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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. [ABSTRACT FROM AUTHOR]

Background: Hemiplegic migraine is a rare form of migraine with aura that includes motor weakness. Diagnosis during thefirst episode can be difficult to make and costly, especially with the sporadic form.Cases: Our study evaluates the ictal magnetic resonance imaging (MRI) features of four sequential pediatric patientsduring a first-time, sporadic hemiplegic migraine. Susceptibility-weighted imaging (SWI) revealed cerebral venous prominenceand increased magnetic susceptibility affecting brain regions that corresponded with each patient's neurologicdeficits. Repeat MRI (performed in three patients) following migraine recovery demonstrated resolution of all susceptibilityabnormalities.Conclusion: When combined with conventional MRI sequences, SWI has diagnostic value in the acute setting of motorweakness and with clinical features consistent with hemiplegic migraine. The sequence may help to further characterizeictal cerebral blood flow changes during the hemiplegic migraine aura. [ABSTRACT FROM AUTHOR]

Background: Ballistic injuries with retained foreign bodies from air guns is a relatively common problem, particularly in children and adolescents. If not removed in a timely fashion, the foreign bodies can result in complications, including pain and infection. Diagnostic methods to identify the presence of the foreign body run the entire gamut of radiology, particularly radiography, ultrasound (US) and computed tomography (CT). Removal of the foreign bodies can be performed by primary care, emergency, surgical, and radiologic clinicians, with or without imaging guidance.Objective: To evaluate the modalities of radiologic detection and the experience of image-guided ballistic foreign body removal related to air gun injuries within the interventional radiology department of a large pediatric hospital.Materials and Methods: A database of more than 1,000 foreign bodies that were removed with imaging guidance by the interventional radiologists at our institution was searched for ballistic foreign bodies from air guns. The location, dimensions, diagnostic modality, duration, complications and imaging modality used for removal were recorded. In addition, the use of sedation and anesthesia required for the procedures was also recorded.Results: Sixty-one patients with ballistic foreign bodies were identified. All foreign bodies were metallic BBs or pellets. The age of the patients ranged from 5 to 20 years. The initial diagnostic modality to detect the foreign bodies was primarily radiography. The primary modality to assist in removal was US, closely followed by fluoroscopy. For the procedure, 32.7% of the patients required some level of sedation. Only two patients had an active infection at the time of the removal. The foreign bodies were primarily in the soft tissues; however, successful removal was also performed from intraosseous, intraglandular and intratendinous locations. All cases resulted in successful removal without complications.Conclusion: Image-guided removal of ballistic foreign bodies secondary to air guns is a very effective procedure that can obviate the need for open surgical procedures in children. [ABSTRACT FROM AUTHOR]

Sex differences after concussion have been studied largely in high school and college athletes, often without reference to comparison groups without concussion. This study sought to evaluate sex differences in outcomes among all children and adolescents presenting to the Emergency Department (ED) for either mild traumatic brain injury (TBI) or orthopedic injury (OI), regardless of mechanism of injury. The study involved a concurrent cohort, prospective study design with longitudinal follow-up. Participants were eight to 16 years old with mild TBI (n = 143) or OI (n = 73). They were recruited and completed an initial assessment at EDs at two children's hospitals. They returned for a post-acute assessment within two weeks of injury and for follow-up assessments at three and six months. Outcomes included child and parent proxy ratings of somatic and cognitive symptoms, and standardized tests of cognitive functioning and balance. Sex did not moderate group differences in balance, fluid or crystallized cognitive ability, or child or parent proxy ratings of somatic or cognitive symptoms. Both parents and children reported more somatic symptoms in girls than boys, but in both groups. Compared with the OI group, the mild TBI group showed significantly lower fluid cognitive ability at the post-acute assessment and significantly higher somatic and cognitive symptoms according to both child and parent proxy ratings across the first two weeks post-injury. The results suggest that sex does not moderate the outcomes of mild TBI in a pediatric ED population. Previous research pointing to sex differences after concussion may reflect the lack of comparison groups, as well as a focus on adolescents and young adults and sport-related concussion. Future research should investigate whether sex moderates the outcomes of pediatric mild TBI in adolescents but not in pre-adolescent children. [ABSTRACT FROM AUTHOR]

This study is the first to examine cognitive outcomes after pediatric mild TBI using the National Institutes of Health Toolbox Cognition Battery (NIHTB-CB), a computerized cognitive test battery. The NIHTB-CB includes two complex measures of attention and executive function that allow differentiation of accuracy and response speed. We compared performance on the NIHTB-CB among children 8–16 years of age with mild TBI (n = 143) versus children with orthopedic injuries (OIs; n = 74) recruited in emergency departments and followed for 6 months post-injury. Mixed-model analyses showed that the mild TBI group showed significantly lower Fluid Cognition composite scores than the OI group at 10 days (group intercept, p = 0.018); the magnitude of group differences declined modestly over time (group × time interaction, p = 0.055). Effect sizes were d = 0.34 at 10 days post-injury, d = 0.27 at 3 months, and d = 0.10 at 6 months. No significant effects of group or time were found for the Crystallized Cognition composite. Analyses of Fluid Cognition subtests indicated that children with mild TBI displayed deficits for as long as 3 months on measures of attention and executive function (e.g., cognitive flexibility, inhibitory control), but not on measures of explicit memory, working memory, or processing speed. The poorer performance of the mild TBI group on measures of attention and executive function was attributable largely to slowed reaction time, not decreased accuracy. The findings suggest that children with mild TBI demonstrate persistent deficits in fluid cognition that are most apparent on tasks that combine demands for both speed and executive function. [ABSTRACT FROM AUTHOR]

To quantify changes in segmented brain volumes over 12 months in children with mucopolysaccharidosis types IIIA and IIIB (MPS IIIA and IIIB). In order to establish suitable outcome measures for clinical trials, twenty-five children greater than 2 years of age were enrolled in a prospective natural history study of MPS IIIA and IIIB at Nationwide Children's Hospital. Data from sedated non-contrast brain 3 T MRIs and neuropsychological measures were reviewed from the baseline visit and at 12-month follow-up. No intervention beyond standard clinical care was provided. Age- and sex-matched controls were gathered from the National Institute of Mental Health Data Archive. Automated brain volume segmentation with longitudinal processing was performed using FreeSurfer. Of the 25 subjects enrolled with MPS III, 17 children (4 females, 13 males) completed at least one MRI with interpretable volumetric data. The ages ranged from 2.8 to 13.7 years old (average 7.2 years old) at enrollment, including 8 with MPS IIIA and 9 with MPS IIIB. At baseline, individuals with MPS III demonstrated reduced cerebral white matter and corpus callosum volumes, but greater volumes of the lateral ventricles, cerebellar cortex, and cerebellar white matter compared to controls. Among the 13 individuals with MPS III with two interpretable MRIs, there were annualized losses or plateaus in supratentorial brain tissue volumes (cerebral cortex −42.10 ± 18.52 cm3/year [mean ± SD], cerebral white matter −4.37 ± 11.82 cm3/year, subcortical gray matter −6.54 ± 3.63 cm3/year, corpus callosum −0.18 ± 0.62 cm3/yr) and in cerebellar cortex (−0.49 ± 12.57 cm3/year), with a compensatory increase in lateral ventricular volume (7.17 ± 6.79 cm3/year). Reductions in the cerebral cortex and subcortical gray matter were more striking in individuals younger than 8 years of age. Greater cerebral cortex volume was associated with higher fine and gross motor functioning on the Mullen Scales of Early Learning, while greater subcortical gray matter volume was associated with higher nonverbal functioning on the Leiter International Performance Scale. Larger cerebellar cortex was associated with higher receptive language performance on the Mullen, but greater cerebellar white matter correlated with worse adaptive functioning on the Vineland Adaptive Behavioral Scales and visual problem-solving on the Mullen. Loss or plateauing of supratentorial brain tissue volumes may serve as longitudinal biomarkers of MPS III age-related disease progression compared to age-related growth in typically developing controls. Abnormally increased cerebellar white matter in MPS III, and its association with worse performance on neuropsychological measures, suggest the possibility of pathophysiological mechanisms distinct from neurodegeneration-associated atrophy that warrant further investigation. [ABSTRACT FROM AUTHOR]

Objective: We analyzed the incidence and demographic features of pediatric intracranial hypertension.Methods: Inpatient and outpatient encounters of children aged 18 years or younger who were diagnosed with primary (idiopathic) or secondary intracranial hypertension between January 2010 and December 2013 were identified. Data were collected from a subspecialty clinic devoted to intracranial hypertension and the sole children's hospital in a large Midwestern city of the United States. Estimated incidence rates were calculated based on the number of newly diagnosed patients in our hospital's primary service area, which includes seven central Ohio counties. Sex, race, body mass index, socioeconomic status, and geographic distribution were also noted.Results: A total of 74 pediatric patients were diagnosed with intracranial hypertension (49 primary/idiopathic and 25 secondary) between January 2010 and December 2013. Using census data, we determined the pediatric population in our service area during the four-year period. The Ohio Hospital Association's database indicated that 92.3% of patients aged 0 to 17 years residing in the region sought care at our institution. By combining these data, we calculated an annual incidence of primary and secondary intracranial hypertension of 0.63 and 0.32 per 100,000 children, respectively.Conclusions: The estimated annual incidence of pediatric primary intracranial hypertension in our seven county service area in central Ohio is similar to previous pediatric reports from other countries and is 67% of that reported in the US adult population. [ABSTRACT FROM AUTHOR]

Introduction: Individuals with Down syndrome (DS) have an increased risk of acute leukemia compared to a markedly decreased incidence of solid tumors. Medulloblastoma, the most common malignant brain tumor of childhood, is particularly rare in the DS population, with only one published case. As demonstrated in a mouse model, DS is associated with cerebellar hypoplasia and a decreased number of cerebellar granule neuron progenitor cells (CGNPs) in the external granule cell layer (EGL). Treatment of these mice with sonic hedgehog signaling pathway (Shh) agonists promote normalization of CGNPs and improved cognitive functioning. Case report: We describe a 21-month-old male with DS and concurrent desmoplastic/nodular medulloblastoma (DNMB)-a tumor derived from Shh dysregulation and over-activation of CGNPs. Molecular profiling further classified the tumor into the new consensus SHH molecular subgroup. Additional testing revealed a de novo heterozygous germ line mutation in the PTCH1 gene encoding a tumor suppressor protein in the Shh pathway. Discussion: The developmental failure of CGNPs in DS patients offers a plausible explanation for the rarity of medulloblastoma in this population. Conversely, patients with PTCH1 germline mutations experience Shh overstimulation resulting in Gorlin (Nevoid Basal Cell Carcinoma) syndrome and an increased incidence of malignant transformation of CGNPs leading to medulloblastoma formation. This represents the first documented report of an individual with DS simultaneously carrying PTCH1 germline mutation. Conclusion: We have observed a highly unusual circumstance in which the PTCH1 mutation appears to 'trump' the effects of DS in causation of Shh-activated medulloblastoma. [ABSTRACT FROM AUTHOR]

Background Social functioning encompasses a range of important skills that an individual uses to interact with the social world. Previous studies suggest that social functioning (outcomes) may be impaired after childhood stroke, but research is limited. Aims We examined the following: (1) the effect of ischemic stroke upon social outcomes in children; (2) the correlation of cognitive abilities and problem behaviors with social outcomes; and (3) the role of infarct characteristics as predictors of social outcomes. Methods We conducted an observational case-controlled study to compare children with neonatal or childhood onset stroke and controls with chronic asthma. Neurological deficits were measured with the Pediatric Stroke Outcome Measure. Cognitive abilities, problem behavior, adaptive behavior, and social outcomes were assessed with standardized measures. Results Overall stroke cases were impaired in several areas of adaptive behaviors but not in cognitive ability, problem behaviors, or social outcomes. Children with more severe neurological deficits had impairments in a range of adaptive behaviors, social adjustment, and social participation. Impaired cognitive ability and more problem behaviors correlated with impaired social adjustment, particularly in stroke cases. Larger infarcts correlated with greater neurological impairment, lower IQ, and poorer social participation. Conclusions Stroke can result in impaired adaptive and social functioning without apparent deficits in IQ or behavior. Infarct size, residual neurological deficits, impaired cognitive ability, and problem behaviors increase the risk for poor social adjustment and participation. These findings can help the clinician anticipate impaired social functioning after pediatric stroke, which is important because age-specific treatments are available. [ABSTRACT FROM AUTHOR]