Dynamic compression in vertebral artery dissection in children: apropos of a new protocol.

PURPOSE: Our goals are (1) to report a consecutive prospective series of children who had posterior circulation stroke caused by vertebral artery dissection at the V3 segment; (2) to describe a configuration of the vertebral artery that may predispose to rotational compression; and (3) to recommend a new protocol for evaluation and treatment of vertebral artery dissection at V3. METHODS: All children diagnosed with vertebral artery dissection at the V3 segment from September 2014 to July 2020 at our institution were included in the study. Demographic, clinical, surgical, and radiological data were collected. RESULTS: Sixteen children were found to have dissection at a specific segment of the vertebral artery. Fourteen patients were male. Eleven were found to have compression on rotation during a provocative angiogram. All eleven underwent C1C2 posterior fusion as part of their treatment. Their mean age was 6.44 years (range 18 months-15 years). Mean blood loss was 57.7 mL. One minor complication occurred: a superficial wound infection treated with oral antibiotics only. There were no vascular or neurologic injuries. There have been no recurrent ischemic events after diagnosis and/or treatment. Mean follow-up was 33.3 months (range 2-59 months). We designed a new protocol to manage V3 dissections in children. CONCLUSION: Posterior C1C2 fusion is a safe and effective option for treatment of dynamic compression in vertebral artery dissection in children. Institution of and compliance with a strict diagnostic and treatment protocol for V3 segment dissections seem to prevent recurrent stroke.

Reduction of bias in the evaluation of fractional anisotropy and mean diffusivity in magnetic resonance diffusion tensor imaging using region-of-interest methodology.

Accurate quantification of fractional anisotropy (FA) and mean diffusivity (MD) in MR diffusion tensor imaging (DTI) requires adequate signal-to-noise ratio (SNR) especially in low FA areas of the brain, which necessitates clinically impractical long image acquisition times. We explored a SNR enhancement strategy using region-of-interest (ROI)-based diffusion tensor for quantification. DTI scans from a healthy male were acquired 15 times and combined into sets with different number of signal averages (NSA = 1-4, 15) at one 1.5-T Philips and three 3-T (Philips, Siemens and GE) scanners. Equivalence test was performed to determine NSA thresholds for bias-free FA and MD quantifications by comparison with reference values derived from images with NSA = 15. We examined brain areas with low FA values including caudate nucleus, globus pallidus, putamen, superior temporal gyrus, and substructures within thalamus (lateral dorsal, ventral anterior and posterior nuclei), where bias-free FA is difficult to obtain using a conventional approach. Our results showed that bias-free FA can be obtained with NSA = 2 or 3 in some cases using ROI-based analysis. ROI-based analysis allows reliable FA and MD quantifications in various brain structures previously difficult to study with clinically feasible data acquisition schemes.

Reversible Cerebral Vasoconstriction Syndrome: A Novel Mechanism for Neurological Complications in Schimke Immuno-osseous Dysplasia.

BACKGROUND: Schimke immuno-osseous dysplasia is a rare autosomal recessive disease resulting from biallelic SMARCAL1 mutations. It presents in early childhood and is characterized by short stature, nephropathy, and immunodeficiency. Approximately 50% of those affected have neurological complications including migraines, transient ischemic attacks, and strokes. METHODS: We present a six-year-old boy with Schimke immuno-osseous dysplasia without evidence of atherosclerosis with recurrent episodes of severe headache, fluctuating hemiparesis, and aphasia. RESULTS: Magnetic resonance imaging and angiography were normal during the initial episode; multiple areas of reversible restricted diffusion with decreased perfusion and arterial stenosis were seen with subsequent attacks. CONCLUSIONS: This constellation of symptoms and imaging findings is suggestive of reversible cerebral vasoconstriction syndrome, which we propose as a mechanism for the transient ischemic attacks and infarcts seen in some patients with Schimke immuno-osseous dysplasia, as opposed to accelerated atherosclerosis alone. This new insight may provide a basis for novel preventative therapy in this rare disorder.

Correcting B0 Field Distortions in MRI Caused by Stainless Steel Orthodontic Appliances at 1.5 T Using Permanent Magnets - A Head Phantom Study.

Susceptibility artifacts caused by stainless steel orthodontic appliances (braces) pose significant challenges in clinical brain MRI examinations. We introduced field correction device (FCD) utilizing permanent magnets to cancel the induced B0 inhomogeneity and mitigate geometric distortions in MRI. We evaluated a prototype FCD using a 3D-printed head phantom in this proof of concept study. The phantom was compartmented into anterior frontal lobe, temporal lobe, fronto-parieto-occipital lobe, basal ganglia and thalami, brain stem, and cerebellum and had built-in orthogonal gridlines to facilitate the quantification of geometric distortions and volume obliterations. Stainless steel braces were mounted on dental models of three different sizes with total induced magnetic moment 0.15 to 0.17 A·m2. With braces B0 standard deviation (SD) ranged from 2.8 to 3.7 ppm in the temporal and anterior frontal lobes vs. 0.2 to 0.3 ppm without braces. The volume of brain regions in diffusion weighted imaging was obliterated by 32-38% with braces vs. 0% without braces in the cerebellum. With the FCD the SD of B0 ranged from 0.3 to 1.2 ppm, and obliterated volume ranged from 0 to 6% in the corresponding brain areas. These results showed that FCD can effectively decrease susceptibility artifacts from orthodontic appliances.

Heterogeneous increases of regional cerebral blood flow during preterm brain development: Preliminary assessment with pseudo-continuous arterial spin labeled perfusion MRI.

The human brain develops rapidly during 32-45 postmenstrual weeks (PMW), a critical stage characterized by dramatic increases of metabolic demand. The increasing metabolic demand can be inferred through measurements of regional cerebral blood flow (CBF), which might be coupled to regional metabolism in preterm brains. Arterial spin labeled (ASL) perfusion MRI is one of the few viable approaches for imaging regional CBF of preterm brains, but must be optimized for the extremely slow blood velocity unique in preterm brains. In this study, we explored the spatiotemporal CBF distribution in newborns scanned at the age of 32-45PMW using a pseudo-continuous ASL (pCASL) protocol adapted to slow blood flow in neonates. A total of 89 neonates were recruited. PCASL MRI was acquired from 34 normal newborns and phase contrast (PC) images from 19 newborns. Diffusion tensor images (DTI) were acquired from all 89 neonates for measuring cortical fractional anisotropy (FA), which characterizes cortical microstructure. Reproducible CBF measurements were obtained with the adjusted pCASL sequence. Global CBF measurement based on PC MRI was found to double its value in the 3rd trimester. Regional CBF increases were heterogeneous across the brain with a significantly higher rate of CBF increase in the frontal lobe and a lower rate of CBF increase in the occipital lobe. A significant correlation was found between frontal cortical CBF and cortical FA measurements (p<0.01). Increasing CBF values observed in the frontal lobe corresponded to lower FA values, suggesting that dendritic arborization and synaptic formation might be associated with an elevated local CBF. These results offer a preliminary account of heterogeneous regional CBF increases in a vital early developmental period and may shed the light on underlying metabolic support for cortical microstructural changes during the developmental period of 32-45PMW. Preterm effects and limitations of pCASL techniques in newborns need to be carefully considered for interpretation these results.

Variability of Ponto-cerebellar Fibers by Diffusion Tensor Imaging in Diverse Brain Malformations.

To describe pontine axonal anomalies across diverse brain malformations. Institutional review board-approved review of magnetic resonance imaging (MRI) and genetic testing of 31 children with brain malformations and abnormal pons by diffusion tensor imaging. Anomalous dorsal pontocerebellar tracts were seen in mid-hindbrain anomalies and in diffuse malformations of cortical development including lissencephaly, gyral disorganization with dysplastic basal ganglia, presumed congenital fibrosis of extraocular muscles type 3, and in callosal agenesis without malformations of cortical development. Heterotopic and hypoplastic corticospinal tracts were seen in callosal agenesis and in focal malformations of cortical development. There were no patterns by chromosomal microarray analysis in the non-lissencephalic brains. In lissencephaly, there was no relationship between severity, deletion size, or appearance of the pontocerebellar tract. Pontine axonal anomalies may relate to defects in precerebellar neuronal migration, chemotactic signaling of the pontine neurons, and/or corticospinal tract pathfinding and collateral branching not detectable with routine genetic testing.

Assessment of diffusion tensor image quality across sites and vendors using the American College of Radiology head phantom.

The purpose of this study was to explore the feasibility of assessing quality of diffusion tensor imaging (DTI) from multiple sites and vendors using American College of Radiology (ACR) phantom. Participating sites (Siemens (n = 2), GE (n= 2), and Philips (n = 4)) reached consensus on parameters for DTI and used the widely available ACR phantom. Tensor data were processed at one site. B0 and eddy current distortions were assessed using grid line displacement on phantom Slice 5; signal-to-noise ratio (SNR) was measured at the center and periphery of the b = 0 image; fractional anisotropy (FA) and mean diffusivity (MD) were assessed using phantom Slice 7. Variations of acquisition parameters and deviations from specified sequence parameters were recorded. Nonlinear grid line distortion was higher with linear shimming and could be corrected using the 2nd order shimming. Following image registration, eddy current distortion was consistently smaller than acquisi-tion voxel size. SNR was consistently higher in the image periphery than center by a factor of 1.3-2.0. ROI-based FA ranged from 0.007 to 0.024. ROI-based MD ranged from 1.90 × 10-3 to 2.33 × 10-3 mm2/s (median = 2.04 × 10-3 mm2/s). Two sites had image void artifacts. The ACR phantom can be used to compare key qual-ity measures of diffusion images acquired from multiple vendors at multiple sites.

Induced magnetic moment in stainless steel components of orthodontic appliances in 1.5 T MRI scanners.

PURPOSE: Most orthodontic appliances are made of stainless steel materials and induce severe magnetic susceptibility artifacts in brain MRI. In an effort for correcting these artifacts, it is important to know the value of induced magnetic moments in all parts of orthodontic appliances. In this study, the induced magnetic moment of stainless steel orthodontic brackets, molar bands, and arch-wires from several vendors is measured. METHODS: Individual stainless steel brackets, molar bands, and short segments of arch-wire were positioned in the center of spherical flask filled with water through a thin plastic rod. The induced magnetic moment at 1.5 T was determined by fitting the B0 map to the z-component of the magnetic dipole field using a computer routine. RESULTS: The induced magnetic moment at 1.5 T was dominated by the longitudinal component mz, with a small contribution from the transverse components. The mz was insensitive to the orientation of the metal parts. The orthodontic brackets collectively dominated the magnetic dipole moment in orthodontic appliances. In brackets from six vendors, the total induced mz from 20 brackets for nonmolar teeth ranged from 0.108 to 0.158 (median 0.122) A ⋅ m(2). The mz in eight molar bands with bracket attachment from two vendors ranged from 0.0004 to 0.0166 (median 0.0035) A ⋅ m(2). Several full length arch wires had induced magnetic moment in the range of 0.006-0.025 (median 0.015) A ⋅ m(2). CONCLUSIONS: Orthodontic brackets collectively contributed most to the total magnetic moment. Different types of brackets, molar bands, and arch wires all exhibit substantial variability in the induced magnetic moment.

Signal-to-noise assessment for diffusion tensor imaging with single data set and validation using a difference image method with data from a multicenter study.

PURPOSE: To describe a quantitative method for determination of SNR that extracts the local noise level using a single diffusion data set. METHODS: Brain data sets came from a multicenter study (eight sites; three MR vendors). Data acquisition protocol required b=0, 700 s/mm2, fov=256×256 mm2, acquisition matrix size 128×128, reconstruction matrix size 256×256; 30 gradient encoding directions and voxel size 2×2×2 mm3. Regions-of-interest (ROI) were placed manually on the b=0 image volume on transverse slices, and signal was recorded as the mean value of the ROI. The noise level from the ROI was evaluated using Fourier Transform based Butterworth high-pass filtering. Patients were divided into two groups, one for filter parameter optimization (N=17) and one for validation (N=10). Six white matter areas (the genu and splenium of corpus callosum, right and left centrum semiovale, right and left anterior corona radiata) were analyzed. The Bland-Altman method was used to compare the resulting SNR with that from the difference image method. The filter parameters were optimized for each brain area, and a set of "global" parameters was also obtained, which represent an average of all regions. RESULTS: The Bland-Altman analysis on the validation group using "global" filter parameters revealed that the 95% limits of agreement of percent bias between the SNR obtained with the new and the reference methods were -15.5% (median of the lower limit, range [-24.1%, -8.9%]) and 14.5% (median of the higher limits, range [12.7%, 18.0%]) for the 6 brain areas. CONCLUSIONS: An FT-based high-pass filtering method can be used for local area SNR assessment using only one DTI data set. This method could be used to evaluate SNR for patient studies in a multicenter setting.

Perinatal asphyxia with hyperoxemia within the first hour of life is associated with moderate to severe hypoxic-ischemic encephalopathy.

OBJECTIVE: To determine whether early hyperoxemia in neonates with severe perinatal acidemia is associated with the development of hypoxic-ischemic encephalopathy (HIE). STUDY DESIGN: We identified 120 infants at ≥ 36 weeks gestational age with perinatal acidosis born at Parkland Hospital who qualified for a screening neurologic exam for cooling therapy. Based on a PaO2 measurement during the first hour of life, the cohort was divided into infants with hyperoxemia (PaO2 >100 mmHg) and those without hyperoxemia (PaO2 ≤ 100 mmHg). The rate of moderate-severe encephalopathy was compared between the groups using χ(2) analysis, as well as multiple logistic regression, taking into account baseline characteristics and confounding variables. RESULTS: Thirty-six infants (30%) had an initial PaO2 >100 mmHg. Infants with and without hyperoxemia had similar baseline maternal and infant characteristics. Infants with hyperoxemia had a higher incidence of HIE than those without hyperoxemia (58% vs 27%; P = .003). Admission hyperoxemia was associated with a higher risk of HIE (OR, 4; 95% CI, 1.4-10.5; adjusted P = .01). Among the neonates with moderate-severe HIE during the first 6 hours of life, those with hyperoxemia had a higher incidence of abnormal brain magnetic resonance imaging results, consistent with hypoxic ischemic injury, compared with those without hyperoxemia (79% vs 33%; P = .015). CONCLUSION: In neonates with perinatal acidemia, admission hyperoxemia is associated with a higher incidence of HIE. Among neonates with HIE, admission hyperoxemia is associated with abnormal brain magnetic resonance imaging findings. The judicious use of oxygen during and after resuscitation is warranted.

Diffusion tensor imaging metrics in neonates-a comparison of manual region-of-interest analysis vs. tract-based spatial statistics.

BACKGROUND: Diffusion tensor data can be analyzed using region-of-interest (ROI) analysis and tract-based spatial statistics (TBSS). There is essentially no literature validating or comparing these techniques in the neonate. OBJECTIVE: The purpose of this study was to perform a direct comparison of fractional anisotropy (FA), axial diffusivity (AD) and radial diffusivity (RD) derived using manual ROI analysis and TBSS modified for use in neonates. MATERIALS AND METHODS: This study was IRB-approved. Thirty-nine infants, 32-49 weeks post-conception age, underwent MRI at 3 T. FA, AD and RD of the callosal genu (CG) and splenium (CS) and posterior limbs of both internal capsules (PLIC) were determined using both techniques. Pearson correlation (r) was used to estimate the concordance of tensor metrics derived from these techniques. RESULTS: The r value for FA in the CG, CS and left and right PLIC was 0.88, 0.75, 0.78 and 0.35, respectively. The r value for axial/radial diffusivity in the CG, CS and left and right PLIC was 0.62/0.72, 0.76/0.64, 0.68/0.9 and 0.3/0.72, respectively. The variable concordance results from problems with spatial correspondence of ROI masks between the native space and the FA skeleton. CONCLUSION: Direct comparison between these methodologies shows tensor metrics varied with location and by degree, suggesting the two techniques do not provide consistently comparable results.

Acute silent cerebral ischemia and infarction during acute anemia in children with and without sickle cell disease.

We hypothesized that the silent cerebral infarcts (SCI), which affect up to 40% of children with sickle cell disease (SCD), could occur in the setting of acute anemic events. In a prospective observational study of children with and without SCD hospitalized for an illness associated with acute anemia, we identified acute silent cerebral ischemic events (ASCIE) in 4 (18.2%) of 22 with SCD and in 2 (6.7%) of 30 without SCD, using diffusion-weighted magnetic resonance imaging. Children with ASCIE had lower hemoglobin concentration than those without (median 3.1 vs 4.4 g/dL, P = .003). The unique temporal features of stroke on diffusion-weighted magnetic resonance imaging permit estimation of incidence rates for ASCIE of 421 (95% confidence interval, 155-920) per 100 patient-years during acute anemic events for all patients. For children with SCD, the estimated incidence was 663 (95% confidence interval, 182-1707) which is much higher than previously reported. Acute anemic events are common in children with SCD and prevalence could partially account for the high SCI. Some ASCIE (1 of 4 in our study) may be reversible. Alterations in management may be warranted for children with severe anemia to identify unrecognized ischemic brain injury that may have permanent neurocognitive sequelae.

Minimum SNR and acquisition for bias-free estimation of fractional anisotropy in diffusion tensor imaging - a comparison of two analytical techniques and field strengths.

Although it is known that low signal-to-noise ratio (SNR) can affect tensor metrics, few studies reporting disease or treatment effects on fractional anisotropy (FA) report SNR; the implicit assumption is that SNR is adequate. However, the level at which low SNR causes bias in FA may vary with tissue FA, field strength and analytical methodology. We determined the SNR thresholds at 1.5 T vs. 3 T in regions of white matter (WM) with different FA and compared FA derived using manual region-of-interest (ROI) analysis to tract-based spatial statistics (TBSS), an operator-independent whole-brain analysis tool. Using ROI analysis, SNR thresholds on our hardware-software magnetic resonance platforms were 25 at 1.5 T and 20 at 3 T in the callosal genu (CG), 40 at 1.5 and 3 T in the anterior corona radiata (ACR), and 50 at 1.5 T and 70 at 3 T in the putamen (PUT). Using TBSS, SNR thresholds were 20 at 1.5 T and 3 T in the CG, and 35 at 1.5 T and 40 at 3 T in the ACR. Below these thresholds, the mean FA increased logarithmically, and the standard deviations widened. Achieving bias-free SNR in the PUT required at least nine acquisitions at 1.5 T and six acquisitions at 3 T. In the CG and ACR, bias-free SNR was achieved with at least three acquisitions at 1.5 T and one acquisition at 3 T. Using diffusion tensor imaging (DTI) to study regions of low FA, e.g., basal ganglia, cerebral cortex, and WM in the abnormal brain, SNR should be documented. SNR thresholds below which FA is biased varied with the analytical technique, inherent tissue FA and field strength. Studies using DTI to study WM injury should document that bias-free SNR has been achieved in the region of the brain being studied as part of quality control.

A quality assurance protocol for diffusion tensor imaging using the head phantom from American College of Radiology.

PURPOSE: To propose a quality assurance procedure for routine clinical diffusion tensor imaging (DTI) using the widely available American College of Radiology (ACR) head phantom. METHODS: Analysis was performed on the data acquired at 1.5 and 3.0 T on whole body clinical MRI scanners using the ACR phantom and included the following: (1) the signal-to-noise ratio (SNR) at the center and periphery of the phantom, (2) image distortion by EPI readout relative to spin echo imaging, (3) distortion of high-b images relative to the b= 0 image caused by diffusion encoding, and (4) determination of fractional anisotropy (FA) and mean diffusivity (MD) measured with region-of-interest (ROI) and pixel-based approaches. Reproducibility of the measurements was assessed by five repetitions of data acquisition on each scanner. RESULTS: The SNR at the phantom center was approximately half of that near the periphery at both 1.5 and 3 T. The image distortion by the EPI readout was up to 7 mm at 1.5 T and 10 mm at 3 T. The typical distortion caused by eddy currents from diffusion encoding was on the order of 0.5 mm. The difference between ROI-based and pixel-based MD quantification was 1.4% at 1.5 T and 0.3% at 3 T. The ROI-based MD values were in close agreement (within 2%) with the reference values. The ROI-based FA values were approximately a factor of 10 smaller than pixel-based values and less than 0.01. The measurement reproducibility was sufficient for quality assurance (QA) purposes. CONCLUSIONS: This QA approach is simple to perform and evaluates key aspects of the scanner performance for DTI data acquisition using a widely available phantom.

Age-related variations in white matter anisotropy in school-age children.

BACKGROUND: Determination of diffusion tensor metrics in typically developing school-age children shows that maturational increases in fractional anisotropy (FA) vary across the brain and that age effects on FA are to increases in axial diffusivity in some regions, to decreases in radial diffusivity in some, and to both increases in axial and decreases in radial diffusivity in others. OBJECTIVE: When studying developing white matter (WM) using diffusion tensor imaging (DTI), knowledge of age-related normative tensor metrics is important, as normal variations can mask or mimic disease effects. MATERIALS AND METHODS: Right-handed English-speaking children (n = 32) 6-18 years old (mean 11.0) were studied over 31 months, 7 longitudinally. Anisotropy data were analyzed using tract-based spatial statistics; 43 regions showing significant (P < 0.05) age effects on fractional anisotropy (FA) were analyzed for age effects (r), coefficient of variability (CV), and FA, axial and radial diffusivity. This study was IRB-approved. RESULTS: The callosal genu and splenium showed the highest FA values, smallest age effects, and lowest between-subject variability. Mean FA was lower and age effects were greatest in the dorsal callosal body. The highest age effects on FA were in the cingulum, centrum semiovale, right corticospinal tract, and right temporal WM. The dorsal callosal body, calcarine WM, superior frontal and temporal gyri, and right corticospinal tract showed the highest CV. Radial diffusivity decreased while axial diffusivity increased in the cingulum, decreased in the optic tracts, and showed minimal or no age effects in most other regions. CONCLUSION: Age effects on FA and variability in FA are location-dependant in developing WM.