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Post-hemorrhagic hydrocephalus (PHH) is a severe complication of intraventricular hemorrhage (IVH) in very preterm infants. PHH monitoring and treatment decisions rely heavily on manual and subjective two-dimensional measurements of the ventricles. Automatic and reliable three-dimensional (3D) measurements of the ventricles may provide a more accurate assessment of PHH, and lead to improved monitoring and treatment decisions. To accurately and efficiently obtain these 3D measurements, automatic segmentation of the ventricles can be explored. However, this segmentation is challenging due to the large ventricular anatomical shape variability in preterm infants diagnosed with PHH. This study aims to (a) propose a Bayesian U-Net method using 3D spatial concrete dropout for automatic brain segmentation (with uncertainty assessment) of preterm infants with PHH; and (b) compare the Bayesian method to three reference methods: DenseNet, U-Net, and ensemble learning using DenseNets and U-Nets. A total of 41 T

Gamma-aminobutyric acid (GABA) and glutamatergic system perturbations following premature birth may explain neurodevelopmental deficits in the absence of structural brain injury. Using GABA-edited spectroscopy (MEscher-GArwood Point Resolved Spectroscopy [MEGA-PRESS] on 3 T MRI), we have described in-vivo brain GABA+ (+macromolecules) and Glx (glutamate + glutamine) concentrations in term-born infants. We report previously unavailable comparative data on in-vivo GABA+ and Glx concentrations in the cerebellum, the right basal ganglia, and the right frontal lobe of preterm-born infants without structural brain injury. Seventy-five preterm-born (gestational age 27.8 ± 2.9 weeks) and 48 term-born (39.6 ± 0.9 weeks) infants yielded reliable MEGA-PRESS spectra acquired at post-menstrual age (PMA) of 40.2 ± 2.3 and 43.0 ± 2 weeks, respectively. GABA+ (median 2.44 institutional units [i.u.]) concentrations were highest in the cerebellum and Glx higher in the cerebellum (5.73 i.u.) and basal ganglia (5.16 i.u.), with lowest concentrations in the frontal lobe. Metabolite concentrations correlated positively with advancing PMA and postnatal age at MRI (Spearman’s rho 0.2–0.6). Basal ganglia Glx and NAA, and frontal GABA+ and NAA concentrations were lower in preterm compared with term infants. Moderate preterm infants had lower metabolite concentrations than term and extreme preterm infants. Our findings emphasize the impact of premature extra-uterine stimuli on GABA–glutamate system development and may serve as early biomarkers of neurodevelopmental deficits.

The aim of this study is to describe the clinical characteristics, perioperative course and neuroimaging abnormalities of infants with congenital heart disease (CHD) undergoing heart surgery without deep hypothermic circulatory arrest (DHCA) and identify variables associated with neurological outcome. Infants with CHD undergoing open-heart surgery without DHCA between 2009 and 2017 were identified from a cardiac surgery database. Full-term infants

BACKGROUND: The third trimester of gestation is a crucial phase of rapid brain development, but little has been reported on the trajectories of cerebral blood flow (CBF) in preterm infants in this period. PURPOSE: To quantify regional CBF in very preterm infants longitudinally across the ex utero third trimester and to determine its relationship with clinical factors associated with brain injury and premature birth. MATERIALS AND METHODS: In this prospective study, very preterm infants were enrolled for three longitudinal MRI scans, and 22 healthy full-term infants were enrolled for one term MRI scan between November 2016 and February 2019. Global and regional CBF in the cortical gray matter, white matter, deep gray matter, and cerebellum were measured using arterial spin labeling with postlabeling delay of 2025 msec at 1.5 T and 3.0 T. Brain injury and clinical risk factors in preterm infants were investigated to determine associations with CBF. Generalized estimating equations were used to account for correlations between repeated measures in the same individual. RESULTS: A total of 75 preterm infants (mean postmenstrual age [PMA]: 29.5 weeks ± 2.3 [standard deviation], 34.9 weeks ± 0.8, and 39.3 weeks ± 2.0 for each scan; 43 male infants) and 22 full-term infants (mean PMA, 42.1 weeks ± 2.0; 13 male infants) were evaluated. In preterm infants, global CBF was 11.9 mL/100 g/min ± 0.2 (standard error). All regional CBF increased significantly with advancing PMA (P ≤ .02); the cerebellum demonstrated the most rapid CBF increase and the highest mean CBF. Lower CBF was associated with intraventricular hemorrhage in all regions (P ≤ .05) and with medically managed patent ductus arteriosus in the white matter and deep gray matter (P = .03). Mean CBF of preterm infants at term-equivalent age was significantly higher compared with full-term infants (P ≤ .02). CONCLUSION: Regional cerebral blood flow increased significantly in preterm infants developing in an extrauterine environment across the third trimester and was associated with intraventricular hemorrhage and patent ductus arteriosus. © RSNA, 2021 Online supplemental material is available for this article.

Background Due to random motion of fetuses and maternal respirations, image quality of fetal brain MRIs varies considerably. To address this issue, visual inspection of the images is performed during acquisition phase and after 3D-reconstruction, and the images are re-acquired if they are deemed to be of insufficient quality. However, this process is time-consuming and subjective. Multi-instance (MI) deep learning methods (DLMs) may perform this task automatically. Purpose To propose an MI count-based DLM (MI-CB-DLM), an MI vote-based DLM (MI-VB-DLM), and an MI feature-embedding DLM (MI-FE-DLM) for automatic assessment of 3D fetal-brain MR image quality. To quantify influence of fetal gestational age (GA) on DLM performance. Study type Retrospective. Subjects Two hundred and seventy-one MR exams from 211 fetuses (mean GA ± SD = 30.9 ± 5.5 weeks). Field strength/sequence T2 -weighted single-shot fast spin-echo acquired at 1.5 T. Assessment The T2 -weighted images were reconstructed in 3D. Then, two fetal neuroradiologists, a clinical neuroscientist, and a fetal MRI technician independently labeled the reconstructed images as 1 or 0 based on image quality (1 = high; 0 = low). These labels were fused and served as ground truth. The proposed DLMs were trained and evaluated using three repeated 10-fold cross-validations (training and validation sets of 244 and 27 scans). To quantify GA influence, this variable was included as an input of the DLMs. Statistical tests DLM performance was evaluated using precision, recall, F-score, accuracy, and AUC values. Results Precision, recall, F-score, accuracy, and AUC averaged over the three cross validations were 0.85 ± 0.01, 0.85 ± 0.01, 0.85 ± 0.01, 0.85 ± 0.01, 0.93 ± 0.01, for MI-CB-DLM (without GA); 0.75 ± 0.03, 0.75 ± 0.03, 0.75 ± 0.03, 0.75 ± 0.03, 0.81 ± 0.03, for MI-VB-DLM (without GA); 0.81 ± 0.01, 0.81 ± 0.01, 0.81 ± 0.01, 0.81 ± 0.01, 0.89 ± 0.01, for MI-FE-DLM (without GA); and 0.86 ± 0.01, 0.86 ± 0.01, 0.86 ± 0.01, 0.86 ± 0.01, 0.93 ± 0.01, for MI-CB-DLM with GA. Data conclusion MI-CB-DLM performed better than other DLMs. Including GA as an input of MI-CB-DLM improved its performance. MI-CB-DLM may potentially be used to objectively and rapidly assess fetal MR image quality. Evidence level 4 TECHNICAL EFFICACY: Stage 3.

BACKGROUND The aims of this study were to find the normal value of fronto-temporal horn ratio (FTHR) as a marker of ventriculomegaly on cranial ultrasound (CUS) in premature newborns and the relation to white matter injury (WMI) and cerebral palsy (CP). METHODS This is a retrospective study of newborns admitted between 2011 and 2014. Inclusion criteria were: (1) gestation

Gamma-aminobutyric acid (GABA) and glutamate are principal neurotransmitters essential for late gestational brain development and may play an important role in prematurity-related brain injury. In vivo investigation of GABA in the preterm infant with standard proton magnetic resonance spectroscopy (1H-MRS) has been limited due to its low concentrations in the developing brain, and overlap in the spectrum by other dominant metabolites. We describe early postnatal profiles of in vivo GABA and glutamate concentrations in the developing preterm brain measured by using the J-difference editing technique, Mescher-Garwood point resolved spectroscopy. We prospectively enrolled very preterm infants born ≤32 weeks gestational age and non-sedated 1H-MRS (echo time 68 ms, relaxation time 2000 ms, 256 signal averages) was acquired on a 3 Tesla magnetic resonance imaging scanner from a right frontal lobe voxel. Concentrations of GABA + (with macromolecules) was measured from the J-difference spectra; whereas glutamate and composite glutamate + glutamine (Glx) were measured from the unedited (OFF) spectra and reported in institutional units. We acquired 42 reliable spectra from 38 preterm infants without structural brain injury [median gestational age at birth of 28.0 (IQR 26.0, 28.9) weeks; 19 males (50%)] at a median postmenstrual age of 38.4 (range 33.4 to 46.4) weeks. With advancing post-menstrual age, the concentrations of glutamate OFF increased significantly, adjusted for co-variates (generalized estimating equation β = 0.22, p = 0.02). Advancing postnatal weeks of life at the time of imaging positively correlated with GABA + (β = 0.06, p = 0.02), glutamate OFF (β = 0.11, p = 0.02) and Glx OFF (β = 0.12, p = 0.04). Male infants had higher GABA + (1.66 ± 0.07 vs. 1.33 ± 0.11, p = 0.01) concentrations compared with female infants. For the first time, we report the early ex-utero developmental profile of in vivo GABA and glutamate stratified by age and sex in the developing brain of very preterm infants. This data may provide novel insights into the pathophysiology of neurodevelopmental disabilities reported in preterm infants even in the absence of structural brain injury.

To determine whether ventilator-related fluctuations in cerebral blood volume (CBV) are associated with cerebral pressure passivity. In a prospective study of newborns undergoing positive-pressure ventilation, we calculated coherence between continuous mean arterial pressure (MAP) and cerebral near-infrared spectroscopy hemoglobin difference (HbD). Significant HbD–MAP coherence indicated cerebral pressure passivity. CBV changes were measured as the spectral power of total hemoglobin (SHbT) at the ventilator frequency. A regression model tested whether SHbT predicts cerebral pressure passivity and/or death/brain injury, controlling for birth gestational age and other factors. We studied 68 subjects with prematurity (n = 19), congenital heart disease (n = 11), and hypoxic–ischemic encephalopathy (n = 38). SHbT, sedative use, and pCO2 were positively associated, and circulating hemoglobin negatively associated, with cerebral pressure passivity (p

ispartof: J Neonatal Perinatal Med vol:15 issue:2 pages:411-426 ispartof: location:Netherlands status: published

We describe a 2-year-old girl with bow hunter syndrome complicated by vertebral artery dissection and multiple ischemic infarcts. Pediatric bow hunter syndrome is a rare and likely under-recognized disorder. Interestingly, our patient had atlanto-occipital ligament calcification on CT scan, an imaging finding that has not been reported in association with bow hunter syndrome and one that might help increase recognition of this dynamic disorder of the posterior circulation.

Here we report the case of a previously healthy 8-year-old boy who presented with altered mental status, right facial droop and right-sided hemiplegia the day after playing in an inflatable bouncer. No head trauma was reported by the patient nor witnessed by the parents. Urgent magnetic resonance imaging (MRI) demonstrated acute ischemic infarction in the left pons; computed tomographic angiography excluded arterial dissection but identified a small hyperdense filling defect in the basilar artery, later confirmed to be a calcification at the origin of a perforating artery. Pediatric National Institutes of Health (PedNIH) Stroke Scale score was 15. Infectious, inflammatory, hypercoagulable and additional vascular causes were excluded. Although the cause of the calcification remains obscure, we speculate that, similarly to mineralizing microangiopathy, a minor trauma led to stroke in this child. To our knowledge, mineralizing microangiopathy, the well-described entity affecting perforating arteries of the anterior circulation in young children leading to basal ganglia stroke following minor head traumas has not been described in the posterior circulation or in previously healthy school-age children.

To determine the prevalence of and risk factors for cerebral sinus venous thrombosis (CSVT) in neonates undergoing congenital heart disease (CHD) repair.Neonates who had CHD repair with cardiopulmonary bypass and postoperative brain magnetic resonance imaging (MRI) between 2013 and 2019 at a single tertiary care center were identified from institutional databases. Demographic, clinical, and surgical data were abstracted from these databases and from the medical record; 278 neonates with CHD had cardiopulmonary bypass, 184 of whom had a postoperative brain MRI.Eight patients (4.3%) had a CSVT. Transposition of the great arteries with an intact ventricular septum (P .01) and interrupted aortic arch (P = .02) were associated with an increased risk for CSVT. Other risk factors for CSVT included cross-clamp time (98 [IQR, 77.5-120] minutes vs 67 [IQR, 44-102] minutes; P = .03), units of platelets (3.63 [IQR, 3-4] vs 2.17 [IQR, 1-4]; P .01) and packed red blood cells (0.81 [IQR, 0.25-1] vs 1.21 [IQR, 1-1]; P = .03) transfused intraoperatively, and time between surgery and MRI (10 [IQR, 7-12.5] days vs 20 [IQR, 12-35] days; P .01). Five patients (62.5%) were treated with anticoagulation. All patients had complete or partial resolution of their CSVT, regardless of treatment.Brain MRI after cardiopulmonary bypass in neonates revealed a low prevalence of CSVT (4.3%). Further studies are needed to establish best practices for surveillance, prevention, and treatment of CSVT in this population.

Brain injury is a serious and common complication of critical congenital heart disease (CHD). Impaired autonomic development (assessed by heart rate variability (HRV)) is associated with brain injury in other high-risk neonatal populations.To determine whether impaired early neonatal HRV is associated with pre-operative brain injury in CHD.In infants with critical CHD, we evaluated HRV during the first 24 h of cardiac ICU (CICU) admission using time-domain (RMS 1, RMS 2, and alpha 1) and frequency-domain metrics (LF, nLF, HF, nHF). Pre-operative brain magnetic resonance imaging (MRI) was scored for injury using an established system. Spearman's correlation coefficient was used to determine the association between HRV and pre-operative brain injury.We enrolled 34 infants with median birth gestational age of 38.8 weeks (IQR 38.1-39.1). Median postnatal age at pre-operative brain MRI was 2 days (IQR 1-3 days). Thirteen infants had MRI evidence of brain injury. RMS 1 and RMS 2 were inversely correlated with pre-operative brain injury.Time-domain metrics of autonomic function measured within the first 24 h of admission to the CICU are associated with pre-operative brain injury, and may perform better than frequency-domain metrics under non-stationary conditions such as critical illness.Autonomic dysfunction, measured by heart rate variability (HRV), in early transition is associated with pre-operative brain injury in neonates with critical congenital heart disease. These data extend our earlier findings by providing further evidence for (i) autonomic dysfunction in infants with CHD, and (ii) an association between autonomic dysfunction and brain injury in critically ill neonates. These data support the notion that further investigation of HRV as a biomarker for brain injury risk is warranted in infants with critical CHD.

Background Craniosynostosis following placement of a ventriculoperitoneal shunt for hydrocephalus has been sporadically described. The purpose of this investigation was to determine the general risk of developing craniosynostosis in this patient population. Methods The authors retrospectively reviewed records and radiographs of infants who underwent ventriculoperitoneal shunt placement for hydrocephalus from 2006 to 2012. Recorded variables included date of shunt placement, demographics, comorbidities, cause of hydrocephalus, shunt type, and number of shunt revisions. Axial computed tomographic images obtained before and immediately after shunt placement and 2 to 4 years after shunt placement were evaluated by a panel of clinicians for evidence of craniosynostosis. Patients with preshunt craniosynostosis, craniosynostosis syndromes, or poor-quality computed tomographic images were excluded. Data were analyzed using STATA Version 15.1 statistical software. Results One hundred twenty-five patients (69 male and 56 female patients) were included. Average age at shunt placement was 2.3 ± 2.58 months. Sixty-one patients (48.8 percent) developed craniosynostosis at a median of 26 months after shunt placement. Of these, 28 patients fused one suture; the majority involved the sagittal suture (n = 25). Thirty-three patients fused multiple sutures; the most common were the coronal (n = 32) and the sagittal (n = 30) sutures. Multivariable logistic regression identified older age at shunt placement and more shunt revisions as independent predictors of craniosynostosis. Shunt valve type was not significant. Conclusions Craniosynostosis developed in nearly half of infants who underwent ventriculoperitoneal shunt placement for hydrocephalus. The sagittal suture was most commonly involved. The effect of suture fusion on subsequent cranial growth, shunt failure, or the development of intracranial pressure is unclear. Clinical quesiton/level of evidence Risk, III.

The objective was to determine the association between perioperative risk factors and brain imaging abnormalities on neurologic outcome in neonates with hypoplastic left heart syndrome (HLHS) or d-Transposition of the great arteries (d-TGA) who underwent cardiac surgery including cardiopulmonary bypass.A retrospective analysis of neonates with HLHS or d-TGA undergoing cardiac surgery including cardiopulmonary bypass between 2009 and 2017 was performed. Perioperative risk factors and Andropoulos' Brain Injury Scores from pre- and postoperative brain magnetic resonant images (MRI) were correlated to outcome assessments on patients between 5 and 23 months of age. Neurologic outcome was measured using the Pediatric Stroke Outcome Measure (PSOM) and Pediatric Version of the Glasgow Outcome Scale-Extended (GOS-E).Fifty-three neonates met our enrollment criteria (24 HLHS, 29 d-TGA). Mechanical ventilation 12 days and DHCA 40 min were associated with worse outcome. MRI measures of brain injuries were not associated with worse outcome by PSOM or GOS-E.For HLHS and d-TGA patients, duration of mechanical ventilation and DHCA are associated with adverse neurologic outcome. Neonatal brain MRI commonly demonstrates acquired brain injuries, but the clinical impact of these abnormalities are not often seen before 2 years of age.Acquired brain injury is common in high-risk neonates with CHD but poor neurological outcome was not predicted by severity of injury or lesion subtype. Longer stay in ICU is associated with postoperative brain injuries on MRI. Total duration of ventilation 12 days is predictive of adverse neurological outcome scores. DHCA 40 min is associated with adverse neurological outcome scores. Neurological outcome before 2 years of age is more affected by the clinical course than by cardiac diagnosis.

Objectives To compare regional cerebral cortical microstructural organization between preterm infants at term-equivalent age (TEA) and healthy full-term newborns, and to examine the impact of clinical risk factors on cerebral cortical micro-organization in the preterm cohort. Study design We prospectively enrolled very preterm infants (gestational age (GA) at birth, Highlights • Microstructure of the cerebral cortex in preterm infants was assessed using DTI. • Preterm birth alters regional micro-organization of the cerebral cortex. • Mild to moderate brain injury did not affect cerebral cortical microstructure. • Hydrocortisone treatment was related to accelerated cortical micro-organization.

Objective The purpose of this study is to highlight the relationship between obstructive hydrocephalus, changes in intracranial pressure, and sensorineural hearing loss. Methods A case of a 10-month old infant with sensorineural hearing loss secondary to obstructive hydrocephalus is reported. A literature review, with a focus on sensorineural hearing loss in the setting of changes in intracranial pressure, was performed. Results The authors report the case of a 10-month old infant with metopic and bicoronal craniosynostosis who presented with bilateral moderately severe sensorineural hearing loss after failing newborn hearing screening. Imaging subsequently demonstrated obstructive hydrocephalus, which was treated with the insertion of a VP shunt. The patient had immediate improvement of her hearing post-operatively, with repeat hearing tests showed resolution of her hearing loss. Conclusion Sensorineural hearing loss is a rare complication of hydrocephalus, but changes in intracranial pressure should be considered in the differential diagnosis. We put forth a flow diagram illustrating the hypothesized relationship between intracranial pressures, alterations in the levels of cochlear fluid, and hearing.

To determine whether ventilator-related fluctuations in cerebral blood volume (CBV) are associated with cerebral pressure passivity.In a prospective study of newborns undergoing positive-pressure ventilation, we calculated coherence between continuous mean arterial pressure (MAP) and cerebral near-infrared spectroscopy hemoglobin difference (HbD). Significant HbD-MAP coherence indicated cerebral pressure passivity. CBV changes were measured as the spectral power of total hemoglobin (SWe studied 68 subjects with prematurity (n = 19), congenital heart disease (n = 11), and hypoxic-ischemic encephalopathy (n = 38). SIn sick newborns, ventilator-related CBV fluctuations may predispose to cerebral pressure passivity, which may predispose to an adverse neonatal outcome.

Congenital heart disease (CHD) is an independent risk factor for brain injury, including stroke, and poor neurodevelopmental outcomes, and placental abnormalities may represent an additional risk factor for brain injury in neonates. The incidence and scope of placental pathology and relationship to fetal brain abnormalities in pregnancies complicated by fetal CHD has not been explored to our knowledge. In order to determine the prevalence of placental pathology findings and whether placental findings are associated with postnatal brain injury in pregnancies complicated by fetal CHD, we reviewed placental pathology reports for 51 pregnancies complicated by CHD and scored available postnatal, pre-operative brain MRI for brain pathology. Overall, 57% of CHD infants had abnormal placental pathology. Pregnancies complicated by CHD with aortic obstruction (AO) were significantly more likely than those with no obstruction to have abnormal placental pathology (79% vs. 44%). There was a trend toward more severe brain lesions amongst patients with brain lesions and placental abnormality (55% moderate/severe) compared to those without placental abnormality (11% moderate/severe). These data suggest that placental abnormalities are common in CHD and may have a compounding effect on brain lesions in this high-risk population.

To evaluate the clinical impact of routine term-equivalent magnetic resonance imaging (TE-MRI) for extremely low-birth-weight infants at a regional neonatal intensive care unit.This is a single-center retrospective study evaluating preterm survivors who underwent TE-MRI. MRI abnormalities were compared between infants with and without cranial ultrasonography (CUS) abnormalities. Cost analysis comparing imaging modalities was also performed.TE-MRI use increased from 17% in 2006 to 76% in 2010. MRI detected new findings in nearly half of infants, whether or not they had known ultrasound abnormalities. MRI detected more cerebellar (18% vs 6%, P=0.04) and moderate white matter injury (12% vs 7%, P0.001), and altered simulated neurological prognosis across developmental domains. The cost of TE-MRI was $1600, which was comparable to serial CUSs.TE-MRI detects new abnormalities and impacts developmental prognosis in the extremely low birth weight, which supports its use despite the added financial cost.

Recent improved understanding of the molecular markers of high grade glioma has shifted the approach to these aggressive CNS tumors to increasingly use molecularly guided targeted therapies. Treatment of patients with BRAFV600E mutant high grade gliomas with BRAF inhibitors has shown efficacy, however the impact of concomitant H3K27M mutation is unknown. ONC201 targets dopamine receptor D2 (DRD2), which is shown to be broadly overexpressed in the thalamus as well as multiple tumor types; its antagonism has demonstrated anti-tumor efficacy and immunomodulatory properties in preclinical studies. ONC201 has also demonstrated clinical efficacy in patients with H3K27M mutant gliomas. We present the case of a 9-year-old male with a right thalamic H3.3K27M mutant diffuse midline glioma with a concomitant BRAFV600E mutation with an ongoing partial response to ONC201 treatment. The patient was diagnosed in May 2018. He underwent biopsy, followed by standard focal proton radiation therapy (54Gy) and subsequent treatment with dasatinib, bevacizumab and everolimus over the course of five months. After continued radiographic progression on serial imaging, in April 2019 he started ONC201 375mg orally once per week through an expanded access trial. He has tolerated the medication well with grade 1 nausea and fatigue. Over the next nine months, he demonstrated clinical and radiographic improvement with modest increased use of his left side and MRIs showing progressive decrease in size of the thalamic lesion with a 70 % decrease in the target lesion (measuring 53x62mm prior to treatment, decreased to 38x26mm in January 2020).

Background Acute Flaccid Myelitis (AFM), diagnosed almost exclusively in children, is characterized by sudden onset flaccid weakness in one or more extremities with distinct gray matter spinal cord lesions on magnetic resonance imaging (MRI), with or without cerebrospinal fluid (CSF) pleocytosis. Outbreaks of AFM have occurred biennially since 2014. Although the definitive causative agent(s) remain unknown, current data support an association with Enteroviruses D68 and A71. Treatment is supportive and long-term prognosis is variable, with many children having persistent motor deficits. Methods In this prospective cohort study, we identified patients with clinical and radiographic presentation consistent with AFM at Children’s National Hospital (CNH) from 2013–2019. Medical records and MRIs of identified patients were then reviewed by members of the multidisciplinary CNH AFM Task Force to identify those meeting diagnostic criteria for AFM. Identified patients had follow-up arranged in the multidisciplinary AFM clinic for exam, functional motor assessment and quality of life questionnaires (Peds QL, PROMIS and NeuroQoL). Results Since 2013, we identified 22 patients meeting criteria for AFM at CNH. The average age of our patients was 7.25 years (range 6 months to 16 years); almost 2/3 of patients had CSF pleocytosis. Half of patients presented with initial neurologic complaint of single limb weakness. Other presenting neurological symptoms included ataxia, bilateral lower extremity weakness and ophthalmoplegia. A potential infectious cause was identified in the CSF, blood, nasopharynx or stool of 9 patients. As of November 2019, 7 of 21 patients have had follow-up evaluation; 1 had no improvement, 4 had partial improvement but with persistent motor deficits, and 2 had complete resolution. Two patients/families have completed quality of life questionnaires to date. CNH AFM Patient Characteristics and Results Conclusion Similar to other centers, the majority of CNH AFM patients with follow-up to date have persistent and significant long term motor deficits. Assessment of quality of life is an important aspect that has not yet been formally assessed in other studies and will provide useful information regarding the experience of these patients and help identify goals for optimizing care in the future. Disclosures All Authors: No reported disclosures

Brain structural changes in premature infants appear before term age. Functional differences between premature infants and healthy fetuses during this period have yet to be explored. Here, we examined brain connectivity using resting state functional MRI in 25 very premature infants (VPT; gestational age at birth

Advanced neuroimaging techniques have improved our understanding of microstructural changes in the preterm supratentorial brain as well as the cerebellum and its association with impaired neurodevelopmental outcomes. However, the metabolic interrogation of the developing cerebellum during the early postnatal period after preterm birth remains largely unknown. Our study investigates the relationship between cerebellar neurometabolites measured by proton magnetic spectroscopy (1H-MRS) in preterm infants with advancing post-menstrual age (PMA) and brain injury during ex-utero third trimester prior to term equivalent age (TEA). We prospectively enrolled and acquired high quality 1H-MRS at median 33.0 (IQR 31.6–35.2) weeks PMA from a voxel placed in the cerebellum of 53 premature infants born at a median gestational age of 27.0 (IQR 25.0–29.0) weeks. 1H-MRS data were processed using LCModel software to calculate absolute metabolite concentrations of N-acetylaspartate (NAA), choline (Cho) and creatine (Cr). We noted positive correlations of cerebellar concentrations of NAA, Cho and Cr (Spearman correlations of 0.59, 0.64 and 0.52, respectively, p value

1. Cherilyn Cecchini, MD* 2. Sabah Iqbal, MD† 3. Jonathan Murnick, MD, PhD* 1. *Departments of Pediatrics, Radiology, and 2. †Pediatric Emergency Medicine, Children's National Health System, Washington, DC A previously healthy 18-month-old boy presents to the emergency department (ED) after a fall. His parents reported that the father was carrying the child down the stairs when he slipped 1 step from the bottom; the child fell out of his arms and landed on his chest on the hardwood floor. The child did not lose consciousness and he cried immediately, but several minutes later his mother noticed that he seemed “dazed.” Emergency medical services was called, and the child was alert and awake on their arrival. The child arrived at the ED 3.5 hours after the fall given the family’s remote location. During his initial examination he was sitting in his mother’s arms with his head held midline and was moving all 4 extremities. The remainder of his examination findings are normal. His mother is very worried that “something” is wrong. He is stratified as intermediate risk for traumatic brain injury, and the decision is made to observe him in the ED. After a trial of oral intake, he stood up to walk, at which point he is noted to be slightly antalgic. Shortly …

Objective This study aims to evaluate individual regional brain biometrics and their association with developmental outcome in extremely low-birth-weight (ELBW) infants. Study Design This is a retrospective study evaluating term-equivalent magnetic resonance imaging (TE-MRI) from 27 ELBW infants with known developmental outcomes beyond 12 months corrected age. Regional biometric measurements were performed by a pediatric neuroradiologist blinded to outcome data. Measures included biparietal width, transcerebellar diameter (TCD), deep gray matter area (DGMA), ventricular dilatation, corpus callosum, and interhemispheric distance. The relationship between regional biometrics and Bayley-II developmental scores were evaluated with linear regression models. Results The study cohort had an average ± standard deviation birth weight of 684 ± 150 g, gestational age of 24.6 ± 2 weeks and 48% males. DGMA was significantly associated with both cognitive and motor outcomes. Significant associations were also observed between TCD and corpus callosum splenium with cognitive and motor outcomes, respectively. Other biometric measures were not associated with outcome ( p > 0.05). DGMA 2 was highly specific for poor motor and cognitive outcome. Conclusion TE-MRI biometrics reflecting impaired deep gray matter, callosal, and cerebellar size is associated with worse early childhood cognitive and motor outcomes. DGMA may be the most robust single biometric measure to predict adverse developmental outcome in preterm survivors.

BACKGROUND AND PURPOSE: Early disturbances in systemic and cerebral hemodynamics are thought to mediate prematurity-related brain injury. However, the extent to which CBF is perturbed by preterm birth is unknown. Our aim was to compare global and regional CBF in preterm infants with and without brain injury on conventional MR imaging using arterial spin-labeling during the third trimester of ex utero life and to examine the relationship between clinical risk factors and CBF. MATERIALS AND METHODS: We prospectively enrolled preterm infants younger than 32 weeks' gestational age and

Traumatic arteriovenous fistulas (AVFs) involving the middle meningeal artery (MMA) are uncommon lesions and rare in the pediatric population. An adolescent with headaches developed a bruit and a rhythmic “swooshing” noise in the left ear 9 weeks after a traumatic brain injury involving a left temporal bone fracture. An MRA of the brain demonstrated an enlarged left MMA and arterialized flow in the left middle meningeal vein (MMV), suggestive of an AVF. Cerebral angiography confirmed and characterized the left MMA-MMV fistula, which drained into the pterygoid plexus. The patient underwent transarterial coil embolization, with successful obliteration of the fistulous connection, and her tinnitus resolved. This case illustrates the importance of recognizing a post-traumatic AVF in a pediatric patient with pulsatile tinnitus, and that endovascular treatment by coil embolization is a safe and effective treatment option.

This study aims to compare the cerebellar biochemical profiles in preterm (PT) infants evaluated at term equivalent age (TEA) and healthy full-term newborns using proton magnetic resonance spectroscopy (1H-MRS). We explore the associations between altered cerebellar metabolite profiles and brain injury topography, severity of injury, and prematurity-related clinical complications. We prospectively collected high quality 1H-MRS in 59 premature infants born ≤32 weeks and 61 healthy full term controls. 1H-MRS data were processed using LCModel software to calculate absolute metabolite concentration for N-acetyl-aspartate (NAA), choline (Cho) and creatine (Cr). PT infants had significantly lower cerebellar NAA (p

A previously healthy 10-year-old female reported a 1-month history of wheezing and hemoptysis. Initial evaluation and treatment were focused on refractory reactive airway disease and infectious etiologies prompted by her recent travels in Africa. Worsening respiratory distress prompted emergent evaluation with imaging and endoscopy. Bronchoscopy diagnosed a distal tracheal tumor; pathology of this tumor was benign fibrous histiocytoma. Successful management of this condition included imaging, rigid bronchoscopy with biopsy, and tracheal resection to surgically excise the lesion. Although rare, tracheal tumors should be considered when presentation of asthma is atypical and nonresponsive to medical interventions. Laryngoscope, 125:1004–1007, 2015

Objectives/Hypothesis We describe management of an infant with chronic aspiration as a result of severe and rarely described laryngeal dysgenesis. Results A neonate with severe maldevelopment of the laryngeal structures required tracheostomy for respiratory distress on day-of-life 1, but the patient continued to have aspiration pneumonias. After failing to improve with conservative measures, the infant underwent laryngotracheal separation (LTS), which was successful in preventing aspiration. The patient has had no further pneumonias. Conclusions The presented case illustrates that LTS may be considered a safe and effective initial treatment option for chronic aspiration in select infants with severe dysgenesis of the larynx. Laryngoscope, 124:2186–2189, 2014

To determine the functional network organization of the brain in infants born very preterm at term-equivalent age and to relate network alterations to known clinical risk factors for poor neurologic outcomes in prematurity.Resting-state functional magnetic resonance imaging data from 66 infants born very preterm (gestational age32 weeks and birth weight1500 g) and 66 healthy neonates born at full term, acquired as part of a prospective, cross-sectional study, were compared at term age using graph theory. Features of resting-state networks, including integration, segregation, and modularity, were derived from correlated hemodynamic activity arising from 93 cortical and subcortical regions of interest and compared between groups.Despite preserved small-world topology and modular organization, resting-state networks of infants born very preterm at term-equivalent age were less segregated and less integrated than those of infants born full term. Chronic respiratory illness (ie, bronchopulmonary dysplasia and the length of oxygen support) was associated with decreased global efficiency and increased path lengths (P .05). In both cohorts, 4 functional modules with similar composition were observed (parietal/temporal, frontal, subcortical/limbic, and occipital). The density of connections in 3 of the 4 modules was decreased in the very preterm network (P .01); however, in the occipital/visual cortex module, connectivity was increased in infants born very preterm relative to control infants (P .0001).Early exposure to the ex utero environment is associated with altered resting-state network functional organization in infants born very preterm at term-equivalent age, likely reflecting disrupted brain maturational processes.