Adverse effects of erenumab on cerebral proliferative angiopathy: A case report.

BACKGROUND: Cerebral proliferative angiopathy is a vascular malformation associated with compromised blood-brain barrier and with migraine-like headache. Treating blood-brain barrier-compromised patients with erenumab, an anti-calcitonin gene-related peptide receptor monoclonal antibody, may be risky. CASE: We describe a case of a 22-year-old chronic migraine patient with cerebral proliferative angiopathy who presented to our hospital in status epilepticus 2 d after his first dose of erenumab. Serial magnetic resonance imaging (MRI) studies demonstrated progressive areas of diffusion restriction including the brain tissue adjacent to the cerebral proliferative angiopathy, bilateral white matter and hippocampi. His 6-month post-presentation magnetic resonance imaging was notable for white matter injury, encephalomalacia surrounding cerebral proliferative angiopathy and bilateral hippocampal sclerosis. He remains clinically affected with residual symptoms, including refractory epilepsy and cognitive deficits. CONCLUSION: The evidence presented in this case supports further investigation into potential deleterious side effects of erenumab in patients with compromised blood-brain barrier, such as individuals with intracranial vascular malformations.

Magnetic resonance imaging in children with implants.

As access to MRI in pediatrics increases, the radiologist needs to become acquainted with the basic principles of MRI safety. As part of the image acquisition, the static magnetic field, gradient system, and the radiofrequency transmit-receive coil interact with medical and non-medical implants and can result in serious injury. The main stage of risk triage is based on the determination of whether the implant is MRI-safe, conditional, unsafe or unknown. Guiding principles include the strict adherence to manufacturer specifications for MRI-conditional implants and the assumption that an unknown implant is MR-unsafe. In this article we review considerations for common medical implants encountered in pediatrics including ventriculoperitoneal shunts, orthopedic hardware, orthodontic hardware, pacemakers, vascular stents, vagal nerve stimulators and cochlear implants. Finally, we review a set of high-yield considerations, including the non-communicative patient (sedated or non-verbal), susceptibility artifacts from unclear source, and the approach to an unknown implant.

A Stroke Alert Protocol Decreases the Time to Diagnosis of Brain Attack Symptoms in a Pediatric Emergency Department.

OBJECTIVE: To determine whether a stroke alert system decreases the time to diagnosis of children presenting to the emergency department (ED) with acute-onset focal neurologic deficits. STUDY DESIGN: We performed a retrospective comparison of clinical and demographic information for patients who presented to the ED of a tertiary children's hospital with acute-onset focal neurologic deficits during the 2.5 years before (n = 14) and after (n = 65) the implementation of a stroke alert system. The primary outcome was the median time to neuroimaging analyzed using a Wilcoxon rank-sum test. RESULTS: The median time from ED arrival to neuroimaging for patients with acute-onset focal neurologic deficits decreased significantly after implementation of a stroke alert system (196 minutes; IQR, 85-230 minutes before [n = 14] vs 82 minutes; IQR, 54-123 minutes after [n = 65]; P < .01). Potential intravenous tissue plasminogen activator candidates experienced the shortest time to neuroimaging after implementation of a stroke alert system (54 minutes; IQR, 34-66 minutes [n = 13] for intravenous tissue plasminogen activator candidates vs 89.5 minutes; IQR, 62-126.5 minutes [n = 52] for non-intravenous tissue plasminogen activator candidates; P < .01). CONCLUSIONS: A stroke alert system decreases the median time to diagnosis by neuroimaging of children presenting to the ED with acute-onset focal neurologic deficits by more than one-half. Such a protocol constitutes an important step in ensuring that a greater proportion of children with arterial ischemic stroke are diagnosed in a time frame that enables hyperacute treatment.

Neck CT angiography examinations for pediatric oropharyngeal trauma: diagnostic yield and proposal of a new targeted technique.

BACKGROUND: Neck computed tomography (CT) angiography is commonly ordered for pediatric patients with soft palate trauma to exclude vascular injury. Debate exists regarding what type of imaging is indicated in this setting, particularly amid growing concern that standard neck CT angiography results in considerable radiation exposure. OBJECTIVE: To assess the diagnostic yield and estimated dose reduction of a novel targeted protocol extending from the skull base to the hyoid bone to evaluate pediatric oropharyngeal trauma. MATERIALS AND METHODS: A retrospective imaging and medical chart review was performed of patients for whom a neck CT angiography was obtained for an indication of oropharyngeal trauma between 2008 and 2018. Effective dose and size-specific dose estimates (SSDEs) were estimated for standard and targeted neck CT angiography protocols with calculation of percent dose reduction of the targeted exams. RESULTS: Ninety-eight CT angiography examinations were reviewed. No cases were positive for neurological or major vessel injury; one case was positive for small vessel extravasation. Clinically significant nonvascular findings included phlegmonous change, retained foreign body, retropharyngeal/mediastinal air and pterygoid process fracture. With the exception of mediastinal air, all findings would have been included in the targeted protocol. Effective dose and SSDE were calculated for all cases where CTDIvol (volume CT dose index) had been reported (n=72). There was a statistically significant reduction in dose for the targeted protocol with an effective dose decrease of 69.7%±10.5% (P=0.009) and SSDE decrease of 53.9%±14.7% (P=0.01). Limiting ionizing radiation to the lung apices, esophagus and thyroid gland provided the greatest dose savings. CONCLUSION: Based on low diagnostic yield and high radiation dose associated with standard neck CT angiography for evaluating oropharyngeal trauma, a targeted protocol is recommended, resulting in significantly less dose to the neck, while preserving diagnostic yield.

Placental Pathology in Neonatal Stroke: A Retrospective Case-Control Study.

OBJECTIVE: To assess the association of placental abnormalities with neonatal stroke. STUDY DESIGN: This retrospective case-control study at 3 academic medical centers examined placental specimens for 46 children with neonatal arterial or venous ischemic stroke and 99 control children without stroke, using a standard protocol. Between-group comparisons used χ2 and Fisher exact t test. Correlations used Spearman correlation coefficient. RESULTS: Case placentas were more likely than controls to meet criteria for ≥1 of 5 major categories of pathologic abnormality (89% vs 62%; OR, 5.1; 95% CI, 1.9-14.0; P = .0007) and for ≥2 categories (38% vs 8%; OR, 7.3; 95% CI, 2.9-19.0; P < .0001). Fetal vascular malperfusion occurred in 50% of cases and 17% of controls (OR, 4.8; 95% CI, 2.2-10.5; P = .0001). Amniotic fluid inflammation occurred in 46% of cases with arterial ischemic stroke vs 25% of controls (OR, 2.6; 95% CI, 1.1-6.1; P = .037). There was evidence of a "stress response" (meconium plus elevated nucleated red blood cells) in 24% of cases compared with 1% of controls (OR, 31; 95% CI, 3.8-247.0; P < .0001). CONCLUSIONS: Placental abnormality was more common in children with neonatal stroke compared with controls. All placental findings represent subacute-to-chronic intrauterine stressors. Placental thrombotic processes were associated with both arterial and venous stroke. Our findings provide evidence for specific mechanisms that may predispose to acute perinatal stroke. Amniotic fluid inflammation associated with neonatal arterial ischemic stroke deserves further investigation.

Workup for Perinatal Stroke Does Not Predict Recurrence.

BACKGROUND AND PURPOSE: Perinatal stroke, including neonatal and presumed perinatal presentation, represents the age in childhood in which stroke occurs most frequently. The roles of thrombophilia, arteriopathy, and cardiac anomalies in perinatal ischemic stroke are currently unclear. We took a uniform approach to perinatal ischemic stroke evaluation to study these risk factors and their association with recurrent stroke. METHODS: We reviewed records of perinatal stroke patients evaluated from August 2008 to February 2016 at a single referral center. Demographics, echocardiography, arterial imaging, and thrombophilia testing were collected. Statistical analysis was performed using Fisher exact test. RESULTS: Across 215 cases, the median follow-up was 3.17 years (1.49, 6.46). Females comprised 42.8% of cases. Age of presentation was neonatal (110, 51.2%) or presumed perinatal (105, 48.8%). The median age at diagnosis was 2.9 days (interquartile range, 2.0-9.9) for neonatal stroke and 12.9 months (interquartile range, 8.7-32.8) for presumed perinatal stroke. Strokes were classified as arterial (149, 69.3%), venous (60, 27.9%), both (4, 1.9%), or uncertain (2, 0.9%) by consensus imaging review. Of the 215 cases, there were 6 (2.8%) recurrent ischemic cerebrovascular events. Abnormal thrombophilia testing was not associated with recurrent stroke, except for a single patient with combined antithrombin deficiency and protein C deficiency. After excluding venous events, 155 patients were evaluated for arteriopathy and cardioembolic risk factors; neither was associated with recurrent stroke. Positive family history of thrombosis was not predictive of abnormal thrombophilia testing. CONCLUSIONS: Thrombophilia, arteriopathy, or cardioembolic risk factors were not predictive of recurrent events after perinatal stroke. Thrombophilia evaluation in perinatal stroke should only rarely be considered.

In child and adult migraineurs the somatosensory cortex stands out again: An arterial spin labeling investigation.

Over the past decade, human brain imaging investigations have reported altered regional cerebral blood flow (rCBF) in the interictal phase of migraine. However, there have been conflicting findings across different investigations, making the use of perfusion imaging in migraine pathophysiology more difficult to define. These inconsistencies may reflect technical constraints with traditional perfusion imaging methods such as single-photon emission computed tomography and positron emission tomography. Comparatively, pseudocontinuous arterial spin labeling (pCASL) is a recently developed magnetic resonance imaging technique that is noninvasive and offers superior spatial resolution and increased sensitivity. Using pCASL, we have previously shown increased rCBF within the primary somatosensory cortex (S1) in adult migraineurs, where blood flow was positively associated with migraine frequency. Whether these observations are present in pediatric and young adult populations remains unknown. This is an important question given the age-related variants of migraine prevalence, symptomology, and treatments. In this investigation, we used pCASL to quantitatively compare and contrast blood flow within S1 in pediatric and young adult migraineurs as compared with healthy controls. In migraine patients, we found significant resting rCBF increases within bilateral S1 as compared with healthy controls. Furthermore, within the right S1, we report a positive correlation between blood flow value with migraine attack frequency and cutaneous allodynia symptom profile. Our results reveal that pediatric and young adult migraineurs exhibit analogous rCBF changes with adult migraineurs, further supporting the possibility that these alterations within S1 are a consequence of repeated migraine attacks. Hum Brain Mapp 38:4078-4087, 2017. © 2017 Wiley Periodicals, Inc.

Predictors of Stroke After Transient Ischemic Attack in Children.

BACKGROUND AND PURPOSE: Transient ischemic attack (TIA) in children has received far less attention compared with TIA in adults. The risk factors of stroke after TIA in children are relatively unknown. We aimed to determine the percentage of children who have stroke after TIA and the risk factors associated with stroke after TIA. METHODS: We searched the medical records at Boston Children's Hospital for the year 2010 to find children who were evaluated for TIA to determine associated risk factors of stroke after TIA. We included children who were evaluated in 2009 through 2010 for TIA and had magnetic resonance imaging. We examined follow-up imaging through August 2014 for subsequent stroke. Logistic regression was used to calculate odds ratios for factors in our cohort who are associated with stroke after presentation with TIA. RESULTS: We identified 63 children who experienced a TIA. The mean time of imaging follow-up was 4.5 years after TIA presentation. Of the 63 children, 10 (16%) developed radiological evidence of ischemic cerebral injury within the follow-up period. Four of the 10 (6%) demonstrated diffusion abnormalities on magnetic resonance imaging at TIA presentation, whereas 8 (13%) had a stroke after their TIA. Arteriopathy, female sex, and autoimmune disorders were significantly associated with stroke after TIA. CONCLUSIONS: In our cohort of children, stroke occurred after TIA at a rate similar to that seen in adults, but the risk factors for stroke after TIA in children are different.

Case 226: Oval Window Atresia.

A 6-year-old girl presented with bilateral hearing loss. Her otologic, birth, and family histories were limited, given that she was adopted, but her parents reported that she had had difficulty hearing and speaking ever since they adopted her at 2 years of age. Her parents denied a history of acute otitis media, otorrhea, otalgia, vertigo, autophony, or tinnitus since her adoption. At 2.5 years of age, a diagnosis of hearing loss was made, and she was given hearing aids. Her parents believed that she had been doing well with both receptive and expressive language since she had received the hearing aids. At examination, she had small bilateral preauricular skin tags and normal pinna. Her external auditory canals were of a normal caliber bilaterally, with no otorrhea or lesions. The tympanic membranes were translucent and mobile at pneumatic otoscopy. There was no evidence of a middle ear lesion, nor was there a Schwartz sign. She had no nystagmus or vertigo at pneumatic otoscopy. Audiometry was performed and revealed moderate to severe conductive hearing loss bilaterally, with a mixed component present at 2000 KHz. She had normal bilateral middle ear pressure at tympanometry. Thin-section computed tomography (CT) of the temporal bone was performed.

Stroke in Acquired and Congenital Heart Disease Patients and Its Relationship to Hospital Mortality and Lasting Neurologic Deficits.

OBJECTIVE: To describe strokes in patients with acquired or congenital heart disease and investigate risk factors for in-hospital mortality and ongoing neurologic deficits. DESIGN: Single-center, retrospective review of cardiac, neurologic, and radiologic patient databases. SETTING: Tertiary care children's hospital. PATIENTS: All patients with acquired or congenital heart disease admitted from January 2010 to October 2014 identified with stroke. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Eighty-four stroke events were identified in 83 patients (median age, 5.9 mo; interquartile range, 0.8-33.4). Thirty-two patients (38%) had single ventricle congenital heart disease. Eight patients (9%) presented with symptoms at home, and the remainder was diagnosed while already admitted to the hospital. Forty patients (48%) presented with clinically evident neurologic deficits (e.g., weakness, seizures). Fifty-eight patients (69%) had arterial ischemic strokes, and 13 (15%) had parenchymal hemorrhages. At diagnosis, 54 patients (64%) were on inotropes. Twenty-nine patients (35%) had greater than or equal to 3 cardiac procedures during their hospitalization before stroke diagnosis. In-hospital mortality occurred in 28 patients (33%). Under multivariate analysis, inotropes, number of cardiac procedures, lack of seizure, and parenchymal hemorrhage were independently associated with in-hospital mortality (p < 0.05). Fifty-four percent of survivors with neurologic follow-up had ongoing neurologic deficits attributable to strokes (median follow-up, 15.3 mo; interquartile range, 7.0-29.9). With multivariate analysis, longer hospital stay (p = 0.02) was independently associated with ongoing deficits. CONCLUSIONS: A majority of patients with acquired or congenital heart disease who suffer stroke present while hospitalized and without focal neurologic findings. In-hospital mortality is associated with inotropes, cardiac procedures, lack of seizure, and parenchymal hemorrhage. The majority of survivors have lasting neurologic deficits associated with longer hospital stay.

Retroclival collections associated with abusive head trauma in children.

Retroclival collections are rare lesions reported almost exclusively in children and strongly associated with trauma. We examine the incidence and imaging characteristics of retroclival collections in young children with abusive head trauma. We conducted a database search to identify children with abusive head trauma ≤ 3 years of age with brain imaging performed between 2007 and 2013. Clinical data and brain images of 65 children were analyzed. Retroclival collections were identified in 21 of 65 (32%) children. Ten (48%) were subdural, 3 (14%) epidural, 2 (10%) both, and 6 (28%) indeterminate. Only 8 of 21 retroclival collections were identifiable on CT and most were low or intermediate in attenuation. Eighteen of 21 retroclival collections were identifiable on MRI: 3 followed cerebral spinal fluid in signal intensity and 15 were bloody/proteinaceous. Additionally, 2 retroclival collections demonstrated a fluid-fluid level and 2 enhanced in the 5 children who received contrast material. Sagittal T1-weighted images, sagittal fluid-sensitive sequences, and axial FLAIR (fluid-attenuated inversion recovery) images showed the retroclival collections best. Retroclival collections were significantly correlated with supratentorial and posterior fossa subdural hematomas and were not statistically correlated with skull fracture or parenchymal brain injury. Retroclival collections, previously considered rare lesions strongly associated with accidental injury, were commonly identified in this cohort of children with abusive head trauma, suggesting that retroclival collections are an important component of the imaging spectrum in abusive head trauma. Retroclival collections were better demonstrated on MRI than CT, were commonly identified in conjunction with intracranial subdural hematomas, and were not significantly correlated with the severity of brain injury or with skull fractures.

Autism Spectrum Disorder in Children With Perinatal Ischemic Stroke Varies by Stroke Type.

Background and Objectives: Perinatal stroke leads to significant morbidity over a child's lifetime, including diagnosis of various neurodevelopmental disorders. Specific studies examining the prevalence of autism spectrum disorder in children with perinatal stroke are scarce. Following the clinical observation of autism spectrum disorder in a pediatric referral stroke center, we evaluated the rate of autism spectrum disorder diagnosis after perinatal ischemic stroke, including analysis by subtypes of perinatal ischemic stroke. Methods: We retrospectively examined all children diagnosed with perinatal ischemic stroke, who were ≥18 months old at the time of last follow-up at a single institution from 2008 through 2021. We classified patients as having autism spectrum disorder if they were diagnosed by a neurologist, neuropsychologist, clinical psychologist, or developmental pediatrician. Multivariable logistic regression was performed to examine the association between ischemic stroke subtype and autism spectrum disorder. Results: Among 260 children with perinatal stroke, 19 children (7.3%) also had autism spectrum disorder. Children with perinatal venous stroke had 3-fold higher odds of autism spectrum disorder compared to those with perinatal arterial ischemic stroke (adjusted odds ratio: 3.01, 95% confidence interval: 1.07-8.47). Conclusion: In our perinatal ischemic stroke population, children with venous stroke had higher odds of autism spectrum disorder compared to those with arterial ischemic stroke alone. Prospective studies are needed to further investigate the role of perinatal stroke in autism spectrum disorder development.

Adverse events in pediatric cochlear implant patients undergoing magnetic resonance imaging.

OBJECTIVE: To investigate the prevalence and nature of adverse events in magnetic resonance imaging (MRI) of pediatric cochlear implant (CI) patients. METHODS: Retrospective chart review at a tertiary pediatric hospital. CI patients who underwent MRI from 2004 through 2019 were identified via our internal radiology database. Comorbidities, CI model, age at MRI, number of MRIs, type of MRIs, indication for MRIs, precautions taken for MRIs, quality of MRIs, anesthesia during MRIs, patient language abilities, and adverse events were recorded from the electronic medical record. The literature was reviewed, and our results were compared to those of previous similar series. RESULTS: From 2004 to 2019, 12 pediatric patients (17 ears) with CIs underwent 22 MRIs. 12 MRIs were performed in CI patients with retained internal magnet. 4/22 MRIs resulted in morbidity; 2 patients experienced pain requiring MRI abortion, 1 experienced magnet rotation requiring surgical replacement, and 1 underwent operative removal of the magnet prior to the scan with surgical replacement thereafter. 19/22 MRIs were performed to evaluate the brain; 17/22 of the radiologic reports noted limitation of evaluation due to artifact. 18/22 MRIs required the administration of anesthesia. 9 of the 22 MRI events involved 2 patients whose CIs had been without internal magnet in anticipation of future MRI requirement. CONCLUSIONS: Adverse events affecting pediatric patients with CI can occur as a result of MRI, despite appropriate precautions. Safety requires consideration of factors unique to a pediatric hearing-impaired population. Clinicians must remain informed on best practices and manufacturer recommendations.

Modeling severe functional impairment or death following ECPR in pediatric cardiac patients: Planning for an interventional trial.

AIM: We aimed to characterize extracorporeal CPR (ECPR) outcomes in our center and to model prediction of severe functional impairment or death at discharge. METHODS: All ECPR events between 2011 and 2019 were reviewed. The primary outcome measure was severe functional impairment or death at discharge (Functional Status Score [FSS] ≥ 16). Organ dysfunction was graded using the Pediatric Logistic Organ Dysfunction Score-2, neuroimaging using the modified Alberta Stroke Program Early Computed Tomography Score. Multivariable logistic regression was used to model FSS ≥ 16 at discharge. RESULTS: Of the 214 patients who underwent ECPR, 182 (median age 148 days, IQR 14-827) had an in-hospital cardiac arrest and congenital heart disease and were included in the analysis. Of the 110 patients who underwent neuroimaging, 52 (47%) had hypoxic-ischemic injury and 45 (41%) had hemorrhage. In-hospital mortality was 52% at discharge. Of these, 87% died from the withdrawal of life-sustaining therapies; severe neurologic injury was a contributing factor in the decision to withdraw life-sustaining therapies in 50%. The median FSS among survivors was 8 (IQR 6-8), and only one survivor had severe functional impairment. At 6 months, mortality was 57%, and the median FSS among survivors was 6 (IQR 6-8, n = 79). Predictive models identified FSS at admission, single ventricle physiology, extracorporeal membrane oxygenation (ECMO) duration, mean PELOD-2, and worst mASPECTS (or DWI-ASPECTS) as independent predictors of FSS ≥ 16 (AUC = 0.931) and at 6 months (AUC = 0.924). CONCLUSION: Mortality and functional impairment following ECPR in children remain high. It is possible to model severe functional impairment or death at discharge with high accuracy using daily post-ECPR data up to 28 days. This represents a prognostically valuable tool and may identify endpoints for future interventional trials.

Stroke After Cardiac Catheterization in Children.

BACKGROUND: Children with cardiac disease are at high risk for stroke. Approximately one-quarter of strokes in children with cardiac disease occur in the peri-procedural period; yet, the risk factors, clinical presentation, and treatment of post-catheterization stroke in children have not been well defined. METHODS: We conducted a retrospective review of the medical records of patients aged zero to 18 years with a new clinically-apparent arterial ischemic stroke after cardiac catheterization at a tertiary children's hospital from 2006 to 2016. We excluded patients who had cardiac surgery, a cardiac arrest, extracorporeal membrane oxygenation, a ventricular assist device, or an arrhythmia proximate to their stroke. RESULTS: Twenty children had a new clinically-apparent post-catheterization arterial ischemic stroke. The median age was one year (range, two days to 16 years). The most common procedures were balloon dilation for pulmonary vein stenosis (n = 6) and systemic pulmonary collateral closure (n = 5). The most common presenting symptoms were arm weakness (n = 10) and seizure (n = 8). The median time from catheterization to symptom discovery was 31.5 hours (interquartile range, 16.2 to 47.8 hours; n = 18). The median Pediatric Stroke Outcome Measure score 12 months post-stroke was 0.75 (range, 0 to 2; n = 6). CONCLUSIONS: Although arterial ischemic stroke after cardiac catheterization is rare, better understanding this entity is important as children with cardiac disease and stroke have ongoing morbidity. Ameliorating this morbidity requires efforts aimed at preventing and rapidly detecting stroke, thereby enabling timely institution of neuroprotective measures and treatment with hyperacute therapies.

Migraine in the Young Brain: Adolescents vs. Young Adults.

Migraine is a disease that peaks in late adolescence and early adulthood. The aim of this study was to evaluate age-related brain changes in resting state functional connectivity (rs-FC) in migraineurs vs. age-sex matched healthy controls at two developmental stages: adolescence vs. young adulthood. The effect of the disease was assessed within each developmental group and age- and sex-matched healthy controls and between developmental groups (migraine-related age effects). Globally the within group comparisons indicated more widespread abnormal rs-FC in the adolescents than in the young adults and more abnormal rs-FC associated with sensory networks in the young adults. Direct comparison of the two groups showed a number of significant changes: (1) more connectivity changes in the default mode network in the adolescents than in the young adults; (2) stronger rs-FC in the cerebellum network in the adolescents in comparison to young adults; and (3) stronger rs-FC in the executive and sensorimotor network in the young adults. The duration and frequency of the disease were differently associated with baseline intrinsic connectivity in the two groups. fMRI resting state networks demonstrate significant changes in brain function at critical time point of brain development and that potentially different treatment responsivity for the disease may result.

Post-arrest therapeutic hypothermia in pediatric patients with congenital heart disease.

BACKGROUND: While therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH. METHODS: Patients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared. RESULTS: Thirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group's post-arrest temperature was significantly lower than control's (33.6 ±â€¯0.2 °C vs 34.7 ±â€¯0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 h post-arrest. CONCLUSION: Our data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures.

Pathways for Neuroimaging of Neonatal Stroke.

PURPOSE: To provide consensus-based, suggested imaging protocols to facilitate the accurate and timely diagnosis of a neonate with symptoms concerning for stroke. METHODS: The Writing Group, an international collaboration of pediatric neurologists and neuroradiologists with expertise in perinatal and childhood stroke, participated in a series of pediatric stroke neuroimaging symposia. These discussions, in conjunction with extensive literature review, led to a consensus for imaging protocols to guide practitioners in the diagnosis of neonatal stroke subtypes as defined by the National Institute of Neurological Disorders and Stroke Common Data Elements. The epidemiology, clinical presentation, and associated risk factors for arterial ischemic stroke, cerebral sinovenous thrombosis, and hemorrhagic stroke are reviewed, with a focused discussion regarding the role of neuroimaging for each subtype. RESULTS: In a neonate with suspected stroke, magnetic resonance imaging is the preferred modality, given the lack of X-irradiation, superior anatomic resolution, and sensitivity for acute ischemia. Core recommended sequences include diffusion-weighted imaging and apparent diffusion coefficient mapping to diagnose acute ischemia, gradient-recalled echo or susceptibility-weighted imaging to detect intracranial blood and its breakdown products, and T1- and T2-weighted imaging to assess for myelination, extra-axial blood, and edema. Magnetic resonance angiography of the brain may be useful to detect vascular abnormalities, with venography if venous sinus thrombosis is suspected. The application of more novel sequences, as well as the utility of follow up-imaging, is also discussed.

Calvarial defects and skeletal dysplasia in patients with neurofibromatosis Type 1.

OBJECT: Skull defects, including sphenoid dysplasia and calvarial defects, are rare but distinct findings in patients with neurofibromatosis Type 1 (NF1). The underlying pathophysiology is unclear. The goal of this study was to identify the clinical characteristics and natural history of skull defects in patients with NF1. METHODS: An electronic search engine of medical records was used to identify patients with NF1 and bony skull anomalies. All clinical, radiographic, pathology, and operative reports were reviewed. The relationship between bony anomalies and significant clinical associations was evaluated. This study received institutional review board approval. RESULTS: Twenty-one patients were identified. The mean age at NF1 diagnosis was 4.2 years. The mean age at skull defect diagnosis was 8.8 years (9.7 years in the sphenoid wing dysplasia group and 11.9 years in the calvarial defect group). Sphenoid dysplasia was associated with a plexiform neurofibroma or dural ectasia in 73.3% and 80.0% of cases, respectively. Calvarial defects were associated with a plexiform neurofibroma or dural ectasia in 66.7% and 33.3% of patients, respectively. An absence of either an associated neurofibroma or ectasia was not noted in any patient with sphenoid wing dysplasia or 25.0% of those with calvarial defects. In 6 patients, both types of skull defects presented simultaneously. Serial imaging studies were obtained for a mean follow-up time of 7.5 years (range 0.4-20.0 years). Of these patients with serial imaging, radiographic progression was found in 60% of cases of calvarial defects and 56% of cases of sphenoid wing dysplasia. Two patients underwent surgical repair of a skull defect, and both required repeat procedures. CONCLUSIONS: The majority of skull defects in patients with NF1 were associated with an adjacent structural lesion, such as a plexiform neurofibroma or dural ectasia. This findings from this cohort also support the concept of progression in defect size in more than half of the patients. Potential mechanisms by which these secondary lesions contribute to pathogenesis of the bony defect may include changes in the bony microenvironment. A better understanding of the pathophysiology of skull defects will help guide detection, improve treatment and outcome, and may contribute to the understanding of the pathogenesis of bony lesions in NF1.