) had persistent vesicoureteral reflux (6.1%), including 19 grade 1/3, 29 grade 2/3 and 11 grade 3/3. Median patient age at reimplantation was 1.9 years (range 0.8 to 5.1) and 62.7% were female. Preoperative vesicoureteral reflux grade was 2/3 in 42.4% and 3/3 in 57.6%, and 30.5% of patients had ureteral tapering. Median followup was 47.1 months (IQR 19.3-650.3). Reflux was resolved in 26 of 36 (72.2%) patients and median time to resolution was 20.4 months. Grade 2 or greater reflux on postoperative cystogram resolved in 21 of 32 (65.6%) patients and median time to resolution was 20.4 months. There were 10 patients with persistent vesicoureteral reflux at last cystogram, grade 1 or 2 in 9 and 3/3 in 1 patient. One patient underwent repeat reimplantation for persistent vesicoureteral reflux and 7 (13%) had postoperative febrile urinary tract infection at a median of 37 months postoperatively (IQR 1.7-64.4). CONCLUSIONS: Persistent vesicoureteral reflux after reimplantation resolves spontaneously in most children and can be managed nonoperatively with good long-term outcomes.

Protective locus against renal scarring on chromosome 11 in affected sib pairs with familial vesicoureteral reflux identified by single nucleotide polymorphism linkage analysis.

PURPOSE: We identified the loci associated with renal scarring risk and protection in affected sib pairs with familial vesicoureteral reflux. MATERIALS AND METHODS: A genome-wide analysis of vesicoureteral reflux with high density single nucleotide polymorphisms was conducted in 43 families with 2 or more affected children. A total of 43 probands and 58 affected siblings were included in the analysis. Genomic DNA was extracted from blood or saliva from all patients. All nuclear families had complete parental genotypes and all were Caucasian. Renal scarring was present in 23 of the 43 probands as detected by dimercapto-succinic acid imaging. easyLINKAGE software was used for the genome-wide linkage analysis. A LOD (logarithm [base 10] of odds) score of 3.3 or greater was considered significant evidence of linkage and a LOD score of 2.4 or greater but less than 3.3 was considered suggestive evidence of linkage. RESULTS: Using the affected sib pair method of analysis, a statistically significant linkage peak with a multipoint LOD score of 3.66 for patients without renal scarring was identified on chromosome 11 at 47.97 cM. For the scarring group a peak with a multipoint LOD score of 2.69 was identified on chromosome 17, which provides suggestive evidence of linkage. CONCLUSIONS: Our results suggest that a locus on chromosome 11 is associated with protection against renal scarring in patients with vesicoureteral reflux. In addition, a new locus on chromosome 17 may be linked to renal scarring. Our results suggest that multiple genes contribute to the formation of the vesicoureteral reflux phenotype, with patients having a unique susceptibility to renal injury/damage.

Development and initial validation of a disease specific quality of life survey for children with vesicoureteral reflux.

PURPOSE: Vesicoureteral reflux is believed to be a largely asymptomatic condition. However, previous studies have failed to assess the precise impact of vesicoureteral reflux on quality of life. We created and validated a survey for assessment of quality of life in children with vesicoureteral reflux. MATERIALS AND METHODS: Two surveys were created by a team of pediatric urologists and survey design specialists, 1 for general assessment and 1 for postoperative assessment in patients undergoing surgical treatment for vesicoureteral reflux. Cronbach alpha test was used to assess internal consistency reliability and the correlation coefficient for test-retest analysis. Surveys were distributed from November 2008 to January 2010 during routine office visits for vesicoureteral reflux. The postoperative survey was distributed 8 to 20 weeks following surgery. RESULTS: The general survey yielded a Cronbach alpha of 0.64 and a correlation coefficient of 0.78, while the postoperative survey results were 0.58 and 0.84, respectively, for the same parameters. Complete general surveys were obtained from 216 patients. All but 4 categories, related to repeat testing, office visits and medicine intake, reached greater than 80% positive quality of life responses. The postoperative survey was completed by 104 patients. Bladder spasm was the only category with less than 86% rate of positive quality of life response. CONCLUSIONS: Vesicoureteral reflux is shown to have little effect on quality of life in pediatric patients. These validated surveys should be applied to compare the effect of different management options on quality of life.

A comparative direct cost analysis of pediatric urologic robot-assisted laparoscopic surgery versus open surgery: could robot-assisted surgery be less expensive?

BACKGROUND AND PURPOSE: Cost in healthcare is an increasing and justifiable concern that impacts decisions about the introduction of new devices such as the da Vinci(®) surgical robot. Because equipment expenses represent only a portion of overall medical costs, we set out to make more specific cost comparisons between open and robot-assisted laparoscopic surgery. MATERIALS AND METHODS: We performed a retrospective, observational, matched cohort study of 146 pediatric patients undergoing either open or robot-assisted laparoscopic urologic surgery from October 2004 to September 2009 at a single institution. Patients were matched based on surgery type, age, and fiscal year. Direct internal costs from the institution were used to compare the two surgery types across several procedures. RESULTS: Robot-assisted surgery direct costs were 11.9% (P=0.03) lower than open surgery. This cost difference was primarily because of the difference in hospital length of stay between patients undergoing open vs robot-assisted surgery (3.8 vs 1.6 days, P<0.001). Maintenance fees and equipment expenses were the primary contributors to robotic surgery costs, while open surgery costs were affected most by room and board expenses. When estimates of the indirect costs of robot purchase and maintenance were included, open surgery had a lower total cost. There were no differences in follow-up times or complication rates. CONCLUSIONS: Direct costs for robot-assisted surgery were significantly lower than equivalent open surgery. Factors reducing robot-assisted surgery costs included: A consistent and trained robotic surgery team, an extensive history of performing urologic robotic surgery, selection of patients for robotic surgery who otherwise would have had longer hospital stays after open surgery, and selection of procedures without a laparoscopic alternative. The high indirect costs of robot purchase and maintenance remain major factors, but could be overcome by high surgical volume and reduced prices as competitors enter the market.

Vesicoureteral reflux: current trends in diagnosis, screening, and treatment.

CONTEXT: Vesicoureteral reflux (VUR) is present in approximately 1% of children in North America and Europe and is associated with an increased risk of pyelonephritis and renal scarring. Despite its prevalence and potential morbidity, however, many aspects of VUR management are controversial. OBJECTIVE: Review the evidence surrounding current controversies in VUR diagnosis, screening, and treatment. EVIDENCE ACQUISITION: A systematic review was performed of Medline, Embase, Prospero, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, clinicaltrials.gov, and the most recent guidelines of relevant medical specialty organizations. EVIDENCE SYNTHESIS: We objectively assessed and summarized the published data, focusing on recent areas of controversy relating to VUR screening, diagnosis, and treatment. CONCLUSIONS: The evidence base for many current management patterns in VUR is limited. Areas that could significantly benefit from additional future research include improved identification of children who are at risk for VUR-related renal morbidity, improved stratification tools for determining which children would benefit most from which VUR treatment option, and improved reporting of long-term outcomes of VUR treatments.

Durability of antireflux effect of ureteral reimplantation for primary vesicoureteral reflux: findings on long-term cystography.

OBJECTIVE: To evaluate the long-term durability of successful ureteral reimplantation (UR) for vesicoureteral reflux (VUR) through a review of late cystography (LC) findings. MATERIALS AND METHODS: We performed a retrospective chart review of all children with primary VUR who underwent successful open UR (grade 0 VUR into the reimplanted ureter[s] on initial cystogram) at our institution from January 1990 to December 2002. We identified successful UR patients who underwent LC ≥ 1 year after UR and reviewed the results for the presence of recurrent VUR into the reimplanted ureter(s). RESULTS: Seven-hundred ninety-four patients underwent successful open UR for primary VUR, of whom 60 (7.6%) had a subsequent LC. Preoperative VUR grade was ≤ II in 20 (34.5%) and ≥ III in 38 (65.5%). Median age at UR was 3.5 years (IQR 1.3-6.2 years); 51 (85%) were female. UR was intravesical in 45 (75%) and bilateral in 19 (32%). LC was performed at a median of 38.7 months after UR (IQR 19.6-66.1 months). Indication for LC was febrile urinary tract infection (UTI) in 16 (27%), nonfebrile UTIs in 15 (25%), follow-up of contralateral VUR in 16 (27%), and other clinical indications in 13 (21%). The recurrence rate was 0%; of the 79 reimplanted ureters, 100% (95% CI 95.4-100) had no VUR (grade 0). CONCLUSION: Among children who underwent successful open UR for primary VUR, there was no VUR recurrence on extended follow-up. This suggests that the late durability of open antireflux surgery is excellent.

Genome gender diversity in affected sib-pairs with familial vesico-ureteric reflux identified by single nucleotide polymorphism linkage analysis.

UNLABELLED: Study Type - Aetiology (case series) Level of Evidence 4 What's known on the subject? and What does the study add? Genetic linkage to distinguish loci for VUR has been previously described in several autosomal chromosomes. Although there are numerous explanations for the dissimilar findings, e.g. multifactorial etiology of VUR and hereditary miscellany among studied populations, clinical diversity between males and females may indicate a central gender-specific genetic susceptibility. Early studies suggested the presence of modified VUR gene(s) on the X-chromosome, accounting for the higher incidence of this disorder among female members in the pedigrees studied. On the other hand male-to-male transmission and a higher ratio of females to males argued against X-linked inheritance. More recently, additional chromosomal regions (i.e. chromosomes 1-7, 10-13, and 18-22) have been identified for VUR by using single nucleotide polymorphism-genome-wide linkage analysis. This is the first study to show that there is autosomal difference in VUR expression in males and females. This genotype variability may be the basis for the clinical differences between genders in children with VUR. OBJECTIVE: To assess gender-specific genetic differences in the susceptibility loci for vesico-ureteric reflux (VUR) in families who have two or more affected children. PATIENTS AND METHODS: A genome-wide linkage analysis of VUR with high-density single nucleotide polymorphisms was conducted in 98 families with two or more affected children. A total of 221 affected offspring (123 sibling pairs) were included in the analysis. Genomic DNA was extracted from blood or saliva from all the patients. Data was stratified and analysed according to clinical presentation and gender of the proband and affected siblings. RESULTS: Using the affected sib-pair method, statistically significant peaks were found on chromosomes 1 (logarithm of odds, base10 [LOD] 4.4) and 5 (LOD 3.7) in males and on chromosomes 3 (LOD 3.5), 13 (LOD 4.5), and 15 (LOD 3.4) in females. CONCLUSION: This genotype variability might be the basis for the clinical differences between genders in children with VUR. Our data might be the first step to understanding the genetic background behind the gender-specific differences of VUR and more clearly defining the genetically different subgroups of VUR.

Development and implementation of a photographic atlas for parental instruction and guidance after outpatient penile surgery.

OBJECTIVE: To develop and evaluate a visual tool to assist parents in assessing healing after surgical circumcision or revision circumcision (SCRC). METHODS: Among children undergoing SCRC, photographs were taken on postoperative days 0-1-2-3-5-7-10-14-21, and compiled into an atlas. Atlas utility was assessed during two 1-month periods. During the first period (M1) families received routine postoperative instructions only; during the second period (M2), families received the atlas in addition to routine instructions. Families were surveyed by phone and calls/contacts were tracked. RESULTS: 33 families (among 83 SCRCs) were surveyed during M1, vs 39 families (among 77 SCRCs) during M2 (p = 0.17). Nearly all reported the atlas helpful (59% very helpful, 27% moderately helpful, 9% somewhat helpful). All but one family used the atlas. There was a trend toward families receiving the atlas being more comfortable (64% vs 82% very comfortable, p = 0.12). Survey scores were similar between M1 and M2 for total score, satisfaction, and the number whose expectations were met (58% vs 55%, p = 0.21). Phone contacts decreased between M1 and M2, both in absolute number (M1 = 24 calls vs M2 = 12 calls), and as a proportion of total cases performed (29% vs 16%, p = 0.04). CONCLUSIONS: The circumcision atlas was well received by families and was associated with a significant decrease in post-surgical telephone calls. The atlas has been put into routine clinical use with excellent response.

Hydronephrosis following ureteral reimplantation: when is it concerning?

OBJECTIVES: Hydronephrosis without obstruction is common prior to ureteral reimplant, especially in patients with high-grade VUR. Consequently, when hydronephrosis is present post-operatively, it is unclear when it should be concerning. We evaluated the finding of hydronephrosis in children undergoing reimplantation and its evolution following surgery. METHODS: After obtaining IRB approval, we identified 938 children who underwent reimplantation at our institution from 1998 to 2006. Their pre- and post-operative US and clinical course were analyzed. RESULTS: Hydronephrosis was observed in 24% pre-operatively and 21% post-operatively. 52% with pre-operative hydronephrosis had it post-operatively, while 12% without pre-operative hydronephrosis had it post-operatively. 71% of post-operative hydronephrosis resolved on average in 1.36 years. 19% didn't resolve and 0.1% had ureteral obstruction. Risk factors for post-operative hydronephrosis included increasing severity of VUR, and high degree of pre-operative hydronephrosis. CONCLUSION: Hydronephrosis following ureteral reimplantation is not rare, and correlated to pre-operative evaluations. Post-operative hydronephrosis is frequently transient and benign, and usually resolves within the first 2 years. These patients do not require follow-up ultrasounds or further imaging, and can be followed clinically. Patients with high-grade VUR and hydronephrosis pre-operatively, however, are at risk for developing worsened hydronephrosis and should be followed with a 3-month post-operative ultrasound.