International Pediatric Multidisciplinary Management Using Telemedicine to Promote Equitable Care.

Objective: To evaluate the use of telemedicine as a collaboration tool between a pediatrician and subspecialists looking to address challenges, such as the lack of health care specialists, which are present in the Dominican Republic. Study design: During this 6-year study, 65 patients were evaluated by a medical team consisting of a local pediatrician and 17 subspecialists from a leading academic medical center in the Unites States. Patient's age ranged from 2 months to 16 years of age (mean 8 years old). The most common reasons for referral were masses or malignancies, vascular malformations, urogenital anomalies, stuttering, and cochlear implant programming. Results: A total of 39 out of 65 cases (60%) carried an initial diagnosis. Of the 65 cases, a change in medical management occurred in 92.31% of cases (60 cases). There was no change in medical diagnosis or treatment in 5 of 65 cases (8%). Conclusion: This protocol exhibited high patient satisfaction with the technology and platform and direct patient savings from transportation costs. It also demonstrated the importance of thorough diagnosis in providing appropriate treatment and solutions. Telemedicine use in comparable practices should be studied further to aid in the development of policies for the diagnosis and management of chronic illnesses that require referrals to subspecialists.

Outcomes according to channel type for continent catheterizable channels in patients undergoing simultaneous urinary and fecal reconstruction.

INTRODUCTION: When creating a continent catheterizable channel (CCC) the choice of bowel segment used as the conduit should be tempered with the morbidity associated with it. The split-appendix technique allows the creation of both a urinary and fecal CCC without the need for a bowel anastomosis. However, there is concern that by splitting the appendix there is compromise to its blood supply and may affect outcomes. We aim to compare what affect the bowel segment used for urinary and fecal CCCs has on perioperative and long-term outcomes in patients undergoing simultaneous urinary and fecal reconstruction. METHODS: A retrospective review was performed analyzing all patients that underwent simultaneous continent catheterizable urinary and fecal CCC between the years 2010-2016. Patient demographics, channel characteristics, perioperative complications and clinical success rate were analyzed. RESULTS: A total of 106 patients were identified that had simultaneous fecal and urinary CCC created at time of reconstruction. For urinary CCC, there were 64 patients (60.4%) that underwent a split-appendix technique, 27 patients (25.4%) underwent a Monti, and 15 patients (14.2%) had the appendix used only for the urinary channel. Those patients undergoing a split-appendix technique had median operative time of 447 min compared to 619 min when a Monti channel was created. The median length of hospital stay was 9 days for the split-appendix technique compared to 12 and 13 day median hospital stay when the appendix was used only for the urinary channel or a Monti was created, respectively. There was no difference seen in revision free survival of the channel following surgery of any of the channels with median follow-up of 44.5 months. However, there were more subfascial revisions of urinary CCC in those that underwent a Monti (5 patients, 18.5%) compared to other bowel segments (0 patients). CONCLUSIONS: Utilizing a split-appendix approach for creation of urinary and fecal CCCs does not affect 30-day complications or long-term revision rates compared to other established techniques. This technique minimizes the potential surgical morbidity of a bowel anastomosis and provide shorter operative times, when feasible, at time of simultaneous creation of fecal and urinary CCC.

Pediatric Partial Nephrectomy for Upper Urinary Tract Duplication Anomalies: A Comparison Between Different Surgical Approaches and Techniques.

OBJECTIVE: To review and compare 4 different surgical approaches for partial nephrectomy of a nonfunctioning moiety in children with upper urinary tract duplication anomalies. MATERIALS AND METHODS: A retrospective review of all pediatric patients who underwent open partial nephrectomy (OPN), laparoscopic partial nephrectomy (LPN), robotic partial nephrectomy (RPN), or laparoendoscopic single site partial nephrectomy (LESS-PN) for the treatment of a nonfunctioning moiety in a duplicated collecting system at 2 medical centers between 2007 and 2017. Patient demographics, perioperative data, surgical techniques, complications, and results were compared. RESULTS: A total of 59 pediatric patients underwent partial nephrectomy for an upper urinary tract duplication anomaly during a 10-year period: 24 OPN, 7 LPN, 18 RPN, and 10 LESS-PN. Median age was 16 months (interquartile range 9-49.7). Median weight was 10.7 Kg (interquartile range 8.8-16.4). Median estimated blood loss was comparable between all minimally invasive approaches, but significantly increased in the open approach. OPN required more narcotics (0.554 mg Morphine equivalent/Kg/day, range 0.03-6.13) and Acetaminophen (72.12 mg/Kg/day, range 0-209.06) than all other groups in the study. Median operating time in OPN (154.5 minutes, range 108-413) and LESS-PN (140 minutes, range 65-245) were found to be significantly shorter in comparison to LPN (190 minutes, range 159-355), and RPN (256 minutes, range 163-458); (P = .03, .005, .02, and .005). CONCLUSION: Minimally invasive approaches (LPN, RPN, and LESS-PN) for partial nephrectomy in upper urinary tract duplication anomalies may be associated with decreased postoperative analgesia requirements, shorter hospital stay, less blood loss, and less use of drains in comparison to the open approach, while demonstrating efficacy and safety.

Comparison of ultrasound versus computed tomography for the detection of kidney stones in the pediatric population: a clinical effectiveness study.

BACKGROUND: The incidence of pediatric nephrolithiasis in the United States is increasing. There is a paucity of literature comparing the diagnostic performance of computed ultrasound (US) to tomography (CT) in the pediatric population. OBJECTIVE: To determine the diagnostic performance of renal US for nephrolithiasis in children using a clinical effectiveness approach. MATERIALS AND METHODS: Institutional review board approval with a waiver of informed consent was obtained for this retrospective, HIPAA-complaint investigation. Billing records and imaging reports were used to identify children (≤18 years old) evaluated for nephrolithiasis by both US and unenhanced CT within 24 h between March 2012 and March 2017. Imaging reports were reviewed for presence, number, size and location of kidney stones. Diagnostic performance of US (reference standard=CT) was calculated per renal unit (left/right kidney) and per renal sector (four sectors per kidney). For sector analysis, US was considered truly positive if a stone was identified at CT in the same or an adjacent sector. RESULTS: There were 68 renal stones identified by CT in 30/69 patients (43%). Mean patient age was 14.7±3.6 years, and 35 were boys. For detecting nephrolithiasis in any kidney, US was 66.7% (48.8-80.8%) sensitive and 97.4% (86.8-99.9%) specific (positive predictive value=95.2% [77.3-99.8%], negative predictive value=79.2% [65.7-88.3%], positive likelihood ratio=26.0). Per renal sector, US was 59.7% (46.7-71.4%) sensitive and 97.4% (95.5-98.5%) specific (positive predictive value=72.3% [58.2-83.1%], negative predictive value=95.4% [93.2-96.9%], positive likelihood ratio=22.5). Of the 30 stones not detected by US, only 3 were >3 mm at CT. CONCLUSION: In clinical practice, US has high specificity for detecting nephrolithiasis in children but only moderate sensitivity and false negatives are common.

Analyte variations in consecutive 24-hour urine collections in children.

PURPOSE: The metabolic evaluation of children with nephrolithiasis begins with a 24-h urine collection. For adults, the diagnostic yield increases with consecutive collections; however, little is known regarding the variability of multiple 24-h studies in the pediatric population. We sought to evaluate the variability of consecutive 24-h urine collection in children through a multi-institutional study hypothesizing that compared with a single collection, consecutive 24-h urine collections would reveal a greater degree of clinically useful information in the evaluation of children at risk for nephrolithiasis. MATERIALS AND METHODS: Including data from six institutions, we identified children less than 18 years of age considered at risk for recurrent nephrolithiasis, undergoing metabolic evaluation. We evaluated a subset of patients performing two collections with urine creatinine varying by 10% or less during a 7-day period. Discordance between repeat collections based on normative urine chemistry values was evaluated. RESULTS: A total of 733 children met inclusion criteria, and in over a third both urine calcium and urine volume differed by 30% or more between samples. Urine oxalate demonstrated greater variation between collections in children <5 years than among older children (p = 0.030) while variation in other parameters did not differ by age. Discordance between repeat samples based on normative values was most common for urine oxalate (22.5%) and the derived relative supersaturation ratios for both calcium phosphate (25.1%) and calcium oxalate (20.5%). The proportion of discordant samples, based on normative thresholds, as well as variability greater ≥30% and 50%, respectively, are shown in the table. CONCLUSIONS: Our analysis indicates that stone risk in as many as one in four children may be misclassified if normative values of only a single 24-h urine are used. In light of these findings, repeat 24-h urine collections prior to targeted intervention to modify stone risk are advised to increase diagnostic yield in children at risk for nephrolithiasis.

A fast-growing, painless, solid scrotal mass.

The history and physical examination of an adolescent male are critical to early detection of scrotal abnormalities. Although rare, paratesticular rhabdomyosarcoma grows quickly and can be life-threatening. This article focuses on the importance of prompt recognition, diagnosis, and appropriate management of paratesticular rhabdomyosarcoma in an adolescent.

Urine risk factors in children with calcium kidney stones and their siblings.

Calcium nephrolithiasis in children is increasing in prevalence and tends to be recurrent. Although children have a lower incidence of nephrolithiasis than adults, its etiology in children is less well understood; hence, treatments targeted for adults may not be optimal in children. To better understand metabolic abnormalities in stone-forming children, we compared chemical measurements and the crystallization properties of 24-h urine collections from 129 stone formers matched to 105 non-stone-forming siblings and 183 normal, healthy children with no family history of stones, all aged 6 to 17 years. The principal risk factor for calcium stone formation was hypercalciuria. Stone formers have strikingly higher calcium excretion along with high supersaturation for calcium oxalate and calcium phosphate, and a reduced distance between the upper limit of metastability and supersaturation for calcium phosphate, indicating increased risk of calcium phosphate crystallization. Other differences in urine chemistry that exist between adult stone formers and normal individuals such as hyperoxaluria, hypocitraturia, abnormal urine pH, and low urine volume were not found in these children. Hence, hypercalciuria and a reduction in the gap between calcium phosphate upper limit of metastability and supersaturation are crucial determinants of stone risk. This highlights the importance of managing hypercalciuria in children with calcium stones.

The risk of recurrent urolithiasis in children is dependent on urinary calcium and citrate.

OBJECTIVES: To determine which risk factors help predict recurrent stone formation. Urinary stone disease is relatively rare in children. At our institution, a full urinary metabolic evaluation is initiated after the first stone episode. METHODS: A retrospective cohort study was performed to assess urinary metabolic profiles in children with urolithiasis. Twenty-four-hour urine collections were performed and evaluated. Urine chemistries were adjusted for creatinine and weight. Abnormal thresholds were obtained from the available published data. The patients were stratified into solitary or recurrent stone formers by review of the medical record. Multivariate analysis was performed with a logistic regression model to assess for independent risk factors for stone recurrence. RESULTS: A total of 148 samples from 88 patients with solitary stones and 84 samples from 51 patients with recurrent stones were evaluated. Age and gender were well-matched between the 2 groups. Most known stones were calcium oxalate, and there were no radiolucent stones in those with unknown composition. A significantly higher number of patients with recurrent stones had abnormal values for calcium (73% vs 57%) and citrate (30% vs 13%) by univariate analysis. Both calcium (odds ratio, 2.3, P <.01) and citrate (odds ratio, 3.5, P <.001) remained independent risk factors for stone recurrence by multivariate analysis. CONCLUSIONS: There are significant differences in the urinary calcium and citrate levels between children with solitary and recurrent calcium stone formation. This may allow identification of patients at risk for stone recurrence that may benefit from more aggressive dietary and/or pharmacologic intervention.

Ureteroscopy: The standard of care in the management of upper tract urolithiasis in children.

OBJECTIVES: Advances in technology and the continued evolution in the design of ureteroscopes now permit a primary endoscopic approach to the upper urinary tract of pediatric patients on a routine basis to treat a diverse group of conditions that include urolithiasis, hematuria and strictures. The purpose of this review article is to demonstrate that ureteroscopic lithotripsy is now to be considered the standard of care in the management of upper tract urolithiasis in the pediatric patient, replacing shockwave lithotripsy (ESWL) as the first line of therapy. Additionally, the article will discuss the available endoscopic equipment and the lessons learned over the years to optimize the success of these procedures in children. MATERIALS AND METHODS: A systematic review of articles written about ureteroscopy (URS) in the contemporary urological literature (1990-2009) on PubMed was undertaken. The success rates and complications of pediatric ureteroscopic procedures were abstracted from the identified publications and the results were tabulated and compared with the success rates of shockwave lithotripsy. RESULTS: In over 832 URS cases, there was a 5.9% complication rate and a stone-free rate of 93.4%. The stone-free rates of URS are superior to those obtained with the published success rates with ESWL of 80.3% in 1,839 cases. CONCLUSIONS: The safety and outcomes of ureteroscopic lithotripsy in the management of pediatric urolithiasis now justify that this treatment modality be considered the standard of care and first line of therapy in the management of children who present with upper tract stones.

Incontinence following bladder neck reconstruction--is there a role for endoscopic management?

PURPOSE: Little has been reported concerning the efficacy of endoscopic injection of dextranomer/hyaluronic acid for the treatment of residual incontinence following bladder neck reconstruction. We present the experience of 2 institutions using endoscopic submucosal injection of dextranomer/hyaluronic acid to correct incontinence in patients who had previously undergone bladder neck reconstruction with or without concomitant enterocystoplasty. MATERIALS AND METHODS: A retrospective chart review was performed with patient demographics, indications for treatment and outcomes recorded. All patients had adequate bladder capacity and compliance on maximized medical therapy before injection. Continence was defined as at least a 3-hour daytime dry interval, while improvement was defined as an increase in the daytime dry interval to at least 2 hours. RESULTS: A total of 14 patients (10 females and 4 males) underwent 21 injections. At a median followup of 17 months 10 patients had successful results (6 continent, 4 improved). CONCLUSIONS: Endoscopic injection of dextranomer/hyaluronic acid to correct incontinence following bladder neck reconstruction appears safe and can increase the daytime dry interval in more than 70% of carefully selected patients. Continued followup is necessary to evaluate the long-term effectiveness of this treatment.

Modulation of germ cell apoptosis with a nitric oxide synthase inhibitor in a murine model of congenital cryptorchidism.

PURPOSE: Apoptosis has been implicated in testicular germ cell loss in experimental models of cryptorchidism. Nitric oxide synthase (NOS) has been shown to have a role in apoptosis in many cell types. The Hoxa 11 knockout mouse has congenital bilateral cryptorchidism and is uniformly sterile. We examined the time course of apoptosis in this model and attempted to attenuate this response in vivo by inhibition of NOS. MATERIALS AND METHODS: The offspring of heterozygous Hoxa 11 knockout mice were genotyped by polymerase chain reaction. Homozygous knockout mice treated with the NOS inhibitor Nomega-nitro-L-arginine methyl ester (L-NAME) and untreated controls were sacrificed at weekly intervals at 3 to 13 weeks of age. Spermatogenesis was evaluated with hematoxylin and eosin staining. Germ cell apoptosis was assessed with a TUNEL assay and DNA staining. Co-localization of NOS activity was measured with a polyclonal antibody to endothelial NOS. RESULTS: Impaired spermatogenesis was observed in Hoxa 11 knockout mice. Testis/body weight ratios were decreased in this group at weeks 6 and 7, while body weights were unchanged. Germ cell apoptosis was significantly higher in the knockout group compared to wild-type controls. Co-localization was observed between endothelial NOS activity and apoptotic cells, while mice treated with L-NAME demonstrated improved spermatogenesis and attenuated apoptosis. CONCLUSIONS: Apoptosis and NOS reactivity appeared to co-localize in the seminiferous tubules in the Hoxa 11 knockout mouse model. Treatment with the NOS inhibitor L-NAME attenuated apoptosis and improved spermatogenesis. This finding suggests that early treatment might serve as an adjunct to early surgical intervention to reduce testicular atrophy, although any impact on long-term fertility remains to be determined.