Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

Ureteral stent extraction strings in children: Stratifying the risk of post operative urinary tract infection.

INTRODUCTION: Ureteral stents facilitate recovery and avoid external drains in pediatric ureteral reconstruction. Extraction strings avoid the need for a secondary cystoscopy and anesthetic. Due to concerns regarding febrile UTIs in children with extraction strings, we retrospectively assessed the relative risk of UTI in children with extraction strings. OBJECTIVE: Our hypothesis was that stents with extraction strings do not increase the risk of UTI after pediatric ureteral reconstruction. METHODS: Records of all children undergoing pyeloplasty and ureteroureterostomy (UU) from 2014 to 2021 were reviewed. The incidences of UTI, fever, and hospitalization were recorded. RESULTS: 245 patients mean age 6.4 years (163M:82F) underwent pyeloplasty (n = 221) or UU (n = 24). 42% (n = 103) received prophylaxis. Of these, 15% developed UTI versus 5% of those not receiving prophylaxis (p < 0.05). 42 females had prior history of UTI, compared to 20 males (p < 0.05). 49 patients had an extraction string. Stents with extraction strings were removed on average 0.6 months post-op while others underwent cystoscopic removal on average 1.26 months post-op (p < 0.05). 9 (18.4%) required hospitalization for febrile UTI while the stent with extraction string was in place, while only 13 (6.6%) of those without extraction string did (p < 0.02). Of the 9 children with a febrile UTI in the extraction string group, 6 had history of prior UTI (46.1%), compared to only 3 (8.3%) without a prior UTI (p < 0.05). With no prior UTI, there was no difference in UTI risk between those with (3, 8.3%) and without (8, 6.4%) extraction string (p = 0.71). Females with prior UTI and extraction string were more likely to develop UTI than those with prior UTI and no extraction string (p = 0.01). There were not enough males with history of UTI to analyze alone. There were 5 (10%) stent dislodgements in the extraction string group, 2 required further intervention with cystoscopy or percutaneous drainage. DISCUSSION: Extraction strings provide the assurance of drainage while avoiding the need for a second general anesthetic procedure. There is not an increased risk of UTI with extraction string in those without prior history of UTI, but we no longer routinely leave extraction strings if there is history of UTI. CONCLUSION: Children, particularly females, with prior history of UTI have a significantly increased risk of febrile UTIs associated with the use of extraction strings. Prophylaxis does not seem to reduce this risk. Patients with no prior UTI had no higher risk of UTI with extraction string use for pyeloplasty or UU.

Clinical utility and interrater reliability of video urodynamics in children with isolated fibrolipoma of filum terminale.

INTRODUCTION: Children with an isolated fibrolipoma of filum terminale (IFFT) but otherwise normal spinal cord are often evaluated with video urodynamics (VUDS). VUDS interpretation is subjective and can be difficult in young children. These patients may undergo detethering surgery if there is concern for current or future symptomatic tethered cord. OBJECTIVE: We hypothesized that VUDS in children with IFFT would have limited clinical utility regarding decision for or against detethering surgery and VUDS interpretation would have poor interrater reliability. METHODS: Patients with IFFT who underwent VUDS for from 2009 to 2021 were retrospectively reviewed to evaluate clinical utility of VUDS. 6 pediatric urologists who were blinded to patient clinical characteristics reviewed the VUDS. Gwet's first order agreement coefficient (AC1) with 95% CI was used to assess interrater reliability. RESULTS: 47 patients (24F:23M) were identified. Median age at initial evaluation was 2.8yrs (IQR:1.5-6.8). 24 (51%) patients underwent detethering surgery (Table). VUDS at initial evaluation were interpreted by treating urologist as normal in 4 (8%), reassuring for normal in 39 (81%), or concerning for abnormal in 4 (9%). Based on neurosurgery clinic and operative notes for the 47 patients, VUDS made no change in management in 37 patients (79%), prompted detethering in 3 (6%), was given as reason for observation in 7 (15%), and was normal or reassuring for normal but not documented as a reason for observation in 16 (34%) (Table). Interrater reliability for VUDS interpretation had fair agreement (AC1 = 0.27) for overall categorization of VUDS and EMG interpretation (AC1 = 0.34). Moderate agreement was seen for detrusor overactivity interpretation (AC1 = 0.54) and bladder neck appearance (AC1 = 0.46). DISCUSSION: In our cohort, 90% of patients had a normal or reassuring for normal interpretation of VUDS. VUDS interpretation affected clinical course in a minority of patients. There was fair interrater reliability for overall VUDS interpretation and therefore clinical course regarding detethering surgery could vary depending upon interpreting urologist. This fair interrater variability appeared to be related to variability in EMG, bladder neck appearance, and detrusor overactivity interpretation. CONCLUSION: VUDS affected clinical management in about 20% of our cohort and supported the choice for observation in around 50% of patients. This suggests VUDS does have clinical utility in pediatric patients with IFFT. The overall VUDS interpretation had fair interrater reliability. This suggest VUDS interpretation has limitations in determining normal versus abnormal bladder function in children with IFFT. Neurosurgeons and urologists should be aware of VUDS limitations in this patient population.

Robot assisted laparoscopic partial cystectomy for inflammatory myofibroblastic tumor with simultaneous intraoperative flexible cystoscopy for tumor mapping.

Bladder masses are an infrequent occurrence rarely suspected in cases of pediatric hematuria. Inflammatory myofibroblastic tumors represent one differential diagnosis that is difficult to characterize as purely benign and should therefore be given special consideration. Although uncommon, this is an important entity to recognize for potential bladder sparing and minimally invasive surgical approaches.

Bladder Necrosis at Time of Presentation with Ulcerative Colitis in an 8-year-old Female: A Case Report.

A case of bladder necrosis in an 8-year-old female at time of presentation of ulcerative colitis (UC) is presented. A case of bladder necrosis in a pediatric patient outside of the neonatal period has not been reported. The patient presented with abdominal pain, bloody stools, hematuria, and acute renal failure. She was acutely management with bilateral nephrostomy tube placement. Bladder and colon biopsies revealed diagnosis of UC and bladder necrosis. The UC was medically managed. The bladder did not regenerate after several months of observation and ileal conduit urinary diversion was performed. A right proximal ureteral stricture was managed by pyeloplasty at time of ileal conduit. The patient is doing well over 1 year after surgery.

Renal artery angiography in pediatric trauma using a national data set.

INTRODUCTION: With limited pediatric renal trauma management literature, treatment pathways for children have been extrapolated from the adult population. A shift to non-operative management has led to higher renal preservation rates; however, characterization of endovascular intervention in the pediatric trauma population is lacking. OBJECTIVE: This study uses the National Trauma Data Bank (NTDB), to evaluate renal outcomes after use of renal artery angiography. We hypothesized that patients undergoing renal artery angiography for renal trauma are unlikely to require additional surgical interventions. STUDY DESIGN: All children ≤18 years old treated for traumatic renal injuries from 2012 to 2015 were identified by the Abbreviated Injury Scaled Score (AISS) codes in the NTDB. AISS codes were converted to American Association for Surgery of Trauma (AAST) grades. ICD-9 codes were used to identify patients that had renal artery angiography, and additional renal interventions such as nephrectomy, partial nephrectomy, percutaneous nephrostomy tube or ureteral stent placement. RESULTS: 536,379 pediatric trauma cases were in the NTDB from 2012 to 2015, with 4506 renal injury cases identified. A total of 88 patients had renal artery angiography (ICD-9 88.45). Only 10% (n = 9) of patients who received renal artery angiography underwent an additional urological intervention. Of those nine, two patients were excluded due to renal angiography taking place after nephrectomy was performed. The remaining seven patients had high grade laceration (AAST grade 4-5). Overall, two patients underwent post angiography nephrectomies, two patients had partial nephrectomies, one percutaneous nephrostomy tube was placed (prior to partial nephrectomy), one aspiration of a kidney (prior to ureteral stent placement), and three had ureteral stent placements. DISCUSSION: The limitations of this study include: the NTDB is a national dataset that is not population based, inclusion is limited to the first hospitalization, inaccuracies exist in encounter coding, and the database is lacking laterality of the renal injury. Based on nonspecific nature of ICD-9 coding for angioembolization, we are unable to discern the number of cases that subsequently had angioembolization after or at the time of angiography. CONCLUSION: Renal artery angiography in children remains a rare procedure, 88/4,506, in children with renal trauma. In pediatric trauma cases that undergo renal artery angiography additional procedures are more common with higher grade injuries. Further studies are needed to create pediatric specific trauma management algorithms.

Design and validation of a low-cost, high-fidelity model for robotic pyeloplasty simulation training.

INTRODUCTION/BACKGROUND: Owing to restrictions in operative experiences, urology residents can no longer solely rely on 'hands-on' operative time to master their surgical skills by the end of residency. Simulation training could help residents master basic surgical skills and steps of a procedure to maximize time in the operative room. However, simulators can be expensive or tedious to set up, limiting the availability to residents and training programs. OBJECTIVE: The authors sought to develop and validate an inexpensive, high-fidelity training model for robotic pyeloplasty. STUDY DESIGN: Pyeloplasty models were created using Dragon Skin® FX-Pro tissue-mimicking silicone cast over 3-dimensional molds. Urology faculty and trainees completed a demographic questionnaire. The participants viewed a brief instructional video and then independently performed robotic dismembered pyeloplasty on the model. Acceptability and content validity were evaluated via post-task evaluation of the model. Construct validity was evaluated by comparing procedure completion time, the Global Evaluative Assessment of Robotic Skills (GEARS) score, blinded subjective physical evaluation of repair quality (1-10 scale), and flow rate between experts and novices. RESULTS: In total, 5 urology faculty, 6 fellows, and 14 residents participated. The median robotic console experience among faculty, fellows, and residents was 8 years (interquartile range [IQR] = 6-11), 3.5 years (IQR = 2-4 years), and 0 years (IQR = 0-0.5 years), respectively. The median procedure completion time was 29 min (IQR = 26-40 min), and the median flow rate was 1.11 mL/s (IQR = 0-1.34 mL/s). All faculty had flow rates >1.25 mL/s and procedure times <30 min compared with 2 of 6 fellows and none of the residents (P < 0.001). All faculty, half of the fellows, and none of the residents achieved a GEARS score ≥20, with a median resident score of 12.5 (IQR = 8-13) (P < 0.001). For repair quality, all faculty scored ≥9 (out of 10), all fellows scored ≥8, and the median score among residents was 6 (IQR = 2-6) (P < 0.001). The material cost was $1.32/model, and the average production time was 0.12 person-hours/model. DISCUSSION AND CONCLUSION: This low-cost pyeloplasty model exhibits acceptability and content validity. Construct validity is supported by significant correlation between participant expertise and simulator performance across multiple assessment domains. The model has excellent potential to be used as a training tool in urology and allows for repetitive practice of pyeloplasty skills before live cases.

Urologic treatment of nephrolithiasis.

PURPOSE OF REVIEW: The purpose of this review is to describe the options and recent developments in the urologic treatment of nephrolithiasis in children. The prevalence of nephrolithiasis in children has increased over time. The urologic treatment of nephrolithiasis ranges from observation to medical expulsive therapy with an alpha blocker for a stone in a ureter to a variety of procedures for stone removal. RECENT FINDINGS: Observation for small, asymptomatic renal stones is a reasonable strategy in children. Medical expulsive therapy with an alpha blocker may have a beneficial effect for passage of ureteral stones in children. Extracorporeal shock wave lithotripsy (ESWL) remains an important treatment for nephrolithiasis in children. Advances in ureteroscopy with clearer digital imaging and single use ureteroscopes have made ureteroscopy more attractive. Percutaneous nephrolithotomy is a more invasive treatment modality but remains a good choice for children with large-stone burdens and instruments are getting smaller. Open or laparoscopic stone surgery should be reserved for unique cases. SUMMARY: Nephrolithiasis is increasing in children with differing urologic management options depending on the clinical scenario. A shared decision-making process with discussion of risks and benefits should be used to help patients and families choose a treatment option.

Prospective Evaluation of Predictors of Testis Atrophy After Surgery for Testis Torsion in Children.

OBJECTIVE: To prospectively correlate pain duration, red scrotal skin, ultrasound appearance of testis, and intraoperative testis color to future testis atrophy after acute testicular torsion. METHODS: Patients 2 months-18 years old with unilateral acute scrotum were consecutively enrolled in a National Institutes of Health transcutaneous near-infrared spectroscopy study, with a subgroup analysis of the true torsion group. Presence or absence of red scrotal skin, pain duration, testicular heterogeneity on preoperative ultrasound, and intraoperative testis color based on a novel visual chart 5 minutes after detorsion were recorded. All testes underwent orchiopexy regardless of appearance. Percent volume difference between normal and torsed testicles on follow-up ultrasound was compared between patients with and without risk factors. RESULTS: Thirty of 56 patients who had surgical detorsion underwent scrotal ultrasound at a mean of 117 days after surgery. A color of black or hemorrhagic 5 minutes after detorsion, pain duration >12 hours, and heterogeneous parenchyma on preoperative ultrasound were associated with significant testis volume loss in follow-up compared with normal testis. All patients with a black or hemorrhagic testis had >80% volume loss. Erythematous scrotal skin was not significantly associated with smaller affected testis volume in follow-up. CONCLUSION: Based on the high atrophy rate, orchiectomy can be considered for testes that are black or hemorrhagic 5 minutes after detorsion. Pain duration >12 hours and parenchymal heterogeneity on preoperative ultrasound were also associated with testis atrophy. Red scrotal skin was not a reliable predictor of atrophy and should not delay exploration.

Straight to the Operating Room: An Emergent Surgery Track for Acute Testicular Torsion Transfers.

OBJECTIVE: To assess the effect of implementing an emergency surgery track for testicular torsion transfers. We hypothesized that transferring children from other facilities diagnosed with torsion straight to the operating room (STOR) would decrease ischemia time, lower costs, and reduce testicular loss. STUDY DESIGN: Demographics, arrival to incision time, hospital cost in dollars, and testicular outcome (determined by testicular ultrasound) at follow-up were retrospectively compared in all patients transferred to our tertiary care children's hospital with a diagnosis of testicular torsion from 2012 to 2016. Clinical data for STOR and non-STOR patients were compared by Wilcoxon rank-sum, 2-tailed t test, or Fisher exact test as appropriate. RESULTS: Sixty-eight patients met inclusion criteria: 35 STOR and 33 non-STOR. Children taken STOR had a shorter median arrival to incision time (STOR: 54 minutes vs non-STOR: 94 minutes, P < .0001) and lower median total hospital costs (STOR: $3882 vs non-STOR: $4419, P < .0001). However, only 46.8% of STOR patients and 48.4% of non-STOR patients achieved surgery within 6 hours of symptom onset. Testicular salvage rates in STOR and non-STOR patients were not significantly different (STOR: 68.4% vs non-STOR: 36.8%, P = .1), but follow-up was poor. CONCLUSIONS: STOR decreased arrival to incision time and hospital cost but did not affect testicular loss. The bulk of ischemia time in torsion transfers occurred before arrival at our tertiary care center. Further interventions addressing delays in diagnosis and transfer are needed to truly improve testicular salvage rates in these patients.

Simple visual review of pre- to post-operative renal ultrasound images predicts pyeloplasty success equally as well as geometric measurements: A blinded comparison with a gold standard.

BACKGROUND: MAG3 diuretic renal scan remains the gold standard for determination of improvement in renal drainage following pyeloplasty for ureteropelvic junction obstruction. We hypothesized that (i) a change in geometric measurements between pre-operative and post-operative renal ultrasound (RUS) images and (ii) blinded simple visual review of images both would predict pyeloplasty success. OBJECTIVE: To determine if simple visual review and/or novel geometric measurement of renal ultrasounds can detect pyeloplasty failure. STUDY DESIGN: This study was a retrospective, blinded comparison with a gold standard. Included were children aged ≤18 years undergoing pyeloplasty at our institution from 2009 to 2015. For each kidney, representative pre-operative and post-operative RUS images were chosen. Our standard for pyeloplasty success was improved drainage curve on MAG3 and lack of additional surgery. Measurements for collecting system circularity, roundness, and renal parenchymal to collecting system area ratio (RPCSR) were obtained by three raters (Figure), who were blinded to the outcome of the pyeloplasty. Changes in geometric measurements were analyzed as a diagnostic test for MAG3-defined pyeloplasty success using ROC curve analysis. In addition, six reviewers blinded to pyeloplasty success reviewed pre-operative and post-operative images visually for improved hydronephrosis and categorized pyeloplasty as success or failure based on simple visual review of RUS. RESULTS: Fifty-three repaired renal units were identified (50 children). There were five pyeloplasty failures, four of which underwent revision or nephrectomy. While all geometric measurements could discriminate pyeloplasty failure and success, the geometric measurements that discriminated best between pyeloplasty failure and success were change in collecting system roundness and change in RPCSR. Consensus opinion among six blinded reviewers using simple visual review had a sensitivity of 94% and PPV of 100% with respect to identifying pyeloplasty success (AUC 0.97 (95% CI 0.93-1.0)). This was not significantly different from AUC for change in roundness (p = 0.09) or change in RPCSR (p = 0.1). DISCUSSION: Change in collecting system roundness and change in RPCSR were the most accurate geometric measurements in predicting pyeloplasty success. Simple visual review of ultrasound images for pyeloplasty success performed as well or better than geometric measurements. However, geometric measurements remain useful as a research tool or to communicate findings between clinicians. CONCLUSIONS: Complex geometric measurements of hydronephrosis or post-operative MAG3 scans are not needed if hydronephrosis is visually significantly improved, as simple visual review is highly sensitive for detecting pyeloplasty failure.

Correction of Residual Ventral Penile Curvature After Division of the Urethral Plate in the First Stage of a 2-Stage Proximal Hypospadias Repair.

PURPOSE OF REVIEW: The first stage of a 2-stage proximal hypospadias repair involves division of the urethral plate and correction of any residual ventral penile curvature (VPC). Options to correct residual VPC include dorsal corporal shortening or ventral corporal lengthening techniques. This review discusses these options and suggests an approach to management. RECENT FINDINGS: Recent reports of 2-stage proximal hypospadias repairs indicate low rates of recurrent VPC with either dorsal corporal shortening or ventral corporal lengthening. Dorsal corporal shortening with dorsal plication may be preferentially used for mild to moderate residual VPC after division of urethral plate and ventral corporal lengthening reserved for severe residual VPC. Ventral corporal lengthening with grafts has been associated with urethroplasty complications after the second stage hypospadias surgery. Ventral corporal lengthening with relaxing incisions of corpora has been reported, but concerns about adverse effects require longer term studies. Little guidance exists to choose the best technique for VPC correction during first stage hypospadias repair after division of urethral plate. Reported literature suggests good results with dorsal plication techniques and ventral corporal lengthening. A practical approach is to use dorsal plication techniques for mild to moderate residual VPC after division of urethral plate (<45°) and reserve ventral corporal lengthening for severe residual VPC (>45°).

Gross Hematuria in Infancy: A Case of Renal Hemangioma Managed Endoscopically.

A case of renal hemangioma in a male infant managed endoscopically is reported. The patient initially presented at 6 months of age to a nephrology clinic with gross hematuria and was found to have what was thought to be a renal cyst. He was referred to urology clinic at 18 months after having continued gross hematuria and negative nephrology evaluation. A magnetic resonance imaging suggested an enhancing mass in the renal pelvis and hydronephrotic upper pole cyst that was mistaken as cyst on ultrasound. A 1.5 × 2.5 cm pedunculated mass in the renal pelvis was resected endoscopically and pathology was consistent with renal hemangioma. He has remained free of hematuria or sign of recurrence on magnetic resonance imaging or renal ultrasound for 9 months.

Diagnosing Testicular Torsion before Urological Consultation and Imaging: Validation of the TWIST Score.

PURPOSE: The TWIST (Testicular Workup for Ischemia and Suspected Torsion) score uses urological history and physical examination to assess risk of testis torsion. Parameters include testis swelling (2 points), hard testis (2), absent cremasteric reflex (1), nausea/vomiting (1) and high riding testis (1). While TWIST has been validated when scored by urologists, its diagnostic accuracy among nonurological providers is unknown. We assessed the usefulness of the TWIST score when determined by nonurological nonphysician providers, mirroring emergency room evaluation of acute scrotal pain. MATERIALS AND METHODS: Children with unilateral acute scrotum were prospectively enrolled in a National Institutes of Health clinical trial. After undergoing basic history and physical examination training, emergency medical technicians calculated TWIST score and determined Tanner stage per pictorial diagram. Clinical torsion was confirmed by surgical exploration. All data were captured into REDCap™ and ROC curves were used to evaluate the diagnostic usefulness of TWIST. RESULTS: Of 128 patients (mean age 11.3 years) 44 (13.0 years) had torsion. TWIST score cutoff values of 0 and 6 derived from ROC analysis identified 31 high, 57 intermediate and 40 low risk cases (positive predictive value 93.5%, negative predictive value 100%). CONCLUSIONS: TWIST score assessed by nonurologists, such as emergency medical technicians, is accurate. Low risk patients do not require ultrasound to rule out torsion. High risk patients can proceed directly to surgery, with more than 50% avoiding ultrasound. In the future emergency medical technicians and/or emergency room triage personnel may be able to calculate TWIST score to guide radiological evaluation and immediate surgical intervention at initial assessment long before urological consultation.

Perioperative and Short-Term Outcomes of Robotic vs Open Bladder Neck Procedures for Neurogenic Incontinence.

PURPOSE: Complex urological reconstruction may be facilitated by the improved magnification and dexterity provided by a robotic approach. Minimally invasive surgery also has the potential advantages of decreased length of stay and improved convalescence. We reviewed perioperative and short-term outcomes between robot-assisted and open bladder neck sling/repair with catheterizable channel in patients with neurogenic bladder. MATERIALS AND METHODS: We performed an institutional review board approved retrospective chart review of all patients who underwent open or robotic bladder neck reconstruction without augmentation cystoplasty for refractory urinary incontinence between 2010 and 2014. Age at surgery, operative time, length of stay, complications within 30 days of surgery and future continence procedures (injection of bladder neck/catheterizable channel, additional bladder neck surgery, botulinum toxin A injection) were compared between the groups. RESULTS: A total of 45 patients underwent bladder neck reconstruction (open in 26, robotic in 19) with a mean follow up of 2.8 years. There was no difference in preoperative urodynamics, age at surgery or length of stay (median 4 days in each group, p >0.9). Operative time was significantly longer in the robotic group (8.2 vs 4.5 hours, p <0.001). Three patients (16%) undergoing robotic and 3 (12%) undergoing open surgery had a complication within 30 days (p >0.9). Of patients undergoing open repair 14 (56%) underwent 23 subsequent surgeries for incontinence. By comparison, 8 patients undergoing robotic repair (42%) underwent 12 additional procedures (p = 0.5). CONCLUSIONS: Although a robotic approach may take longer to perform, it does not result in increased complications or length of stay, or worsened continence outcomes.

Long-Term Outcomes of Bladder Neck Reconstruction without Augmentation Cystoplasty in Children.

PURPOSE: Bladder outlet procedures without augmentation cystoplasty remain controversial. We hypothesized that bladder outlet procedures without augmentation cystoplasty may lead to unfavorable bladder dynamics, upper tract changes and/or continued incontinence. We reviewed long-term urodynamic, upper tract and continence outcomes following bladder outlet procedures without augmentation cystoplasty. MATERIALS AND METHODS: We retrospectively reviewed all patients who underwent bladder neck reconstruction/closure/sling without augmentation cystoplasty between 2000 and 2014. Because of variation in length of followup, we calculated the cumulative incidence and proportion of cases of upper tract and urodynamic changes, augmentation cystoplasty and subsequent continence procedures. Preoperative factors were compared between patients with and without adverse outcomes. RESULTS: A total of 109 patients underwent bladder outlet procedures without augmentation cystoplasty at a mean age of 8.5 years. At a mean of 4.9 years of followup 59 patients (54%) had undergone additional continence surgery, 20 (18%) had undergone augmentation cystoplasty, 50 (46%) manifested vesicoureteral reflux or hydronephrosis and 23 (21%) had newly diagnosed or worsening renal scarring. At augmentation cystoplasty 13 of 18 patients (72%) had upper tract changes, 15 (83%) had continued incontinence and 11 (61%) had an end fill pressure of greater than 40 cm H2O. All patients had resolution of these changes after augmentation cystoplasty. Patients who had previously undergone vesicostomy or surgery for vesicoureteral reflux were significantly more likely to undergo a subsequent augmentation cystoplasty or to show upper tract changes. CONCLUSIONS: Following bladder outlet procedures without augmentation cystoplasty the estimated 10-year cumulative incidence of augmentation cystoplasty is 30%, continence procedures 70%, upper tract changes greater than 50% and chronic kidney disease 20%. Because of these risks, careful patient selection and close followup are essential if considering a bladder outlet procedure without augmentation cystoplasty.

Cumulative incidence of outcomes and urologic procedures after augmentation cystoplasty.

OBJECTIVE: Augmentation cystoplasty (AC) is a major surgery that can be associated with long-term morbidity. This study aimed to describe the cumulative incidence of outcomes and urologic procedures in a large cohort of children who underwent AC, identify significant sources of morbidity, and to evaluate baseline factors associated with outcomes of interest. METHODS: Children≤18 years who underwent AC in the Pediatric Health Information System from 1999 to 2010 were included. All follow-up encounters up to June 2012 were included. Cumulative incidences for 15 outcomes and urologic procedures were calculated using non-informative censoring. Sensitivity analyses were performed to determine effect of censoring assumptions and including hospitals without complete datasets. As an exploratory analysis, baseline patient factors were evaluated for associations with outcomes and urologic procedures of interest using multivariable Cox proportional hazards models adjusted for clustering by hospital. RESULTS: 2831 AC patients were identified. Based on cumulative incidence calculations and sensitivity analyses; the cumulative incidence ranges of outcomes and procedures at 1, 3, 5, and 10 years were calculated. Examples of 10-year cumulative incidence ranges are given for the following outcomes and procedures: bladder rupture (2.9-6.4%), small bowel obstruction (5.2-10.3%), bladder stones (13.3-36.0%), pyelonephritis (16.1-37.1%), cystolithopaxy (13.3-35.1%), and reaugmentation (5.2-13.4%). The development of chronic kidney disease was strongly associated with a diagnosis of lower urinary tract obstruction (HR 13.7; 95% CI 9.4-19.9). Bladder neck surgery and stoma creation at time of AC were associated with an increased hazard of bladder rupture (HR 1.9; 95% CI 1.1-3.3) and bladder stones (HR 1.4; 95% CI 1.1-1.8) respectively. CONCLUSIONS: Outcomes of interest and urologic procedures after AC are common. Results from this large cohort can be used to counsel patients and families about expectations after AC. Pyelonephritis, chronic kidney disease, further reconstructive surgery, and calculus disease appear to cause significant morbidity. Collaborative efforts are needed to further reduce morbidity in this patient population.

National trends in augmentation cystoplasty in the 2000s and factors associated with patient outcomes.

PURPOSE: Augmentation cystoplasty is a major surgery performed by pediatric urologists. We evaluated national estimates of children undergoing augmentation cystoplasty in the United States for trends during the 2000s, and analyzed patient and hospital factors associated with outcomes. MATERIALS AND METHODS: Patients who underwent augmentation cystoplasty registered in the 2000 to 2009 Kids' Inpatient Database were included. Estimates of total number of augmentation cystoplasties performed and patient and hospital characteristics were evaluated for trends. Hierarchical models were created to evaluate patient and hospital factors associated with length of stay, total hospital charges and odds of having a postoperative complication. RESULTS: An estimated 792 augmentation cystoplasties were performed in 2000, which decreased to 595 in 2009 (p = 0.02). Length of stay decreased from 10.5 days in 2000 to 9.2 days in 2009 (p = 0.04). A total of 1,622 augmentation cystoplasties were included in the hierarchical models and 30% of patients had a complication identified. Patient factors associated with increased length of stay and increased odds of any complication included bladder exstrophy-epispadias complex diagnosis and older age. Pediatric hospitals had 31% greater total hospital charges (95% CI 7-55). CONCLUSIONS: The estimated number of augmentation cystoplasties performed in children in the United States decreased by 25% in the 2000s, and mean length of stay decreased by 1 day. The cause of the decrease is multifactorial but could represent changing practice patterns in the United States. Of the patients 30% had a potential complication during hospitalization after augmentation cystoplasty. Older age and bladder exstrophy-epispadias complex diagnosis were associated with greater length of stay and increased odds of having any complication.

Analysis of the effect of estrogen/androgen perturbation on penile development in transgenic and diethylstilbestrol-treated mice.

Because both androgens and estrogens have been implicated in penile morphogenesis, we evaluated penile morphology in transgenic mice with known imbalance of androgen and estrogen signaling using scanning electron microscopy (SEM), histology, and immunohistochemistry of androgen and estrogen receptors α/ß. Penises of adult wild-type, estrogen receptor-α knockout (αERKO), estrogen receptor-ß knockout (ßERKO), aromatase knockout (Arom-KO), and aromatase overexpression (Arom+) mice were evaluated, as well as adult mice treated with diethylstilbestrol (DES) from birth to day 10. Adult penises were examined because the adult pattern is the endpoint of development. The urethral orifice is formed by fusion of the MUMP (male urogenital mating protuberance) with the MUMP ridge, which consists of several processes fused to each other and to the MUMP. Similarly, the internal prepuce is completed ventrally by fusion of a ventral cleft. In adult murine penises the stromal processes that form the MUMP ridge are separated from their neighbors by clefts. αERKO, ßERKO, and Arom-KO mice have penises with a MUMP ridge clefting pattern similar to that of wild-type mice. In contrast, Arom+ mice and neonatally DES-treated mice exhibit profound malformations of the MUMP, MUMP ridge clefting pattern, and internal prepuce. Abnormalities observed in Arom+ and neonatally DES-treated mice correlate with the expression of estrogen receptor-beta (ERß) in the affected structures. This study demonstrates that formation of the urethal orifice and internal prepuce is due to fusion of separate epithelial-surfaced mesenchymal elements, a process dependent upon both androgen and estrogen signaling, in which ERß signaling is strongly implicated.

Single institution experience with Tru-Cut renal mass biopsy for diagnosing Wilms tumor.

PURPOSE: To evaluate the efficacy of needle biopsy for diagnosing Wilms tumor (WT) before chemotherapy. MATERIALS AND METHODS: We reviewed our institutional experience with Tru-Cut biopsy of pediatric renal masses in patients who subsequently underwent nephrectomy. We compared biopsy pathology with nephrectomy specimens to determine if biopsy accurately predicted final pathology. RESULTS: Seven children underwent Tru-Cut renal mass biopsy followed by surgical resection. In 4 patients, the final biopsy pathology was definitively read as WT and in 3 subjects, the pathology was read as WT versus hyperplastic nephrogenic rest. In all 7 patients, the nephrectomy pathology confirmed a diagnosis of WT. There were no complications after biopsy, and no patients have had local or regional recurrence. CONCLUSION: In our experience, pre-therapy Tru-Cut biopsy safely provides an adequate specimen for pathologic review in diagnosing WT.