National Imaging Trends of Recurrent Pediatric Urolithiasis.

OBJECTIVES: The aim of this study was to examine computed tomography (CT) and ultrasound (US) utilization trends in incident and prevalent pediatric emergency department (ED) urolithiasis patients before and after imaging guideline release. METHODS: We reviewed imaging modalities for children with 2 or more ED encounters between January 1, 2006, and September 1, 2013, for urolithiasis using the Pediatric Health Information System database. Z scores compared the proportion of patient encounters receiving CT and US before (January 1, 2006, to December 31, 2010) and after (January 1, 2011, to September 1, 2013) the release of imaging guidelines. McNemar test for paired proportions compared the percentage of US and CT use between initial versus subsequent visits. Piecewise logistic regression was used to determine the probability of US use and CT use over time before and after the implementation of imaging guidance. RESULTS: Analysis was completed on 2041 patients with 4930 unique encounters for urolithiasis. During 1758 encounters (35.7%), CT was performed initially. Ultrasound was performed 1585 times (32.2%). Fourteen percent fewer CT procedures were performed during first urolithiasis visits after guideline release (P < 0.01), whereas US use increased by 15% (P < 0.01). Fewer CT procedures were performed at later visits compared with the first (P < 0.05), and US was used more during second or later visits than the first (P < 0.05). CONCLUSIONS: Medical providers at large academic pediatric EDs have decreased use of CT and increased use of US over the study time frame to diagnose urolithiasis and are now similar during initial visits (US 36.4% vs CT 36.2%, P = 0.94). Physicians are still more likely to use US as the initial urolithiasis imaging modality during second and later encounters.

Spontaneous Cystic Penile Vascular Malformation in an Adolescent and Review of the Literature.

Vascular malformations rarely involve the male genitalia, and even fewer appear as cystic lesions on the penile shaft. We report an uncommon case of spontaneous swelling near the coronal margin of the penis that was found to arise from a vascular malformation. We review the pathologic findings and the literature regarding vascular malformations of the penis and other penile cystic structures that have similar clinical appearance, and discuss treatment options for penile vascular malformations.

Novel Use of Pudendal Neuromodulation in a Pediatric Patient With Caudal Regression and Partial Sacral Agenesis for Refractory Bowel Bladder Dysfunction.

Sacral neuromodulation (SNM) has been used off-label in the United States for over a decade in the pediatric population. Many published studies have demonstrated efficacy with SNM in this population; however, a significant number of children with refractory bowel bladder dysfunction (BBD) also have underlying comorbidities. Children with certain spinal abnormalities pose a problem for the urologist treating BBD. Patients with caudal regression can have various sacral anomalies, making SNM challenging or impossible. We present the first case in the United States of pudendal neuromodulation in a pediatric BBD patient with caudal regression.

Ultrasound-Guided Transgluteal Percutaneous Nephrolithotomy for a Patient with Complex Urogenital Reconstruction and Ectopic Kidney.

BACKGROUND AND PURPOSE: Pediatric patients with urolithiasis and complex reconstructed genitourinary anatomy pose a significant surgical challenge. We describe a technique utilized to treat an obstructing calculus in the ectopic kidney of a patient with a history of cloacal exstrophy, bladder augmentation, Monti catheterizable channel, and reconstructed abdominal wall. Case and Technique: A 5-year-old female with a history of cloacal exstrophy, pelvic kidney, and reconstructed urologic and abdominal wall anatomy presented after prior shockwave lithotripsy with an obstructing ureteropelvic junction calculus with signs of sepsis. Because of the patient's previous abdominal wall reconstruction with polytetrafluoroethylene mesh and the location of her pelvic kidney, traditional methods of percutaneous nephrostomy tube placement could not be performed. Transgluteal percutaneous nephrostomy tube was placed by interventional radiology. Subsequently, a percutaneous nephrolithotomy (PCNL) was performed through this tract. CONCLUSIONS: Transgluteal PCNL is a feasible option in children with complex congenital genitourinary anomalies with a history of reconstructed anatomy.

Robot-assisted laparoscopic management of duplex renal anomaly: Comparison of surgical outcomes to traditional pure laparoscopic and open surgery.

INTRODUCTION: Surgical management of duplex renal anomaly (DRA) is complex because of individual anatomic variation, competing priorities of vesicoureteral reflux (VUR) and ureteral obstruction present in the same child, the varied differential function of the different renal moieties, and the presence of voiding dysfunction and recurrent urinary tract infection (UTI). Robot-assisted laparoscopic (RAL) surgical management has been under-reported in this group of children but is becoming a viable alternative to traditional open surgery. OBJECTIVE: The aim was to report the surgical outcomes of a series of children with DRA who had RAL surgery and compare these outcomes to historical cohorts of open and laparoscopic surgery. STUDY DESIGN: This was a retrospective analysis of a prospective series of children who had RAL surgery for DRA over an 8-year period. Forty-five RAL surgeries were performed in 47 children. RAL heminephrectomy (RAL HN) was performed in 19 children for poorly or non-functional renal moiety. One had staged bilateral RAL HN. RAL ureteroureterostomy (RAL UU) was performed in 14 children for upper pole ureteral obstruction. Thirteen RAL common sheath ureteral reimplants (RAL csUN) with or without ureteral tapering were performed in 12 children with VUR and UTI. Diagnosis and demographics, results of preoperative imaging, intraoperative time stamps, perioperative complications, success rate, and renal outcomes were recorded. RESULTS: Low-grade VUR present preoperatively in the RAL UU group all resolved within the follow-up period. Four (25%) children in the RAL HN group developed de novo VUR after surgery, which resolved in two (50%) and required surgery in two (50%). Grade I VUR after RAL csUR that occurred in two (14.3%) children was asymptomatic and observed when off preventative antibiotics. DISCUSSION: Most children with DRA who need surgical treatment can be offered RAL surgery. We report good outcomes and improved operative times for RAL HN and UU that approach historical open and pure laparoscopic cohorts. However, RAL csUR, especially with ureteral tapering, is more complex and inherently susceptible to complications, and has not met the outcomes of the open gold standard. The limits of this study are that selection bias is present, and there is no study control cohort. CONCLUSIONS: This report achieves its aim to report surgical outcomes of children who had RAL surgery for DRA.

The Interaction between Enterobacteriaceae and Calcium Oxalate Deposits.

BACKGROUND: The role of calcium oxalate crystals and deposits in UTI pathogenesis has not been established. The objectives of this study were to identify bacteria present in pediatric urolithiasis and, using in vitro and in vivo models, to determine the relevance of calcium oxalate deposits during experimental pyelonephritis. METHODS: Pediatric kidney stones and urine were collected and both cultured and sequenced for bacteria. Bacterial adhesion to calcium oxalate was compared. Murine kidney calcium oxalate deposits were induced by intraperitoneal glyoxalate injection and kidneys were transurethrally inoculated with uropathogenic Escherichia coli to induce pyelonephritis. RESULTS: E. coli of the family Enterobacteriaceae was identified in patients by calcium oxalate stone culture. Additionally Enterobacteriaceae DNA was sequenced from multiple calcium oxalate kidney stones. E. coli selectively aggregated on and around calcium oxalate monohydrate crystals. Mice inoculated with glyoxalate and uropathogenic E. coli had higher bacterial burdens, increased kidney calcium oxalate deposits and an increased kidney innate immune response compared to mice with only calcium oxalate deposits or only pyelonephritis. CONCLUSIONS: In a murine model, the presence of calcium oxalate deposits increases pyelonephritis risk, likely due to preferential aggregation of bacteria on and around calcium oxalate crystals. When both calcium oxalate deposits and uropathogenic bacteria were present, calcium oxalate deposit number increased along with renal gene transcription of inner stone core matrix proteins increased. Therefore renal calcium oxalate deposits may be a modifiable risk factor for infections of the kidney and urinary tract. Furthermore, bacteria may be present in calcium oxalate deposits and potentially contribute to calcium oxalate renal disease.

Pediatric Origins of Nephrolithiasis-Associated Atherosclerosis.

OBJECTIVES: To determine if nephrolithiasis-associated atherosclerosis has pediatric origins and to consider possible association between kidney stones and atherosclerosis-related proteins. STUDY DESIGN: We matched children aged 12-17 years with kidney stones and without kidney stones. Carotid artery intima-media thickness (cIMT) was measured by ultrasound. Participants' urine was investigated by enzyme-linked immunosorbent assay for the atherosclerosis-related proteins fibronectin 1, macrophage scavenger receptor 1, osteopontin, and vascular cell adhesion molecule 1 levels, and normalized to urine creatinine levels. RESULTS: Subjects with nephrolithiasis had higher cIMT in the right common carotid artery and overall mean measurement. Urine osteopontin and fibronectin 1 were significant predictors of cIMT. CONCLUSIONS: We have provided initial preliminary evidence that nephrolithiasis-associated atherosclerosis has pediatric origins and performed studies that begin to identify potential reasons for the association of nephrolithiasis and vascular disease.

Sacral Nerve Stimulation for Pediatric Lower Urinary Tract Dysfunction: Development of a Standardized Pathway with Objective Urodynamic Outcomes.

PURPOSE: We propose that sacral nerve stimulation is a valid adjunctive therapy for refractory pediatric lower urinary tract dysfunction, and that prospective collection of preoperative and postoperative validated questionnaires and urodynamic data in a standardized fashion is beneficial in characterizing patient response. MATERIALS AND METHODS: Patients were candidates for sacral nerve stimulation if they had refractory voiding dysfunction and standard treatments had failed. Preoperative evaluation included urodynamic studies, spinal magnetic resonance imaging, and validated bladder and bowel related questionnaires. Children were stratified into 2 groups, ie overactive bladder with or without incontinence (group 1) and detrusor underactivity/urinary retention requiring clean intermittent catheterization (group 2). A staged procedure was used with initial test lead placement, followed by permanent device insertion 2 weeks later if patients demonstrated symptom improvement with test lead. Postoperatively children were followed with questionnaires and at least 1 urodynamic study. RESULTS: A total of 26 children underwent sacral nerve stimulation. Mean patient age was 10.8 years and median followup was 1.2 years. There were 23 patients in group 1 and 4 in group 2 (1 patient was included in both groups). In group 1 voiding dysfunction scores improved significantly, and urodynamic studies revealed a significant decrease in mean number of uninhibited contractions and maximum detrusor pressure during the filling phase. In group 2 there was significant improvement in mean post-void residual. CONCLUSIONS: Sacral nerve stimulation is a treatment option that may produce significant improvement in objective and subjective measures of bladder function in children with refractory lower urinary tract dysfunction.

The diagnosis, evaluation and treatment of acute and recurrent pediatric urinary tract infections.

Urinary tract infection (UTI) is one of the most common bacterial infections encountered by pediatricians. Currently, the diagnosis and management of acute UTI and recurrent UTI in children remain controversial. Recently published guidelines and large clinical trials have attempted to clarify UTI diagnostic and management strategies. In this manuscript, we review the diagnosis and management of acute and recurrent UTI in the pediatric population.

Vesicoscopic ureteral reimplant: is there a role in the age of robotics?

Vesicoscopic ureteral reimplantation is a challenging procedure to learn but does have outcomes equivalent to standard open repair. Children objectively have less pain than after an open cross-trigonal repair. Operative times compare favorably to other forms of minimally invasive surgery.

Spontaneous clitoral hood epidermal inclusion cyst mimicking clitoromegaly in a pediatric patient.

Epidermal inclusion cysts are benign lesions that can be found in many parts of the body. They are rarely seen in the clitoral region in pediatric patients but when these are found, they are most commonly seen with a history of trauma. We report an uncommon case of a spontaneous nontraumatic epidermal inclusion cyst in the clitoral hood of a female child. This presentation mimicked clitoromegaly but was ultimately found to be a large epidermal cyst that was successfully excised surgically. We present the important pathologic findings and review the relevant literature.

Prostate-derived sterile 20-like kinases (PSKs/TAOKs) phosphorylate tau protein and are activated in tangle-bearing neurons in Alzheimer disease.

In Alzheimer disease (AD), the microtubule-associated protein tau is highly phosphorylated and aggregates into characteristic neurofibrillary tangles. Prostate-derived sterile 20-like kinases (PSKs/TAOKs) 1 and 2, members of the sterile 20 family of kinases, have been shown to regulate microtubule stability and organization. Here we show that tau is a good substrate for PSK1 and PSK2 phosphorylation with mass spectrometric analysis of phosphorylated tau revealing more than 40 tau residues as targets of these kinases. Notably, phosphorylated residues include motifs located within the microtubule-binding repeat domain on tau (Ser-262, Ser-324, and Ser-356), sites that are known to regulate tau-microtubule interactions. PSK catalytic activity is enhanced in the entorhinal cortex and hippocampus, areas of the brain that are most susceptible to Alzheimer pathology, in comparison with the cerebellum, which is relatively spared. Activated PSK is associated with neurofibrillary tangles, dystrophic neurites surrounding neuritic plaques, neuropil threads, and granulovacuolar degeneration bodies in AD brain. By contrast, activated PSKs and phosphorylated tau are rarely detectible in immunostained control human brain. Our results demonstrate that tau is a substrate for PSK and suggest that this family of kinases could contribute to the development of AD pathology and dementia.