Use of Perirectal Hyaluronic Acid Spacer Prior to Radiotherapy in a Pediatric Patient With Bladder Rhabdomyosarcoma: A Case Report.

Rhabdomyosarcoma (RMS) treatment involves surgery, chemotherapy, and radiotherapy. A radioprotective space between the bladder/prostate and rectum reduces postradiation complications, as reported in adult patients. Describe pediatric preradiotherapy perirectal hyaluronic acid (HA) spacer injection for bladder/prostate RMS. We present a case of a 17-month-old male with bladder/prostate RMS. Before radiotherapy, an HA spacer was injected peri-rectally. Under general anesthesia, a transrectal ultrasound was positioned and 1mL of HA spacer was injected into the perirectal space. No complications were reported at 6-month follow-up. This is the first report of pre-radiation therapy spacer injection for pediatric bladder/prostate RMS.

The Utilization of an Opioid-Free Anesthetic for Pediatric Circumcision in an Ambulatory Surgery Center.

Circumcision is one of the most common urologic procedures performed at pediatric ambulatory centers. Emerging data on the short- and long-term effects of perioperative opioid administration has highlighted the importance of an opioid-free anesthetic regimen. We sought to evaluate the effectiveness of an opioid-free anesthetic in pediatric circumcision and its correlation with ambulatory surgery center efficiency. Patients, 3 years of age and younger, who underwent circumcision or circumcision revision by two surgeons pre and post introduction of an opioid-free anesthetic fast-track regimen at an outpatient surgical center were included. There were 100 patients included in this analysis, with 50 patients in each cohort. On univariate analysis, fast-tracking was associated with a decrease in median combined in-room and post-anesthesia care unit times (102.5 vs. 129.0 min, p-value < 0.001). This difference continued after multivariable analysis with an adjusted median combined in-room and post-anesthesia care unit time difference of -15.6 min (95% CI -34.2 to -12.7 min, p-value 0.018). In addition, the fast-track cohort received less intraoperative morphine equivalents without an increase in post-operative analgesic administration or change in postoperative questionnaire score. This demonstrates that opioid-free anesthesia may be used effectively in pediatric circumcision while also allowing for significant time savings for surgical centers.

Accuracy of subjective vesicoureteral reflux timing assessment: supporting new voiding cystourethrogram guidelines.

BACKGROUND: Bladder volume at the onset of vesicoureteral reflux (VUR) is an important prognostic indicator of spontaneous resolution and the risk of pyelonephritis. OBJECTIVE: We aim to determine whether pediatric urologists and pediatric radiologists can accurately estimate the timing of reflux by examining voiding cystourethrogram (VCUG) images without prior knowledge of the instilled contrast volume. MATERIALS AND METHODS: Total bladder volume and the volume at the time of reflux were collected from VCUG reports to determine the volume at the onset of VUR. Thirty-nine patients were sorted into three groups: early-/mid-filling reflux, late-filling and voiding only. Thirty-nine images were shown to three pediatric urologists and two pediatric radiologists in a blinded fashion and they were then asked to estimate VUR timing based on the above categories. A weighted kappa statistic was calculated to assess rater agreement with the gold standard volume-based report of VUR timing. RESULTS: The mean patient age at VCUG was 3.1±2.9 months, the median VUR was grade 3, and 20 patients were female. Overall agreement among all five raters was moderate (k=0.43, 95% confidence interval [CI] 0.36-0.50). Individual agreement between rater and gold standard was slight to moderate with kappa values ranging from 0.13 to 0.43. CONCLUSION: Pediatric radiologists and urologists are unable to accurately and reliably characterize VUR timing on fluoroscopic VCUG. These findings support the recently published American Academy of Pediatrics protocol recommending the routine recording of bladder volume at the onset of VUR as a standard component of all VCUGs to assist in a more accurate assessment of the likelihood of resolution and risk of recurrent urinary tract infections.

Opioid utilization is minimal after outpatient pediatric urologic surgery.

INTRODUCTION: There are no guidelines for opioid use after pediatric urologic surgery, and it is unknown to what extent prescriptions written for these patients may be contributing to the opioid epidemic in the United States. We sought to characterize opioid utilization in a prospective fashion following outpatient pediatric urologic surgery at our institution. MATERIALS AND METHODS: After obtainingapproval from the Institutional Review Board, we prospectively recruited pediatric patients undergoing outpatient urologic surgery. All patients and families were counseled regarding appropriate use of over-the-counter pain medications as first-line agents, with opioids for breakthrough pain only. All patients received an opioid prescription (ORx), which we attempted to standardize to 10 doses. Parents were provided with a log for keeping track of pain medication administration. Postoperative surveys were sent at various time points after surgery to assess utilization of pain medications at home. We quantified unused opioids prescribed and evaluated factors potentially associated with opioid use. RESULTS: Two hundred and two patients were recruited. All patients were male, with a median age of 2.7 years (interquartile range (IQR) 5.5, range 0.5-17.9 years). One hundred and fifty-four children underwent penile surgery, 22 underwent scrotal surgery, and 27 underwent inguinal surgery. Nearly half of our study patients were black, 33.2% were white, 12.9% were Latino, and 4.0% were Asian. The median number of doses prescribed was 10 (IQR 0, range 4.0-20.8). Postoperative surveys were completed by 80.7% of study patients. The median number of opioid doses used was 0 (IQR 2), whereas the mean was 1.28 (standard deviation (SD) 1.98). None of the factors evaluated (including patient age, surgery type, perioperative pain management techniques, length of surgery, and insurance type) were associated with the amount of opioid used at home after surgery, as utilization was equally low across all groups. DISCUSSION AND CONCLUSIONS: Ensuring adequate postoperative pain control for children is critical, yet it is also important to minimize excess ORx. We found that the majority of pediatric patients used 0-2 doses of prescription pain medication after discharge following outpatient urologic surgery, representing a small percentage of the total prescribed amount. Low utilization was seen irrespective of patient age, procedure, and perioperative factors. These data can be used to guide perioperative patient and family counseling and to guide future efforts to standardize ORx following outpatient pediatric urologic surgery.

Parental Preferences for Vesicoureteral Reflux Treatment: A Crowd-sourced, Best-worst Scaling Study.

OBJECTIVE: To quantitatively evaluate parental preferences for the various treatments for vesicoureteral reflux using crowd-sourced best-worst scaling, a novel technique in urologic preference estimation. METHODS: Preference data were collected from a community sample of parents via 2 best-worst scaling survey instruments published to Amazon's Mechanical Turk online community. Attributes and attribute levels were selected following extensive review of the reflux literature. Respondents completed an object case best-worst scaling exercise to prioritize general aspects of reflux treatments and multiprofile case best-worst scaling to elicit their preferences for the specific differences in reflux treatments. Data were analyzed using multinomial logistic regression. Results from the object-case provided probability scaled values (PSV) that reflected the order of importance of attributes. RESULTS: We analyzed data for 248 and 228 respondents for object and multiprofile case BWS, respectively. When prioritizing general aspects of reflux treatment, effectiveness (PSV = 20.37), risk of future urinary tract infection (PSV = 14.85), and complication rate (PSV = 14.55) were most important to parents. Societal cost (PSV = 1.41), length of hospitalization (PSV = 1.09), and cosmesis (PSV = 0.91) were least important. Parents perceived no difference in preference for the cosmetic outcome of open vs minimally invasive surgery (P = .791). Bundling attribute preference weights, parents in our study would choose open surgery 74.9% of the time. CONCLUSION: High treatment effectiveness was the most important and preferred attribute to parents. Alternatively, cost and cosmesis were among the least important. Our findings serve to inform shared parent-physician decision-making for vesicoureteral reflux.

Prospective multicenter study on robot-assisted laparoscopic extravesical ureteral reimplantation (RALUR-EV): Outcomes and complications.

BACKGROUND: Robot-assisted laparoscopic extravesical ureteral reimplantation (RALUR-EV) is a minimally invasive alternative to open surgery. We have previously reported retrospective outcomes from our study group, and herein provide an updated prospective analysis with a focus on success rate, surgical technique, and complications among surgeons who have overcome the initial learning curve. OBJECTIVE: To assess the safety and efficacy of RALUR-EV in children, among experienced surgeons. DESIGN AND METHODS: We reviewed our prospective database of children undergoing RALUR-EV by pediatric urologists at eight academic centers from 2015 to 2017. Radiographic success was defined as absence of vesicoureteral reflux (VUR) on postoperative voiding cystourethrogram. Complications were graded using the Clavien scale. Univariate regression analysis was performed to assess for association among various patient and technical factors and radiographic failure. RESULTS: In total, 143 patients were treated with RALUR-EV for primary VUR (87 unilateral, 56 bilateral; 199 ureters). The majority of ureters (73.4%) had grade III or higher VUR preoperatively. Radiographic resolution was present in 93.8% of ureters, as shown in the summary table. Ureteral complications occurred in five ureters (2.5%) with mean follow-up of 7.4 months (SD 4.0). Transient urinary retention occurred in four patients following bilateral procedure (7.1%) and in no patients after unilateral. On univariate analysis, there were no patient or technical factors associated with increased odds of radiographic failure. DISCUSSION: We report a radiographic success rate of 93.8% overall, and 94.1% among children with grades III-V VUR. In contemporary series, alternate management options such as endoscopic injection and open UR have reported radiographic success rates of 90% and 93.5% respectively. We were unable to identify specific patient or technical factors that influenced outcomes, although immeasurable factors such as tissue handling and intraoperative decision-making could not be assessed. Ureteral complications requiring operative intervention were rare and occurred with the same incidence reported in a large open series. Limitations include lack of long-term follow-up and absence of radiographic follow-up on a subset of patients. CONCLUSIONS: Radiographic resolution of VUR following RALUR is on par with contemporary open series, and the incidence of ureteral complications is low. RALUR should be considered as one of several viable options for management of VUR in children.

Molecular Characterization of Renal Cell Carcinoma: A Potential Three-MicroRNA Prognostic Signature.

Background: Aberrantly expressed miRNAs promote renal cell carcinoma (RCC) growth and metastasis and are potentially useful biomarkers for metastatic disease. However, a consensus clinically significant miRNA signature has not been identified. To identify an miRNA signature for predicting clinical outcome in RCC patients, we used a four-pronged interconnected approach.Methods: Differentially expressed miRNAs were identified and analyzed in 113 specimens (normal kidney: 59; tumor: 54). miRNA profiling was performed in matched normal and tumor specimens from 8 patients and extended to 32 specimens. Seven aberrantly expressed miRNAs were analyzed by qPCR, and their levels were correlated with RCC subtypes and clinical outcome. miRNA signature was confirmed in The Cancer Genome Atlas RCC dataset (n = 241).Results: Discovery phase identified miR-21, miR-142-3p, miR-142-5p, miR-150, and miR-155 as significantly upregulated (2-4-fold) and miR-192 and miR-194 as downregulated (3-60-fold) in RCC; miR-155 distinguished small tumors (<4 cm) from benign oncocytomas. In univariate and multivariate analyses, miRNA combinations (miR-21+194; miR-21+142-5p+194) significantly predicted metastasis and/or disease-specific mortality; miR-21+142-5p+194 (for metastasis): P = 0.0017; OR, 0.53; 95% confidence interval (CI), 0.75-0.33; 86.7% sensitivity; 82% specificity. In the TCGA dataset, combined biomarkers associated with metastasis and overall survival (miR-21+142-5p+194: P < 0.0001; OR, 0.37; 95% CI, 0.58-0.23).Conclusions: The interconnected discovery-validation approach identified a three-miRNA signature as a potential predictor of disease outcome in RCC patients.Impact: With 10% survival at 5 years, metastatic disease presents poor prognosis for RCC patients. The three-miRNA signature discovered and validated may potentially at an early stage detect and predict metastasis, to allow early intervention for improving patient prognosis. Cancer Epidemiol Biomarkers Prev; 27(4); 464-72. ©2018 AACR.

National Trends in the Management of Primary Vesicoureteral Reflux in Children.

PURPOSE: In September 2011 the AAP (American Academy of Pediatrics) released updated guidelines for the evaluation of children 2 to 24 months old with a febrile urinary tract infection. We documented the impact of the guideline on diagnosis and surgical management of vesicoureteral reflux at U.S. children's hospitals. We hypothesized that voiding cystourethrogram studies and the vesicoureteral reflux treatment rate decreased concurrent with the national guideline release. MATERIALS AND METHODS: The Pediatric Health Information System was queried for children (younger than 18 years) with primary vesicoureteral reflux and their antireflux surgical history from January 2004 to June 2015. Voiding cystourethrogram orders were recorded. Interrupted time series analysis quantified trends surrounding several seminal vesicoureteral reflux publications (2007) and guideline publication (2011). RESULTS: A total of 43,341 voiding cystourethrogram encounters (male 23,946 [55.3%]) were identified for patients at a median age of 3 months (IQR 1-20). For all children monthly voiding cystourethrogram orders increased (+1.0 to +1.6 encounters per month, p <0.034) to September 2011, then sharply declined by 106 encounters per month from September to October 2011 (p <0.001) then did not change significantly (p=0.096, R2=0.79). For those children 2 to 24 months old with a urinary tract infection (3,379 records; male 1,384 [41.0%], median age 4 months [IQR 3-7]) voiding cystourethrograms gradually increased from January 2007 to September 2011 (+0.1 encounters per month, p=0.036), then similarly decreased by 21 encounters per month from September to October 2011 (p <0.001), then did not change significantly (p=0.064, R2=0.78). Overall 28,484 procedures for primary vesicoureteral reflux were identified (male 5,950 [20.9%], median age 4.8 years [IQR 2.5-7.2]). Total surgical procedures did not change significantly until October 2011, then declined (-1.5 procedures per month, p <0.001, R2=0.66). CONCLUSIONS: The number of voiding cystourethrograms ordered nationally in all children and those with a urinary tract infection decreased sharply with the 2011 AAP urinary tract infection guideline release and did not change thereafter. A steady decline in procedures for primary vesicoureteral reflux occurred after October 2011.

Hyaluronic acid family in bladder cancer: potential prognostic biomarkers and therapeutic targets.

BACKGROUND: Molecular markers of clinical outcome may aid in designing targeted treatments for bladder cancer. However, only a few bladder cancer biomarkers have been examined as therapeutic targets. METHODS: Data from The Cancer Genome Atlas (TCGA) and bladder specimens were evaluated to determine the biomarker potential of the hyaluronic acid (HA) family of molecules - HA synthases, HA receptors and hyaluronidase. The therapeutic efficacy of 4-methylumbelliferone (4MU), a HA synthesis inhibitor, was evaluated in vitro and in xenograft models. RESULTS: In clinical specimens and TCGA data sets, HA synthases and hyaluronidase-1 levels significantly predicted metastasis and poor survival. 4-Methylumbelliferone inhibited proliferation and motility/invasion and induced apoptosis in bladder cancer cells. Oral administration of 4MU both prevented and inhibited tumour growth, without dose-related toxicity. Effects of 4MU were mediated through the inhibition of CD44/RHAMM and phosphatidylinositol 3-kinase/AKT axis, and of epithelial-mesenchymal transition determinants. These were attenuated by HA, suggesting that 4MU targets oncogenic HA signalling. In tumour specimens and the TCGA data set, HA family expression correlated positively with ß-catenin, Twist and Snail expression, but negatively with E-cadherin expression. CONCLUSIONS: This study demonstrates that the HA family can be exploited for developing a biomarker-driven, targeted treatment for bladder cancer, and 4MU, a non-toxic oral HA synthesis inhibitor, is one such candidate.

Vesicoureteral Reflux Index: Predicting Primary Vesicoureteral Reflux Resolution in Children Diagnosed after Age 24 Months.

PURPOSE: The Vesicoureteral Reflux Index is a validated tool that reliably predicts spontaneous resolution of reflux or at least 2 grades of improvement for patients diagnosed before age 24 months. We evaluated the Vesicoureteral Reflux Index in children older than 2 years. MATERIALS AND METHODS: Patients younger than 18 years who were diagnosed with primary vesicoureteral reflux after age 24 months and had undergone 2 or more voiding cystourethrograms were identified. Disease severity was scored using the Vesicoureteral Reflux Index, a 6-point scale based on gender, reflux grade, ureteral abnormalities and reflux timing. Proportional subdistribution hazard models for competing risks identified variables associated with resolution/improvement at different time points. RESULTS: A total of 21 males and 250 females met inclusion criteria. Mean ± SD age was 4.0 ± 2.1 years and patients had a median vesicoureteral reflux grade of 2. The Vesicoureteral Reflux Index score improved by 1 point in 1 patient (100%), 2 points in 25 (67.6%), 3 points in 48 (37%), 4 points in 18 (21.4%) and 5 to 6 points in 4 (18.2%). Female gender (p = 0.005) and vesicoureteral reflux timing (late filling, p = 0.002; early/mid filling, p <0.001) independently predicted nonresolution. Median resolution time based on Vesicoureteral Reflux Index score was 2 months or less in 15.6% of patients (95% CI 11.0-13.8), 3 months in 34.7% (95% CI 25.4-44.1), 4 months in 55.9% (95% CI 40.1 to infinity) and 5 months or more in 30.3% (95% CI 29.5 to infinity). High grade (IV or V) reflux was not associated with resolution at any point. Ureteral abnormalities were associated with lack of resolution in the first 12 to 18 months (HR 0.29, 95% CI 0.29-0.80) but not in later followup. Vesicoureteral Reflux Index scores of 3, 4 and 5 were significantly associated with lack of resolution/improvement compared to scores of 2 or less (p = 0.031). CONCLUSIONS: The Vesicoureteral Reflux Index reliably predicts primary vesicoureteral reflux improvement/resolution in children diagnosed after age 24 months. Spontaneous resolution/improvement is less likely as Vesicoureteral Reflux Index score and time from diagnosis increase.

Surgical Scar Location Preference for Pediatric Kidney and Pelvic Surgery: A Crowdsourced Survey.

PURPOSE: The benefits of minimally invasive surgery in pediatric urology, such as reduced length of hospital stay and postoperative pain, are less predictable compared to findings in the adult literature. We evaluated the choices that adult patients make for themselves and their children regarding scar location. MATERIALS AND METHODS: We surveyed the preference for scar location/size based on surgery for bladder and kidney procedures with additional questions assessing the impact of a hidden incision, length of hospital stay and pain. The survey was posted to Amazon® Mechanical Turk®. RESULTS: We analyzed a total of 954 completed surveys. Surgical history was reported in 660 surveys (69%) with scar bother reported in 357 (54.2%). For pelvic surgery the initial choice was a Pfannenstiel incision for 434 respondents (45.5%), laparoscopy port incisions for 392 (41.1%) and no preference for incision location for 126 (13.2%). When incisions were illustrated relative to undergarments, 718 respondents (75.3%) chose Pfannenstiel. For kidney surgery 567 respondents (59.4%) initially chose the dorsal lumbotomy incision, 170 (17.8%) chose a flank incision, 105 (11.0%) chose laparoscopy ports and 110 (11.5%) had no preference. Respondents were told that minimally invasive surgery might result in less pain/length of hospital stay and were asked to restate the incision choice. For pelvic surgery 232 of 434 respondents (53.5%) who had chosen Pfannenstiel and 282 of 394 (71.6%) who had chosen laparoscopy remained consistent (p <0.001). For kidney surgery 96 respondents (56.5%) who chose a flank incision, 322 (56.8%) who chose dorsal lumbotomy and 68 (64.2%) who chose laparoscopy remained consistent (p = 0.349). Agreement between the incision choice by respondent as a child and for a child was 82% (κ = 0.69) for pelvic surgery and 84.6% (κ = 0.75) for kidney surgery. CONCLUSIONS: The smallest incision is not always the patient preferred incision, particularly in childhood when pain, length of hospital stay and blood loss may be equivocal among approaches. Discussion of surgical treatment options should include scar length, location and relationship to undergarments.

Delayed upper tract drainage on voiding cystourethrogram may not be associated with increased risk of urinary tract infection in children with vesicoureteral reflux.

INTRODUCTION: Urinary stasis in the setting of obstruction provides an opportunistic environment for bacterial multiplication and is a well-established risk factor for UTI. Vesicoureteral reflux (VUR) with delayed upper tract drainage (UTD) on VCUG has been reported to correlate with increased UTI risk. We sought to determine whether delayed UTD can be reliably classified, and whether it correlates with UTI incidence, VCUG, or endoscopic findings. METHODS: Children undergoing endoscopic surgery for primary VUR (2009-2012) were identified. VUR grade, timing, and laterality were abstracted. Demographics, hydrodistention (HD) grade, reported febrile and culture-proven UTI were assessed. UTD on VCUG was graded on post-void images as 1 = partial/complete UTD or 2 = no/increased UTD. Inter-observer agreement was calculated. Patients were excluded for incomplete imaging or inability to void during VCUG. RESULTS: The cohort included 128 patients (10M, 118F), mean age 4.1 ± 2.1 years. Mean age at diagnosis was 2.8 ± 2.8 years. Mean maximum VUR grade was 3 ± 0.9: 1 (7.8%), 2 (20.3%), 3 (43%), 4 (25.8%), 5 (3.1%). UTD occurred in 45 (35%), and no drainage in 83 (65%) patients. Agreement coefficient between graders was 0.596 (p < 0.0001). Cultures were available in 100 patients (70 positive). Patients experienced a mean of 2 ± 1.2 parent-reported fUTIs and 1.2 ± 1.2 culture-proven UTIs from birth to surgery. UTI rate did not differ by UTD status for parent or culture-proven UTI (Table). On multivariate analysis, no patient characteristic was a significant predictor of UTI based on drainage status. Children diagnosed with VUR before 1 year of age had a higher verified UTI rate (p < 0.001). However, drainage was not a significant predictor of UTI rate and when testing the interaction of drainage and age. CONCLUSION: We sought to determine whether UTD was an accurate predictor of UTI risk to maximize available prognostic information from a single VCUG. Delayed UTD was not a predictor of infection in our patients, nor was it associated with previously described UTI risk factors, such as VUR timing or grade, and voiding dysfunction. Limitations included the retrospective nature of the study in patients undergoing endoscopic VUR treatment, and possible inaccurate UTI reports from parents and pediatricians. UTD can be reliably scored using a binary system with high inter-observer correlation. Our data call into question the previous finding that children with poor UTD are at increased risk of recurrent UTI. Delayed UTD is also not associated with higher HD, or VUR grade compared with those with more prompt UTD.

Vesicoureteral Reflux Index: 2-Institution Analysis and Validation.

PURPOSE: The vesicoureteral reflux index is a novel tool designed to predict spontaneous reflux resolution in infants younger than 2 years. We performed a multi-institutional validation study to confirm the discriminatory power of the vesicoureteral reflux index to predict the vesicoureteral reflux resolution rate in young children. MATERIALS AND METHODS: We identified patients diagnosed with primary vesicoureteral reflux before age 24 months who had 2 or more voiding cystourethrograms available. Demographics, vesicoureteral reflux grade and timing, ureteral anomalies and radiographic outcomes were evaluated. The C-index was estimated for time to event model assessment. RESULTS: A total of 219 girls and 150 boys met study inclusion criteria. Mean ± SD age at diagnosis was 4.7 ± 4.9 months. Of the patients 101 (27.4%) had early to mid filling, 214 (58%) had late filling and 54 (14.6%) had voiding only vesicoureteral reflux. High grade reflux was present in 87 patients (23.6%) and ureteral anomalies were observed in 50 (13.6%). A vesicoureteral reflux index of 1, 2, 3, 4 and 5 or greater showed an improvement/resolution rate of 88.2%, 77.3%, 62.3%, 32.1% and 14.3%, respectively. On time to event analysis children with filling phase vesicoureteral reflux (p <0.001), grade 4-5 reflux (p <0.001) and ureteral anomalies (p = 0.003) had significantly longer median time to resolution. Median time to spontaneous resolution was 12.6, 12.7, 15.1, 25.6 and 31 months or greater for a vesicoureteral reflux index of 1, 2, 3, 4 and 5 or greater, respectively (C-index 0.305, 95% CI 0.252-0.357). During the study period 65 patients (17.6%) underwent surgical intervention. CONCLUSIONS: The vesicoureteral reflux index is a simple tool that reliably predicts significant improvement and spontaneous resolution of primary reflux in patients diagnosed at younger than 2 years. The index provides valuable prognostic information, facilitating individualized patient care.

The dilemma of adolescent varicocele.

Adolescent varicocele is associated with ipsilateral testicular hypotrophy and the concern for future infertility. A testicular size discrepancy greater than 15-20 % between left and right testicle is an indication for treatment to allow catch-up growth in the hope of preventing a future decline in fertility. Some authors advocate for a period of watchful waiting, as normal testicular growth may occur asymmetrically. We review the current literature to highlight some controversies and challenges in management.

Sigmoid vaginoplasty with a modified single Monti tube: a pediatric case series.

PURPOSE: No consensus exists regarding the most effective procedure for neovagina formation. We describe our experience with modified single Monti tube colovaginoplasty in pediatric patients with disorders of sexual differentiation. MATERIALS AND METHODS: Six patients were retrospectively identified who underwent primary sigmoid vaginoplasty with a modified single Monti tube between 2009 and 2012. Data were collected from patient charts. The procedure is performed by isolating an 8 to 10 cm segment of distal sigmoid colon or proximal rectum, which is detubularized along the anterior mesentery, folded and retubularized longitudinally, leaving the mesentery in a cephalad position. A channel is dissected in the pelvis to accommodate the neovagina. RESULTS: Mean patient age was 12.7 years (range 6 to 17). The primary diagnosis was androgen insensitivity in 3 cases (50%), and Mayer-Rokitansky syndrome, partial androgen insensitivity and persistent cloaca in 1 each (16.7%). Chromosomal analysis revealed 46XY in 4 patients (66.7%). Median followup was 7.9 months (range 3 to 41). One patient who engages in vaginal intercourse reported satisfactory vaginal length without discomfort. In 1 patient an anastomotic stricture developed, which was managed by buccal mucosal grafting. CONCLUSIONS: Modified single Monti tube sigmoid vaginoplasty is a safe, effective technique for neovagina formation in pediatric patients with disorders of sexual differentiation. Compared to other existing methods, our technique allows for the use of shorter bowel segments with decreased tension of the vascular pedicle.

Molecular marker for predicting treatment response in advanced renal cell carcinoma: does the promise fulfill clinical need?

Renal cell carcinoma (RCC) is largely diagnosed incidentally on imaging taken for unrelated reasons. The management of localized lesions is primarily extirpative with excellent results. Treatment of advanced RCC has evolved over recent years with the use of targeted therapies such as tyrosine kinase inhibitors, mammalian target of rapamycin inhibitors, and antibody-mediated therapies. The treatment response to these targeted therapies is highly variable, with no clear clinical method of identifying patients who will benefit from or not tolerate therapy. The field of molecular markers has evolved significantly in the last decade, with a multitude of markers identified that predict treatment response and drug toxicity. The following review critically evaluates those molecular markers that have been assessed for their utility in predicting treatment response in patients with advanced/metastatic renal cell carcinoma (mRCC). Identifying the ideal treatment for these patients will improve responses to therapy, minimize morbidity, and save significant healthcare dollars.

Inferior vena cava resection and reconstruction: technical considerations in the surgical management of renal cell carcinoma with tumor thrombus.

The mainstay of treatment for renal cell carcinoma with invasion of the inferior vena cava (IVC) is complete surgical extirpation. Cases complicated by adherent or invasive tumor thrombus represent a special technical challenge due to the need for resection of a large segment of the IVC. The aim of this review is to describe the indications and surgical approach for radical nephrectomy with en bloc resection of the IVC with or without venous reconstruction. In addition, special attention is paid to the relevant anatomical and hemodynamic considerations related to the development of venous collateral pathways secondary to IVC obstruction.

Transplantation techniques for the resection of renal cell carcinoma with tumor thrombus: a technical description and review.

Renal cell carcinoma with tumor thrombus of the inferior vena cava presents a special surgical challenge. The use of surgical techniques derived from the field of transplantation surgery have previously been shown to enable optimal control of inferior vena cava, allowing for the extirpation of most tumors via a transabdominal approach without the need for venovenous or cardiopulmonary bypass. In this report, we provide a stepwise description of the transplantation techniques employed at the University of Miami/Jackson Memorial Hospital for the resection of renal cell carcinoma with intracaval tumor. In addition, we review the results of centers that have utilized these surgical maneuvers.

Atherosclerosis within the non-neoplastic margin of partial nephrectomy specimens: implications for medical management.

PURPOSE: To describe the frequency and severity of atherosclerosis contained within the non-neoplastic tissue of partial nephrectomy (PN) specimens. METHODS: Archived open PN specimens were evaluated for histological evidence of atherosclerosis. Clinically significant atherosclerosis was defined as arterial luminal narrowing of >25% due to fibrointimal thickening. Histological findings were correlated with clinical data including history of major medical comorbidities and preoperative statin use. RESULTS: The study cohort was comprised of 114 patients (71 men and 43 women). The mean age at the time of surgery was 59.3 years, and 69 (60.5%) patients had a history of at least one major medical comorbidity including hypertension (54.5%), diabetes (16.7%) and coronary artery disease (12.3%). Clinically significant atherosclerosis was observed in 29 (25.4%) patients. These individuals were older (p = 0.001), and three times more likely to have greater than one major medical comorbidity (p = 0.002). In addition, only 17 (58.6%) were prescribed a statin at the time of surgery. CONCLUSIONS: Atherosclerosis is frequently observed in the non-neoplastic tissue of PN specimens. Patients found to have atherosclerosis can potentially benefit from intensive lifestyle modification and medical therapy with lipid-lowering medications. These measures would likely have the greatest clinical impact on those patients without an existing history of major medical comorbidities.

Pathologic evaluation of non-neoplastic renal parenchyma in partial nephrectomy specimens.

PURPOSE: This study aimed to identify non-neoplastic pathologic changes in partial nephrectomy specimens of patients without a known history of medical comorbidities. Routine analysis of this tissue may allow the clinician to identify subclinical renal disease. METHODS: We retrospectively reviewed our database of patients who underwent open partial nephrectomy for a small renal mass. Non-neoplastic tissue of partial nephrectomy specimens of patients without a known history of chronic kidney disease, diabetes mellitus, hypertension, or coronary artery disease was evaluated for glomerular, interstitial, and vascular pathologic changes. RESULTS: A rim of non-neoplastic tissue was adequate for pathologic evaluation in 91.8% of specimens. A total of 45 patients were studied with a median age of 52.0 years. Atherosclerosis was the most commonly identified pathologic finding in 9 (20%) patients, followed by mesangial expansion and interstitial fibrosis, each found in 8 (17.8%) patients. Linear regression found interstitial fibrosis to be the only pathologic lesion associated with preoperative serum creatinine (coefficient = 0.697, P = 0.001). Male gender was also associated with a higher preoperative creatinine (coefficient = 0.270, P = 0.034). Postoperative serum creatinine was not associated with any of the examined lesions. CONCLUSIONS: Current surgical techniques provide adequate non-neoplastic tissue for pathologic evaluation. We observed a striking degree of pathologic disease in patients without a known history of medical comorbidities. Routine inspection of the non-neoplastic parenchyma of partial nephrectomy specimens should be performed as it can alert the clinician to presence subclinical renal disease allowing for medical intervention.